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Sample records for antibody-positive interstitial lung

  1. An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

    Science.gov (United States)

    Sato, Yuki; Otsuka, Kojiro; Tamai, Koji; Ono, Yuichiro; Hamaguchi, Yasuhito; Tomii, Keisuke

    2017-01-01

    Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. These findings indicate that the ILD in anti-MDA5 antibody-positive patients may not only be rapidly progressive, but may also be chronic and recurrent. PMID:28154280

  2. Interstitial Lung Diseases

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    Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and ... is responsible for some types of interstitial lung diseases. Specific types include Black lung disease among coal ...

  3. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008429 The predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis/dermatomyositis. WANG Peizhen(王培珍), et al. Dept Rheumatol & Immunol, Changhai Hosp, Milit Med Univ, Shanghai 200433. Chin J Tuberc Respir Dis 2008;31(6):417-420. Objective To analyze the predictive factors and the unfavourable prognostic factors of interstitial lung disease (ILD) in patients with polymyositis

  4. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930124 The effect of glycosaminoglycans inthe genesis of pulmonary interstitial fibrosis.LIBaoyu(李保玉),et al.Dept Pathol,Jilin MedColl,132001.Chin J Tuberc & Respir Dis 1992;15(4):204-205.The pulmonary interstitial fibrosis was causedby injecting Bleomycin into mouse trachea.Afterthe injection,the volume of glycosaminoglycans(GAG)in bronchoalveolar lavage fluid and lungtissues was increased.The observation underhistochemical stain and electron microscopeshowed that the distribution of GAG in lung tis-sues was varied at different time after the injec-tion,and related to the volume of collagen pro-teins and the formation of pulmonary interstitialfibrosis.

  5. Interstitial lung disease in scleroderma.

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    Schoenfeld, Sara R; Castelino, Flavia V

    2015-05-01

    Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.

  6. Smoking and interstitial lung diseases.

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    Margaritopoulos, George A; Vasarmidi, Eirini; Jacob, Joseph; Wells, Athol U; Antoniou, Katerina M

    2015-09-01

    For many years has been well known that smoking could cause lung damage. Chronic obstructive pulmonary disease and lung cancer have been the two most common smoking-related lung diseases. In the recent years, attention has also focused on the role of smoking in the development of interstitial lung diseases (ILDs). Indeed, there are three diseases, namely respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia and pulmonary Langerhans cell histiocytosis, that are currently considered aetiologically linked to smoking and a few others which are more likely to develop in smokers. Here, we aim to focus on the most recent findings regarding the role of smoking in the pathogenesis and clinical behaviour of ILDs.

  7. Pneumoproteins in interstitial lung diseases

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    Janssen, Rob

    2006-01-01

    The interstitial lung diseases (ILD)s are a diverse group of pulmonary disorders that are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations, compromising over 100 different members that have been broadly classified into several categories. T

  8. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950308 Inhibition of mRNA expression of sillicoticcollagen gene by tetrandrine.HE Yuxian(何玉先),etal.Occup Med Instit,CAMS,Beijing,100050.Chin JPrev Med 1995;29(1):18-20.Effects of tetrandrine(TT) on types Ⅰ and Ⅱ col-lagen gene mRNA in lung tissues of silicotic rats werestudied with RNA dot blot and in situ hybridizatin bycDNA coding human and mouse Proα1(Ⅰ) and Proα1(Ⅲ) collagen.Results revealed that types Ⅰ and Ⅲcollagen gene mRNA content in lung tissues of rats ex-posed to silica dust for two to four months was obvi-

  9. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930512 Changes of interleukin—I released bypulmonary alveolar macrophage in patients withinterstitial lung disease.LI Zhenhua(李振华),etal.Respir Dis Instit,China Med Univ,Shengyang,110001.Chin J Tuberc & Respir Dis1993;16(2):90—92.To evaluate the activity of PAM,levels of IL-l released by PAM in patients with ILD(nonsmokers)were measured by usinglipopolysacharide(LPS)stimulation and thymo-cyte proliferation method,with healthy non-smokers as control group.The results showed

  10. A case of anti-nuclear matrix protein 2 antibody positive myopathy associated with lung cancer.

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    Ohta, Shin; Unoda, Ki-Ichi; Nakajima, Hideto; Ikeda, Soichiro; Hamaguchi, Yasuhito; Kimura, Fumiharu

    2016-08-31

    Myositis-specific autoantibodies (MSAs) are associated with myositis. Anti-nuclear matrix protein 2 (NXP-2) antibody was recently identified as a major MSA and was observed mostly in juvenile dermatomyositis. We report the case of a 44-year-old man who presented with myopathy with anti-NXP-2 antibody and large cell carcinoma of the lung. He was hospitalized because of myalgia and edema of limbs. Neurological examination revealed mild proximal-dominant weakness in all four extremities, and laboratory studies showed elevated creatine kinase level (6,432 IU/l). Needle electromyography showed myogenic patterns. MRI of the lower limbs demonstrated inflammatory lesions in the thighs. Biopsied specimen from the left quadriceps femoris muscle showed mild mononuclear inflammatory infiltrate surrounding muscle fibres but no fiber necrosis. He was diagnosed with myopathy based on neurological examinations and clinical symptoms. His chest X-ray and CT showed tumor shadow on the right upper lung field, but CT didn't indicate the findings of interstitial lung disease. This was surgically removed, and a histological diagnosis of non-small cell lung cancer was suspected. He was also treated with definitive chemoradiotherapy before and after operation. His symptoms of myopathy promptly remitted with the preoperative chemotherapy. His serum analysis was positive for the anti-NXP-2. Further investigation and experience of MSAs are necessary to evaluate the therapeutic strategy against cancer-associated myopathy/myositis.

  11. Interstitial lung disease: Diagnostic approach

    Directory of Open Access Journals (Sweden)

    Kaushik Saha

    2014-01-01

    Full Text Available Interstitial lung disease (ILD is a final common pathway of a broad heterogeneous group of parenchymal lung disorders. It is characterized by progressive fibrosis of the lung leading to restriction and diminished oxygen transfer. Clinically, the presenting symptoms of ILD are non-specific (cough and progressive dyspnea on exertion and are often attributed to other diseases, thus delaying diagnosis and timely therapy. Clues from the medical history along with the clinical context and radiologic findings provide the initial basis for prioritizing diagnostic possibilities for a patient with ILD. An accurate prognosis and optimal treatment strategy for patients with ILDs can only be after an accurate diagnosis. This review will assist pulmonary physicians and medicine specialist in recognition of ILD. Extensive literature search has been made through PubMed and also Book References has been used for writing this review.

  12. Lung alveolar epithelium and interstitial lung disease.

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    Corvol, Harriet; Flamein, Florence; Epaud, Ralph; Clement, Annick; Guillot, Loic

    2009-01-01

    Interstitial lung diseases (ILDs) comprise a group of lung disorders characterized by various levels of inflammation and fibrosis. The current understanding of the mechanisms underlying the development and progression of ILD strongly suggests a central role of the alveolar epithelium. Following injury, alveolar epithelial cells (AECs) may actively participate in the restoration of a normal alveolar architecture through a coordinated process of re-epithelialization, or in the development of fibrosis through a process known as epithelial-mesenchymal transition (EMT). Complex networks orchestrate EMT leading to changes in cell architecture and behaviour, loss of epithelial characteristics and gain of mesenchymal properties. In the lung, AECs themselves may serve as a source of fibroblasts and myofibroblasts by acquiring a mesenchymal phenotype. This review covers recent knowledge on the role of alveolar epithelium in the pathogenesis of ILD. The mechanisms underlying disease progression are discussed, with a main focus on the apoptotic pathway, the endoplasmic reticulum stress response and the developmental pathway.

  13. Cough in interstitial lung disease.

    Science.gov (United States)

    Garner, Justin; George, Peter M; Renzoni, Elisabetta

    2015-12-01

    Cough in the context of interstitial lung disease (ILD) has not been the focus of many studies. However, chronic cough has a major impact on quality of life in a significant proportion of patients with ILD. For the purpose of this review, we have chosen to highlight some of the more frequently encountered diffuse lung diseases including idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis and systemic sclerosis associated ILD. Many of the underlying mechanisms remain speculative and further research is now required to elucidate the complex pathways involved in the pathogenesis of chronic cough in ILD. This will hopefully pave the way for the identification of new therapeutic agents to alleviate this distressing and often intractable symptom.

  14. [Interstitial lung diseases. The pattern is important].

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    Fink, L

    2014-11-01

    Interstitial lung diseases (ILDs) comprise a number of rare entities with an estimated incidence of 10-25 per 100,000 inhabitants but the incidence greatly increases beyond the age of 65 years. The prognosis depends on the underlying cause. The fibrotic disorders show a set of radiological and histopathological patterns that are distinct but not entirely specific. In the absence of a clear clinical picture and consistent high resolution computed tomography (HRCT) findings, patients are advised to undergo surgical lung biopsies from two or three lung lobes (or transbronchial biopsies) to determine the histopathological pattern. The ILDs are differentiated into disorders of known causes (e.g. collagen vascular disease, drug-related), idiopathic interstitial pneumonia (IIP), granulomatous ILDs (e.g. sarcoidosis) and other forms of ILD (e.g. Langerhans' cell histiocytosis). The IIPs encompass idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease, cryptogen organizing pneumonia, lymphocytic interstitial pneumonia and acute interstitial pneumonia. Additionally, a category of unclassified interstitial pneumonia exists. The pathologist has to recognize and address the histopathological pattern. In a multidisciplinary discussion the disorder is allocated to a clinicopathological entity and the histopathological pattern plays a major role in the classification of the entity. Recognition of the underlying pattern and the respective histopathological differential diagnoses is important as the therapy varies depending on the cause and ranges from elimination of the stimulus (if possible) to antifibrotic drug therapy up to preparation for lung transplantation.

  15. Rheumatoid arthritis-associated interstitial lung disease

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    Brown KK

    2012-03-01

    Full Text Available Joshua J Solomon, Kevin K BrownAutoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, USAAbstract: Rheumatoid arthritis (RA is a systemic inflammatory disorder affecting 1% of the US population. Patients can have extra-articular manifestations of their disease and the lungs are commonly involved. RA can affect any compartment of the respiratory system and high resolution computed tomography (HRCT of the lung is abnormal in over half of these patients. Interstitial lung disease is a dreaded complication of RA. It is more prevalent in smokers, males, and those with high antibody titers. The pathogenesis is unknown but data suggest an environmental insult in the setting of a genetic predisposition. Smoking may play a role in the pathogenesis of disease through citrullination of protein in the lung leading to the development of autoimmunity. Patients usually present in middle age with cough and dyspnea. Pulmonary function testing most commonly shows reduced diffusion capacity for carbon monoxide and HRCT reveals a combination of reticulation and ground glass abnormalities. The most common pattern on HRCT and histopathology is usual interstitial pneumonia (UIP, with nonspecific interstitial pneumonia seen less frequently. There are no large-scale well-controlled treatment trials. In severe or progressive cases, treatment usually consists of corticosteroids with or without a cytotoxic agent for 6 months or longer. RA interstitial lung disease is progressive; over half of patients show radiographic progression within 2 years. Patients with a UIP pattern on biopsy have a survival similar to idiopathic pulmonary fibrosis.Keywords: rheumatoid arthritis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, anti-CCP

  16. [Modern Views on Children's Interstitial Lung Disease].

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    Boĭtsova, E V; Beliashova, M A; Ovsiannikov, D Iu

    2015-01-01

    Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. Children's interstitial lung diseases is an topical problem ofpediatricpulmonoogy. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children's ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail. Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children's ILD, therapy options are presented in this article.

  17. [Interstitial lung disease in rheumatoid arthritis].

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    Froidevaux-Janin, Sylvie; Dudler, Jean; Nicod, Laurent P; Lazor, Romain

    2011-11-23

    Interstitial lung disease (ILD) is found in up to 30% of patients with rheumatoid arthritis (RA) and is clinically manifest in 5 to 10%, resulting in significant morbidity and mortality. The most frequent histopathological forms are usual interstitial pneumonia and nonspecific interstitial pneumonia. Another recently described presentation is combined pulmonary fibrosis and emphysema. Similarly to idiopathic pulmonary fibrosis, acute exacerbation of ILD may occur in RA and is associated with severe prognosis. Smoking is a known risk factor of RA and may also play a role in the pathogenesis of RA-associated ILD, in combination with genetic and immunologic mechanisms. Several treatments of RA may also lead to drug-induced ILD.

  18. Acute exacerbations of fibrotic interstitial lung disease.

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    Churg, Andrew; Wright, Joanne L; Tazelaar, Henry D

    2011-03-01

    An acute exacerbation is the development of acute lung injury, usually resulting in acute respiratory distress syndrome, in a patient with a pre-existing fibrosing interstitial pneumonia. By definition, acute exacerbations are not caused by infection, heart failure, aspiration or drug reaction. Most patients with acute exacerbations have underlying usual interstitial pneumonia, either idiopathic or in association with a connective tissue disease, but the same process has been reported in patients with fibrotic non-specific interstitial pneumonia, fibrotic hypersensitivity pneumonitis, desquamative interstitial pneumonia and asbestosis. Occasionally an acute exacerbation is the initial manifestation of underlying interstitial lung disease. On biopsy, acute exacerbations appear as diffuse alveolar damage or bronchiolitis obliterans organizing pneumonia (BOOP) superimposed upon the fibrosing interstitial pneumonia. Biopsies may be extremely confusing, because the acute injury pattern can completely obscure the underlying disease; a useful clue is that diffuse alveolar damage and organizing pneumonia should not be associated with old dense fibrosis and peripheral honeycomb change. Consultation with radiology can also be extremely helpful, because the fibrosing disease may be evident on old or concurrent computed tomography scans. The aetiology of acute exacerbations is unknown, and the prognosis is poor; however, some patients survive with high-dose steroid therapy.

  19. Diffuse interstitial lung disease: overlaps and uncertainties

    Energy Technology Data Exchange (ETDEWEB)

    Walsh, Simon L.F.; Hansell, David M. [Royal Brompton Hospital, Department of Radiology, London (United Kingdom)

    2010-08-15

    Histopathological analysis of lung biopsy material allows the diagnosis of idiopathic interstitial pneumonias; however, the strength of this diagnosis is sometimes subverted by interobserver variation and sampling. The American Thoracic Society and European Respiratory Society recommendations of 2002 provide a framework for the diagnosis of interstitial lung disease (ILD) and proposed an integrated clinical, radiological and histopathological approach. These recommendations represent a break with tradition by replacing the 'gold standard' of histopathology with the combined 'silver standards' of clinical, imaging and histopathological information. One of the pitfalls of a rigid classification system for the diagnosis of interstitial lung disease is its failure to accommodate the phenomenon of overlapping disease patterns. This article reviews the various ways that interstitial lung disease may be classified and discusses their applicability. In addition the issue of overlap disease patterns is considered in the context of histopathological interobserver variation and sampling error and how a pigeonhole approach to disease classification may overlook these hybrid entities. (orig.)

  20. Interstitial lung disease probably caused by imipramine.

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    Deshpande, Prasanna R; Ravi, Ranjani; Gouda, Sinddalingana; Stanley, Weena; Hande, Manjunath H

    2014-01-01

    Drugs are rarely associated with causing interstitial lung disease (ILD). We report a case of a 75-year-old woman who developed ILD after exposure to imipramine. To our knowledge, this is one of the rare cases of ILD probably caused due to imipramine. There is need to report such rare adverse effects related to ILD and drugs for better management of ILD.

  1. Surfactant gene polymorphisms and interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    Pantelidis Panagiotis

    2001-11-01

    Full Text Available Abstract Pulmonary surfactant is a complex mixture of phospholipids and proteins, which is present in the alveolar lining fluid and is essential for normal lung function. Alterations in surfactant composition have been reported in several interstitial lung diseases (ILDs. Furthermore, a mutation in the surfactant protein C gene that results in complete absence of the protein has been shown to be associated with familial ILD. The role of surfactant in lung disease is therefore drawing increasing attention following the elucidation of the genetic basis underlying its surface expression and the proof of surfactant abnormalities in ILD.

  2. Lung Cancer and Interstitial Lung Diseases: A Systematic Review

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    Kostas Archontogeorgis

    2012-01-01

    Full Text Available Interstitial lung diseases (ILDs represent a heterogeneous group of more than two hundred diseases of either known or unknown etiology with different pathogenesis and prognosis. Lung cancer, which is the major cause of cancer death in the developed countries, is mainly attributed to cigarette smoking and exposure to inhaled carcinogens. Different studies suggest a link between ILDs and lung cancer, through different pathogenetic mechanisms, such as inflammation, coagulation, dysregulated apoptosis, focal hypoxia, activation, and accumulation of myofibroblasts as well as extracellular matrix accumulation. This paper reviews current evidence on the association between lung cancer and interstitial lung diseases such as idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis, dermatomyositis/polymyositis, rheumatoid arthritis, systemic lupus erythematosus, and pneumoconiosis.

  3. Smoking-related interstitial lung diseases: histopathological and imaging perspectives

    Energy Technology Data Exchange (ETDEWEB)

    Desai, S.R.; Ryan, S.M.; Colby, T.V

    2003-04-01

    The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed.

  4. Interstitial lung disease in systemic sclerosis.

    Science.gov (United States)

    Wells, Athol U

    2014-10-01

    Based on international collaborative data, interstitial lung disease is now the most frequent cause of death in systemic sclerosis (SSc), having supplanted renal crisis in that regard. Despite detailed explorations of candidate mediators, no primary pathway in the pathogenesis of interstitial lung disease associated with SSc (SSc-ILD) has been definitively identified and, therefore, treatment with current agents is only partially successful. However, as immunomodulatory agents do, on average, retard progression of lung disease, early identification of SSc-ILD, using thoracic high resolution computed tomography (HRCT), is highly desirable. The decision whether to introduce therapy immediately is often difficult as the balance of risk and benefit favours a strategy of careful observation when lung disease is very limited, especially in long-standing SSc. The threshold for initiating treatment is substantially reduced when lung disease is severe, systemic disease is short in duration or ongoing progression is evident, based on pulmonary function tests and symptoms. This review summarises epidemiology, pathogenesis, difficult clinical problems and management issues in SSc-ILD.

  5. Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis

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    A. Laria

    2015-01-01

    Full Text Available According to the American Thoracic Society (ATS/European Respiratory Society consensus classification, idiopathic interstitial pneumonias (IIPs include several clinic-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF, usual interstitial pneumonia (UIP, nonspecific interstitial pneumonia (NSIP, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Ultrasound Lung Comets (ULCs are an echographic chest-sonography hallmark of pulmonary interstitial fibrosis. We describe the ultrasound (US findings in the follow-up of a NSIP’s case in rheumatoid arthritis (RA.

  6. Respiratory failure due to infliximab induced interstitial lung disease.

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    Kakavas, Sotiris; Balis, Evangelos; Lazarou, Vasiliki; Kouvela, Marousa; Tatsis, Georgios

    2013-01-01

    Although poorly understood, interstitial lung disease has been reported as a possible complication of tumor necrosis factor alpha inhibitors. We report a case of interstitial lung disease in a 64-year-old man with psoriasis 3 weeks after the initiation of infliximab treatment. The patient had received two fortnightly infusions of infliximab following a short course of methotrexate. Thoracic computed tomography showed bilateral ground glass and interstitial infiltrates, while the results of microbiology and immunologic workup were negative. Likewise, bronchoalveolar lavage detected neither typical nor atypical pathogens. Infliximab-induced interstitial lung injury was suspected and corticosteroid therapy was administered which resulted in rapid clinical and radiological improvement. This is one of the few reported cases of interstitial lung disease due to infliximab in the psoriasis population. The patient had no pre-existing lung pathology, while his previous exposure to methotrexate was minimal and was not temporally associated with the induction of interstitial lung disease.

  7. Facts and promises on lung biomarkers in interstitial lung diseases.

    Science.gov (United States)

    Campo, Ilaria; Zorzetto, Michele; Bonella, Francesco

    2015-08-01

    Interstitial lung diseases (ILDs) are a heterogeneous group of >100 pulmonary disorders. ILDs are characterized by an irreversible architectural distortion and impaired gas exchange; however, there is great variability in the clinical course. ILD diagnosis requires a combination of clinical data, radiological imaging and histological findings (when a lung biopsy is required). At the same time, successful management of ILD patients strictly depends on an accurate and confident diagnosis. In this context, the detection of reliable biomarkers able to identify ILD subtypes, avoiding lung biopsy, as well as the capacity to stratify patients and predict over time the disease course, has become a primary aim for all research studies in this field.

  8. Interstitial lung involvement in rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    David Vladimirovich Bestaev

    2014-01-01

    Full Text Available Rheumatoid arthritis (RA is a systemic autoimmune rheumatic disease of unknown etiology, characterized by chronic erosive arthritis and extraarticular manifestations. Pulmonary involvement is one of the common extraarticular manifestations of RA and may show itself as bronchial tree lesions, rheumatoid nodules, Caplan's syndrome, and lesions in the pleura or pulmonary interstitium (interstitial lung involvement (ILI. High-resolution computed tomography allows the diagnosis of ILI in RA in nearly 70% of cases although the incidence of ILI may be lower (4 to 30% depending on diagnostic methods and patient selection criteria. There are several histopathological types of ILI, the differential diagnosis of which can be troublesome. Usual interstitial pneumonia (UIP and nonspecific interstitial pneumonia are major types of RA-associated ILI. UIP-pattern ILI has a more severe course than ILI with other histological patterns. The clinical presentation of ILI may be complicated by the likely toxic effect of a number of disease-modifying antirheumatic drugs (DMARDs used to treat RA, such as methotrexate and leflunomide, and biological agents (BAs, tumor necrosis factor-α (TNF-α inhibitors. The pathogenesis of pulmonary involvement in RA and the role of synthetic DMARDs and BAs in the development of ILI call for further investigations.An extraarticular manifestation, such as ILI, affects the choice of treatment policy in patients with RA.The relevance of a study of ILI is beyond question. The paper discusses the state-of-the-art of investigations in this area.

  9. Unclassifiable interstitial lung disease: A review.

    Science.gov (United States)

    Skolnik, Kate; Ryerson, Christopher J

    2016-01-01

    Accurate classification of interstitial lung disease (ILD) requires a multidisciplinary approach that incorporates input from an experienced respirologist, chest radiologist and lung pathologist. Despite a thorough multidisciplinary evaluation, up to 15% of ILD patients have unclassifiable ILD and cannot be given a specific diagnosis. The objectives of this review are to discuss the definition and features of unclassifiable ILD, identify the barriers to ILD classification and outline an approach to management of unclassifiable ILD. Several recent studies have described the characteristics of these patients; however, there are inconsistencies in the definition and terminology of unclassifiable ILD due to limited research in this population. Additional studies are required to determine the appropriate evaluation and management of patients with unclassifiable ILD.

  10. CLINICAL PROFILE OF INTERSTITIAL LUNG DISEASES CASES

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    Gagiya Ashok K

    2012-02-01

    Full Text Available Background: There are very few studies are done on interstitial lung diseases (ILD in India. Methods: We conducted a retrospective study of 30 patients of high resolution computed tomography (HRCT proven interstitial lung diseases in tertiary care centre. Results: Most common etiological causes of ILD were occupational (46.62%, Rheumatoid Arthritis (13.32%, and idiopathic pulmonary fibrosis (33.33 %. Majority were in age group 40-49 years (mean age-45.23 years and 66.5% male patients. Common symptoms were breathlessness on exertion (100%, dry cough (43.29%, anorexia (50% and joint pain (16.65%. Clubbing and bilateral crepitations were present in 50% and 63.27% of patients respectively. X- ray chest showed reticulo-nodular pattern (60%. Restrictive pattern (96.57% was present in majority patients in spirometry. Conclusion: Availability of non-invasive investigations like HRCT chest has increased our early recognitions of ILDs. Association of ILD in patients with autoimmune diseases must be ruled out. [National J of Med Res 2012; 2(1.000: 2-4

  11. Heterogeneity of mononuclear phagocytes in interstitial lung diseases

    NARCIS (Netherlands)

    H.C. Hoogsteden (Henk)

    1990-01-01

    textabstractInterstitial lung diseases are a heterogeneous group of illnesses with different pathogeneses. In interstitial lung diseases there often is an increased influx of cells from the peripheral blood (PB) to the interstitium and alveoli. Besides the increase in total cell numbers, often marke

  12. Smoking-related interstitial lung diseases; Interstitielle Lungenerkrankungen bei Rauchern

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    Marten, K. [Technische Univ. Muenchen (Germany). Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik

    2007-03-15

    The most important smoking-related interstitial lung diseases (ILD) are respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and Langerhans' cell histiocytosis. Although traditionally considered to be discrete entities, smoking-related ILDs often coexist, thus accounting for the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). Further studies are needed to elucidate the causative role of smoking in the development of pulmonary fibrosis.

  13. 11.5.Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930305 Immunoenzymatic labeling of mono-clonal antibodies for surface antigens of T—cellby using immune complexes of APAAP in pa-tients with interstitial lung disease.HOU Xian-ming(侯显明),et al.Respir Dis Res Instit,Chi-na Med Univ,Shenyang,110001.Chin J InternMed 1992;31(12):748—751.The use of unlabeled antibody bridging tech-nique with alkaline phosphatase moncional an-tialkaline phosphatase (APAAP) complexesmakes it possible to solve the problem of shortdurability of immunofluorescent staining and theproblem of nonspecific endogenous enzyme in-terference of blood cells with immunoperoxidasemethod.The technique of APAAP allows saris-

  14. Rituximab-induced interstitial lung disease

    DEFF Research Database (Denmark)

    Naqibullah, Matiuallah; Shaker, Saher B; Bach, Karen S

    2015-01-01

    Rituximab (RTX), a mouse/human chimeric anti-CD20 IgG1 monoclonal antibody has been effectively used as a single agent or in combination with chemotherapy regimen to treat lymphoma since 1997. In addition, it has been used to treat idiopathic thrombocytopenic purpura, systemic lupus erythematous......, rheumatoid arthritis, and autoimmune hemolytic anemia. Recently, RTX has also been suggested for the treatment of certain connective tissue disease-related interstitial lung diseases (ILD) and hypersensitivity pneumonitis. Rare but serious pulmonary adverse reactions are reported. To raise awareness about...... this serious side effect of RTX treatment, as the indication for its use increases with time, we report five cases of probable RTX-ILD and discuss the current literature on this potentially lethal association....

  15. Pemphigus vulgaris-associated interstitial lung disease.

    Science.gov (United States)

    Bai, Yi-Xiu; Chu, Jin-Gang; Xiao, Ting; Chen, Hong-Duo

    2016-07-01

    Autoimmune bullous diseases (AIBDs)-associated interstitial lung disease (ILD) is extremely rare. Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease caused by circulating autoantibodies against desmoglein. To date, PV-associated ILD has rarely been reported in English literature. We report a rare association of PV and ILD. A 53-year-old Chinese female with PV for 8 months developed ILD after a relapse of PV for 2 months due to discontinuation of oral prednisone by herself. She was successfully treated by systemic methylprednisolone. Taken previously reported bullous pemphigoid-associated ILD and linear IgA/IgG bullous dermatosis-associated ILD together, in general, AIBDs-associated ILD occurs when AIBDs relapse or are not controlled, responds well to systemic corticosteroids, and has a relatively better prognosis when compared with rheumatoid arthritis- or dermatomyositis-associated ILD.

  16. Pulmonary hypertension in chronic interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    Antonella Caminati

    2013-09-01

    Full Text Available Pulmonary hypertension (PH is a common complication of interstitial lung diseases (ILDs, particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs.

  17. Rheumatoid arthritis associated interstitial lung disease: a review

    Directory of Open Access Journals (Sweden)

    Deborah Assayag

    2014-04-01

    Full Text Available Rheumatoid arthritis is a common inflammatory disease affecting about 1% of the population. Interstitial lung disease is a serious and frequent complication of rheumatoid arthritis. Rheumatoid arthritis associated interstitial lung disease (RA-ILD is characterized by several histopathologic subtypes. This article reviews the proposed pathogenesis and risk factors for RA-ILD. We also outline the important steps involved in the work-up of RA-ILD and review the evidence for treatment and prognosis.

  18. A Case of IgG4-Related Lung Disease Presenting as Interstitial Lung Disease.

    Science.gov (United States)

    Ahn, Jee Hwan; Hong, Sun In; Cho, Dong Hui; Chae, Eun Jin; Song, Joon Seon; Song, Jin Woo

    2014-08-01

    Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

  19. CT in the diagnosis of interstitial lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Bergin, C.J.; Mueller, N.L.

    1985-09-01

    The computed tomographic (CT) appearance of interstitial lung disease was assessed in 23 patients with known interstitial disease. These included seven patients with fibrosing alveolitis, six with silicosis, two with hypersensitivity pneumonitis, three with lymphangitic spread of tumor, two with sarcoidosis, one with rheumatoid lung disease, and two with neurofibromatosis. The CT appearance of the interstitial changes in the different disease entities was assessed. Nodules were a prominent CT feature in silicosis, sarcoidosis, and lymphangitic spread of malignancy. Distribution of nodules and associated interlobular septal thickening provided further distinguishing features in these diseases. Reticular densities were the predominant CT change in fibrosing alveolitis, rheumatoid lung disease, and extrinsic allergic alveolitis. CT can be useful in the investigation of selected instances of interstitial pulmonary disease.

  20. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease.

    Science.gov (United States)

    Kim, E J; Elicker, B M; Maldonado, F; Webb, W R; Ryu, J H; Van Uden, J H; Lee, J S; King, T E; Collard, H R

    2010-06-01

    Interstitial lung disease is a common manifestation of rheumatoid arthritis; however, little is known about factors that influence its prognosis. The aim of the present study was to determine whether or not the usual interstitial pneumonia pattern found on high-resolution computed tomography (HRCT) is of prognostic significance in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Patients with RA-ILD were identified retrospectively (n = 82). The relationship of a definite usual interstitial pneumonia pattern on HRCT to survival was determined and compared to that in a cohort of patients with radiologically diagnosed idiopathic pulmonary fibrosis (n = 51). A definite usual interstitial pneumonia pattern was seen in 20 (24%) out of 82 patients with RA-ILD. These patients showed worse survival than those without this pattern (median survival 3.2 versus 6.6 yrs), and a similar survival to those with idiopathic pulmonary fibrosis. On multivariate analysis, a definite usual interstitial pneumonia pattern on HRCT was associated with worse survival (hazard ratio of 2.3). Analysis of specific HRCT features demonstrated that traction bronchiectasis and honeycomb fibrosis were associated with worse survival (hazard ratio of 2.6 and 2.1, respectively). Female sex (hazard ratio of 0.30) and a higher baseline diffusing capacity of the lung for carbon monoxide (hazard ratio of 0.96) were associated with better survival. A definite usual interstitial pneumonia pattern on HRCT has important prognostic implications in RA-ILD.

  1. [HRCT patterns of the most important interstitial lung diseases

    NARCIS (Netherlands)

    Schaefer-Prokop, C.M.

    2014-01-01

    Interstitial lung diseases are a mixed group of diffuse parenchymal lung diseases which can have an acute or chronic course. Idiopathic diseases and diseases with an underlying cause (e.g. collagen vascular diseases) share the same patterns. Thin section computed tomography (CT) plays a central role

  2. Smoking-related interstitial lung diseases: radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Hidalgo, Alberto [Universidad Autonoma de Barcelona, Department of Radiology, Hospital de Sant Pau, Barcelona (Spain); Hospital de la Santa Creu i Sant Pau, Thoracic Radiology, Department of Radiology, Barcelona (Spain); Franquet, Tomas; Gimenez, Ana; Pineda, Rosa; Madrid, Marta [Universidad Autonoma de Barcelona, Department of Radiology, Hospital de Sant Pau, Barcelona (Spain); Bordes, Ramon [Universidad Autonoma de Barcelona, Department of Pathology, Hospital de Sant Pau, Barcelona (Spain)

    2006-11-15

    Smoking-related interstitial lung diseases (SRILD) are a heterogeneous group of entities of unknown cause. These diseases include desquamative interstitial pneumonia (DIP), respiratory-bronchiolitis-related interstitial lung disease (RB-ILD), pulmonary Langerhans' cell histiocytosis (LCH) and idiopathic pulmonary fibrosis (IPF). High-resolution CT is highly sensitive in the detection of abnormalities in the lung parenchyma and airways. Ground-glass attenuation can occur in DIP and RB-ILD. Whereas DIP is histologically characterized by intra-alveolar pigmented macrophages, RB-ILD shows alveolar macrophages in a patchy peribronchiolar distribution. LCH shows nodular infiltrates on histopathological examination containing varying amounts of characteristic Langerhans' histiocytes. The HRCT findings are characteristically bilateral, symmetrical and diffuse, involving the upper lobe zones with sparing of the costophrenic angles. The most prominent CT features are nodules (sometimes cavitary) measuring 1 to 10 mm in diameter, cysts and areas of ground-glass attenuation. Pathologically, IPF is characterized by its heterogeneity with areas of normal clung, alveolitis and end-stage fibrosis shown in the same biopsy specimen. High-resolution CT findings consist of honeycombing, traction bronchiectasis and intralobular interstitial thickening with subpleural and lower lung predominance. Since coexisting lesions in the same cases have been observed, a better understanding of the different smoking-related interstitial lung diseases (SRILD) allows a more confident and specific diagnosis. (orig.)

  3. Leflunomide-Induced Interstitial Lung Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Aygül Güzel

    2015-04-01

    Full Text Available Leflunomide (LEF induced interstitial pneumonitis is a very rare condition but potentially fatal. We report a case of LEF induced interstitial pneumonitis. A 63-year-old woman followed-up for 37 years with the diagnosis of rheumatoid arthritis treated with LEF (20 mg/day since 5 months were admitted to our hospital with cough, dyspnea, fever, and dark sputum.Chest radiography represented bilateral alveolar consolidation. High-resolution computed tomography demonstrated diffuse ground-glass appearance and interlobular septal thickening. Since the patient’s clinics and radiologic findings improved dramatically after the cessation of LEF and recieving oral steriod therapy, she was diagnosed as drug-induced interstitial lung disease. In conclusion, when nonspecific clinical signs such as respiratory distress, cough and fever seen during the use of LEF, drug-induced interstitial lung disease should be kept in mind for the differantial diagnosis.

  4. Treatment of intractable interstitial lung injury with alemtuzumab after lung transplantation

    DEFF Research Database (Denmark)

    Kohno, M; Perch, M; Andersen, E;

    2011-01-01

    of transplantation. Routine examination of a lung biopsy, 4 months after transplantation, showed nonspecific, diffuse interstitial inflammation with alveolar septal fibrosis. The patient's clinical status and imaging studies, consistent with nonspecific interstitial pneumonitis, which was considered as signs......, posttransplant antirejection drug regimen. We have since successfully treated with alemtuzumab three additional patients who developed interstitial lung injury after lung transplantation, who are also summarized in this report.......A 44-year-old woman underwent left single-lung transplantation for end-stage emphysema due to α1-antitrypsin deficiency in January 2010. Cyclosporine, azathioprine, and prednisolone were administered for immunosuppression and antithymocyte globulin for induction therapy at the time...

  5. An approach to interstitial lung disease in India

    Directory of Open Access Journals (Sweden)

    J N Pande

    2014-07-01

    Full Text Available Interstitial lung diseases are common and have varied etiology, clinical presentation, clinical course and outcome. They pose a diagnostic challenge to physicians and pulmonologists. Patients present with dry cough, exertional dyspnoea, interstitial lesions on X-ray of the chest and restrictive ventilatory defect on spirometry. A sharp decline in oxygen saturation with exercise is characteristic. Careful evaluation of the history of the patient and physical examination help in narrowing down diagnostic probabilities. HRCT of the chest has emerged as an important tool in the evaluation of these disorders. Idiopathic Interstitial Pneumonias (IIP are a group of conditions which are classified into several types based on pathological features. Bronchoscopic procedures are helpful in diagnosis of certain disorders but are of limited value in classification of IIP which requires surgical biopsy. Usual Interstitial Pneumonia (UIP, also referred to as Idiopathic Pulmonary Fibrosis, has a progressive course and an unfavourable outcome. Certain new drugs have recently become available for treatment of UIP. Our approach towards diagnosis and management of interstitial lung diseases based on personal experience over the past three decades is reported here. Key words: Usual interstitial pneumonia – sarcoidosis – pneumoconiosis – bronchoscopy – lung biopsy 

  6. Interstitial lung disease in the connective tissue diseases.

    Science.gov (United States)

    Antin-Ozerkis, Danielle; Rubinowitz, Ami; Evans, Janine; Homer, Robert J; Matthay, Richard A

    2012-03-01

    The connective tissue diseases (CTDs) are inflammatory, immune-mediated disorders in which interstitial lung disease (ILD) is common and clinically important. Interstitial lung disease may be the first manifestation of a CTD in a previously healthy patient. CTD-associated ILD frequently presents with the gradual onset of cough and dyspnea, although rarely may present with fulminant respiratory failure. Infection and drug reaction should always be ruled out. A diagnosis of idiopathic ILD should never be made without a careful search for subtle evidence of underlying CTD. Treatment of CTD-ILD typically includes corticosteroids and immunosuppressive agents.

  7. Detection of interstitial lung disease in PA chest radiographs

    Science.gov (United States)

    Loog, Marco; van Ginneken, Bram; Nielsen, Mads

    2004-05-01

    A computer-aided diagnosis scheme for the detection of interstitial disease in standard digital posteroanterior (PA) chest radiographs is presented. The detection technique is supervised-manually labelled data should be provided for training the algorithm-and fully automatic, and can be used as part of a computerized analysis scheme for X-ray lung images. Prior to the detection, a segmentation should be performed which delineates the lung field boundaries. Subsequently, a quadratic decision rule is employed for every pixel within the lung fields to associate with each pixel a probabilistic measure indicating interstitial disease. The locally obtained per-pixel probabilities are fused to a single global probability indicating to what extent there is interstitial disease present in the image. Finally, a threshold on this quantity classifies the image as containing interstitial disease or not. The probability combination scheme presented utilizes the quantiles of the local posterior probabilities to fuse the local probability into a global one. Using this nonparametric technique, reasonable results are obtained on the interstitial disease detection task. The area under the receiver operating characteristic equals 0.92 for the optimal setting.

  8. Rheumatoid interstitial lung disease presenting as cor pulmonale

    Directory of Open Access Journals (Sweden)

    Acharya Sourya

    2010-01-01

    Full Text Available Rheumatiod arthritis (RA is a multisystem connective tissue disorder. The predominant presentation is polyarticular, symmetric peripheral arthritis with relative sparing of axial skeleton. Inflammatory synovitis is the pathologic hallmark. Extra-articular manifestations of RA can involve several other organ systems and amongst them pulmonary manifestations occur commonly. We report a case of rheumatoid interstitial lung disease presenting as cor pulmonale.

  9. Rheumatoid interstitial lung disease presenting as cor pulmonale.

    Science.gov (United States)

    Acharya, Sourya; Mahajan, S N; Shukla, Samarth; Diwan, S K; Banode, Pankaj; Kothari, Nirmesh

    2010-10-01

    Rheumatiod arthritis (RA) is a multisystem connective tissue disorder. The predominant presentation is polyarticular, symmetric peripheral arthritis with relative sparing of axial skeleton. Inflammatory synovitis is the pathologic hallmark. Extra-articular manifestations of RA can involve several other organ systems and amongst them pulmonary manifestations occur commonly. We report a case of rheumatoid interstitial lung disease presenting as cor pulmonale.

  10. Rare Lung Diseases: Interstitial Lung Diseases and Lung Manifestations of Rheumatological Diseases.

    Science.gov (United States)

    Ramamurthy, Mahesh Babu; Goh, Daniel Y T; Lim, Michael Teik Chung

    2015-10-01

    The concept of Childhood Interstitial Lung Disease (ChILD) is relatively young. There has been tremendous progress in this field in the last decade. The key advance has been the recognition of interstitial lung diseases that are often distinct and occur mainly in infants. Diagnosis is challenging because the incidence is low and no single center in the world has enough cases to promote experience and clinical skills. This has led to formation of international groups of people interested in the field and the "Children's interstitial and diffuse lung disease research network" (ChILDRN) is one such group which contributed to the progress of this field. Clinically, these disorders overlap with those of other common respiratory disorders. Hence, clinical practice guidelines emphasize the additional role of chest imaging, genetic testing and lung biopsy in the diagnostic evaluation. Genetic testing, in particular, has shown tremendous progress in this field. Being noninvasive, it has the potential to help early recognition in a vast majority. Despite progress, definitive therapeutic modalities are still lacking and supportive care is still the backbone of management in the majority. Early recognition of the definitive diagnosis helps in the management, even if, in a significant number, it helps in avoiding unnecessary therapy. Also discussed in this article, is the pulmonary manifestation of rheumatic diseases in children. The incidence and spectrum of pulmonary involvement in rheumatic conditions vary and can be result of the primary disease or its management or due to an concurrent infection.

  11. Update in diagnosis and management of interstitial lung disease.

    Science.gov (United States)

    Mikolasch, Theresia A; Garthwaite, Helen S; Porter, Joanna C

    2016-12-01

    The field of interstitial lung disease (ILD) has undergone significant evolution in recent years, with an increasing incidence and more complex, ever expanding disease classification. In their most severe forms, these diseases lead to progressive loss of lung function, respiratory failure and eventually death. Despite notable advances, progress has been challenged by a poor understanding of pathological mechanisms and patient heterogeneity, including variable progression. The diagnostic pathway is thus being continually refined, with the introduction of tools such as transbronchial cryo lung biopsy and a move towards genetically aided, precision medicine. In this review, we focus on how to approach a patient with ILD and the diagnostic process.

  12. [Subclinical interstitial lung disease associated with rheumatoid arthritis].

    Science.gov (United States)

    Bestaev, D V

    2014-01-01

    Interstitial lung disease (ILD) in rheumatoid arthritis (RA) is its extra-articular manifestation. At the same time, ILD considerably worsens the prognosis of the disease. Mortality rates for interstitial pulmonary fibrosis are 6% of all-cause mortality in RA patients. ILD can be identified by clinical examination only in 2-6% of cases, by plain chest X-ray in 1-6%, and by high-resolution computed tomography in 50-60%. The paper deals with subclinical ILD and discusses the state-of-the-art of investigations in this area.

  13. Histopathologic approach to the surgical lung biopsy in interstitial lung disease.

    Science.gov (United States)

    Jones, Kirk D; Urisman, Anatoly

    2012-03-01

    Interpretation of lung biopsy specimens is an integral part in the diagnosis of interstitial lung disease (ILD). The process of evaluating a surgical lung biopsy for disease involves answering several questions. Unlike much of surgical pathology of neoplastic lung disease, arriving at the correct diagnosis in nonneoplastic lung disease often requires correlation with clinical and radiologic findings. The topic of ILD or diffuse infiltrative lung disease covers several hundred entities. This article is meant to be a launching point in the clinician's approach to the histologic evaluation of lung disease.

  14. Gastroesophageal Reflux Disease in Children with Interstitial Lung Disease.

    Science.gov (United States)

    Dziekiewicz, M A; Karolewska-Bochenek, K; Dembiński, Ł; Gawronska, A; Krenke, K; Lange, J; Banasiuk, M; Kuchar, E; Kulus, M; Albrecht, P; Banaszkiewicz, A

    2016-01-01

    Gastroesophageal reflux disease is common in adult patients with interstitial lung disease. However, no data currently exist regarding the prevalence and characteristics of the disease in pediatric patients with interstitial lung disease. The aim of the present study was to prospectively assess the incidence of gastroesophageal reflux disease and characterize its features in children with interstitial lung disease. Gastroesophageal reflux disease was established based on 24 h pH-impedance monitoring (MII-pH). Gastroesophageal reflux episodes (GERs) were classified according to widely recognized criteria as acid, weakly acid, weakly alkaline, or proximal. Eighteen consecutive patients (15 boys, aged 0.2-11.6 years) were enrolled in the study. Gastroesophageal reflux disease was diagnosed in a half (9/18) of children. A thousand GERs were detected by MII-pH (median 53.5; IQR 39.0-75.5). Of these, 585 (58.5 %) episodes were acidic, 407 (40.7 %) were weakly acidic, and eight (0.8 %) were weakly alkaline. There were 637 (63.7 %) proximal GERs. The patients in whom gastroesophageal reflux disease was diagnosed had a significantly higher number of proximal and total GERs. We conclude that the prevalence of gastroesophageal reflux disease in children with interstitial lung disease is high; thus, the disease should be considered regardless of presenting clinical symptoms. A high frequency of non-acid and proximal GERs makes the MII-pH method a preferable choice for the detection of reflux episodes in this patient population.

  15. Neural network approach for differential diagnosis of interstitial lung diseases

    Science.gov (United States)

    Asada, Naoki; Doi, Kunio; MacMahon, Heber; Montner, Steven M.; Giger, Maryellen L.; Abe, Chihiro; Wu, Chris Y.

    1990-07-01

    A neural network approach was applied for the differential diagnosis of interstitial lung diseases. The neural network was designed for distinguishing between 9 types of interstitial lung diseases based on 20 items of clinical and radiographic information. A database for training and testing the neural network was created with 10 hypothetical cases for each of the 9 diseases. The performance of the neural network was evaluated by ROC analysis. The optimal parameters for the current neural network were determined by selecting those yielding the highest ROC curves. In this case the neural network consisted of one hidden layer including 6 units and was trained with 200 learning iterations. When the decision performances of the neural network chest radiologists and senior radiology residents were compared the neural network indicated high performance comparable to that of chest radiologists and superior to that of senior radiology residents. Our preliminary results suggested strongly that the neural network approach had potential utility in the computer-aided differential diagnosis of interstitial lung diseases. 1_

  16. [Severe interstitial lung disease from pathologic gastroesophageal reflux in children].

    Science.gov (United States)

    Ahrens, P; Weimer, B; Hofmann, D

    1999-07-01

    Interstitial lung diseases comprise a heterogeneous group of pulmonary conditions that cause restrictive lung disease of poor prognosis, especially if growth failure, pulmonary hypertension and fibrosis appears. We report on the case of a girl of 11 years of age who suffered from severe nonallergic asthma in early childhood and who developed severe interstitial pulmonary disease caused by gastro-oesophageal reflux at the age of 8 years. This diagnosis was established by lung biopsy, bronchoalveolar lavage and a high amount of lipid-laden alveolar macrophages, 2-level pH measurement and oesophageal biopsy. Because therapy with oral and inhaled steroids failed and Omeprazol showed benificial effects, hemifundoplication according to THAL was performed. At present the lung function is clearly normal and there is no need of any medicaments. Following the history, we can assume the pathological gastro-oesophageal reflux to be the cause of the disease. It is important to state that there were no typical symptoms at any time pointing to gastro-oesophageal reflux disease. The development of pulmonary disease by pathological reflux is very often caused by "silent aspiration". Very typically there are no symptoms such as vomiting, heartburn and pain but only signs of chronic lung disease.

  17. Interstitial lung disease induced by alectinib (CH5424802/RO5424802).

    Science.gov (United States)

    Ikeda, Satoshi; Yoshioka, Hiroshige; Arita, Machiko; Sakai, Takahiro; Sone, Naoyuki; Nishiyama, Akihiro; Niwa, Takashi; Hotta, Machiko; Tanaka, Tomohiro; Ishida, Tadashi

    2015-02-01

    A 75-year-old woman with anaplastic lymphoma kinase (ALK)-rearranged Stage IV lung adenocarcinoma was administered the selective anaplastic lymphoma kinase inhibitor, alectinib, as a third-line treatment in a Phase 1-2 study. On the 102nd day, chest computed tomography showed diffuse ground glass opacities. Laboratory data revealed high serum levels of KL-6, SP-D and lactate dehydrogenase without any clinical symptoms. There was no evidence of infection. Marked lymphocytosis was seen in bronchoalveolar lavage fluid analysis, and transbronchial lung biopsy showed mild thickening of alveolar septa and lymphocyte infiltration. Interstitial lung disease was judged to be related to alectinib based on improvements in imaging findings and serum biomarkers after discontinuation of alectinib. To our knowledge, this is the first reported case of alectinib-induced interstitial lung disease. Alectinib is a promising drug for ALK-rearranged non-small cell lung cancer. Clinical trials of this selective anaplastic lymphoma kinase inhibitor will facilitate the meticulous elucidation of its long-term safety profile.

  18. Classification of interstitial lung disease patterns with topological texture features

    Science.gov (United States)

    Huber, Markus B.; Nagarajan, Mahesh; Leinsinger, Gerda; Ray, Lawrence A.; Wismüller, Axel

    2010-03-01

    Topological texture features were compared in their ability to classify morphological patterns known as 'honeycombing' that are considered indicative for the presence of fibrotic interstitial lung diseases in high-resolution computed tomography (HRCT) images. For 14 patients with known occurrence of honey-combing, a stack of 70 axial, lung kernel reconstructed images were acquired from HRCT chest exams. A set of 241 regions of interest of both healthy and pathological (89) lung tissue were identified by an experienced radiologist. Texture features were extracted using six properties calculated from gray-level co-occurrence matrices (GLCM), Minkowski Dimensions (MDs), and three Minkowski Functionals (MFs, e.g. MF.euler). A k-nearest-neighbor (k-NN) classifier and a Multilayer Radial Basis Functions Network (RBFN) were optimized in a 10-fold cross-validation for each texture vector, and the classification accuracy was calculated on independent test sets as a quantitative measure of automated tissue characterization. A Wilcoxon signed-rank test was used to compare two accuracy distributions and the significance thresholds were adjusted for multiple comparisons by the Bonferroni correction. The best classification results were obtained by the MF features, which performed significantly better than all the standard GLCM and MD features (p interstitial lung diseases when compared to standard texture analysis methods.

  19. Macrophage inflammatory protein-1 alpha expression in interstitial lung disease.

    Science.gov (United States)

    Standiford, T J; Rolfe, M W; Kunkel, S L; Lynch, J P; Burdick, M D; Gilbert, A R; Orringer, M B; Whyte, R I; Strieter, R M

    1993-09-01

    Mononuclear phagocyte (M phi) recruitment and activation is a hallmark of a number of chronic inflammatory diseases of the lung, including sarcoidosis and idiopathic pulmonary fibrosis (IPF). We hypothesized that macrophage inflammatory protein-1 (MIP-1 alpha), a peptide with leukocyte activating and chemotactic properties, may play an important role in mediating many of the cellular changes that occur in sarcoidosis and IPF. In initial experiments, we demonstrated that human rMIP-1 alpha exerted chemotactic activities toward both polymorphonuclear leukocytes and monocytes, and these activities were inhibited by treatment with rabbit anti-human MIP-1 alpha antiserum. In support of the potential role of MIP-1 alpha in interstitial lung disease, we detected MIP-1 alpha in the bronchoalveolar lavage fluid of 22/23 patients with sarcoidosis (mean 443 +/- 76 pg/ml) and 9/9 patients with IPF (mean 427 +/- 81 pg/ml), whereas detectable MIP-1 alpha was found in only 1/7 healthy subjects (mean 64 +/- 64 pg/ml). In addition, we found a 2.5- and 1.8-fold increase in monocyte chemotactic activity in BALF obtained from patients with sarcoidosis and IPF respectively, as compared to healthy subjects, and this monocyte chemotactic activity, but not neutrophil chemotactic activity, was reduced by approximately 22% when bronchoalveolar lavage fluid from sarcoidosis and IPF patients were preincubated with rabbit antihuman MIP-1 alpha antibodies. To determine the cellular source(s) of MIP-1 alpha within the lung, we performed immunohistochemical analysis of bronchoalveolar lavage cell pellets, transbronchial biopsies, and open lung biopsies obtained from patients with IPF and sarcoidosis. Substantial expression of cell-associated MIP-1 alpha was detected in M phi, including both alveolar AM phi and interstitial M phi. In addition, interstitial fibroblasts within biopsies obtained from sarcoid and IPF patients also expressed immunoreactive MIP-1 alpha. Minimal to no detectable MIP-1

  20. Building a reference multimedia database for interstitial lung diseases.

    Science.gov (United States)

    Depeursinge, Adrien; Vargas, Alejandro; Platon, Alexandra; Geissbuhler, Antoine; Poletti, Pierre-Alexandre; Müller, Henning

    2012-04-01

    This paper describes the methodology used to create a multimedia collection of cases with interstitial lung diseases (ILDs) at the University Hospitals of Geneva. The dataset contains high-resolution computed tomography (HRCT) image series with three-dimensional annotated regions of pathological lung tissue along with clinical parameters from patients with pathologically proven diagnoses of ILDs. The motivations for this work is to palliate the lack of publicly available collections of ILD cases to serve as a basis for the development and evaluation of image-based computerized diagnostic aid. After 38 months of data collection, the library contains 128 patients affected with one of the 13 histological diagnoses of ILDs, 108 image series with more than 41l of annotated lung tissue patterns as well as a comprehensive set of 99 clinical parameters related to ILDs. The database is available for research on request and after signature of a license agreement.

  1. Classification of interstitial lung disease patterns with topological texture features

    CERN Document Server

    Huber, Markus B; Leinsinger, Gerda; Ray, Lawrence A; Wismüller, Axel; 10.1117/12.844318

    2010-01-01

    Topological texture features were compared in their ability to classify morphological patterns known as 'honeycombing' that are considered indicative for the presence of fibrotic interstitial lung diseases in high-resolution computed tomography (HRCT) images. For 14 patients with known occurrence of honey-combing, a stack of 70 axial, lung kernel reconstructed images were acquired from HRCT chest exams. A set of 241 regions of interest of both healthy and pathological (89) lung tissue were identified by an experienced radiologist. Texture features were extracted using six properties calculated from gray-level co-occurrence matrices (GLCM), Minkowski Dimensions (MDs), and three Minkowski Functionals (MFs, e.g. MF.euler). A k-nearest-neighbor (k-NN) classifier and a Multilayer Radial Basis Functions Network (RBFN) were optimized in a 10-fold cross-validation for each texture vector, and the classification accuracy was calculated on independent test sets as a quantitative measure of automated tissue characteriza...

  2. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.

    Science.gov (United States)

    Miura, Yukiko; Saito, Takefumi; Fujita, Kazutaka; Tsunoda, Yoshiya; Tanaka, Toru; Takoi, Hiroyuki; Yatagai, Yohei; Rin, Shigen; Sekine, Akimasa; Hayashihara, Kenji; Nei, Takahito; Azuma, Arata

    2014-10-20

    Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

  3. Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Castillo, Monette; Vade, Aruna; Lim-Dunham, Jennifer Eden [Loyola University Health System, Department of Radiology, Maywood, IL (United States); Masuda, Emi [Henry Ford Hospital, Department of Radiology, Detroit, MI (United States); Massarani-Wafai, Rasan [Loyola University Health System, Department of Pathology, Maywood, IL (United States)

    2010-09-15

    Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces. An open lung biopsy demonstrated a minor component of PIG superimposed upon poor alveolarization. PIG in the setting of lung growth abnormality might be more common than previously described. Additionally, radiographic findings associated with most pediatric interstitial lung diseases are nonspecific, and histopathologic correlation is essential for diagnosis. (orig.)

  4. A case of interstitial lung disease associated with clinically amyopathic dermatomyositis: radiologic-pathologic correlation.

    Science.gov (United States)

    Okubo, Gosuke; Noma, Satoshi; Nishimoto, Yuko; Sada, Ryuichi; Kobashi, Yoichiro

    2013-01-01

    This case report describes a 64-year-old woman with interstitial lung disease associated with clinically amyopathic dermatomyositis. Chest computed tomography revealed consolidations along bronchovascular bundles in the periphery of the lower lungs. Interstitial lung disease developed acutely, and the patient died 3 months after the clinical diagnosis. An autopsy was performed, and a large section of the lung specimen was prepared. Various interstitial lesions including organizing pneumonia, cellular and fibrotic nonspecific interstitial pneumonia, and diffuse alveolar damage were seen in the large section. Correlating the large section and computed tomography images was useful for determining the distribution of diffuse alveolar damage.

  5. Rheumatoid Arthritis-Associated Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis: Shared Mechanistic and Phenotypic Traits Suggest Overlapping Disease Mechanisms.

    Science.gov (United States)

    Paulin, Francisco; Doyle, Tracy J; Fletcher, Elaine A; Ascherman, Dana P; Rosas, Ivan O

    2015-01-01

    The prevalence of clinically evident interstitial lung disease in patients with rheumatoid arthritis is approximately 10%. An additional 33% of undiagnosed patients have interstitial lung abnormalities that can be detected with high-resolution computed tomography. Rheumatoid arthritis-interstitial lung disease patients have three times the risk of death compared to those with rheumatoid arthritis occurring in the absence of interstitial lung disease, and the mortality related to interstitial lung disease is rising. Rheumatoid arthritis-interstitial lung disease is most commonly classified as the usual interstitial pneumonia pattern, overlapping mechanistically and phenotypically with idiopathic pulmonary fibrosis, but can occur in a non-usual interstitial pneumonia pattern, mainly nonspecific interstitial pneumonia. Based on this, we propose two possible pathways to explain the coexistence of rheumatoid arthritis and interstitial lung disease: (i) Rheumatoid arthritis-interstitial lung disease with a non-usual interstitial pneumonia pattern may come about when an immune response against citrullinated peptides taking place in another site (e.g. the joints) subsequently affects the lungs; (ii) Rheumatoid arthritis-interstitial lung disease with a usual interstitial pneumonia pattern may represent a disease process in which idiopathic pulmonary fibrosis-like pathology triggers an immune response against citrullinated proteins that promotes articular disease indicative of rheumatoid arthritis. More studies focused on elucidating the basic mechanisms leading to different sub-phenotypes of rheumatoid arthritis-interstitial lung disease and the overlap with idiopathic pulmonary fibrosis are necessary to improve our understanding of the disease process and to define new therapeutic targets.

  6. Segmentation of interstitial lung disease patterns in HRCT images

    Science.gov (United States)

    Dash, Jatindra K.; Madhavi, Vaddepalli; Mukhopadhyay, Sudipta; Khandelwal, Niranjan; Kumar, Prafulla

    2015-03-01

    Automated segmentation of pathological bearing region is the first step towards the development of lung CAD. Most of the work reported in the literature related to automated analysis of lung tissue aims towards classification of fixed sized block into one of the classes. This block level classification of lung tissues in the image never results in accurate or smooth boundaries between different regions. In this work, effort is taken to investigate the performance of three automated image segmentation algorithms those results in smooth boundaries among lung tissue patterns commonly encountered in HRCT images of the thorax. A public database that consists of HRCT images taken from patients affected with Interstitial Lung Diseases (ILDs) is used for the evaluation. The algorithms considered are Markov Random Field (MRF), Gaussian Mixture Model (GMM) and Mean Shift (MS). 2-fold cross validation approach is followed for the selection of the best parameter value for individual algorithm as well as to evaluate the performance of all the algorithms. Mean shift algorithm is observed as the best performer in terms of Jaccard Index, Modified Hausdorff Distance, accuracy, Dice Similarity Coefficient and execution speed.

  7. Rheumatoid Arthritis (RA) associated interstitial lung disease (ILD).

    LENUS (Irish Health Repository)

    O'Dwyer, David N

    2013-10-01

    Rheumatoid Arthritis (RA) is the most common Connective Tissue Disease (CTD) and represents an increasing burden on global health resources. Interstitial lung disease (ILD) has been recognised as a complication of RA but its potential for mortality and morbidity has arguably been under appreciated for decades. New studies have underscored a significant lifetime risk of ILD development in RA. Contemporary work has identified an increased risk of mortality associated with the Usual Interstitial Pneumonia (UIP) pattern which shares similarity with the most devastating of the interstitial pulmonary diseases, namely Idiopathic Pulmonary Fibrosis (IPF). In this paper, we discuss recent studies highlighting the associated increase in mortality in RA-UIP. We explore associations between radiological and histopathological features of RA-ILD and the prognostic implications of same. We emphasise the need for translational research in this area given the growing burden of RA-ILD. We highlight the importance of the respiratory physician as a key stakeholder in the multidisciplinary management of this disorder. RA-ILD focused research offers the opportunity to identify early asymptomatic disease and define the natural history of this extra articular manifestation. This may provide a unique opportunity to define key regulatory fibrotic events driving progressive disease. We also discuss some of the more challenging and novel aspects of therapy for RA-ILD.

  8. The Multifaceted Aspects of Interstitial Lung Disease in Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Lorenzo Cavagna

    2013-01-01

    Full Text Available Interstitial lung disease (ILD is a relevant extra-articular manifestation of rheumatoid arthritis (RA that may occur either in early stages or as a complication of long-standing disease. RA related ILD (RA-ILD significantly influences the quoad vitam prognosis of these patients. Several histopathological patterns of RA-ILD have been described: usual interstitial pneumonia (UIP is the most frequent one, followed by nonspecific interstitial pneumonia (NSIP; other patterns are less commonly observed. Several factors have been associated with an increased risk of developing RA-ILD. The genetic background plays a fundamental but not sufficient role; smoking is an independent predictor of ILD, and a correlation with the presence of rheumatoid factor and anti-cyclic citrullinated peptide antibodies has also been reported. Moreover, both exnovo occurrence and progression of ILD have been related to drug therapies that are commonly prescribed in RA, such as methotrexate, leflunomide, anti-TNF alpha agents, and rituximab. A greater understanding of the disease process is necessary in order to improve the therapeutic approach to ILD and RA itself and to reduce the burden of this severe extra-articular manifestation.

  9. Treatment of intractable interstitial lung injury with alemtuzumab after lung transplantation

    DEFF Research Database (Denmark)

    Kohno, M; Perch, M; Andersen, E;

    2011-01-01

    of acute rejection, worsened within 2 weeks, despite high-dose steroids, change of calcineurin inhibitor, and plasmapheresis. Within a few days after a single, 10-mg, intravenous dose of alemtuzumab, the patient's health improved markedly. She has remained stable for 4 months on a standard, ambulatory......, posttransplant antirejection drug regimen. We have since successfully treated with alemtuzumab three additional patients who developed interstitial lung injury after lung transplantation, who are also summarized in this report....

  10. Sequential change of high-resolution CT findings of interstitial lung disease in polymyositis and dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Tsukada, Hiroshi; Furuizumi, Naoya; Akita, Shinichi; Oda, Junichi; Sakai, Kunio; Suzuki, Eiichi; Emura, Iwao (Niigata Univ. (Japan). School of Medicine)

    1994-01-01

    Sequential changes of interstitial lung disease in fourteen patients of polymyositis/dermatomyositis (PM/DM) were followed up by high-resolution CT (HRCT). Most frequent CT findings were intense lung attenuation (ILA) with volume loss and slightly increased lung attenuation (SILA). Open lung biopsy was performed in a case with ILA shadow which revealed so-called usual interstitial pneumonia (UIP). Most intense ILA and SILA shadows resolved after steroid therapy. Some of ILA, however, reappeared and accompanied more prominent volume loss findings than before treatment. We think HRCT findings of interstitial lung disease in PM/DM may indicate prognosis of these diagnoses to some degree. (author).

  11. Interstitial lung disease associated with human papillomavirus vaccination

    Directory of Open Access Journals (Sweden)

    Yasushi Yamamoto

    2015-01-01

    Full Text Available Vaccinations against the human papillomavirus (HPV have been recommended for the prevention of cervical cancer. HPV-16/18 AS04-adjuvanted vaccines (Cervarix are said to have favourable safety profiles. Interstitial lung diseases (ILDs can occur following exposure to a drug or a biological agent. We report a case of ILD associated with a Cervarix vaccination. A woman in her 40's, with a history of conisation, received three inoculations of Cervarix. Three months later, she presented with a cough and shortness of breath. Findings from a computed tomography of the chest and a transbronchial lung biopsy were consistent with non-specific interstitial pneumonia. Workup eliminated all other causes of the ILD, except for the vaccination. Over the 11 months of the follow-up period, her symptoms resolved without steroid therapy. The onset and spontaneous resolution of the ILD showed a chronological association with the HPV vaccination. The semi-quantitative algorithm revealed that the likelihood of an adverse drug reaction to Cervarix was “Probable”. The outcome was relatively good, but more attention should be paid to a potential risk for HPV vaccinations to cause ILDs. Wherever possible, chest radiographic examinations should be performed in order not to overlook any ILDs.

  12. Clinical features of rheumatoid arthritis-associated interstitial lung disease.

    Science.gov (United States)

    Wang, Ting; Zheng, Xing-Ju; Liang, Bin-Miao; Liang, Zong-An

    2015-10-07

    Interstitial lung disease (ILD) is the most common extra-articular manifestations of rheumatoid arthritis (RA) in the lung. This study aimed to identify clinical features of RA-associated ILD (RA-ILD). Patients with RA were retrospectively enrolled and sub-classified as RA-ILD or RA without ILD based on high-resolution computed tomography imaging. Pulmonary function testing parameters and levels of RA-related biomarkers, tumour markers, and acute-phase proteins were compared between the two groups. Logistic regression model was used to assess the strength of association between RA-ILD and clinical features of interest. Receiver operating characteristic analysis was performed to assess potential predictive value of clinical features for detecting RA-ILD. Comparison analysis indicated that the percentage of predicted value of total lung capacity, inspiratory capacity, and diffusion capacity of the lung for carbon monoxide (DLCO) were reduced in patients with RA-ILD. Tumour markers CA15-3 and CA125 were increased in patients with RA-ILD. Logistic regression analysis revealed that decreased DLCO was related to the increased likelihood of RA-ILD (OR = 0.94, 95%CI = [0.91, 0.98]). The cut-off point at 52.95 percent of predicted value could sensitively discriminate RA patients with or without ILD. Our study suggested that DLCO value could be a useful tool for detecting ILD in patients with RA.

  13. Interstitial irradiation for unresectable carcinoma of the lung.

    Science.gov (United States)

    Hilaris, B S; Martini, N; Batata, M; Beattie, E J

    1975-11-01

    From 1963 to 1971, 105 patients with histologically proved cancer of the lung were explored at Memorial Hospital and underwent interstitial implantation using encapsulated sources of radon 222 (53 patients) or iodine 125 (52 patients). These lung cancers were considered unresectable because of extension of the disease into the mediastinum with fixation or invasion of the major vessels, trachea, and esophagus or chest wall involvement. No apical lesions, which have a better prognosis, are included in this review. Sixty-nine patients had epidermoid cancer, 24 had adenocarcinoma, and the remaining 12 had various other histological types. All patients were staged according to the criteria proposed by the American Joint Committee using the TNM definitions (standing for tumor, nodes, and metastasis). Local control was obtained in 8 of 10 patients (80% with clinical Stage I and II unresectable cancers of the lung and in 44 of the 95 (46%) with clinical Stage III lung cancer. The two-year survival was 50% for Stages I and II and 7% for Stage III. Five patients have survived for five years or more. The complications, disease-free interval, local recurrences, distant metastases, and survival are presented and indications for this type of therapy outlined.

  14. Advanced sickle cell associated interstitial lung disease presenting with cor pulmonale in a Nigerian.

    Science.gov (United States)

    Fawibe, Ademola Emmanuel; Kolo, Philip Manman; Ogunmodede, James Ayodele; Desalu, Olufemi Olumuyiwa; Salami, Kazeem Alakija

    2012-04-01

    Previous studies have reported abnormal pulmonary function and pulmonary hypertension among Nigerians with sickle cell disease, but there is no report of interstitial lung disease among them. We report a Nigerian sickle cell patient who presented with computed tomography proven interstitial lung disease complicated by pulmonary hypertension and cor pulmonale.

  15. Advanced sickle cell associated interstitial lung disease presenting with cor pulmonale in a Nigerian

    Directory of Open Access Journals (Sweden)

    Ademola Emmanuel Fawibe

    2012-01-01

    Full Text Available Previous studies have reported abnormal pulmonary function and pulmonary hypertension among Nigerians with sickle cell disease, but there is no report of interstitial lung disease among them. We report a Nigerian sickle cell patient who presented with computed tomography proven interstitial lung disease complicated by pulmonary hypertension and cor pulmonale.

  16. Driving performance in patients with chronic obstructive lung disease, interstitial lung disease and healthy controls

    DEFF Research Database (Denmark)

    Prior, Thomas Skovhus; Troelsen, Thomas Tværmose; Hilberg, Ole

    2015-01-01

    INTRODUCTION: Cognitive deficits in patients suffering from chronic obstructive pulmonary disease (COPD) have been described and hypoxaemia has been addressed as a possible cause. Cognitive functions in patients with interstitial lung disease (ILD) are not well studied. These patients are taking....... METHODS: 16 patients with COPD (8 receivers and 8 non-receivers of long-term oxygen therapy (LTOT)), 8 patients with ILD (consisting of idiopathic interstitial pneumonias) and 8 healthy controls were tested in a driving simulator. Each test lasted 45 min. In the oxygen intervention part of the study...

  17. Prevalence and prognosis of unclassifiable interstitial lung disease.

    Science.gov (United States)

    Ryerson, Christopher J; Urbania, Thomas H; Richeldi, Luca; Mooney, Joshua J; Lee, Joyce S; Jones, Kirk D; Elicker, Brett M; Koth, Laura L; King, Talmadge E; Wolters, Paul J; Collard, Harold R

    2013-09-01

    The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour. Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour. Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p = 0.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p = 0.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p = 0.001) and a radiological fibrosis score (p = 0.02). Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables.

  18. Statin Use Is Associated with Reduced Mortality in Patients with Interstitial Lung Disease

    DEFF Research Database (Denmark)

    Vedel-Krogh, Signe; Nielsen, Sune F; Nordestgaard, Børge G

    2015-01-01

    INTRODUCTION: We hypothesized that statin use begun before the diagnosis of interstitial lung disease is associated with reduced mortality. METHODS: We studied all patients diagnosed with interstitial lung disease in the entire Danish population from 1995 through 2009, comparing statin use versus...... no statin use in a nested 1:2 matched study. RESULTS: The cumulative survival as a function of follow-up time from the date of diagnosis of interstitial lung disease (n = 1,786 + 3,572) and idiopathic lung fibrosis (n = 261 + 522) was higher for statin users versus never users (log-rank: P = 7 · 10......(-9) and P = 0.05). The median survival time in patients with interstitial lung disease was 3.3 years in statin users and 2.1 years in never users. Corresponding values in patients with idiopathic lung fibrosis were 3.4 versus 2.4 years. After multivariable adjustment, the hazard ratio for all...

  19. Rheumatoid Arthritis-Associated Interstitial Lung Disease: Diagnostic Dilemma

    Directory of Open Access Journals (Sweden)

    Mark J. Hamblin

    2011-01-01

    Full Text Available Interstitial lung disease (ILD is an increasingly recognized complication of rheumatoid arthritis (RA contributing to significantly increased morbidity and mortality. Diagnosis can be challenging since patients are unlikely to report dyspnea due to an overall decrease in physical activity with advanced arthritic symptoms. Additionally, infections, drug toxicity, and environmental toxins can mimic ILD, creating significant diagnostic dilemmas for the clinician. In this paper we will explore an effective clinical algorithm for the diagnosis of RA-ILD. We will also discuss features of drug-related toxicities, infections, and environmental toxins that comprise the main entities in the differential diagnosis of RA-ILD. Finally, we will explore the known and experimental treatment options that may have some benefit in the treatment of RA-ILD.

  20. Cellular interactions in the pathogenesis of interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    Gianluca Bagnato

    2015-03-01

    Full Text Available Interstitial lung disease (ILD encompasses a large and diverse group of pathological conditions that share similar clinical, radiological and pathological manifestations, despite potentially having quite different aetiologies and comorbidities. Idiopathic pulmonary fibrosis (IPF represents probably the most aggressive form of ILD and systemic sclerosis is a multiorgan fibrotic disease frequently associated with ILD. Although the aetiology of these disorders remains unknown, in this review we analyse the pathogenic mechanisms by cell of interest (fibroblast, fibrocyte, myofibroblast, endothelial and alveolar epithelial cells and immune competent cells. New insights into the complex cellular contributions and interactions will be provided, comparing the role of cell subsets in the pathogenesis of IPF and systemic sclerosis.

  1. Beyond pneumonoconiosis: Recently described occupational interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    Evangelia Nena

    2010-01-01

    Full Text Available SUMMARY. Recent technological innovations have resulted in the introduction of new substances in different manufacturing procedures. Unfortunately, lack of knowledge of the adverse effects of some novel substances has led to the development of interstitial lung disease (ILD among exposed workers. Exposure to diacetyl can cause bronchiolitis obliterans (“popcorn lung”, while exposure to nylon flock, Acramin-FWN, indium-tin oxide, biomass fuels or nanoparticles is associated with ILD. In addition, hypersensitivity pneumonitis can occur after exposure to additives in animal feed. Finally, new applications of substances already known to be hazardous can result in the occurrence of ILD in exposed workers. Pneumon 2010, 23(3:293-300.

  2. Usefulness of texture features for segmentation of lungs with severe diffuse interstitial lung disease

    Science.gov (United States)

    Wang, Jiahui; Li, Feng; Li, Qiang

    2010-03-01

    We developed an automated method for the segmentation of lungs with severe diffuse interstitial lung disease (DILD) in multi-detector CT. In this study, we would like to compare the performance levels of this method and a thresholdingbased segmentation method for normal lungs, moderately abnormal lungs, severely abnormal lungs, and all lungs in our database. Our database includes 31 normal cases and 45 abnormal cases with severe DILD. The outlines of lungs were manually delineated by a medical physicist and confirmed by an experienced chest radiologist. These outlines were used as reference standards for the evaluation of the segmentation results. We first employed a thresholding technique for CT value to obtain initial lungs, which contain normal and mildly abnormal lung parenchyma. We then used texture-feature images derived from co-occurrence matrix to further segment lung regions with severe DILD. The segmented lung regions with severe DILD were combined with the initial lungs to generate the final segmentation results. We also identified and removed the airways to improve the accuracy of the segmentation results. We used three metrics, i.e., overlap, volume agreement, and mean absolute distance (MAD) between automatically segmented lung and reference lung to evaluate the performance of our segmentation method and the thresholding-based segmentation method. Our segmentation method achieved a mean overlap of 96.1%, a mean volume agreement of 98.1%, and a mean MAD of 0.96 mm for the 45 abnormal cases. On the other hand the thresholding-based segmentation method achieved a mean overlap of 94.2%, a mean volume agreement of 95.8%, and a mean MAD of 1.51 mm for the 45 abnormal cases. Our new method obtained higher performance level than the thresholding-based segmentation method.

  3. Interstitial lung disease in systemic sclerosis: where do we stand?

    Directory of Open Access Journals (Sweden)

    Susanna Cappelli

    2015-09-01

    Full Text Available Interstitial lung disease (ILD is common in systemic sclerosis (SSc patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease. Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described. Nonselective immunosuppressors are still the main treatment for ILD, with cyclophosphamide (CYC most widely used to obtain remission. Novel therapies towards specific molecular and cellular targets have been suggested; in particular, rituximab (RTX has shown promising results, but further research is needed. It is of paramount importance to define the severity of the disease and the risk of progression in order to define the need for treatment and the treatment intensity. We propose the division of the treatment strategies at our disposal to induce remission into three categories: high intensity (haematopoietic stem cell transplantation, medium intensity (CYC and RTX and low intensity (azathioprine (AZA and mycophenolate mofetil (MMF. After obtaining remission, maintenance treatment with AZA or MMF should be started. In this review we explore new advances in the pathogenesis, diagnosis and treatment of SSc-ILD.

  4. High-resolution CT study of interstitial lung disease in polymyositis and dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Tomii, Keisuke; Iwata, Takekuni; Oida, Kazukiyo (Tenri Hospital, Nara (Japan)) (and others)

    1992-01-01

    High-resolution CT scans of lung parenchyma were obtained in 8 patients with pure polymyositis or dermatomyositis (PM-DM) with interstitial lung involvement. The most frequent findings were subpleural curved band-like shadows (A 2), which were demonstrated in 7 patients (88%). Open lung biopsy was performed in a case of A 2 shadow, which revealed non-specific interstitial pneumonia (chronic interstitial pneumonia, not otherwise specified). Most of the A 2 shadows gradually shrinked with steroids or immunosuppressants, and then disappeared completely or changed into subpleural curvilinear shadows (A 1). Peri-bronchovascular shadows (B) were detected in 4 patients (50%). Acute exacerbation had occurred in the two of them, but after all every B shadow reduced or disappeared with therapy. We think A 2 and B shadows are characteristic HRCT findings of interstitial lung disease in PM-DM and their prognosis is fairly good. (author).

  5. Video-Assisted Thoracoscopic Lung Biopsy as a Possible Cause of Acute Interstitial Pneumonia in a Patient with Nonspecific Interstitial Pneumonia

    Directory of Open Access Journals (Sweden)

    D Jeffrey Moore

    2004-01-01

    Full Text Available The present case report describes a 44-year-old woman who presented with dyspnea due to diffuse interstitial lung disease. High-resolution computed tomography showed features of usual interstitial pneumonia, but the lung biopsy obtained by video-assisted thoracoscopy was consistent with a histological pattern of nonspecific interstitial pneumonia. Following the procedure, the patient developed progressive respiratory distress and died on postoperative day 13 with a clinical picture of acute interstitial pneumonia. The autopsy showed evidence of diffuse alveolar damage superimposed on the background pattern of nonspecific interstitial pneumonia. The present case report supports the notion that patients with a variety of subtypes of idiopathic interstitial pneumonias may be at risk of exacerbation of their underlying disease following thoracic procedures, including video-assisted thoracoscopic lung biopsy.

  6. Ultrasound lung comets: new echographic sign of lung interstitial fibrosis in systemic sclerosis

    Directory of Open Access Journals (Sweden)

    C. Giacomelli

    2011-09-01

    Full Text Available Objective: Interstitial lung disease (ILD and pulmonary arterial hypertension (PAH are common complications of systemic sclerosis (SSc. Echocardiography evaluates PAH, and chest sonography detects even mild ILC as ultrasound lung comets (ULC, i.e. multiple comet-tails fanning out from the lung surface and originating from subpleural interlobular septa thickened by fibrosis. Aim: to assess ILaD and PAH by integrated cardiac and chest ultrasound in SSc. Methods: We enrolled 30 consecutive SSc patients (age= 54±13 years, 23 females in the Rheumatology Clinic of Pisa University. In all, we assessed systolic pulmonary arterial pressure (SPAP, from maximal velocity of tricuspid regurgitation flow, and ULC score with chest sonography (summing the number of ULC from each scanning space of anterior and posterior right and left chest, from second to fifth intercostal space. All patients underwent plasma assay for anti-topoisomerase antibodies (anti-Scl70, and antiicentromere associated with development of pulmonary involvement. Twenty-eight patients also underwent high resolution computed tomography, HRCT (from 0= no fibrosis to 3= honey combing. Results: ULC number - but not SPAP - was correlated to HRCT fibrosis and presence Scl-70 antibodies. ULC number was similar in localized or diffuse forms (16±20 vs 21±19, p=ns and was unrelated to SPAP (r=0.216, p=ns. Conclusions: Chest sonography assessment and ULC allow a complete, simple, radiation-free characterization of interstitial lung involvement in SSc - all in one setting and with the same instrument, same transducer and the same sonographer. In particular, ULC number is associated with HRCT evidence of lung fibrosis and presence of Scl-70 antibodies.

  7. Natural history of five children with surfactant protein C mutations and interstitial lung disease.

    Science.gov (United States)

    Avital, Avraham; Hevroni, Avigdor; Godfrey, Simon; Cohen, Shlomo; Maayan, Channa; Nusair, Samir; Nogee, Lawrence M; Springer, Chaim

    2014-11-01

    Interstitial lung diseases in infants and children are uncommon and may be caused by specific inborn errors of surfactant metabolism. Five children with open lung biopsy diagnosed interstitial lung disease were followed (mean of 27.2 years) and evaluated for surfactant protein gene mutations. Four of the children were originally diagnosed as desquamative interstitial pneumonitis and one as chronic interstitial pneumonitis. All had good response to chloroquine or hydroxychloroquine treatment for periods of 7-38 months. Lung function tests, incremental exercise tests, and rentgenological studies were performed in the children. Surfactant protein gene mutations were searched in all the patients and in part of their families. Three of the patients, aged now 32, 29, and 37 years, feel well and have normal lung function, while two of the patients, both females, aged 28 and 37 years, conduct normal activities of daily living, have healthy children but have clinical, physiological and rentgenological evidence of restrictive lung disease. All five patients were found to have surfactant protein C gene (SFTPC) mutations, three of them with the most common mutation (p.I73T) and the other two with new mutations of surfactant protein C gene (p.I38F and p.V39L). We conclude that detection of surfactant protein mutations should be attempted in all children presenting with interstitial lung disease. Furthermore, treatment with hydroxychloroquine should be considered in children with SFTPC mutations. Prospective evaluation of hydroxychloroquine therapy in a greater number of patients is needed.

  8. [Epidemiological, clinical and evolutionary peculiarities of interstitial lung disease in systemic sclerosis].

    Science.gov (United States)

    Aydi, Z; Rachdi, I; Ben Dhaou, B; Dridi, M; Daoud, F; Baili, L; Boussema, F

    2016-04-01

    Pulmonary involvement during systemic sclerosis (SS) is dominated by interstitial lung disease and arterial pulmonary hypertension. It is about a retrospective study analyzing 65 cases of SS over a period of 13 years. We compared cases with and without interstitial lung disease. The diagnosis of SS was retained according to American College of Rheumatology (ACR)/EULAR 2013 criteria. The diagnosis of interstitial lung disease was retained in TDM and EFR. Pulmonary hypertension is defined by a pulmonary arterial pression higher than 25 mmHg. The mean delay of diagnosis of interstitial lung disease and the diagnosis was of 48 months (extremes 0-78 months). The comparison between both groups according to average age of the patients, prevalence of pulmonary hypertension, frequency of Raynaud phenomenon and trophic disorders did not find any significant difference. Lung involvement was associated with an esophageal involvement in 71% of the cases (P=0.059). Antibodies anti-Scl 70 were noted more frequently in patient's with interstitial lung disease (79% of the cases, P=0.001). Patients were treated with colchicine and vitamin E. A corticotherapy had been indicated at a single patient. The evolution of SS was marked by the stabilisation of the restrictive syndrome in 71.8% of the cases and a worsening in 25% of the cases. Early and appropriate diagnosis of SS and screening of lung involvement are essential for a early care.

  9. Diagnosis of Interstitial Lung Diseases An Ideal Choice: Video-Assisted Thoracoscopic

    Directory of Open Access Journals (Sweden)

    Fuat Sayır

    2012-01-01

    Full Text Available Aim: Interstitial lung diseases are a heterogeneous group of diseases with a known or unknown etiology affecting the interstitium of the lung. In this study, our experience in the lung biopsy performed by video-assisted thoracoscopic surgery and open lung biopsy for interstitial lung diseases is discussed. Material and Method: In this study, we reviewed 31 patients with a clinical diagnosis of interstitial lung disease who underwent open or thoracoscopic lung biopsy between the years of 2004 and 2010. The cases were examined retrospectively for the age, sex, radiological appearance, operative time, chest tube duration, postoperative hospital stay and the complications. Thoracotomy was performed to 19 of the patients (61.30% while twelve patients (38.70% underwent video-assisted thoracoscopic surgery. Result: Fourteen of the cases (45.16% were male while 17 patients were female (58.06% with a mean age of 40.83 15.537 (18-69. Nonspecific interstitial fibrosis constituted the most of the certain diagnoses (29.27%. Operative time, chest tube duration and postoperative hospital stay were significantly shorter in video-assisted thoracoscopic surgery group (p values were 0.018, 0.001 and 0.011 respectively. The overall morbidity rate was 12.90% and there was no mortality. Discussion: In spite of recent advances in clinical diagnostic techniques, lung biopsy is the gold standard for the diagnosis of interstitial lung disease. In our opinion, video-assisted thoracoscopic surgery should be the first choice in the diagnosis of interstitial lung diseases, since these patients can be discharged early as a result of shorter chest tube duration and hospital length of stay

  10. A novel model of rheumatoid arthritis-associated interstitial lung disease in SKG mice.

    Science.gov (United States)

    Keith, Rebecca C; Powers, Jennifer L; Redente, Elizabeth F; Sergew, Amen; Martin, Richard J; Gizinski, Alison; Holers, V Michael; Sakaguchi, Shimon; Riches, David W H

    2012-03-01

    Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is associated with increased mortality in up to 10% of patients with rheumatoid arthritis. Lung exposure to cigarette smoke has been implicated in disease development. Little is known about the mechanisms underlying the development of RA-ILD, in part due to the lack of an appropriate mouse model. The objectives of this study were (i) to test the suitability of SKG mice as a model of cellular and fibrotic interstitial pneumonia in the setting of autoimmune arthritis, and (ii) to determine the role of lung injury in the development of arthritis in SKG mice. Lung tissues were evaluated in arthritic SKG mice by quantifying cell accumulation in bronchoalveolar lavage, static compliance, collagen levels, and infiltrating cell phenotypes by flow cytometry and histology. Lung injury was induced by exposure to cigarette smoke or bleomycin. Arthritic SKG mice developed a patchy cellular and fibrotic interstitial pneumonia associated with reduced static compliance, increased collagen levels, and accumulation of inflammatory cells. Infiltrating cells comprised CD4+ T cells, B cells, macrophages, and neutrophils. Chronic exposure to cigarette smoke or initiation of lung injury with bleomycin did not cause arthritis. The pattern of lung disease suggests that arthritic SKG mice represent an authentic model of nonspecific interstitial pneumonia in RA-ILD patients. The lack of arthritis development after cigarette smoke or lung injury suggests that a model where breaches in immunologic tolerance are induced by lung inflammation and injury alone may be overly simplistic.

  11. Successful EGFR-TKI rechallenge of leptomeningeal carcinomatosis after gefitinib-induced interstitial lung disease.

    Science.gov (United States)

    Nakamichi, Shinji; Kubota, Kaoru; Horinouchi, Hidehito; Kanda, Shintaro; Fujiwara, Yutaka; Nokihara, Hiroshi; Yamamoto, Noboru; Tamura, Tomohide

    2013-04-01

    We report the case of a 49-year-old non-smoking Japanese woman with backache and difficulty in walking. She was diagnosed as having advanced lung adenocarcinoma, and an epithelial growth factor receptor mutation (in-frame deletions in exon 19) was found. After radiation therapy of bone metastases with spinal cord compression and brain metastases, gefitinib was administered. On day 2, she developed acute interstitial lung disease. Gefitinib therapy was discontinued and treatment with high-dose steroid therapy improved the interstitial lung disease. Cisplatin plus pemetrexed was initiated as second-line chemotherapy, but she was hospitalized again for leptomeningeal carcinomatosis. Considering the poor prognosis of leptomeningeal carcinomatosis, we decided that erlotinib was our only choice of treatment. As a third-line treatment, erlotinib was administered after informing the patient about the high risk of interstitial lung disease. Neurological symptoms were improved within a week and interstitial lung disease did not recur. The patient has received erlotinib successfully for 18 months without the recurrence of leptomeningeal carcinomatosis. Erlotinib rechallenge after gefitinib-induced interstitial lung disease must be carefully chosen based on the balance of a patient's risk and benefit.

  12. Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function.

    Science.gov (United States)

    Kinder, Brent W; Shariat, Cyrus; Collard, Harold R; Koth, Laura L; Wolters, Paul J; Golden, Jeffrey A; Panos, Ralph J; King, Talmadge E

    2010-04-01

    Undifferentiated connective tissue disease (UCTD) is a distinct clinical entity that may be accompanied by interstitial lung disease (ILD). The natural history of UCTD-ILD is unknown. We hypothesized that patients with UCTD-ILD would be more likely to have improvement in lung function than those with idiopathic pulmonary fibrosis (IPF) during longitudinal follow-up. We identified subjects enrolled in the UCSF ILD cohort study with a diagnosis of IPF or UCTD. The primary outcome compared the presence or absence of a > or = 5% increase in percent predicted forced vital capacity (FVC) in IPF and UCTD. Regression models were used to account for potential confounding variables. Ninety subjects were identified; 59 subjects (30 IPF, 29 UCTD) had longitudinal pulmonary function data for inclusion in the analysis. After accounting for baseline pulmonary function tests, treatment, and duration between studies, UCTD was associated with substantial improvement in FVC (odds ratio = 8.23, 95% confidence interval, 1.27-53.2; p = 0.03) during follow-up (median, 8 months) compared with IPF. Patients with UCTD-ILD are more likely to have improved pulmonary function during follow-up than those with IPF. These findings demonstrate the clinical importance of identifying UCTD in patients presenting with an "idiopathic" interstitial pneumonia.

  13. A descriptive epidemiological study of interstitial lung disease in the United Kingdom general population

    OpenAIRE

    Amar, Rajeev K.

    2012-01-01

    Interstitial lung disease (ILD) is a heterogeneous group of parenchymal lung disorders having varied histopathologies. Although histologically different, the ILDs have rather similar clinical presentations consisting of increasing dyspnea, a restrictive lung function, impaired gas exchange, and widespread shadowing on chest radiography. Approximately two-thirds of ILD cases have no known etiology. The remaining one-third is either associated with connective tissue disease (CTD) or caused by v...

  14. Reversible Lansoprazole-Induced Interstitial Lung Disease Showing Improvement after Drug Cessation

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Kyu Won; Woo, Ok Hee; Yong, Hwan Seok; Shin, Bong Kyung; Shim, Jae Jeong; Kang, Eun Young [College of Medicine, Korea University, Guro Hospital, Seoul (Korea, Republic of)

    2008-04-15

    Lansoprazole is an acid proton-pump inhibitor that is similar to omeprazole. It is used to treat duodenal or gastric ulcers, H. pylori infection, gastroesophageal reflux disease (GERD) or Zollinger-Ellison syndrome. Common adverse effects of lansoprazole are diarrhea, abdominal pain, skin rash and/or itching. Information from U.S. National Library of Medicine warns that this drug can on rare occasion cause cough or cold-like symptoms. The pathophysiological mechanisms of lansoprazole-related pulmonary symptoms are not yet understood. In particular, there are no known reports regarding lansoprazole-induced interstitial lung diseases. We report here a case of interstitial lung disease (ILD) induced by oral administration of lansoprazole, which showed a pattern of nonspecific interstitial pneumonia (NSIP) as detected from a video-assisted thoracoscopic lung biopsy. We believe that this is the first report of a case of pathologically proven lansoprazole-induced ILD for which a surgical lung biopsy was performed. To the best of our knowledge, this is the first description of DI-ILD caused by lansoprazole. The diagnosis was made by considering the radiological, histopathological and clinical findings, including the close temporal relationship between lansoprazole exposure and symptom severity. Other possible causes were excluded due to a lack of a temporal relationship between the symptoms and work history or prednisolone therapy, and no other history of specific allergen exposure. When there is diffuse interstitial lung disease with an unknown etiology, it is important to remember that drugs can be the cause of pulmonary symptoms and it is crucial to take a careful patient history. If there is a recent history of taking lansoprazole in a patient with clinical and radiological findings of diffuse interstitial lung disease, we recommend stopping the medication to see if there is clinical and radiological improvement. That way, one can avoid using invasive procedures to

  15. Intravascular Large B-Cell Lymphoma Presenting as Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Elham Vali Khojeini

    2014-01-01

    Full Text Available Intravascular large B-cell lymphoma (IVLBL is a rare subtype of diffuse large B-cell lymphoma that resides in the lumen of blood vessels. Patients typically present with nonspecific findings, particularly bizarre neurologic symptoms, fever, and skin lesions. A woman presented with shortness of breath and a chest CT scan showed diffuse interstitial thickening and ground glass opacities suggestive of an interstitial lung disease. On physical exam she was noted to have splenomegaly. The patient died and at autopsy was found to have an IVLBL in her lungs as well as nearly all her organs that were sampled. Although rare, IVLBL should be included in the differential diagnosis of interstitial lung disease and this case underscores the importance of the continuation of autopsies.

  16. Ulcerative colitis and steroid-responsive, diffuse interstitial lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Balestra, D.J.; Balestra, S.T.; Wasson, J.H.

    1988-07-01

    The authors describe a patient with ulcerative colitis and extracolonic manifestations in whom diffuse interstitial pulmonary disease developed that was responsive to glucocorticoid therapy one year after total proctocolectomy. The patient presented in December 1983 with a subacute course marked by cough and progressive exertional dyspnea, abnormal chest examination results, and a chest roentgenogram that revealed diffuse interstitital and alveolar infiltrates. A transbronchial biopsy specimen revealed a polymorphic interstitial infiltrate, mild interstitial fibrosis without apparent intraluminal fibrosis, and no vasculitis, granulomas, or significant eosinophilic infiltration. Within one week of the initiation of daily high-dose steroid therapy, the patient's symptoms dramatically improved; chest roentgenogram and forced vital capacity (60%) improved at a slower rate. All three measures deteriorated when alternate-day prednisone therapy was started but once again improved until the patient was totally asymptomatic, chest roentgenograms were normal, and forced vital capacity was 80% of the predicted value 2 1/2 years later.

  17. Interstitial lung disease in connective tissue disease--mechanisms and management.

    Science.gov (United States)

    Wells, Athol U; Denton, Christopher P

    2014-12-01

    Pulmonary complications are an important extra-articular feature of autoimmune rheumatic diseases and a major cause of mortality. The underlying pathogenesis probably involves multiple cellular compartments, including the epithelium, lung fibroblasts, and the innate and adaptive immune system. Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in underlying pathogenic mechanisms. Growing understanding of the key pathogenic drivers of lung fibrosis might lead to the development of more effective targeted therapies to replicate the treatment advances in other aspects of these diseases. Interstitial lung disease (ILD) in connective tissue disease (CTD) is characterized using the classification of the idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD is most often usual interstitial pneumonia. The key goals of clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease.

  18. Preclinical studies on the use of interstitial laser photocoagulation in the lung parenchyma

    Science.gov (United States)

    Fielding, D. I.; Buonaccorsi, Giovanni A.; Hanby, A.; Hetzel, M. R.; Bown, Stephen G.

    1996-12-01

    A preliminary assessment was made of the effect of interstitial laser photocoagulation on normal lung parenchyma. Using rats lesions were created by passing the laser fiber percutaneously into the normal lung under general anaesthetic. The lungs were removed post mortem at 3 days. The lesions were ellipsoid in shape and well circumscribed. Histology showed central charring surrounded by zones of coagulative and hemorrhagic necrosis. there was a clear margin between the treated and normal tissue. These results indicate that further examination is warranted of the use of ILP for treatment of small primary lung tumors in patients unsuitable for surgery.

  19. HRCT patterns of the most important interstitial lung diseases; HRCT-Muster der wichtigsten interstitiellen Lungenerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Schaefer-Prokop, C. [Meander Medisch Centrum, Abt. Radiologie, Amersfoort (Netherlands); Radboud Universitaet, Abt. Radiologie und Nuklearmedizin, Nijmegen (Netherlands)

    2014-12-15

    Interstitial lung diseases are a mixed group of diffuse parenchymal lung diseases which can have an acute or chronic course. Idiopathic diseases and diseases with an underlying cause (e.g. collagen vascular diseases) share the same patterns. Thin section computed tomography (CT) plays a central role in the diagnostic work-up. The article describes the most important interstitial lung diseases following a four pattern approach with a predominant nodular or reticular pattern or a pattern with increased or decreased lung density. (orig.) [German] Interstitielle Lungenerkrankungen stellen eine gemischte Gruppe diffuser Lungenparenchymerkrankungen dar, die einen akuten oder chronischen Verlauf haben koennen. Idiopathische Erkrankungen und Erkrankungen mit definierter Ursache (z. B. kollagenvaskulaere Erkrankungen) weisen ein gemeinsames Muster auf. Die Duennschichtcomputertomographie spielt eine zentrale Rolle in der diagnostischen Abklaerung. In dem vorliegenden Beitrag werden die wichtigsten interstitiellen Lungenerkrankungen beschrieben. Dabei gibt es 4 Grundmuster: ueberwiegend nodulaere Verdichtungen, vorwiegend retikulaere Verdichtungen, erhoehte oder erniedrigte Lungenparenchymdichte. (orig.)

  20. [Severe hypoxemic respiratory insufficiency in a patient with hepato-pulmonary syndrome coexisting with interstitial lung disease of unknown etiology].

    Science.gov (United States)

    Gorzkowska-Pasik, Katarzyna; Wiatr, Elżbieta; Burakowska, Barbara; Nowicka, Urszula; Kober, Jarosław; Malong, Przemysław; Pasik, Piotr; Folcik, Krystyna

    2013-01-01

    The coexistence of the interstitial lung disease and respiratory failure is rarely associated with extrapulmonary pathology. In patients with liver cirrhosis, hypoxemia may develop in the course of hepato-pulmonary syndrome (HPS), but radiological pathology seen in the course of HPS is of vascular origin, and thus typically not classified as interstitial lung disease. We present a patient with severe hypoxemic respiratory insufficiency in whom hepato-pulmonary syndrome coexisted with interstitial lung disease of unknown etiology. The mechanisms of hypoxemia in the course of hepatic diseases and reasons of possible coincidence of lung and hepatic pathology are discussed.

  1. Automated estimation of progression of interstitial lung disease in CT images.

    NARCIS (Netherlands)

    Arzhaeva, Y.; Prokop, M.; Murphy, K.; Rikxoort, E.M. van; Jong, P.A. de; Gietema, H.A.; Viergever, M.A.; Ginneken, B. van

    2010-01-01

    PURPOSE: A system is presented for automated estimation of progression of interstitial lung disease in serial thoracic CT scans. METHODS: The system compares corresponding 2D axial sections from baseline and follow-up scans and concludes whether this pair of sections represents regression, progressi

  2. Texture feature ranking with relevance learning to classify interstitial lung disease patterns

    NARCIS (Netherlands)

    Huber, Markus B.; Bunte, Kerstin; Nagarajan, Mahesh B.; Biehl, Michael; Ray, Lawrence A.; Wismueller, Axel

    2012-01-01

    Objective: The generalized matrix learning vector quantization (GMLVQ) is used to estimate the relevance of texture features in their ability to classify interstitial lung disease patterns in high-resolution computed tomography images. Methodology: After a stochastic gradient descent, the GMLVQ algo

  3. Ganciclovir-resistant, cytomegalic interstitial lung disease in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Finger, Eduardo; Romaldini, Helio; Lewi, David Salomão; Scheinberg, Morton Aaron

    2007-10-01

    A patient with systemic lupus erythematosus developed interstitial lung disease initially felt to be a manifestation of the disease but that, on further workup, proved to be a manifestation of cytomegalic disease resistant to ganciclovir. Treatment with foscarnet was associated with prompt improvement.

  4. The First Successful Heart-Lung Transplant in a Korean Child with Humidifier Disinfectant-Associated Interstitial Lung Disease.

    Science.gov (United States)

    Jhang, Won Kyoung; Park, Seong Jong; Lee, Eun; Yang, Song I; Hong, Soo Jong; Seo, Ju-Hee; Kim, Hyung-Young; Park, Jeong-Jun; Yun, Tae-Jin; Kim, Hyeong Ryul; Kim, Yong-Hee; Kim, Dong Kwan; Park, Seung-Il; Lee, Sang-Oh; Hong, Sang-Bum; Shim, Tae-Sun; Choi, In-Cheol; Yu, Jinho

    2016-05-01

    From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure. She could survive by 100 days of extracorporeal membrane oxygenation support and finally, underwent heart-lung transplantation. This is the first successful pediatric heart-lung transplantation carried out in Korea.

  5. Pulmonary hypertension in chronic obstructive and interstitial lung diseases

    DEFF Research Database (Denmark)

    Andersen, Charlotte U; Mellemkjær, Søren; Nielsen-Kudsk, Jens Erik

    2013-01-01

    , and is considered one of the most frequent types of PH. However, the prevalence of PH among patients with COPD and ILD is not clear. The diagnosis of PH in chronic lung disease is often established by echocardiographic screening, but definitive diagnosis requires right heart catheterization, which...... is not systematically performed in clinical practice. Given the large number of patients with chronic lung disease, biomarkers to preclude or increase suspicion of PH are needed. NT-proBNP may be used as a rule-out test, but biomarkers with a high specificity for PH are still required. It is not known whether specific...... treatment with existent drugs effective in pulmonary arterial hypertension (PAH) is beneficial in lung disease related PH. Studies investigating existing PAH drugs in animal models of lung disease related PH have indicated a positive effect, and so have case reports and open label studies. However...

  6. Narrow bandwidth spectral analysis of the textures of interstitial lung diseases

    Science.gov (United States)

    Krasner, Brian; Lo, Shih-Chung B.; Mun, Seong K.

    1994-05-01

    The object of this study was to develop a classifier for distinguishing between regions-of-interest (ROIs) from normal lung radiographs and ROIs from radiographs showing interstitial lung disease. The method used was to estimate the covariance statistics of the ROIs of the lung interstitial space and, based on the estimate, to design filters for isolating statistically significant components of the spectrum. The energy of filtered images was used as a classifier. Additionally, the filtered images were analyzed and classified using a convolution neural network (CNN). The procedure used to generate the filters was: (1) Convert 2D neighborhoods of pixels to vectors. (2) Form the sample covariance matrix from the vectors. (3) Compute the eigenvectors and eigenvalues of the matrix. (4) Convert the eigenvectors back to 2D form and use as filters. The images selected for study included normal lungs, and lungs with different types and profusions of pneumoconiosis opacities. One group of ROIs of the interstitial space was used to design filters. Another group was used as a test of classification accuracy. The results showed that the designed classifier was effective in discriminating ROIs with small pneumoconiosis opacities from normal ROIs.

  7. T2 mapping of CT remodelling patterns in interstitial lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Buzan, Maria T.A. [Iuliu Hatieganu University of Medicine and Pharmacy, Department of Pneumology, Cluj-Napoca (Romania); Thoraxklinik at Heidelberg University Hospital, Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Heidelberg (Germany); University Hospital Heidelberg, Department of Diagnostic and Interventional Radiology, Heidelberg (Germany); Eichinger, Monika; Heussel, Claus Peter [Thoraxklinik at Heidelberg University Hospital, Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Heidelberg (Germany); Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg (Germany); Kreuter, Michael; Herth, Felix J. [Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg (Germany); Thoraxklinik at Heidelberg University Hospital, Department of Pneumology, Center for Rare and Interstitial Lung Diseases, Heidelberg (Germany); Kauczor, Hans-Ulrich [University Hospital Heidelberg, Department of Diagnostic and Interventional Radiology, Heidelberg (Germany); Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg (Germany); Warth, Arne [Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg (Germany); University Hospital Heidelberg, Institute for Pathology, Heidelberg (Germany); Pop, Carmen Monica [Iuliu Hatieganu University of Medicine and Pharmacy, Department of Pneumology, Cluj-Napoca (Romania); Dinkel, Julien [Thoraxklinik at Heidelberg University Hospital, Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Heidelberg (Germany); Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg (Germany); Comprehensive Pneumology Center Munich (CPC-M), German Center for Lung Research (DZL), Munich (Germany); Ludwig-Maximilians-University Hospital Munich, Institute for Clinical Radiology, Munich (Germany)

    2015-11-15

    To evaluate lung T2 mapping for quantitative characterization and differentiation of ground-glass opacity (GGO), reticulation (RE) and honeycombing (HC) in usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP). Twelve patients with stable UIP or NSIP underwent thin-section multislice CT and 1.5-T MRI of the lung. A total of 188 regions were classified at CT into normal (n = 29) and pathological areas, including GGO (n = 48), RE (n = 60) and HC (n = 51) predominant lesions. Entire lung T2 maps based on multi-echo single shot TSE sequence (TE: 20, 40, 79, 140, 179 ms) were generated from each subject with breath-holds at end-expiration and ECG-triggering. The median T2 relaxation of GGO was 67 ms (range 60-72 ms). RE predominant lesions had a median relaxation of 74 ms (range 69-79 ms), while for HC pattern this was 79 ms (range 74-89 ms). The median T2 relaxation for normal lung areas was 41 ms (ranged 38-49 ms), and showed significant difference to pathological areas (p < 0.001). A statistical difference was found between the T2 relaxation of GGO, RE and HC (p < 0.05). The proposed method provides quantitative information for pattern differentiation, potentially allowing for monitoring of progression and response to treatment, in interstitial lung disease. (orig.)

  8. The clinical utility of bronchoalveolar lavage in interstitial lung disease - is it really useful?

    Science.gov (United States)

    Meyer, Keith C

    2014-04-01

    Bronchoalveolar lavage (BAL) can be a very useful tool in the diagnosis of interstitial lung disease, but BAL must be performed properly and the retrieved BAL fluid adequately processed and analyzed to allow accurate conclusions to be drawn from BAL analysis. A differential cell count of nucleated immune cells can show cell patterns that suggest or support certain diagnoses, and other testing (stains and cultures for infectious pathogens, malignant cell cytology) can be performed on BAL fluid that can also aid in diagnosis. When combined with the results of a careful history, physical examination, thoracic imaging, and other pertinent laboratory testing, the BAL analysis may allow a confident diagnosis of a specific interstitial lung disease to be made without proceeding to more invasive testing (e.g., surgical lung biopsy) that is associated with increased risk of complications.

  9. Increased prevalence of high anti-Cladosporium antibody titers in interstitial lung diseases.

    Science.gov (United States)

    Watanuki, Zenta; Okada, Shinji; Chiba, Shigeki; Kamei, Katsuhiko; Suzuki, Yasuko; Yamada, Norihiro

    2012-01-01

    Interstitial lung diseases (ILDs) represent a large group of different diseases, with a large part comprising idiopathic interstitial pneumonias. Differentiating hypersensitivity pneumonitis (HP), especially its chronic form and other ILDs, is difficult because of similarities in radiological manifestation and clinical course, and the difficulty of identifying causative antigens. We recently experienced a patient with Cladosporium-induced chronic HP that developed in a household environment, but the cause had been misdiagnosed as idiopathic interstitial pneumonia for several years. This case highlighted the need for measures differentiating HP from idiopathic interstitial pneumonia. In this study, we examined fungal exposure in ILDs using an antibody titer in serum to identify possible fungus-related HP. We measured the antibody titer to Cladosporium spp. in 34 patients with various ILDs, 17 patients with bronchial asthma, and 21 control subjects using an immunofluorescence assay. ILDs included HP (5 patients), idiopathic interstitial pneumonias (21 patients), and ILDs with collagen vascular diseases (8 patients). Results showed a significantly higher tendency for high anti-Cladosporium antibody titers in ILD groups (12 patients out of 34 patients), compared to patients with bronchial asthma (0/17) or control subjects (0/21). This increase in antibody titers was observed not only in patients with HP, but also in those with idiopathic interstitial pneumonias and those exhibiting collagen vascular diseases with ILDs. This report highlights the pathogenic role of fungal antigens in various ILDs. In conclusion, fungi commonly observed in our living environment such as Cladosporium could be involved in the development of ILDs.

  10. Paraseptal emphysema: Prevalence and distribution on CT and association with interstitial lung abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Araki, Tetsuro, E-mail: taraki@partners.org [Department of Radiology, Center for Pulmonary Functional Imaging, Brigham and Women' s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02215 (United States); Department of Radiology, Kinki University Faculty of Medicine, Osaka-Sayama (Japan); Nishino, Mizuki [Department of Radiology, Center for Pulmonary Functional Imaging, Brigham and Women' s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02215 (United States); Zazueta, Oscar E. [The Pulmonary and Critical Care Division, Brigham and Women' s Hospital, Harvard Medical School, Boston, MA (United States); Gao, Wei [Department of Biostatistics, Boston University School of Public Health, Boston, MA (United States); Dupuis, Josée [Department of Biostatistics, Boston University School of Public Health, Boston, MA (United States); The National Heart Lung and Blood Institute' s Framingham Heart Study, Framingham, MA (United States); Okajima, Yuka [Department of Radiology, Center for Pulmonary Functional Imaging, Brigham and Women' s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02215 (United States); Latourelle, Jeanne C. [Department of Medicine and Neurology, Boston University School of Medicine, Boston, MA (United States); Rosas, Ivan O. [The Pulmonary and Critical Care Division, Brigham and Women' s Hospital, Harvard Medical School, Boston, MA (United States); Murakami, Takamichi [Department of Radiology, Kinki University Faculty of Medicine, Osaka-Sayama (Japan); O’Connor, George T. [The National Heart Lung and Blood Institute' s Framingham Heart Study, Framingham, MA (United States); Pulmonary Center and Department of Medicine, Boston University School of Medicine, Boston, MA (United States); Washko, George R.; Hunninghake, Gary M. [The Pulmonary and Critical Care Division, Brigham and Women' s Hospital, Harvard Medical School, Boston, MA (United States); and others

    2015-07-15

    Highlights: • The prevalence of pure paraseptal emphysema was 3% (85/2633) in the Framingham Heart Study population, predominantly affects the upper lung zone, and contributes to slightly decreased pulmonary function. • There was significant association between paraseptal emphysema and interstitial lung abnormalities, which is a novel finding. • Prevalence of paraseptal emphysema and its impact on pulmonary function could have been underestimated in the previous reports. - Abstract: Objective: To investigate the prevalence and distribution of paraseptal emphysema on chest CT images in the Framingham Heart Study (FHS) population, and assess its impact on pulmonary function. Also pursued was the association with interstitial lung abnormalities. Materials and methods: We assessed 2633 participants in the FHS for paraseptal emphysema on chest CT. Characteristics of the participants, including age, sex, smoking status, clinical symptoms, and results of pulmonary function tests, were compared between those with and without paraseptal emphysema. The association between paraseptal emphysema and interstitial lung abnormalities was investigated. Results: Of the 2633 participants, 86 (3%) had pure paraseptal emphysema (defined as paraseptal emphysema with no other subtypes of emphysema other than paraseptal emphysema or a very few centrilobular emphysema involved) in at least one lung zone. The upper zone of the lungs was almost always involved. Compared to the participants without paraseptal emphysema, those with pure paraseptal emphysema were significantly older, and were more frequently male and smokers (mean 64 years, 71% male, mean 36 pack-years, P < 0.001) and had significantly decreased FEV{sub 1}/FVC% (P = 0.002), and diffusion capacity of carbon monoxide (DLCO) (P = 0.002). There was a significant association between pure paraseptal emphysema and interstitial lung abnormalities (P < 0.001). Conclusions: The prevalence of pure paraseptal emphysema was 3% in the

  11. Enhancement of CD147 on M1 macrophages induces differentiation of Th17 cells in the lung interstitial fibrosis.

    Science.gov (United States)

    Geng, Jie-jie; Zhang, Kui; Chen, Li-na; Miao, Jin-lin; Yao, Meng; Ren, Ying; Fu, Zhi-guang; Chen, Zhi-nan; Zhu, Ping

    2014-09-01

    Lung interstitial fibrosis is a chronic lung disease, and few effective therapies are available to halt or reverse the progression of the disease. In murine and human lung fibrosis, the expression of CD147 is increased. However, the role of CD147 in lung fibrosis has not been identified, and it remains to be determined whether lung fibrosis would be improved by decreasing the expression of CD147. A murine bleomycin-induced lung interstitial fibrosis model was used in the experiments, and HAb18 mAbs and CsA were administered during the induction of lung fibrosis. In our study, we found that the HAb18 mAbs markedly reduced the collagen score and down-regulated M1 macrophages and Th17 cells. In vitro, flow cytometry analysis showed that M1 macrophages induced higher Th17 differentiation than M2 macrophages. After treatment with HAb18 mAbs or after reducing the expression of CD147 by lentivirus interference in M1 macrophages, the level of Th17 cells were significantly inhibited. In conclusion, HAb18 mAbs or CsA treatment ameliorates lung interstitial fibrosis. CD147 promoted M1 macrophage and induced the differentiation of Th17 cells in lung interstitial fibrosis, perhaps by regulating some cytokines such as IL-6, IL-1β, IL-12 and IL-23. These results indicated that CD147 may play an important role in the development of lung interstitial fibrosis.

  12. Study of fractal dimension in chest images using normal and interstitial lung disease cases

    Science.gov (United States)

    Tucker, Douglas M.; Correa, Jose L.; Souto, Miguel; Malagari, Katerina S.

    1993-09-01

    A quantitative computerized method which provides accurate discrimination between chest radiographs with positive findings of interstitial disease patterns and normal chest radiographs may increase the efficacy of radiologic screening of the chest and the utility of digital radiographic systems. This report is a comparison of fractal dimension measured in normal chest radiographs and in radiographs with abnormal lungs having reticular, nodular, reticulonodular and linear patterns of interstitial disease. Six regions of interest (ROI's) from each of 33 normal chest radiographs and 33 radiographs with positive findings of interstitial disease were studied. Results indicate that there is a statistically significant difference between the distribution of the fractal dimension in normal radiographs and radiographs where disease is present.

  13. Novel level-set based segmentation method of the lung at HRCT images of diffuse interstitial lung disease (DILD)

    Science.gov (United States)

    Lee, Jeongjin; Seo, Joon Beom; Kim, Namkug; Park, Sang Ok; Lee, Ho; Shin, Yeong Gil; Kim, Soo-Hong

    2009-02-01

    In this paper, we propose an algorithm for reliable segmentation of the lung at HRCT of DILD. Our method consists of four main steps. First, the airway and colon are segmented and excluded by thresholding(-974 HU) and connected component analysis. Second, initial lung is identified by thresholding(-474 HU). Third, shape propagation outward the lung is performed on the initial lung. Actual lung boundaries exist inside the propagated boundaries. Finally, subsequent shape modeling level-set inward the lung from the propagated boundary can identify the lung boundary when the curvature term was highly weighted. To assess the accuracy of the proposed algorithm, the segmentation results of 54 patients are compared with those of manual segmentation done by an expert radiologist. The value of 1 minus volumetric overlap is less than 5% error. Accurate result of our method would be useful in determining the lung parenchyma at HRCT, which is the essential step for the automatic classification and quantification of diffuse interstitial lung disease.

  14. Interstitial Lung Disease in Rheumatoid Arthritis in the Era of Biologics

    Directory of Open Access Journals (Sweden)

    A. Picchianti Diamanti

    2011-01-01

    Full Text Available Interstitial lung disease (ILD represents a severe manifestation in connective tissue diseases (CTD, with an overall incidence of 15%, and it is still a challenge for clinicians evaluation and management. ILD is the most common manifestation of lung involvement in Rheumatoid Arthritis (RA, observed in up to 80% of biopsies, 50% of chest Computed Tomography (CT and only 5% of chest radiographs. Histopatological patterns of ILD in RA may present with different patterns, such as: usual interstitial pneumonia, non specific interstitial pneumonia, desquamative interstitial pneumonia, organizing pneumonia, and eosinophilic infiltration. The incidence of ILD in RA patients is not only related to the disease itself, many drugs may be in fact associated with the development of pulmonary damage. Some reports suggest a causative role for TNFα inhibitors in RA-ILD development/worsening, anyway, no definitive statement can be drawn thus data are incomplete and affected by several variables. A tight control (pulmonary function tests and/or HRCT is mandatory in patients with preexisting ILD, but it should be also performed in those presenting risk factors for ILD and mild respiratory symptoms. Biologic therapy should be interrupted, and, after excluding triggering infections, corticosteroids should be administered.

  15. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease.

    Science.gov (United States)

    Solomon, Joshua J; Chung, Jonathan H; Cosgrove, Gregory P; Demoruelle, M Kristen; Fernandez-Perez, Evans R; Fischer, Aryeh; Frankel, Stephen K; Hobbs, Stephen B; Huie, Tristan J; Ketzer, Jill; Mannina, Amar; Olson, Amy L; Russell, Gloria; Tsuchiya, Yutaka; Yunt, Zulma X; Zelarney, Pearlanne T; Brown, Kevin K; Swigris, Jeffrey J

    2016-02-01

    Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; pdisease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.

  16. Erlotinib Induced Fatal Interstitial Lung Disease in a Patient with Metastatic Non-Small Cell Lung Cancer: Case Report and Review of Literature.

    Science.gov (United States)

    Mangla, Ankit; Agarwal, Nikki; Carmel, Chou; Lad, Thomas

    2016-09-05

    Erlotinib is one of the most widely used tyrosine kinase inhibitor targeting human epidermal growth factor receptor. Since its introduction, it has revolutionized the treatment of advanced non-small cell lung cancer. Skin rashes and diarrhea are the most often reported side effects of erlotinib however it is also associated with interstitial pneumonitis or interstitial lung disease, which often turns out to be fatal complication of using this medicine. Though reported scarcely in the western world, the association of interstitial lung disease with epidermal growth factor receptor has attracted a lot of attention in the recent times. Various researches working with murine models of bleomycin-induced pulmonary fibrosis have found a pro and con role of the receptor in development of the interstitial lung disease. We present the case of a patient diagnosed with stage IV adenocarcinoma of the lung with metastasis to brain. He was found to be positive for the human epidermal growth factor mutation and was hence started on erlotinib. Within a few weeks of starting the medicine the patient was admitted with diarrhea. During the course of this admission he developed acute shortness of breath diagnosed as interstitial pneumonitis. The purpose of this case report is to review the literature associated with erlotinib induced interstitial pneumonitis and make the practicing oncologists aware of this rare yet fatal complication of erlotinib. Here we will also review literature, pertaining to the role of epidermal growth factor receptor in development of interstitial lung disease.

  17. Visual assessment of early emphysema and interstitial abnormalities on CT is useful in lung cancer risk analysis

    DEFF Research Database (Denmark)

    Wille, Mathilde M W; Thomsen, Laura H.; Petersen, Jens;

    2016-01-01

    Objectives: Screening for lung cancer should be limited to a high-risk-population, and abnormalities in low-dose computed tomography (CT) screening images may be relevant for predicting the risk of lung cancer. Our aims were to compare the occurrence of visually detected emphysema and interstitial...... abnormalities in subjects with and without lung cancer in a screening population of smokers. Methods: Low-dose chest CT examinations (baseline and latest possible) of 1990 participants from The Danish Lung Cancer Screening Trial were independently evaluated by two observers who scored emphysema and interstitial.......499). Conclusions: Even early signs of emphysema and interstitial abnormalities are associated with lung cancer. Quantitative measurements of emphysema—regardless of type—do not show the same association. Key Points: • Visually detected emphysema on CT is more frequent in individuals who develop lung cancer...

  18. March 2014 ciritcal care case of the month: interstitial lung disease

    Directory of Open Access Journals (Sweden)

    Yun S

    2014-03-01

    Full Text Available No abstract available. Article truncated after 150 words. History of Present Illness An 80 year-old man with a history of chronic obstructive pulmonary disease, asbestosis and interstitial lung disease, presented to the outpatient clinic with cough, sinus congestion and mild sputum. He was sent home with amoxicillin for the treatment of a sinus infection. However, he came back to emergency department with worsening respiratory symptoms including shortness of breath and persistent cough. He required 8-10 L/min of oxygen to maintain an oxygen saturation above 90 %. PMH: COPD, Asbestosis, Interstitial lung disease, Diabetes mellitus type II, Hypertension, Aortic valve replacement. Medications: Fluticasone-salmeterol 250-50 mcg inhaler, Mometasone 50 mcg/actuation nasal spray, Furosemide 40 mg PO daily, Felodipine 5 mg PO BID, Warfarin 3 mg PO daily, Insulin aspart 5 units SC injection before meals, Insulin glargine 15 units SC injection night time. Social History 50 pack-year ...

  19. Interstitial Lung Disease of the UIP Variant as the Only Presenting Symptom of Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Abhinav Agrawal

    2015-01-01

    Full Text Available Rheumatoid arthritis is a chronic inflammatory disease primarily manifesting with symptoms of joint pain. It also involves multiple organ systems in the body, including the lungs. Interstitial lung disease (ILD is the most common form of pulmonary involvement in rheumatoid arthritis (RA. Without the typical symptoms such as chronic joint pain, establishing the diagnosis of RA could be quite challenging and a high index of suspicion is thereby required to diagnose ILD in patients with RA, thereby delaying treatment and increasing morbidity and mortality. We report a case of a 67-year-old Hispanic male with no previous history of rheumatoid arthritis or symptoms of typical joint pain who comes to the hospital only with the chief complaints of progressive worsening of shortness of breath for a duration of 6 months and was eventually diagnosed with ILD of the usual interstitial pneumonia variant with serologies positive for rheumatoid arthritis.

  20. Calcium Channel Blockers and Esophageal Sclerosis: Should We Expect Exacerbation of Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Charalampos Seretis

    2012-01-01

    Full Text Available Esophageal sclerosis is the most common visceral manifestation of systemic sclerosis, resulting in impaired esophageal clearance and retention of ingested food; in addition, co-existence of lung fibrosis with esophageal scleroderma is not uncommon. Both the progression of generalized connective tissue disorders and the damaging effect of chronic aspiration due to esophageal dysmotility appear to be involved in this procedure of interstitial fibrosis. Nifedipine is a widely prescribed calcium antagonist in a significant percentage of rheumatologic patients suffering from Raynaud syndrome, in order to inhibit peripheral vasospasm. Nevertheless, blocking calcium channels has proven to contribute to exacerbation of gastroesophageal reflux, which consequently can lead to chronic aspiration. We describe the case of severe exacerbation of interstitial lung disease in a 76-year-old female with esophageal sclerosis who was treated with oral nifedipine for Raynaud syndrome.

  1. Optimal management of interstitial lung disease associated with dermatomyositis/polymyositis: lessons from the Japanese experience

    OpenAIRE

    Kurasawa K; Arai S

    2014-01-01

    Kazuhiro Kurasawa,1,2 Satoko Arai2 1Center of Rheumatic Diseases, Dokkyo Medical University, Mibu, Tochigi, Japan; 2Department of Clinical Immunology, Dokkyo Medical University, Mibu, Tochigi, Japan Abstract: Interstitial lung disease (ILD) is a serious complication in dermatomyositis (DM) and polymyositis (PM). In Japan, patients with DM/PM develop acute life-threatening ILD with high frequency. Physicians in Japan have shown the following: refractory acute/subacute (A/S)-ILD is not a rare ...

  2. Non-invasive mechanical ventilation in patients with diffuse interstitial lung diseases

    OpenAIRE

    Aliberti, S.; Messinesi, G.; Gamberini, S; Maggiolini, S.; Visca, D.; Galavotti, V; Giuliani, F.; Cosentini, R; Brambilla, A M; Blasi, F; Scala, R; Carone, M.; Luisi, F; Harari, S.; Voza, A.

    2014-01-01

    Background To evaluate noninvasive ventilation (NIV) in diffuse interstitial lung diseases (DILD) patients with acute respiratory failure (ARF) according to baseline radiological patterns and the etiology of ARF. Methods In a multicenter, observational, retrospective study, consecutive DILD patients undergoing NIV because of an episode of ARF were evaluated in six Italian high dependency units. Three groups of patients were identified based on the etiology of ARF: those with pneumonia (Group ...

  3. Interstitial lung disease in patients with rheumatoid arthritis: spontaneous and drug induced.

    Science.gov (United States)

    Hallowell, Robert W; Horton, Maureen R

    2014-03-01

    Rheumatoid arthritis (RA) is an inflammatory autoimmune disease characterized by the destruction of articular joint structures. RA is a systemic condition that often affects multiple organs, including the heart, lungs, and kidneys. Pulmonary complications of RA are relatively common and include pleural effusion, rheumatoid nodules, bronchiectasis, obliterative bronchiolitis, and opportunistic infections. Interstitial lung disease (ILD) is a common occurrence in patients with RA, and can range in severity from an asymptomatic incidental finding to a rapidly progressing life-threatening event. Usual interstitial pneumonia and non-specific interstitial pneumonia are the two most common patterns, though others have been reported. Various disease-modifying anti-rheumatic drugs-in particular, methotrexate and the tumor necrosis factor-alpha inhibitors-have been associated with RA-ILD in numerous case reports and case series, though it is often difficult to distinguish association from causality. Treatment for RA-ILD typically involves the use of high-dose corticosteroids, often in conjunction with alternative immunosuppressant agents such as azathioprine or mycophenolate mofetil, and outcomes vary widely depending on the initial pattern of lung disease. Additional research into the mechanisms driving RA-ILD is needed to guide future therapy.

  4. Drug induced interstitial lung disease in oncology phase I trials.

    Science.gov (United States)

    Yonemori, Kan; Hirakawa, Akihiro; Kawachi, Asuka; Kinoshita, Fumie; Okuma, Hitomi; Nishikawa, Tadaaki; Tamura, Kenji; Fujiwara, Yasuhiro; Takebe, Naoko

    2016-12-01

    Interstitial lung disease is a serious drug-related condition that can cause life threatening organ failure. The incidence and risk factors of drug-induced interstitial lung disease (DILD) are unknown in oncology phase I trials. This study analyzed clinical information from 8906 patients with malignancies who were enrolled in 470 phase I trials sponsored by the Cancer Therapy Evaluation Program, National Cancer Institute, from 1988 to 2014. Logistic and Cox statistical analyses were utilized to determine clinical differences between patients who developed DILD and patients who did not. In this study, the overall incidence rate of patients with pulmonary toxicity was 2.7%. The overall incidence rate for DILD was 0.77%, whereas for grade 3 or 4 DILD it was 0.31%. Median time to occurrence of DILD was 1.4 months. The Cox hazard analysis indicated smaller body surface area and a combination of thoracic radiation with investigational drug regimens were significant risk factors for time to occurrence of interstitial lung disease. Investigators should carefully monitor for DILD in oncology patients enrolled in phase I trials with identified risk factors. A 6-month observation period would be sufficient to detect the onset of most DILD in such patients.

  5. The A2B adenosine receptor modulates pulmonary hypertension associated with interstitial lung disease.

    Science.gov (United States)

    Karmouty-Quintana, Harry; Zhong, Hongyan; Acero, Luis; Weng, Tingting; Melicoff, Ernestina; West, James D; Hemnes, Anna; Grenz, Almut; Eltzschig, Holger K; Blackwell, Timothy S; Xia, Yang; Johnston, Richard A; Zeng, Dewan; Belardinelli, Luiz; Blackburn, Michael R

    2012-06-01

    Development of pulmonary hypertension is a common and deadly complication of interstitial lung disease. Little is known regarding the cellular and molecular mechanisms that lead to pulmonary hypertension in patients with interstitial lung disease, and effective treatment options are lacking. The purpose of this study was to examine the adenosine 2B receptor (A(2B)R) as a regulator of vascular remodeling and pulmonary hypertension secondary to pulmonary fibrosis. To accomplish this, cellular and molecular changes in vascular remodeling were monitored in mice exposed to bleomycin in conjunction with genetic removal of the A(2B)R or treatment with the A(2B)R antagonist GS-6201. Results demonstrated that GS-6201 treatment or genetic removal of the A(2B)R attenuated vascular remodeling and hypertension in our model. Furthermore, direct A(2B)R activation on vascular cells promoted interleukin-6 and endothelin-1 release. These studies identify a novel mechanism of disease progression to pulmonary hypertension and support the development of A(2B)R antagonists for the treatment of pulmonary hypertension secondary to interstitial lung disease.

  6. Visual assessment of early emphysema and interstitial abnormalities on CT is useful in lung cancer risk analysis

    Energy Technology Data Exchange (ETDEWEB)

    Wille, Mathilde M.W.; Dirksen, Asger; Shaker, Saher B. [Gentofte Hospital, Department of Respiratory Medicine, Hellerup (Denmark); Thomsen, Laura H. [Hvidovre Hospital, Department of Respiratory Medicine, Hvidovre (Denmark); Petersen, Jens [University of Copenhagen, Department of Computer Science, DIKU, Koebenhavn Oe (Denmark); Bruijne, Marleen de [University of Copenhagen, Department of Computer Science, DIKU, Koebenhavn Oe (Denmark); Erasmus MC -University Medical Center Rotterdam, Biomedical Imaging Group Rotterdam, Departments of Radiology and Medical Informatics, Rotterdam (Netherlands); Pedersen, Jesper H. [Copenhagen University Hospital, Department of Thoracic Surgery, Rigshospitalet, Koebenhavn Oe (Denmark)

    2016-02-15

    Screening for lung cancer should be limited to a high-risk-population, and abnormalities in low-dose computed tomography (CT) screening images may be relevant for predicting the risk of lung cancer. Our aims were to compare the occurrence of visually detected emphysema and interstitial abnormalities in subjects with and without lung cancer in a screening population of smokers. Low-dose chest CT examinations (baseline and latest possible) of 1990 participants from The Danish Lung Cancer Screening Trial were independently evaluated by two observers who scored emphysema and interstitial abnormalities. Emphysema (lung density) was also measured quantitatively. Emphysema was seen more frequently and its extent was greater among participants with lung cancer on baseline (odds ratio (OR), 1.8, p = 0.017 and p = 0.002) and late examinations (OR 2.6, p < 0.001 and p < 0.001). No significant difference was found using quantitative measurements. Interstitial abnormalities were more common findings among participants with lung cancer (OR 5.1, p < 0.001 and OR 4.5, p < 0.001).There was no association between presence of emphysema and presence of interstitial abnormalities (OR 0.75, p = 0.499). Even early signs of emphysema and interstitial abnormalities are associated with lung cancer. Quantitative measurements of emphysema - regardless of type - do not show the same association. (orig.)

  7. Giant cell interstitial pneumonia: unusual lung disorder and an update

    Institute of Scientific and Technical Information of China (English)

    Dai Jinghong; Huang Mei; Cao Min; Miao Liyun; Xiao Yonglong; Shi Yi; Meng Fanqing

    2014-01-01

    Background Giant cell interstitial pneumonia (GIP) was a rare form of pneumoconiosis,associated with exposure to hard metals,which had been reported mostly as isolated case reports.We described eight cases of GIP diagnosed in our hospital during the past seven years,with particular reference to new findings.Methods Eight patients with GIP confirmed by biopsy in the Nanjing Drum Tower Hospital affiliated to Medical School of Nanjing University from 2005 to 2011 were retrospectively analyzed.For each patient,the occupy histories and medical records were thoroughly reviewed and clinic data were extracted.Two radiologists,without knowledge of any of the clinical and functional findings,independently reviewed the HRCT scans of all patients.Follow-up data were collected.Results Among the eight patients,seven had a history of exposure to hard metal dusts,one denied an exposure history.The most common manifestations were cough and dyspnea.One patient initiated with pneumothorax and another pleural effusion,both of which were uncommon to GIP.The main pathologic appearances were the presence of macrophages and multinucleated giant cells in the alveolar space.The clinical symptoms and radiographic abnormalities were obviously improved after cessation of exposure and receiving corticosteroid treatments,recurrences were observed in two patients when they resumed work.In spite of exposure cessation and corticosteroid treatment,one patient developed pulmonary fibrosis at seven years follow-up.Conclusions Awareness of the patients' occupational history often provided clues to the diagnosis of GIP.Histopathologic examinations were necessary to establish the right diagnosis.Exposure cessation was of benefit to most patients; however,pulmonary fibrosis was possible in spite of exposure cessation and corticosteroid treatment.Better ways should be found out to improve the outcome and quality of life.

  8. Effect of Stem Cell Therapy on Amiodarone Induced Fibrosing Interstitial Lung Disease in Albino Rat

    Science.gov (United States)

    Zaglool, Somaya Saad; Zickri, Maha Baligh; Abd El Aziz, Dalia Hussein; Mabrouk, Doaa; Metwally, Hala Gabr

    2011-01-01

    Background and Objectives: The fibrosing forms of interstitial lung disease (ILD) are associated with significant morbidity and mortality. ILD may be idiopathic, secondary to occupational, infection, complicate rheumatic diseases or drug induced. Efficacy of antifibrotic agents is as far as, limited and uncertain. No effective treatment was confirmed for pulmonary fibrosis except lung transplantation. The present study aimed at investigating the possible effect of human cord blood mesenchymal stem cell (MSC) therapy on fibrosing ILD. This was accomplished by using amiodarone as a model of induced lung damage in albino rat. Methods and Results: Seventeen adult male albino rats were divided into 3 groups. Rats of amiodarone group were given 30 mg/kg of amiodarone orally 6 days/ week for 6 weeks. Rats of stem cell therapy group were injected with stem cells in the tail vein following confirmation of lung damage and left for 4 weeks before sacrifice. Obstructed bronchioles, thickened interalveolar septa and thickened wall of pulmonary vessels were found and proved morphometrically. Reduced type I pneumocytes and increased area% of collagen fibers were recorded. All findings regressed on stem cell therapy. Conclusions: Cord blood MSC therapy proved definite amelioration of fibrosing interstitial lung disease provided therapy starts early in the development of the pathogenesis. PMID:24298346

  9. Dramatic improvement of anti-SS-A/Ro-associated interstitial lung disease after immunosuppressive treatment.

    Science.gov (United States)

    Paola, Caramaschi; Giuliana, Festi; Giovanni, Orsolini; Cristian, Caimmi; Domenico, Biasi

    2016-07-01

    The aim of the study was to report three patients affected by interstitial lung disease associated with positive anti-SS-A/Ro autoantibody who showed a dramatic improvement after immunosuppressive treatment. Medical charts were reviewed to obtain clinical data, laboratory parameters, lung function tests, high-resolution computed tomography results and response to immunosuppressive treatment. The three patients showed a clinical picture of a lung-dominant connective tissue disease characterized by a sudden onset with dyspnea, cough and subtle extrathoracic features together with positive anti-SS-A/Ro antibody and weak titer antinuclear antibodies. All three patients responded favorably to immunosuppressive therapy: Two cases were treated with a combination of corticosteroid and cyclophosphamide followed by mycophenolate mofetil; in the third patient, clinical benefit was obtained after rituximab was added to corticosteroid and immunosuppressant drug. In spite of an abrupt onset with significant lung function impairment, all three patients had a favorable clinical response to immunosuppressive therapy. This report may be useful in making therapeutic decisions in case of interstitial lung disease associated with anti-SS-A antibody.

  10. Image-based diagnostic aid for interstitial lung disease with secondary data integration

    Science.gov (United States)

    Depeursinge, Adrien; Müller, Henning; Hidki, Asmâa; Poletti, Pierre-Alexandre; Platon, Alexandra; Geissbuhler, Antoine

    2007-03-01

    Interstitial lung diseases (ILDs) are a relatively heterogeneous group of around 150 illnesses with often very unspecific symptoms. The most complete imaging method for the characterisation of ILDs is the high-resolution computed tomography (HRCT) of the chest but a correct interpretation of these images is difficult even for specialists as many diseases are rare and thus little experience exists. Moreover, interpreting HRCT images requires knowledge of the context defined by clinical data of the studied case. A computerised diagnostic aid tool based on HRCT images with associated medical data to retrieve similar cases of ILDs from a dedicated database can bring quick and precious information for example for emergency radiologists. The experience from a pilot project highlighted the need for detailed database containing high-quality annotations in addition to clinical data. The state of the art is studied to identify requirements for image-based diagnostic aid for interstitial lung disease with secondary data integration. The data acquisition steps are detailed. The selection of the most relevant clinical parameters is done in collaboration with lung specialists from current literature, along with knowledge bases of computer-based diagnostic decision support systems. In order to perform high-quality annotations of the interstitial lung tissue in the HRCT images an annotation software and its own file format is implemented for DICOM images. A multimedia database is implemented to store ILD cases with clinical data and annotated image series. Cases from the University & University Hospitals of Geneva (HUG) are retrospectively and prospectively collected to populate the database. Currently, 59 cases with certified diagnosis and their clinical parameters are stored in the database as well as 254 image series of which 26 have their regions of interest annotated. The available data was used to test primary visual features for the classification of lung tissue patterns

  11. Estimating local scaling properties for the classification of interstitial lung disease patterns

    Science.gov (United States)

    Huber, Markus B.; Nagarajan, Mahesh B.; Leinsinger, Gerda; Ray, Lawrence A.; Wismueller, Axel

    2011-03-01

    Local scaling properties of texture regions were compared in their ability to classify morphological patterns known as 'honeycombing' that are considered indicative for the presence of fibrotic interstitial lung diseases in high-resolution computed tomography (HRCT) images. For 14 patients with known occurrence of honeycombing, a stack of 70 axial, lung kernel reconstructed images were acquired from HRCT chest exams. 241 regions of interest of both healthy and pathological (89) lung tissue were identified by an experienced radiologist. Texture features were extracted using six properties calculated from gray-level co-occurrence matrices (GLCM), Minkowski Dimensions (MDs), and the estimation of local scaling properties with Scaling Index Method (SIM). A k-nearest-neighbor (k-NN) classifier and a Multilayer Radial Basis Functions Network (RBFN) were optimized in a 10-fold cross-validation for each texture vector, and the classification accuracy was calculated on independent test sets as a quantitative measure of automated tissue characterization. A Wilcoxon signed-rank test was used to compare two accuracy distributions including the Bonferroni correction. The best classification results were obtained by the set of SIM features, which performed significantly better than all the standard GLCM and MD features (p interstitial lung diseases when compared to standard texture analysis methods.

  12. Benefit of adjunctive tacrolimus in connective tissue disease-interstitial lung disease.

    Science.gov (United States)

    Witt, Leah J; Demchuk, Carley; Curran, James J; Strek, Mary E

    2016-02-01

    We evaluated the safety and effectiveness of adjunctive tacrolimus therapy with conventional immunosuppression in patients with severe connective tissue disease-related interstitial lung disease (CTD-ILD). We included patients from our interstitial lung disease (ILD) registry with CTD-ILD, in whom tacrolimus was added to corticosteroids and an additional immunosuppressive agent. Demographic data, clinical features, lung function, radiographic images, and pathologic findings were reviewed. Effectiveness was assessed by comparing pulmonary function tests (PFTs) closest to tacrolimus initiation to PFTs approximately 6-12 months later. Corticosteroid dose at these time points was also evaluated. We report adverse events attributed to tacrolimus. Seventeen patients with CTD-ILD were included in adverse event analysis; twelve were included in efficacy analysis. Length of tacrolimus therapy ranged from 6 to 110 months (mean 38.8 months ± 31.4). The mean improvement in percent predicted total lung capacity was 7.5% ± 11.7 (p = 0.02). Forced vital capacity mean improvement was 7.4% ± 12.5 (p = 0.06). The average decrease in corticosteroid dose at follow-up was 20.3 mg ± 25.2 (p = 0.02) with complete discontinuation in six patients. No patients experienced a life-threatening adverse event attributed to tacrolimus. Tacrolimus can be effective and is well tolerated as an adjunctive therapy and allows tapering of corticosteroids.

  13. Interstitial lung disease associated with Equine Infectious Anemia Virus infection in horses.

    Science.gov (United States)

    Bolfa, Pompei; Nolf, Marie; Cadoré, Jean-Luc; Catoi, Cornel; Archer, Fabienne; Dolmazon, Christine; Mornex, Jean-François; Leroux, Caroline

    2013-12-01

    EIA (Equine Infectious Anemia) is a blood-borne disease primarily transmitted by haematophagous insects or needle punctures. Other routes of transmission have been poorly explored. We evaluated the potential of EIAV (Equine Infectious Anemia Virus) to induce pulmonary lesions in naturally infected equids. Lungs from 77 EIAV seropositive horses have been collected in Romania and France. Three types of lesions have been scored on paraffin-embedded lungs: lymphocyte infiltration, bronchiolar inflammation, and thickness of the alveolar septa. Expression of the p26 EIAV capsid (CA) protein has been evaluated by immunostaining. Compared to EIAV-negative horses, 52% of the EIAV-positive horses displayed a mild inflammation around the bronchioles, 22% had a moderate inflammation with inflammatory cells inside the wall and epithelial bronchiolar hyperplasia and 6.5% had a moderate to severe inflammation, with destruction of the bronchiolar epithelium and accumulation of smooth muscle cells within the pulmonary parenchyma. Changes in the thickness of the alveolar septa were also present. Expression of EIAV capsid has been evidenced in macrophages, endothelial as well as in alveolar and bronchiolar epithelial cells, as determined by their morphology and localization. To summarize, we found lesions of interstitial lung disease similar to that observed during other lentiviral infections such as FIV in cats, SRLV in sheep and goats or HIV in children. The presence of EIAV capsid in lung epithelial cells suggests that EIAV might be responsible for the broncho-interstitial damages observed.

  14. Noninfectious interstitial lung disease during infliximab therapy: case report and literature review.

    Science.gov (United States)

    Caccaro, Roberta; Savarino, Edoardo; D'Incà, Renata; Sturniolo, Giacomo Carlo

    2013-08-28

    Pulmonary abnormalities are not frequently encountered in patients with inflammatory bowel diseases. However, lung toxicity can be induced by conventional medications used to maintain remission, and similar evidence is also emerging for biologics. We present the case of a young woman affected by colonic Crohn's disease who was treated with oral mesalamine and became steroid-dependent and refractory to azathioprine and adalimumab. She was referred to our clinic with a severe relapse and was treated with infliximab, an anti-tumor necrosis factor α (TNF-α) antibody, to induce remission. After an initial benefit, with decreases in bowel movements, rectal bleeding and C-reactive protein levels, she experienced shortness of breath after the 5(th) infusion. Noninfectious interstitial lung disease was diagnosed. Both mesalamine and infliximab were discontinued, and steroids were introduced with slow but progressive improvement of symptoms, radiology and functional tests. This represents a rare case of interstitial lung disease associated with infliximab therapy and the effect of drug withdrawal on these lung alterations. Given the increasing use of anti-TNF-α therapies and the increasing reports of pulmonary abnormalities in patients with inflammatory bowel diseases, this case underlines the importance of a careful evaluation of respiratory symptoms in patients undergoing infliximab therapy.

  15. Computed tomography of the lungs in acquired immunodeficiency syndrome. An early indicator of interstitial pneumonia

    Energy Technology Data Exchange (ETDEWEB)

    Hartelius, H.; Gaub, J.; Jensen, L.I.; Jensen, J.; Faber, V.

    Computed tomography of the chest was performed on 42 occasions as part of the diagnostic work-up in 26 homosexual men with, or suspected of the acquired immunodeficiency syndrome (AIDS). In 17 cases both the chest radiographs and the lung scans were abnormal, and bronchoscopy and/or lung biopsy established an etiologic diagnosis in the majority of these cases. In 9 cases CT of the lungs revealed unequivocal interstitial infiltration in the presence of a normal chest radiography, and subsequently and etiologic agent was demonstrated in all these cases. In 9 cases, patients with symptoms indicative of pulmonary infection had both a normal chest radiograph and a normal lung scan, and in none of these cases did the clinical course or additional diagnostic procedures indicate the presence of current opportunistic lung infection. CT of the lungs seems to identify accurately those patients with severe HIV-related diseases in whom invasive diagnostic procedures such as bronchoalveolar lavage and/or lung biopsy should be done.

  16. Drug-induced interstitial lung diseases. Often forgotten; Medikamenteninduzierte interstitielle Lungenerkrankungen. Haeufig vergessen

    Energy Technology Data Exchange (ETDEWEB)

    Poschenrieder, F.; Stroszczynski, C. [Universitaetsklinikum Regensburg, Institut fuer Roentgendiagnostik, Regensburg (Germany); Hamer, O.W. [Universitaetsklinikum Regensburg, Institut fuer Roentgendiagnostik, Regensburg (Germany); Lungenfachklinik Donaustauf, Donaustauf (Germany)

    2014-12-15

    Drug-induced interstitial lung diseases (DILD) are probably more common than diagnosed. Due to their potential reversibility, increased vigilance towards DILD is appropriate also from the radiologist's point of view, particularly as these diseases regularly exhibit radiological correlates in high-resolution computed tomography (HRCT) of the lungs. Based on personal experience typical relatively common manifestations of DILD are diffuse alveolar damage (DAD), eosinophilic pneumonia (EP), hypersensitivity pneumonitis (HP), organizing pneumonia (OP), non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). These patterns are presented based on case studies, whereby emphasis is placed on the clinical context. This is to highlight the relevance of interdisciplinary communication and discussion in the diagnostic field of DILD as it is a diagnosis of exclusion or of probability in most cases. Helpful differential diagnostic indications for the presence of DILD, such as an accompanying eosinophilia or increased attenuation of pulmonary consolidations in amiodarone-induced pneumopathy are mentioned and the freely available online database http://www.pneumotox.com is presented. (orig.) [German] Medikamenteninduzierte interstitielle Lungenerkrankungen (engl. ''drug-induced interstitial lung diseases'', DILD) sind wahrscheinlich haeufiger, als sie diagnostiziert werden. Aufgrund ihrer potenziellen Reversibilitaet ist eine erhoehte Vigilanz gegenueber DILD auch seitens der Radiologie angebracht, da diese regelmaessig ein radiomorphologisches Korrelat in der hochaufloesenden Computertomographie (''high-resolution CT'', HRCT) der Lunge aufweisen. Typische, nach eigener Erfahrung relativ haeufige Manifestationsformen von DILD sind der diffuse Alveolarschaden (engl. ''diffuse alveolar damage'', DAD), die eosinophile Pneumonie (EP), die Hypersensitivitaetspneumonitis (HP), die organisierende

  17. Efficacy of immunosoppressive therapy and steroid sparing effect in interstitial lung disease associated to antisynthetase syndrome

    Directory of Open Access Journals (Sweden)

    G. De Marchi

    2011-09-01

    Full Text Available Objective: To evaluate the role of bronchoalveolar lavage (BAL in patients with interstitial lung disease associated to antisynthetase syndrome. Methods: We describe 5 patients, anti-Jo1 positive, with interstitial lung disease (lung fibrosis and/or diffusion capacity of CO <80%. Patients were monitored with lung function tests every 6 months, with high-resolution computed tomography (HRCT every 12 months, and with bronchoalveolar lavage (BAL at baseline and in the subsequent follow-up. Patients were treated as follows: a azathioprine with colchicine, or cyclosporine alone b cyclophosphamide when high neutrophil or eosinophil count on BAL was observed. Only low-dose steroids were used for mild muscular or articular involvement. Results: Pulmonary involvement remained stable in all patients at months +24. Lung function remained unchanged compared to the baseline evaluation; HRCT was stable in patients with fibrosis and no progression into fibrosis was observed in patients with ground glass areas at baseline. Bacterial pneumonia occurred in one patient treated with cyclophosphamide and resolved after antibiotic therapy. Conclusions: Clinical manifestations, instrumental tests and BAL may be of value to choice the best immunosuppressive therapy in the single case. An early less aggressive approach (azathioprine with colchicine, or cyclosporine alone may be useful. BAL could be performed when a progression of the lung involvement is demonstrated in the subsequent follow-up. Cyclophosphamide may be a valid alternative treatment in the presence of a neutrophilic or eosinophilic alveolitis. Efficacy and safety of the aforementioned immunosuppressive approach were observed in our series, avoiding prolonged high-dose steroid administration.

  18. Metallic elements in exhaled breath condensate of patients with interstitial lung diseases.

    Science.gov (United States)

    Corradi, Massimo; Acampa, Olga; Goldoni, Matteo; Adami, Elena; Apostoli, Pietro; de Palma, Giuseppe; Pesci, Alberto; Mutti, Antonio

    2009-12-01

    Epidemiological data support the hypothesis that environmental and occupational agents play an important role in the development of interstitial lung diseases such as idiopathic interstitial pneumonia (IIPs) and sarcoidosis. The aim of this study was to assess the elemental composition of exhaled breath condensate (EBC) in patients with interstitial lung diseases (ILDs) of unknown etiology and healthy subjects as an indirect evaluation of tissue burden, which could improve our understanding of the role of metals in the pathogenesis of ILDs. EBC was obtained from 33 healthy subjects, 22 patients with sarcoidosis, 15 patients with non-specific interstitial pneumonia (NSIP) and 19 with IIPs. Trace elements and toxic metals in the samples were measured by means of inductively coupled plasma-mass spectrometry. There are only small overall differences in the EBC levels of a number of metallic elements among patients with idiopathic pulmonary fibrosis (IPF), NSIP or sarcoidosis, and no pattern is capable of distinguishing them with a high degree of sensitivity and specificity. However, a pattern of pneumotoxic (Si, Ni) and essential elements (Zn, Se and Cu) with the addition of Co distinguished the patients with ILDs from healthy non-smokers with relatively high degrees of sensitivity (96.4%) and specificity (90.9%). Assessing the elemental composition of EBC in patients with different ILDs seems to provide useful information. The non-invasiveness of the EBC method makes it suitable for patients with pulmonary diseases, although further studies are required to confirm the usefulness of this approach and to better understand the underlying pathophysiological processes.

  19. αvβ6 integrin may be a potential prognostic biomarker in interstitial lung disease.

    Science.gov (United States)

    Saini, Gauri; Porte, Joanne; Weinreb, Paul H; Violette, Shelia M; Wallace, William A; McKeever, Tricia M; Jenkins, Gisli

    2015-08-01

    Idiopathic pulmonary fibrosis (IPF) and fibrotic nonspecific interstitial pneumonitis are progressive interstitial lung diseases (ILDs) with limited treatment options and poor survival. However, the rate of disease progression is variable, implying there may be different endotypes of disease. We hypothesised that immunophenotyping biopsies from ILD patients might reveal distinct endotypes of progressive fibrotic disease, which may facilitate stratification when undertaking clinical trials of novel therapies for IPF.43 paraffin-embedded, formalin-fixed lung tissue sections were immunostained for five molecules implicated in the pathogenesis of the fibrosis: α-smooth muscle actin (αSMA), αvβ6 integrin, pro-surfactant protein C (SP-C), hepatocyte growth factor (HGF) and tenascin-C (TenC). Levels of immunostaining and numbers of fibroblastic foci were quantified using operator-dependent and -independent methods. The relationship of all these markers to overall survival was analysed.Staining revealed high levels of αSMA, αvβ6 integrin, pro-SP-C, HGF and TenC, and fibroblastic foci. Immunostaining varied across samples for all molecules but only the extent of αvβ6 integrin immunostaining was associated with increased mortality. There was no association with the other markers measured.Our data suggest high levels of αvβ6 integrin may identify a specific endotype of progressive fibrotic lung disease.

  20. Biopsia en enfermedad intersticial pulmonar Lung biopsy for the diagnosis of interstitial lung disease

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    Silvia Quadrelli

    2007-12-01

    Full Text Available El objetivo del presente estudio fue determinar el rédito diagnóstico y los factores asociados a mayor morbimortalidad en la biopsia quirúrgica de pulmón en pacientes con enfermedad intersticial difusa. Se analizaron en forma retrospectiva los registros clínicos de 71 pacientes. Se registraron complicaciones en 16 pacientes (22.5%. La mortalidad operatoria fue 11.2%. Los pacientes en quienes la biopsia se realizó por videotoracoscopia (n = 52 y por toracotomía (n = 17 tuvieron la misma duración de estadía en terapia intensiva y de estadía hospitalaria. La tasa de complicaciones (22.2% vs. 21.0%, p = 1.0000 y la mortalidad (9.2 vs. 15.7%, p = 0.2738 no fueron diferentes. Ocho pacientes murieron dentro de los 30 días. La prevalencia de inmunosupresión (4/8 vs. 9/63, p = 0.0325 fue significativamente superior en el grupo de pacientes fallecidos. Estos pacientes tuvieron valores preoperatorios más elevados de urea (50 ± 20.1 mg/dl vs. 31.2 ± 10.3 mg/ dl, p = 0.0013 y menores valores de saturación de O2: 82.7 ± 14.8% vs. 92.8 ± 3.4%, p = 0.0009. En los 11 pacientes con iniciación aguda la mortalidad fue significativamente más elevada (36.3% vs. 7.1%, p = 0.0223. La biopsia aportó un diagnóstico específico en 100% de los pacientes y cambió la estrategia terapéutica en 66.7%. En conclusión, la biopsia de pulmón por vía toracoscópica es un procedimiento útil y seguro en los pacientes con enfermedad intersticial difusa del pulmón. Sin embargo, en el grupo de pacientes inmunocomprometidos, con cuadros de presentación aguda y con insuficiencia respiratoria preoperatoria, la mortalidad es elevada y deben balancearse muy críticamente los riesgos contra los beneficios en ese grupo de enfermos.The objective of this study was to determine the morbidity, mortality and diagnostic yield of video assisted thoracoscopy (VATS and thoracotomy lung biopsy in interstitial lung disease (ILD. Clinical records of 71 patients were

  1. Drug-induced interstitial lung diseases%药源性间质性肺疾病

    Institute of Scientific and Technical Information of China (English)

    王晓芳; 张运剑; 夏国光

    2012-01-01

    Drug-induced interstitial lung disease is the most common type of drug-induced respiratory diseases. It is known that a lot of drugs can cause interstitial lung diseases. The underlying mechanism may be associated with allergy and the direct cellular toxicity. Its clinical symptoms, imaging, and histopathology were untypical. Clinical diagnosis of the disease was based on the medical history ( medication history), clinical features, chest imaging, hi sto pathological changes, and response to treatment. If interstitial lung diseases were suspected to be; drug-induced, the drug should be discontinued immediately and glucocorticoid could be given. The prognosis is good if intervention is rapid.%药源性间质性肺疾病(DI-ILD)是最常见的药源性肺部疾病,目前已知多种药物可导致间质性肺疾病,其发病机制与药物引起的变态反应和直接细胞毒性作用有关.此类疾病临床症状、影像学及组织病理学表现均无特异性,临床诊断DI-ILD需根据病史(用药史)、临床特点、胸部影像学、组织病理学改变及治疗效果.临床疑诊DI-ILD时应立刻停药并应用糖皮质激素.若及时干预,预后良好.

  2. Clinical features of interstitial lung disease in primary Sjgren’s syndrome

    Institute of Scientific and Technical Information of China (English)

    李娅

    2013-01-01

    Objective To detect the clinical features of interstitial lung disease (ILD) in patients with primary Sjgren’s syndrome (pSS) .Methods From January 2009 to November 2011,368 patients with pSS from 16 clinical centers in China were collected.Patients were divided into the ILD group and the non ILD group according to their chest high resolution CT (HRCT) presentation.Ttest,nonparametric test and Chisquare test were applied to compare data in the subgroups.Logistic regression

  3. Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

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    Daniel Antunes Silva Pereira

    2015-04-01

    Full Text Available OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD. METHODS: This was a retrospective study of patients with interstitial lung disease (ILD, positive antinuclear antibody (ANA results (≥ 1/320, with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD. RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89% and anti-SSA (anti-Ro, 27%. The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05. Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.

  4. Erlotinib-associated interstitial lung disease in advanced pancreatic carcinoma: a case report and literature review.

    Science.gov (United States)

    Macerelli, Marianna; Mazzer, Micol; Foltran, Luisa; Cardellino, Giovanni Gerardo; Aprile, Giuseppe

    2015-07-24

    The combination of erlotinib and gemcitabine is a recognized option for patients with metastatic pancreatic cancer whose common adverse events such as skin rash, diarrhea, or fatigue are usually easily manageable. Interstitial lung disease (ILD) is a life-threatening toxicity reported in patients with non-small-cell lung cancers treated with epidermal growth factor receptor-tyrosine kinase inhibitors or gemcitabine. This side effect is extremely rare in patients with pancreatic cancer. We report fatal treatment-related ILD that occurred in a 67-year-old patient with metastatic pancreatic cancer. Risk factors and pathophysiology of ILD need further investigation but caution is highly recommended for patients with an underlying pulmonary disease when using erlotinib in monotherapy or combination therapy.

  5. State of the art in the diagnosis and management of interstitial lung disease.

    Science.gov (United States)

    Buzan, Maria T A; Pop, Carmen Monica

    2015-01-01

    The interstitial lung diseases (ILDs) are a diverse group of disorders characterized by a varying combination of inflammation and fibrosis of the pulmonary parenchyma. Treatment and prognosis of ILD typically depend on the underlying ILD subtype, highlighting the importance of accurate classification and diagnosis. Besides a thorough history and clinical examination, the protocol should include a 6-minute walk test, chest radiography, high-resolution computed tomography, biochemical analysis, pulmonary function tests, blood gas analysis, bronchoalveolar lavage, and, when necessary, a lung biopsy. The final diagnosis of ILD entities requires dynamic interaction between clinicians, radiologists and pathologists to reach a clinico-radiologic-pathologic diagnosis, the gold standard no longer being the histology but rather a multidisciplinary approach.

  6. Classification of interstitial lung disease patterns using local DCT features and random forest.

    Science.gov (United States)

    Anthimopoulos, M; Christodoulidis, S; Christe, A; Mougiakakou, S

    2014-01-01

    Over the last decade, a plethora of computer-aided diagnosis (CAD) systems have been proposed aiming to improve the accuracy of the physicians in the diagnosis of interstitial lung diseases (ILD). In this study, we propose a scheme for the classification of HRCT image patches with ILD abnormalities as a basic component towards the quantification of the various ILD patterns in the lung. The feature extraction method relies on local spectral analysis using a DCT-based filter bank. After convolving the image with the filter bank, q-quantiles are computed for describing the distribution of local frequencies that characterize image texture. Then, the gray-level histogram values of the original image are added forming the final feature vector. The classification of the already described patches is done by a random forest (RF) classifier. The experimental results prove the superior performance and efficiency of the proposed approach compared against the state-of-the-art.

  7. Acquired lobar emphysema in a case of interstitial chronic lung disease. Surgical implications and review of the literature

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    Sancho Hernández Rogelio

    2014-07-01

    Full Text Available Acquired lobar interstitial emphysema (ALIE, initially described in infants with bronchopulmonary dysplasia is now known to occur in other conditions such as a secondary post-traumatic or inflammatory injury as in patients requiring ventilatory sup- port with chronic lung disease. The ALIE leads to progressive deterioration of lung function with massive distension and com- pression of lung parenchyma with consequent mediastinal shift in the course of chronic interstitial lung disease. This situation requires adequate clinical and radiological interpretation in order to make the proper patient selection i.e., between a candidate for lung biopsy for the benefit of a definitive diagnosis or one who in the course of a superimposed ALIE requires a diagnostic and therapeutic lobectomy. Two cases of this entity and descriptive decision-making diagnostic and surgical about pre-and intraop- erative findings are presented.

  8. [Annual review of Archivos de Neumología in interventional pneumology, interstitial diseases and lung transplantation].

    Science.gov (United States)

    Disdier, Carlos; Bollo, Elena; Morales, Pilar; Montero, Carmen

    2009-01-01

    A review has been made of original articles on invasive pneumology techniques, interstitial diseases and lung transplantation, published in the Archivos de Bronconeumología during the year 2008. We have selected the publication by Martínez-Olondrins et al on the mediastinal staging of bronchogenic carcinoma by transbronchial needle aspiration to highlight the role of this simple, safe and cost-effective technique at a time when aspiration by ultrasound-guided bronchoscopy is profiled as an alternative to staging by mediastinoscopy. Besides its usefulness in the study of lymph nodes, transbronchial needle aspiration increases the overall performance of bronchoscopy by 20%, which means that it should be considered as a basic tool in the study of lung cancer. We also comment on the work by Galvis-Caravajal et al, who describe percutaneous radiofrequency as an alternative to radiotherapy in small lung or metastasic tumours. In diffuse interstitial disease, Morell et al analysed the diagnostic methods in 500 patients with this clinical-radiological presentation in which a definitive diagnosis was achieved in 85%, with 25% of them being obtained by non-invasively. Baloira et al analysed the characteristics of 19 patients with desquamative interstitial pneumonia and respiratory bronchiolitis associated-interstitial lung disease obtained from the National register of Interstitial Diseases.

  9. Undifferentiated connective tissue disease presenting with prevalent interstitial lung disease: Case report and review of literature

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    Sfriso Paolo

    2011-06-01

    Full Text Available Abstract Undifferentiated connective tissue diseases (UCTDs are clinical entities characterised by signs and symptoms suggestive of a systemic autoimmune disease, which do not fulfil the diagnostic criteria for a defined connective tissue disease. Lung involvement can complicate the course and management of the disease, often determining a worse outcome. Respiratory dysfunction as the first clinical manifestation has seldom been reported. We describe a case of a female patient who developed significant respiratory dysfunction as the principal clinical sign. Video-assisted thoracoscopy was performed and a histological pattern of nonspecific interstitial pneumonia (NSIP was found. A pathological diagnosis suggested careful follow-up with extensive immunological screening which then detected Raynaud's phenomenon and positivity of antinuclear antibodies. After a multidisciplinary discussion (pneumologist, radiologist, pathologist and rheumatologist a final diagnosis of NSIP associated with UCTD was made. The diagnosis of UCTD should be considered when NSIP is diagnosed even in cases with evident first clinical manifestations of severe respiratory dysfunction. A multidisciplinary approach in the field of interstitial lung disease with NSIP, also including rheumatologic expertise, is fundamental to achieve a prompt and correct diagnosis.

  10. An integrated approach in the diagnosis of smoking-related interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    Sergio Harari

    2012-09-01

    Full Text Available Cigarette smoke consists of several chemical compounds with a variety of effects in many organs. In the lung, apart being the main cause of chronic obstructive pulmonary disease, carcinoma and idiopathic spontaneous pneumothorax, tobacco smoke is associated with interstitial lung diseases (ILDs, including respiratory bronchiolitis-associated ILD (RB-ILD, desquamative interstitial pneumonia (DIP, pulmonary Langerhans’ cell histiocytosis (PLCH, idiopathic pulmonary fibrosis, acute eosinophilic pneumonia, ILD in rheumatoid arthritis and pulmonary haemorrhage in Goodpasture syndrome. This review will focus on the diseases with a stronger epidemiological association with tobacco smoke, namely RB-ILD, DIP and PLCH. Although the exact pathogenetic evidence linking smoking with these disorders is still not completely understood, there is growing evidence that tobacco smoke targets the terminal or respiratory bronchioles in these diseases, and the differences are reflective of the degree of severity of small airway and parenchymal reaction to the smoke exposure. Despite considerable clinical, radiological and histological overlap between RB-ILD, DIP and PLCH, it is useful to retain the separate classifications for prognostic and therapeutic implications.

  11. Interstitial lung disease in an adult with Fanconi anemia: Clues to the pathogenesis

    Energy Technology Data Exchange (ETDEWEB)

    Rubinstein, W.S.; Wenger, S.L.; Hoffman, R.M. [Univ. of Pittsburgh, PA (United States)] [and others

    1997-03-31

    We have studied a 38-year-old man with a prior diagnosis of Holt-Oram syndrome, who presented with diabetes mellitus. He had recently taken prednisone for idiopathic interstitial lung disease and trimethoprim-sulfamethoxazole for sinusitis. Thrombocytopenia progressed to pancytopenia. The patient had skeletal, cardiac, renal, cutaneous, endocrine, hepatic, neurologic, and hematologic manifestations of Fanconi anemia (FA). Chest radiographs showed increased interstitial markings at age 25, dyspnea began in his late 20s, and he stopped smoking at age 32. At age 38, computerized tomography showed bilateral upper lobe fibrosis, lower lobe honeycombing, and bronchiectasis. Pulmonary function tests, compromised at age 29, showed a moderately severe obstructive and restrictive pattern by age 38. Serum alpha-1 antitrypsin level was 224 (normal 85-213) mg/dL and PI phenotype was M1. Karyotype was 46,X-Y with a marked increase in chromosome aberrations induced in vitro by diepoxybutane. The early onset and degree of pulmonary disease in this patient cannot be fully explained by environmental or known genetic causes. The International Fanconi Anemia Registry (IFAR) contains no example of a similar pulmonary presentation. Gene-environment (ecogenetic) interactions in FA seem evident in the final phenotype. The pathogenic mechanism of lung involvement in FA may relate to oxidative injury and cytokine anomalies. 49 refs., 2 figs., 1 tab.

  12. Transbronchial cryobiopsy in interstitial lung disease: experience in 106 cases – how to do it

    Science.gov (United States)

    Bango-Álvarez, Antonio; Torres-Rivas, Hector; Fernández-Fernández, Luis; Prieto, Amador; Sánchez, Inmaculada; Gil, Maria; Pando-Sandoval, Ana

    2017-01-01

    Transbronchial biopsy using forceps (TBB) is the first diagnostic technique performed on patients with interstitial lung disease (ILD). However, the small size of the samples and the presence of artefacts in the tissue obtained make the yield variable. Our objectives were 1) to attempt to reproduce transbronchial cryobiopsy under the same conditions with which we performed conventional TBB, that is, in the bronchoscopy unit without intubating the patient and without fluoroscopy or general anaesthesia; 2) to describe the method used for its execution; and 3) to analyse the diagnostic yield and its complications. We carried out a prospective study that included 106 patients with clinical and radiological features suggestive of ILD who underwent cryo-transbronchial lung biopsy (cryo-TBB) under moderate sedation without endotracheal intubation, general anaesthesia or use of fluoroscopy. We performed the procedure using two flexible bronchoscopes connected to two video processors, which we alternated until obtaining the number of desired samples. A definitive diagnosis was obtained in 91 patients (86%). As for complications, there were five pneumothoraces (4.7%) and in no case was there severe haemorrhage or exacerbation of the underlying interstitial disease. Cryo-TBB following our method is a minimally invasive, rapid, safe and economic technique that can be performed in a bronchoscopy suite under moderate sedation without the need for intubating the patient or using fluoroscopy and without requiring general anaesthesia. PMID:28344982

  13. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review.

    Science.gov (United States)

    Iqbal, Kundan; Kelly, Clive

    2015-12-01

    Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting 0.5-1% of the worldwide population. Whilst predominantly causing chronic pain and inflammation in synovial joints, it is also associated with significant extra-articular manifestations in a large proportion of patients. Among the various pulmonary manifestations, interstitial lung disease (ILD), a progressive fibrotic disease of the lung parenchyma, is the commonest and most important, contributing significantly to increased morbidity and mortality. The most frequent patterns of RA-associated ILD (RA-ILD) are usual interstitial pneumonia and nonspecific interstitial pneumonia. New insights during the past several years have highlighted the epidemiological impact of RA-ILD and have begun to identify factors contributing to its pathogenesis. Risk factors include smoking, male sex, human leukocyte antigen haplotype, rheumatoid factor and anticyclic citrullinated protein antibodies (ACPAs). Combined with clinical information, chest examination and pulmonary function testing, high-resolution computed tomography of the chest forms the basis of investigation and allows assessment of subtype and disease extent. The management of RA-ILD is a challenge. Several therapeutic agents have been suggested in the literature but as yet no large randomized controlled trials have been undertaken to guide clinical management. Therapy is further complicated by commonly prescribed drugs of proven articular benefit such as methotrexate, leflunomide (LEF) and anti-tumour necrosis factor α agents having been implicated in both ex novo occurrence and acceleration of existing ILD. Agents that offer promise include immunomodulators such as mycophenolate and rituximab as well as newly studied antifibrotic agents. In this review, we discuss the current literature to evaluate recommendations for the management of RA-ILD and discuss key gaps in our knowledge of this important disease.

  14. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis

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    Bonnefoy, Olivier; Ferretti, Gilbert; Calaque, Olivier; Coulomb, Max; Begueret, Hugues; Beylot-Barry, Marie; Laurent, Francois E-mail: francois.laurent@chu-bordeaux.fr

    2004-03-01

    Objective: A retrospective study was carried out in two institutions to determine serial changes in the pattern, distribution, and extent of interstitial lung disease (ILD) associated with polymyositis (PM)-dermatomyositis (DM) using HRCT. Subjects and methods: Twenty patients with PM-DM and clinical suspicion of ILD who underwent at least two serial HRCT examinations were retrospectively evaluated by two readers. Patients were classified according to the dominant CT pattern which was correlated with clinical evolution and underlying histology when available (n=6). Results: Patients were classified into four groups according to the dominant pattern: ground-glass attenuation and reticulation (group 1, n=9); honeycombing (group 2, n=4); airspace consolidation (group 3, n=4), and normal or almost normal lung (group 4, n=3). Under medical treatment, serial HRCT showed that the extent of areas of ground-glass opacities (group 1) decreased in five patients, stabilized in two, and increased in two. Pathologic findings demonstrated usual interstitial pneumonia (UIP) in two cases and unspecified interstitial pneumonia in one. In group 2, extent of honeycombing increased in three cases and stabilized in one. In group 3, dramatic resolution of airspace consolidation occurred in three cases. Clinical deterioration with extensive consolidation at CT and diffuse alveolar damage (DAD) at histology occurred in one patient of each of the three previous groups. Lesions stayed invisible or progressed slightly in the fourth group. Conclusion: In ILD associated with PM-DM, clinical deterioration and DAD in the follow-up can be observed whatever the HRCT pattern. However, unfavorable evolution is constant when honeycombing is present at the initial CT.

  15. Up-to-Date Information on Rheumatoid Arthritis-Associated Interstitial Lung Disease.

    Science.gov (United States)

    Suda, Takafumi

    2015-01-01

    Pulmonary involvement is common in rheumatoid arthritis (RA) and affects all the components of the lung. Interstitial lung disease (ILD) is the most predominant pulmonary manifestation and has been identified as the main cause of morbidity and mortality in RA. Clinically significant RA-ILD occurs in approximately 10% of RA patients. Several risk factors, such as old age, male gender, and smoking, have been reported to date. Histologically, the proportion of the usual interstitial pneumonia (UIP) pattern is higher in RA-ILD than in ILD associated with other connective tissue diseases, and RA-ILD also shows nonspecific interstitial pneumonia and organizing pneumonia patterns. High-resolution computed tomography scans are highly predictive of the histological UIP pattern with a specificity of 96%-100%. Acute exacerbation, which is the acute deterioration of the respiratory status characterized by newly developed bilateral infiltrates with unknown etiologies, has been reported in RA-ILD. Although acute exacerbation of RA-ILD has high mortality, similar to that of idiopathic pulmonary fibrosis, its incidence is lower in RA-ILD than in idiopathic pulmonary fibrosis. A consensus treatment has not yet been established. Current therapeutic regimens typically include corticosteroids with or without cytotoxic agents. Recent large longitudinal studies reported that the prognosis of RA-ILD was poor with a median survival of 2.6-3.0 years. Furthermore, histological and/or radiological patterns, such as UIP or non-UIP, have significant prognostic implications. RA-ILD patients with histological or radiological UIP patterns have poorer prognoses than those with non-UIP patterns. This review assessed the characteristics of RA-ILD by overviewing recent studies in the field and focused on the clinical significance of histological and/or radiological patterns in RA-ILD.

  16. MRI of interstitial lung diseases. What is possible?; MRT bei interstitiellen Lungenerkrankungen. Was ist moeglich

    Energy Technology Data Exchange (ETDEWEB)

    Biederer, J. [Kreisklinik Gross-Gerau, Radiologie Darmstadt, Gross-Gerau (Germany); Universitaetsklinikum Heidelberg, Klinik fuer Diagnostische und Interventionelle Radiologie, Heidelberg (Germany); Deutsches Zentrum fuer Lungenforschung (DZL), Translational Lung Research Center (TLRC) Heidelberg, Heidelberg (Germany); Thoraxklinik, Universitaetsklinikum Heidelberg, Klinik fuer Diagnostische und Interventionelle Radiologie mit Nuklearmedizin, Heidelberg (Germany); Wielpuetz, M.O.; Jobst, B.J.; Dinkel, J. [Universitaetsklinikum Heidelberg, Klinik fuer Diagnostische und Interventionelle Radiologie, Heidelberg (Germany); Deutsches Zentrum fuer Lungenforschung (DZL), Translational Lung Research Center (TLRC) Heidelberg, Heidelberg (Germany); Thoraxklinik, Universitaetsklinikum Heidelberg, Klinik fuer Diagnostische und Interventionelle Radiologie mit Nuklearmedizin, Heidelberg (Germany)

    2014-12-15

    Magnetic resonance imaging (MRI) of the lungs is becoming increasingly appreciated as a third diagnostic imaging modality besides chest x-ray and computed tomography (CT). Its value is well acknowledged for pediatric patients or for scientific use particularly when radiation exposure should be strictly avoided. However, the diagnosis of interstitial lung disease is the biggest challenge of all indications. The objective of this article is a summary of the current state of the art for diagnostic MRI of interstitial lung diseases. This article reflects the results of a current search of the literature and discusses them against the background of the authors own experience with lung MRI. Due to its lower spatial resolution and a higher susceptibility to artefacts MRI does not achieve the sensitivity of CT for the detection of small details for pattern recognition (e.g. fine reticulation and micronodules) but larger details (e.g. coarse fibrosis and honeycombing) can be clearly visualized. Moreover, it could be shown that MRI has the capability to add clinically valuable information on regional lung function (e.g. ventilation, perfusion and mechanical properties) and inflammation with native signal and contrast dynamics. In its present state MRI can be used for comprehensive cardiopulmonary imaging in patients with sarcoidosis or for follow-up of lung fibrosis after initial correlation with CT. Far more indications are expected when the capabilities of MRI for the assessment of regional lung function and activity of inflammation can be transferred into robust protocols for clinical use. (orig.) [German] Die MRT der Lunge entwickelt sich zu einer ernstzunehmenden dritten Saeule der Thoraxdiagnostik neben dem Thoraxroentgen und der Computertomographie (CT). Ihr Wert in der paediatrischen Lungendiagnostik oder fuer den wissenschaftlichen Einsatz, insbesondere wenn eine Strahlenexposition vermieden werden soll, ist unbestritten. Von allen Indikationen stellt die Diagnostik

  17. Establishment of a Rat Adjuvant Arthritis-Interstitial Lung Disease Model

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    Liu-nan Song

    2016-01-01

    Full Text Available Introduction. Development of an animal model of rheumatoid arthritis-interstitial lung disease (RA-ILD and improved knowledge of the pathogenesis of RA-ILD may facilitate earlier diagnosis and the development of more effective targeted therapies. Methods. Adult male Wistar rats were studied in an adjuvant arthritis (AA model induced by the injection of Freund’s complete adjuvant (FCA. Rats were sacrificed on days 7, 14, 21, and 28 after FCA injection. Lung tissue was obtained for histopathological examination and evaluation of Caveolin-1 (Cav-1 and transforming growth factor-β (TGF-β1 protein expression levels. Results. Pulmonary inflammation was evident in lung tissue from day 21 after FCA injection. Inflammation and mild fibrosis were observed in lung tissue on day 28 after FCA injection. Cav-1 protein expression was significantly decreased from day 7 through day 28 and TGF-β1 protein expression was significantly increased on day 28 after FCA injection compared to control (P<0.05. Conclusion. We established an AA rat model that exhibited the extra-articular complication of RA-ILD. We identified Cav-1 and TGF-β1 as protein biomarkers of RA-ILD in this model and propose their signaling pathway as a possible target for therapeutic intervention.

  18. Automated detection and classification of interstitial lung diseases from low-dose CT images

    Science.gov (United States)

    Zheng, Bin; Leader, Joseph K.; Fuhrman, Carl R.; Sciurba, Frank C.; Gur, David

    2004-05-01

    We developed a computer-aided diagnosis (CAD) scheme to detect and quantitatively assess interstitial lung diseases (ILD) depicted on low-dose and multi-slice helical high-resolution computed tomography (CT) examinations. Eighteen CT cases acquired from patients who underwent routine low-dose whole-lung screening examinations for the detection of lung cancer were used to test the scheme. ILD was identified in all of these cases. The CAD scheme involves multiple steps to segment lung areas, identify suspicious ILD regions depicted on each CT slice, and generate volumetric ILD lesions by grouping and matching ILD regions detected on multiple adjacent slices. The scheme computes five "global" features for each identified ILD region, which include size (or volume), contrast, average local pixel value fluctuation, mean of stochastic fractal dimension, and geometric fractal dimension. Two sets of classification rules are applied to remove false-positive detections. The severity of ILD in each case was rated by one experienced chest radiologist into one of the three categories (mild, moderate, and severe). A distance-weighted k-nearest neighbor algorithm and round-robin validation method was applied to classify each testing case into one of the three categories of severity. In this experiment, the CAD scheme classified 78% (14 out of 18) cases into the same categories as rated by the radiologist.

  19. Detection and classification of interstitial lung diseases and emphysema using a joint morphological-fuzzy approach

    Science.gov (United States)

    Chang Chien, Kuang-Che; Fetita, Catalin; Brillet, Pierre-Yves; Prêteux, Françoise; Chang, Ruey-Feng

    2009-02-01

    Multi-detector computed tomography (MDCT) has high accuracy and specificity on volumetrically capturing serial images of the lung. It increases the capability of computerized classification for lung tissue in medical research. This paper proposes a three-dimensional (3D) automated approach based on mathematical morphology and fuzzy logic for quantifying and classifying interstitial lung diseases (ILDs) and emphysema. The proposed methodology is composed of several stages: (1) an image multi-resolution decomposition scheme based on a 3D morphological filter is used to detect and analyze the different density patterns of the lung texture. Then, (2) for each pattern in the multi-resolution decomposition, six features are computed, for which fuzzy membership functions define a probability of association with a pathology class. Finally, (3) for each pathology class, the probabilities are combined up according to the weight assigned to each membership function and two threshold values are used to decide the final class of the pattern. The proposed approach was tested on 10 MDCT cases and the classification accuracy was: emphysema: 95%, fibrosis/honeycombing: 84% and ground glass: 97%.

  20. The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease

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    Shahrzad M Lari

    2014-05-01

    Full Text Available Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL in patients with Scleroderma-Interstitial Lung Disease (SSc-ILD and its relationship with pulmonary function parameters. Materials and Methods: Considering the inclusion and exclusion criteria, 25 patients with SSc-ILD were enrolled in this cross-sectional study from April 2012 to June 2013. Full tests of lung function, including body plethysmography and diffusing capacity of the lungs for carbon monoxide (DLCO, 6-minute walk distance (6MWD, and pulse oximetry were performed. The HRQoL was assessed using St. George’s and CAT questionnaires; also, dyspnea was evaluated for all the patients, using modified medical research council (MMRC scale. Afterwards, the relationship between the total scores of HRQoL questionnaires and the severity of lung disease was analyzed, based on the recorded variables. Results: The mean age of the patients was 40.36±9.50 years and the mean duration of the disease was 7.16±4.50 years. A statistically significant inverse correlation was observed between 6MWD (r=-0.50, P=0.01, DLCO (r=-0.67, P

  1. Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

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    Kasra Karvandian

    2011-01-01

    Full Text Available A case of moyamoya syndrome and spherocytosis with concurrent interstitial lung disease who underwent laparoscopic splenectomy is being reported. A theory regarding their coexistence is being forwarded together with their anesthetic management. According to our search, this is the fourth case of moyamoya syndrome and the first case with an associated interstitial lung disease in a 10-year-old child.

  2. Automated diagnosis of interstitial lung diseases and emphysema in MDCT imaging

    Science.gov (United States)

    Fetita, Catalin; Chang Chien, Kuang-Che; Brillet, Pierre-Yves; Prêteux, Françoise

    2007-09-01

    Diffuse lung diseases (DLD) include a heterogeneous group of non-neoplasic disease resulting from damage to the lung parenchyma by varying patterns of inflammation. Characterization and quantification of DLD severity using MDCT, mainly in interstitial lung diseases and emphysema, is an important issue in clinical research for the evaluation of new therapies. This paper develops a 3D automated approach for detection and diagnosis of diffuse lung diseases such as fibrosis/honeycombing, ground glass and emphysema. The proposed methodology combines multi-resolution 3D morphological filtering (exploiting the sup-constrained connection cost operator) and graph-based classification for a full characterization of the parenchymal tissue. The morphological filtering performs a multi-level segmentation of the low- and medium-attenuated lung regions as well as their classification with respect to a granularity criterion (multi-resolution analysis). The original intensity range of the CT data volume is thus reduced in the segmented data to a number of levels equal to the resolution depth used (generally ten levels). The specificity of such morphological filtering is to extract tissue patterns locally contrasting with their neighborhood and of size inferior to the resolution depth, while preserving their original shape. A multi-valued hierarchical graph describing the segmentation result is built-up according to the resolution level and the adjacency of the different segmented components. The graph nodes are then enriched with the textural information carried out by their associated components. A graph analysis-reorganization based on the nodes attributes delivers the final classification of the lung parenchyma in normal and ILD/emphysematous regions. It also makes possible to discriminate between different types, or development stages, among the same class of diseases.

  3. Improved pulmonary function following pirfenidone treatment in a patient with progressive interstitial lung disease associated with systemic sclerosis

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    Zarir F Udwadia

    2015-01-01

    Full Text Available Pirfenidone is an anti-fibrotic drug which has been approved for the management of patients with Idiopathic Pulmonary Fibrosis (IPF. However, its role in interstitial lung disease (ILD due to other causes such as systemic sclerosis (SSc is not clear. We present a case of a patient with SSc associated ILD who showed a subjective as well as objective improvement in lung function with pirfenidone.

  4. Case of interstitial lung disease with anti-EJ and anti-CCP antibodies preceding rheumatoid arthritis.

    Science.gov (United States)

    Tomioka, Hiromi; Kaneko, Masahiro; Kogata, Yoshinori; Katsuyama, Eiji; Ishikawa, Seiko; Fujii, Takao

    2012-06-01

    Autoantibodies against aminoacyl-tRNA synthetases (ARSs) are highly specific for myositis and/or interstitial lung disease. We report a rare case of double positive antibodies (anti-EJ antibody, the least common among anti-aminoacyl-tRNA synthetase antibodies, and anti-cyclic citrullinated peptide antibody, reported to be specific for rheumatoid arthritis) in a patient who presented with interstitial lung disease and later developed rheumatoid arthritis. The patient did not have clinically apparent myositis over a period of careful follow-up of several years. The initial pulmonary pathologic findings showed a nonspecific interstitial pneumonia pattern, with the formation of lymphoid follicles, which should be recognized as the first manifestation of rheumatoid arthritis.

  5. Computer-aided detection of early interstitial lung diseases using low-dose CT images

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    Park, Sang Cheol; Kim, Soo Hyung [School of Electronics and Computer Engineering, Chonnam National University, Gwangju 500-757 (Korea, Republic of); Tan, Jun; Wang Xingwei; Lederman, Dror; Leader, Joseph K; Zheng Bin, E-mail: zhengb@upmc.edu [Department of Radiology, University of Pittsburgh, Pittsburgh, PA 15213 (United States)

    2011-02-21

    This study aims to develop a new computer-aided detection (CAD) scheme to detect early interstitial lung disease (ILD) using low-dose computed tomography (CT) examinations. The CAD scheme classifies each pixel depicted on the segmented lung areas into positive or negative groups for ILD using a mesh-grid-based region growth method and a multi-feature-based artificial neural network (ANN). A genetic algorithm was applied to select optimal image features and the ANN structure. In testing each CT examination, only pixels selected by the mesh-grid region growth method were analyzed and classified by the ANN to improve computational efficiency. All unselected pixels were classified as negative for ILD. After classifying all pixels into the positive and negative groups, CAD computed a detection score based on the ratio of the number of positive pixels to all pixels in the segmented lung areas, which indicates the likelihood of the test case being positive for ILD. When applying to an independent testing dataset of 15 positive and 15 negative cases, the CAD scheme yielded the area under receiver operating characteristic curve (AUC = 0.884 {+-} 0.064) and 80.0% sensitivity at 85.7% specificity. The results demonstrated the feasibility of applying the CAD scheme to automatically detect early ILD using low-dose CT examinations.

  6. Computer-aided detection of early interstitial lung diseases using low-dose CT images

    Science.gov (United States)

    Park, Sang Cheol; Tan, Jun; Wang, Xingwei; Lederman, Dror; Leader, Joseph K.; Kim, Soo Hyung; Zheng, Bin

    2011-02-01

    This study aims to develop a new computer-aided detection (CAD) scheme to detect early interstitial lung disease (ILD) using low-dose computed tomography (CT) examinations. The CAD scheme classifies each pixel depicted on the segmented lung areas into positive or negative groups for ILD using a mesh-grid-based region growth method and a multi-feature-based artificial neural network (ANN). A genetic algorithm was applied to select optimal image features and the ANN structure. In testing each CT examination, only pixels selected by the mesh-grid region growth method were analyzed and classified by the ANN to improve computational efficiency. All unselected pixels were classified as negative for ILD. After classifying all pixels into the positive and negative groups, CAD computed a detection score based on the ratio of the number of positive pixels to all pixels in the segmented lung areas, which indicates the likelihood of the test case being positive for ILD. When applying to an independent testing dataset of 15 positive and 15 negative cases, the CAD scheme yielded the area under receiver operating characteristic curve (AUC = 0.884 ± 0.064) and 80.0% sensitivity at 85.7% specificity. The results demonstrated the feasibility of applying the CAD scheme to automatically detect early ILD using low-dose CT examinations.

  7. A role for MCP-1/CCR2 in interstitial lung disease in children

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    Reinhardt Dietrich

    2005-08-01

    Full Text Available Abstract Background Interstitial lung diseases (ILD are chronic inflammatory disorders leading to pulmonary fibrosis. Monocyte chemotactic protein 1 (MCP-1 promotes collagen synthesis and deletion of the MCP-1 receptor CCR2 protects from pulmonary fibrosis in ILD mouse models. We hypothesized that pulmonary MCP-1 and CCR2+ T cells accumulate in pediatric ILD and are related to disease severity. Methods Bronchoalveolar lavage fluid was obtained from 25 children with ILD and 10 healthy children. Levels of pulmonary MCP-1 and Th1/Th2-associated cytokines were quantified at the protein and the mRNA levels. Pulmonary CCR2+, CCR4+, CCR3+, CCR5+ and CXCR3+ T cells were quantified by flow-cytometry. Results CCR2+ T cells and MCP-1 levels were significantly elevated in children with ILD and correlated with forced vital capacity, total lung capacity and ILD disease severity scores. Children with lung fibrosis had significantly higher MCP-1 levels and CCR2+ T cells in bronchoalveolar lavage fluid compared to non-fibrotic children. Conclusion The results indicate that pulmonary CCR2+ T cells and MCP-1 contribute to the pathogenesis of pediatric ILD and might provide a novel target for therapeutic strategies.

  8. mTOR inhibitor-induced interstitial lung disease in cancer patients : Comprehensive review and a practical management algorithm

    NARCIS (Netherlands)

    Willemsen, Annelieke E C A B; Grutters, JC; Gerritsen, Winald R; van Erp, Nielka P; van Herpen, Carla M L; Tol, Jolien

    2016-01-01

    Mammalian target of rapamycin inhibitors (mTORi) have clinically significant activity against various malignancies, such as renal cell carcinoma and breast cancer, but their use can be complicated by several toxicities. Interstitial lung disease (ILD) is an adverse event of particular importance. Mo

  9. Fatal interstitial lung disease associated with high erlotinib and metabolite levels. A case report and a review of the literature.

    NARCIS (Netherlands)

    Heine, R. ter; Bosch, R.T. van den; Schaefer-Prokop, C.M.; Lankheet, N.A.; Beijnen, J.H.; Staaks, G.H.; Westerlaken, M.M. van der; Malingre, M.M.; Brand, J.J. van den

    2012-01-01

    INTRODUCTION: Erlotinib is an agent in the class of oral epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors. Although this class of agents is considered to be relatively safe, the most serious, but rare, adverse reaction is drug-associated interstitial lung disease (ILD). This potent

  10. Study the level of sputum matrix metalloproteinase-9 and tissue inhibitor metaloprotienase-1 in patients with interstitial lung diseases

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    Sherif A. Esa

    2016-01-01

    Results: In this study, we have demonstrated that levels of sputum MMP-9 and TIMP-1 were significantly increased in patients with interstitial lung diseases than normal persons with highly significant statistical differences (p = 0.001. MMP-9 was positively correlated with number of neutrophils in the airway with highly significant statistical difference (p = 0.001.

  11. Artificial neural networks in chest radiographs: detection and characterization of interstitial lung disease

    Science.gov (United States)

    Ishida, Takayuki; Katsuragawa, Shigehiko; Ashizawa, Kazuto; MacMahon, Heber; Doi, Kunio

    1997-04-01

    We have developed a computerized scheme for detection of interstitial lung disease by using artificial neural networks (ANNs) on quantitative analysis of digital image data. Three separate ANNs wee applied for the ANN scheme. The first ANN was trained with horizontal profiles in the ROIs selected from digital chest radiographs. The second ANN was trained with vertical output pattern obtained from the 1st ANN in each ROI. The output from the 2nd ANN was used to distinguish between normal and abnormal ROIs. In order to improve the performance, we attempted a density correction and rib edge removal. The Az value was improved from 0.906 to 0.934 by incorporating density correction. For the classification of each chest image, we employed a rule-based method and a rule-based plus the third ANN method. A high Az value was obtained with the rule-based plus ANN method. The ANNs can learn certain statistical properties associate with patterns of interstitial infiltrates in chest radiographs.

  12. Characterization of interstitial lung disease in chest radiographs using SOM artificial neural network

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    Azevedo-Marques, P.M. de; Ambrosio, P.E.; Pereira, R.R. Jr.; Valini, R. de A.; Salomao, S.C. [Faculty of Medicine of Ribeirao Preto, Univ. of Sao Paulo (Brazil)

    2007-06-15

    This paper presents an automated approach to apply a self-organizing map (SOM) artificial neural network (ANN) as a tool for feature extraction and dimensionality reduction to recognize and characterize radiologic patterns of interstitial lung diseases in chest radiography. After feature extraction and dimensionality reduction a multilayer perceptron (MLP) ANN is applied for radiologic patterns classification in normal, linear, nodular or mixed. A leave-one-out methodology was applied for training and test over a database containing 17 samples of linear pattern, 9 samples of nodular pattern, 9 samples of mixed pattern and 18 samples of normal pattern. The MLP network provided an average result of 88.7% of right classification, with 100% of right classification for linear pattern, 55.5% for nodular pattern, 77.7% for mixed pattern and 100% for normal pattern. (orig.)

  13. A systematic review of occupational exposure to coal dust and the risk of interstitial lung diseases

    Science.gov (United States)

    Beer, Christiane; Kolstad, Henrik A.; Søndergaard, Klaus; Bendstrup, Elisabeth; Heederik, Dick; Olsen, Karen E.; Omland, Øyvind; Petsonk, Edward; Sigsgaard, Torben; Sherson, David L.; Schlünssen, Vivi

    2017-01-01

    ABSTRACT Objective: Exposure to coal dust can cause interstitial lung disease (ILD), but whether this is due to pure coal or to the contents of quartz in coal is less clear. Here, we systematically reviewed the relation between ‘pure coal’ and ILD. Methods: In a systematic review based on PRISMA criteria 2945 articles were identified. Strict eligibility criteria, which evaluated the ‘pure coal effect’, led to the inclusion of only nine studies. Results: Among these nine studies six studies indicated an independent effect of the non-quartz part of coal on the development and progression of ILD, two did not demonstrate an effect and one was inconclusive. Conclusions: Although an independent effect of non-quartz coal dust on the development of ILD is supported, due to methodological limitations the evidence is limited and further evidence is needed.

  14. Interstitial lung disease and infection%间质性肺疾病与感染

    Institute of Scientific and Technical Information of China (English)

    李惠萍

    2008-01-01

    间质性肺疾病(interstitial lung disease,ILD)种类繁多,病因也各不相同,大部分病因未明,部分病种可能与感染有关。ILD和感染时常纠缠在一起难以严格区分。有些间质性肺疾病实为感染所致;有些常因感染而加重病情,治疗难度很大。因此,ILD患者的感染问题往往是影响其疗效的重要因素之一。

  15. Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease.

    Science.gov (United States)

    Li, Xue-Ren; Peng, Shou-Chun; Wei, Lu-Qing

    2015-01-01

    Here, we reported two cases of nonspecific interstitial pneumonia overlap organizing pneumonia (NSIP/OP) with lung-dominant connective tissue disease (LD-ILD). The first case is a patient with hands of chapped skin, right-sided pleuritic chest discomfort, weakness, positive ANA and antibodies to Ro/SS-A (+++) and Ro-52 (++). In the second case, there were Reynaud's disease, and nucleolus-ANA increased (1:800). Chest high resolution CT scan in both cases showed ground-glass opacifications, predominantly in basal and subpleural region and the pathologic manifestation were correlated with NSIP/OP, which were previously discovered in Sjogren syndrome, PM/DM and other rheumatic diseases. The two cases of NSIP/OP with LD-CTD we reported expand disease spectrum of NSIP/OP pathological types in ILD. However, it is necessary to process large-scale studies.

  16. Adalimumab-induced acute interstitial lung disease in a patient with rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Olivia Meira Dias

    2014-01-01

    Full Text Available The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs. There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD.

  17. Autoimmune pulmonary proteinosis in a Chilean teenager, a rare aetiology of interstitial lung disease.

    Science.gov (United States)

    Strickler, Alexis; Boza, Maria Lina; Koppmann, Andres; Gonzalez, Sergio

    2014-05-23

    Interstitial lung disease (ILD) is rare and encompasses a heterogeneous group of diseases, and is even rarer in children than in adults. ILDs compromise more than 100 different entities, including pulmonary alveolar proteinosis (PAP). There are many causes of PAP in children, including surfactant protein gene mutations (SFTPB, SFTPC, ABCA3, TTF-1), GMCSF receptor mutations and antigranulocyte-macrophage colony-stimulating factor autoantibodies. We report a case of a 13-year-old Chilean girl who presented with an 8-month history of progressive exercise intolerance, fatigability and diminished school performance. Physical examination revealed resting tachypnoea, a few basal bilateral inspiratory crackles, and hypoxaemia on minimal exertion. Clinical suspicion and evaluation, including international collaboration, led to the diagnosis of autoimmune PAP and specific therapy for the condition.

  18. Optimal management of interstitial lung disease associated with dermatomyositis/polymyositis: lessons from the Japanese experience

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    Kurasawa K

    2014-11-01

    Full Text Available Kazuhiro Kurasawa,1,2 Satoko Arai2 1Center of Rheumatic Diseases, Dokkyo Medical University, Mibu, Tochigi, Japan; 2Department of Clinical Immunology, Dokkyo Medical University, Mibu, Tochigi, Japan Abstract: Interstitial lung disease (ILD is a serious complication in dermatomyositis (DM and polymyositis (PM. In Japan, patients with DM/PM develop acute life-threatening ILD with high frequency. Physicians in Japan have shown the following: refractory acute/subacute (A/S-ILD is not a rare complication in DM and amyopathic DM (ADM; anti-anti-melanoma differentiation-associated gene 5 (anti-MDA5 antibody (Ab is closely related to A/S-ILD with poor outcomes in DM/ADM; and poor prognostic factors in A/S-ILD in DM/PM are ADM, DM with low creatine kinase elevation, positivity for anti-MDA5 Ab, serum ferritin elevation, and consolidation with ground-glass opacities on high-resolution computed tomography. There are subtypes in DM/PM-ILD: refractory DM/ADM A/S-ILD positive for anti-MDA5 Ab with poor prognosis; DM A/S-ILD with glucocorticoid (GC resistance; PM A/S-ILD with GC sensitivity; chronic ILD positive for anti-aminoacyl-tRNA-synthetases (anti-ARS Abs with GC responsiveness; and C-ILD negative for anti-ARS Abs. For patients with A/S-ILD with poor prognosis, initial combination therapy with cyclosporine and cyclophosphamide in addition to GC has been developed, which rescues 50%–80% of the patients, although elucidation of the efficacy of the combination therapy is required. A/S-ILD with potentially fatal outcomes is found worldwide, not only in Japan. Clinicians caring for patients with DM/PM should be cautious when dealing with A/S-ILD and treat the patients based on clinical subtypes. Keywords: interstitial lung disease, dermatomyositis, polymyositis, management, cyclosporine, cyclophosphamide

  19. Classical patterns of interstitial lung diseases; Klassische Muster der interstitiellen Lungenerkrankungen

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    Mueller-Mang, C. [Institut fuer CT und MRT, Gaenserndorf (Austria); Medizinische Universitaet Wien, Universitaetsklinik fuer Radiologie und Nuklearmedizin, Wien (Austria)

    2014-12-15

    High resolution computed tomography (HRCT) is the most important non-invasive tool in the diagnostics and follow-up of patients with interstitial lung disease (ILD). A systematic review of the HRCT patterns of ILD was carried out and the most relevant differential diagnoses are discussed in order to provide a road map for the general radiologist to successfully navigate the complex field of ILD. Using HRCT four basic patterns of ILD can be identified: linear and reticular patterns, the nodular pattern, the high attenuation and low attenuation patterns. These patterns can be further differentiated according to their localization within the secondary pulmonary lobule (SPL), e.g. centrilobular or perilymphatic and their distribution within the lungs (e.g. upper or lower lobe predominance). Relevant clinical data, such as smoking history and course of the disease provide useful additional information in the diagnosis of ILD. On the basis of the pattern and anatomical distribution on HRCT, an accurate diagnosis can be achieved in some cases of ILD; however, due to morphological and clinical overlap the final diagnosis of many ILDs requires close cooperation between clinicians, radiologists and pathologists. (orig.) [German] Die hochaufloesende CT (High-resolution[HR]-CT) ist das wichtigste nichtinvasive Verfahren zur Identifikation und Verlaufsbeurteilung von Patienten mit interstitiellen Lungenerkrankungen (''interstitial lung diseases'', ILD). Systematische Darstellung der HRCT-Muster interstitieller Lungenerkrankungen und Diskussion der relevanten Differenzialdiagnosen, um dem Radiologen eine erfolgreiche Analyse der komplexen CT-Morphologie der ILD zu ermoeglichen. Mit der HRCT koennen 4 Grundmuster interstitieller Grunderkrankungen identifiziert werden: das retikulaere und lineare Muster, das nodulaere Muster, das Muster mit erhoehter Lungendichte und das Muster mit verminderter Lungendichte. Diese Muster koennen anhand ihrer Lage im sekundaeren

  20. Pediatric Interstitial Lung Disease Masquerading as Difficult Asthma: Management Dilemmas for Rare Lung Disease in Children

    Directory of Open Access Journals (Sweden)

    EY Chan

    2005-01-01

    Full Text Available Idiopathic nontransplant-related childhood bronchiolitis obliterans is an uncommon disease. Most patients present with chronic recurrent dyspnea, cough and wheezing, which are also features of asthma, by far a much more common condition. The present case study reports on a six-year-old girl who presented to a tertiary care centre with recurrent episodes of respiratory distress on a background of baseline tachypnea, chronic hypoxemia and exertional dyspnea. Her past medical history revealed significant lung disease in infancy, including respiratory syncytial virus bronchiolitis and repaired gastroesophageal reflux. She was treated for 'asthma exacerbations' throughout her early childhood years. Bronchiolitis obliterans was subsequently diagnosed with an open lung biopsy. She did not have sustained improvement with systemic corticosteroids, hydroxychloroquine or clarithromycin. Cardiac catheterization confirmed the presence of secondary pulmonary hypertension. Treatment options remain a dilemma for this patient because there is no known effective treatment for this condition, and the natural history is not well understood. The present case demonstrates the need for careful workup in 'atypical asthma', and the urgent need for further research into the rare lung diseases of childhood.

  1. Severe acute interstitial lung disease in a patient with anaplastic lymphoma kinase rearrangement-positive non-small cell lung cancer treated with alectinib.

    Science.gov (United States)

    Yamamoto, Yuzo; Okamoto, Isamu; Otsubo, Kohei; Iwama, Eiji; Hamada, Naoki; Harada, Taishi; Takayama, Koichi; Nakanishi, Yoichi

    2015-10-01

    Alectinib, the second generation anaplastic lymphoma kinase (ALK) inhibitor, has significant potency in patients with ALK rearrangement positive non-small cell lung cancer (NSCLC), and its toxicity is generally well tolerable. We report a patient who developed severe acute interstitial lung disease after alectinib treatment. An 86-year-old woman with stage IV lung adenocarcinoma positive for rearrangement of ALK gene was treated with alectinib. On the 215th day after initiation of alectinib administration, she was admitted to our hospital with the symptom of progressive dyspnea. Computed tomography (CT) revealed diffuse ground glass opacities and consolidations in both lungs, and analysis of bronchoalveolar lavage fluid revealed pronounced lymphocytosis. There was no evidence of infection or other specific causes of her condition, and she was therefore diagnosed with interstitial lung disease induced by alectinib. Her CT findings and respiratory condition improved after steroid pulse therapy. As far as we are aware, this is the first reported case of alectinib-induced severe interstitial lung disease (ILD). We should be aware of the possibility of such a severe adverse event and should therefore carefully monitor patients treated with this drug.

  2. Enfermedad pulmonar difusa asociada al consumo de tabaco Smoking related interstitial lung disease. Case report

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    JORGE YÁNEZ V

    2008-03-01

    Full Text Available la enfermedad pulmonar difusa asociada al consumo de tabaco no ha sido claramente definida, la bronquiolitis respiratoria (RB es un hallazgo morfológico frecuente en fumadores asintomáticos, se caracteriza por la acumulación de macrófagos pigmentados en los bronquiolos respiratorios. Sólo una pequeña proporción de los sujetos fumadores presenta una respuesta inflamatoria exagerada que compromete el intersticio y espacio alveolar, lo cual corresponde a la bronquiolitis respiratoria asociada a enfermedad pulmonar difusa (RBIID, que se manifiesta por disnea de esfuerzos y tos. la neumonía intersticial descamativa (DIP se caracteriza por compromiso panlobular, fibrosis intersticial discreta e infiltración masiva del espacio aéreo por macrófagos. El patrón histopatológico de RBIID y DIP se pueden sobreponer, siendo los principales elementos diferenciadores entre ambas entidades, la distribución y extensión de las lesiones: compromiso bronquiolo-céntrico en RBIID y difuso en DIP. Se ha planteado que la RB, RBIID y DIP pueden constituir diferentes fases de una misma enfermedad asociada al consumo de tabaco, lo cual aún es motivo de controversia. Con el propósito de ilustrar este problema, se presenta el caso clínico de un paciente fumador que consultó por disnea progresiva, tos e infiltrados pulmonares bilaterales sugerentes de enfermedad pulmonar difusa asociada al tabaquismoThe relationship between cigarette smoke and interstitial lung diseases (ILD is not clear. Respiratory bronchiolitis (RB, usually found as an incidental histologic abnormality in otherwise asymptomatic smokers, is characterized by the accumulation of cytoplasmic golden-brown-pigmented macrophages within respiratory bronchioles. A small proportion of smokers have a more exaggerated response that, in addition to the bronchiole-centered lesions, provokes interstitial and air spaces inflammation and fibrosis extending to the nearby alveoli. This set of histologic

  3. Procoagulant, tissue factor-bearing microparticles in bronchoalveolar lavage of interstitial lung disease patients: an observational study.

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    Federica Novelli

    Full Text Available Coagulation factor Xa appears involved in the pathogenesis of pulmonary fibrosis. Through its interaction with protease activated receptor-1, this protease signals myofibroblast differentiation in lung fibroblasts. Although fibrogenic stimuli induce factor X synthesis by alveolar cells, the mechanisms of local posttranslational factor X activation are not fully understood. Cell-derived microparticles are submicron vesicles involved in different physiological processes, including blood coagulation; they potentially activate factor X due to the exposure on their outer membrane of both phosphatidylserine and tissue factor. We postulated a role for procoagulant microparticles in the pathogenesis of interstitial lung diseases. Nineteen patients with interstitial lung diseases and 11 controls were studied. All subjects underwent bronchoalveolar lavage; interstitial lung disease patients also underwent pulmonary function tests and high resolution CT scan. Microparticles were enumerated in the bronchoalveolar lavage fluid with a solid-phase assay based on thrombin generation. Microparticles were also tested for tissue factor activity. In vitro shedding of microparticles upon incubation with H₂O₂ was assessed in the human alveolar cell line, A549 and in normal bronchial epithelial cells. Tissue factor synthesis was quantitated by real-time PCR. Total microparticle number and microparticle-associated tissue factor activity were increased in interstitial lung disease patients compared to controls (84±8 vs. 39±3 nM phosphatidylserine; 293±37 vs. 105±21 arbitrary units of tissue factor activity; mean±SEM; p<.05 for both comparisons. Microparticle-bound tissue factor activity was inversely correlated with lung function as assessed by both diffusion capacity and forced vital capacity (r² = .27 and .31, respectively; p<.05 for both correlations. Exposure of lung epithelial cells to H₂O₂ caused an increase in microparticle-bound tissue factor

  4. Role of Oxidants in Interstitial Lung Diseases: Pneumoconioses, Constrictive Bronchiolitis, and Chronic Tropical Pulmonary Eosinophilia

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    William N. Rom

    2011-01-01

    Full Text Available Oxidants such as superoxide anion, hydrogen peroxide, and myeloperoxidase from activated inflammatory cells in the lower respiratory tract contribute to inflammation and injury. Etiologic agents include inorganic particulates such as asbestos, silica, or coal mine dust or mixtures of inorganic dust and combustion materials found in World Trade Center dust and smoke. These etiologic agents are phagocytosed by alveolar macrophages or bronchial epithelial cells and release chemotactic factors that recruit inflammatory cells to the lung. Chemotactic factors attract and activate neutrophils, eosinophils, mast cells, and lymphocytes and further activate macrophages to release more oxidants. Inorganic dusts target alveolar macrophages, World Trade Center dust targets bronchial epithelial cells, and eosinophils characterize tropical pulmonary eosinophilia (TPE caused by filarial organisms. The technique of bronchoalveolar lavage in humans has recovered alveolar macrophages (AMs in dust diseases and eosinophils in TPE that release increased amounts of oxidants in vitro. Interestingly, TPE has massively increased eosinophils in the acute form and after treatment can still have ongoing eosinophilic inflammation. A course of prednisone for one week can reduce the oxidant burden and attendant inflammation and may be a strategy to prevent chronic TPE and interstitial lung disease.

  5. Computer-aided diagnosis of interstitial lung disease: a texture feature extraction and classification approach

    Science.gov (United States)

    Vargas-Voracek, Rene; McAdams, H. Page; Floyd, Carey E., Jr.

    1998-06-01

    An approach for the classification of normal or abnormal lung parenchyma from selected regions of interest (ROIs) of chest radiographs is presented for computer aided diagnosis of interstitial lung disease (ILD). The proposed approach uses a feed-forward neural network to classify each ROI based on a set of isotropic texture measures obtained from the joint grey level distribution of pairs of pixels separated by a specific distance. Two hundred ROIs, each 64 X 64 pixels in size (11 X 11 mm), were extracted from digitized chest radiographs for testing. Diagnosis performance was evaluated with the leave-one-out method. Classification of independent ROIs achieved a sensitivity of 90% and a specificity of 84% with an area under the receiver operating characteristic curve of 0.85. The diagnosis for each patient was correct for all cases when a `majority vote' criterion for the classification of the corresponding ROIs was applied to issue a normal or ILD patient classification. The proposed approach is a simple, fast, and consistent method for computer aided diagnosis of ILD with a very good performance. Further research will include additional cases, including differential diagnosis among ILD manifestations.

  6. Modeling using clinical examination indicators predicts interstitial lung disease among patients with rheumatoid arthritis

    Science.gov (United States)

    Wang, Yao; Song, Wuqi; Wu, Jing; Li, Zhangming; Mu, Fengyun; Li, Yang; Huang, He; Zhu, Wenliang

    2017-01-01

    Interstitial lung disease (ILD) is a severe extra-articular manifestation of rheumatoid arthritis (RA) that is well-defined as a chronic systemic autoimmune disease. A proportion of patients with RA-associated ILD (RA-ILD) develop pulmonary fibrosis (PF), resulting in poor prognosis and increased lifetime risk. We investigated whether routine clinical examination indicators (CEIs) could be used to identify RA patients with high PF risk. A total of 533 patients with established RA were recruited in this study for model building and 32 CEIs were measured for each of them. To identify PF risk, a new artificial neural network (ANN) was built, in which inputs were generated by calculating Euclidean distance of CEIs between patients. Receiver operating characteristic curve analysis indicated that the ANN performed well in predicting the PF risk (Youden index = 0.436) by only incorporating four CEIs including age, eosinophil count, platelet count, and white blood cell count. A set of 218 RA patients with healthy lungs or suffering from ILD and a set of 87 RA patients suffering from PF were used for independent validation. Results showed that the model successfully identified ILD and PF with a true positive rate of 84.9% and 82.8%, respectively. The present study suggests that model integration of multiple routine CEIs contributes to identification of potential PF risk among patients with RA.

  7. Content-based image retrieval for interstitial lung diseases using classification confidence

    Science.gov (United States)

    Dash, Jatindra Kumar; Mukhopadhyay, Sudipta; Prabhakar, Nidhi; Garg, Mandeep; Khandelwal, Niranjan

    2013-02-01

    Content Based Image Retrieval (CBIR) system could exploit the wealth of High-Resolution Computed Tomography (HRCT) data stored in the archive by finding similar images to assist radiologists for self learning and differential diagnosis of Interstitial Lung Diseases (ILDs). HRCT findings of ILDs are classified into several categories (e.g. consolidation, emphysema, ground glass, nodular etc.) based on their texture like appearances. Therefore, analysis of ILDs is considered as a texture analysis problem. Many approaches have been proposed for CBIR of lung images using texture as primitive visual content. This paper presents a new approach to CBIR for ILDs. The proposed approach makes use of a trained neural network (NN) to find the output class label of query image. The degree of confidence of the NN classifier is analyzed using Naive Bayes classifier that dynamically takes a decision on the size of the search space to be used for retrieval. The proposed approach is compared with three simple distance based and one classifier based texture retrieval approaches. Experimental results show that the proposed technique achieved highest average percentage precision of 92.60% with lowest standard deviation of 20.82%.

  8. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis.

    Science.gov (United States)

    Xu, Y; Yang, C S; Li, Y J; Liu, X D; Wang, J N; Zhao, Q; Xiao, W G; Yang, P T

    2016-01-01

    Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD. We found that skin ulceration, CRP, serum ferritin, anti-MDA5 Ab, and lymphocytopenia were significantly associated with ILD. Multivariate logistic regression analysis indicated that anti-MDA5 Ab(+), elevated CRP, and decreased counts of lymphocyte were independent risk factors for RP-ILD, which can provide a precise predict for RP-ILD in CADM patients. When anti-MDA5 Ab(+) was removed from the multivariate regression model, using skin ulcerations, elevated serum ferritin and decreased counts of lymphocyte can also precisely predict RP-ILD. Except for MDA-5, more commonly available clinical characteristics, such as skin ulcerations, serum ferritin, and count of lymphocyte may also help to predict prognosis in CADM.

  9. Peripheral-type benzodiazepine receptors in bronchoalveolar lavage cells of patients with interstitial lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Branley, Howard M. [Imperial College London, Hammersmith Campus, W12 OHS London (United Kingdom)]. E-mail: howard.branley@whittington.nhs.uk; Bois, Roland M. du [Royal Brompton Hospital, SW3 6NP London (United Kingdom); Wells, Athol U. [Royal Brompton Hospital, SW3 6NP London (United Kingdom); Jones, Hazel A. [Imperial College London, Hammersmith Campus, W12 OHS London (United Kingdom)

    2007-07-15

    Introduction: PK11195 is a ligand with high affinity for peripheral benzodiazepine receptors (PBRs), which are present in large numbers in macrophages. PBRs play a role in antioxidant pathways and apoptosis, key factors in control of lung health. Intrapulmonary PBRs, assessed in vivo by positron emission tomography (PET), are decreased in interstitial lung disease (ILD) despite increased macrophage numbers. We wished to ascertain whether the observed decrease in in vivo expression of PBRs in the PET scans could be accounted for by a reduction in PBRs per cell by saturation-binding assays of R-PK11195 in cells obtained by bronchoalveolar lavage (BAL). Methods: We performed receptor saturation-binding assays with [{sup 3}H]-R-PK11195 on a mixed population of cells recovered by BAL to quantify the number of R-PK11195 binding sites per macrophage in 10 subjects with ILD and 10 normal subjects. Results: Receptor affinity [dissociation constant (Kd)] was similar in ILD patients and controls. However, R-PK11195 binding sites per cell [(maximal binding sites available (B {sub max})] were decreased in macrophages obtained by BAL from subjects with ILD compared to normal (P<.0005). Microautoradiography confirmed localization of R-PK11195 to macrophages in a mixed inflammatory cell population obtained by BAL. Conclusion: These results demonstrate that in vitro PBR expression per cell on macrophages obtained by BAL is reduced in patients with ILD indicating a potentially functionally different macrophage phenotype. As PBRs are involved in the orchestration of lung inflammatory responses, this finding offers further insight into the role of macrophages in the pathogenesis of ILDs and offers a potential avenue for pharmacological strategy.

  10. Identifying decreased diaphragmatic mobility and diaphragm thickening in interstitial lung disease: the utility of ultrasound imaging

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    Pauliane Vieira Santana

    2016-04-01

    Full Text Available Objective: To investigate the applicability of ultrasound imaging of the diaphragm in interstitial lung disease (ILD. Methods: Using ultrasound, we compared ILD patients and healthy volunteers (controls in terms of diaphragmatic mobility during quiet and deep breathing; diaphragm thickness at functional residual capacity (FRC and at total lung capacity (TLC; and the thickening fraction (TF, proportional diaphragm thickening from FRC to TLC. We also evaluated correlations between diaphragmatic dysfunction and lung function variables. Results: Between the ILD patients (n = 40 and the controls (n = 16, mean diaphragmatic mobility was comparable during quiet breathing, although it was significantly lower in the patients during deep breathing (4.5 ± 1.7 cm vs. 7.6 ± 1.4 cm; p < 0.01. The patients showed greater diaphragm thickness at FRC (p = 0.05, although, due to lower diaphragm thickness at TLC, they also showed a lower TF (p < 0.01. The FVC as a percentage of the predicted value (FVC% correlated with diaphragmatic mobility (r = 0.73; p < 0.01, and an FVC% cut-off value of < 60% presented high sensitivity (92% and specificity (81% for indentifying decreased diaphragmatic mobility. Conclusions: Using ultrasound, we were able to show that diaphragmatic mobility and the TF were lower in ILD patients than in healthy controls, despite the greater diaphragm thickness at FRC in the former. Diaphragmatic mobility correlated with ILD functional severity, and an FVC% cut-off value of < 60% was found to be highly accurate for indentifying diaphragmatic dysfunction on ultrasound.

  11. COMPARATIVE CLINICAL, LABORATORY, AND INSTRUMENTAL EVALUATION OF INTERSTITIAL LUNG CHANGES IN RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    D. V. Bestaev

    2014-01-01

    Full Text Available Interstitial lung disease (ILD is a common extra-articular manifestation of rheumatoid arthritis (RA. Objective: to study the associations of the data of high-resolution computed tomography (HRCT and the esti- mate of diffusing lung capacity (DLC with clinical and laboratory parameters in RA patients with and without ILD. Subjects and methods. 79 RA patients fulfilling the 1987 American College of Rheumatology criteria (61 women and 18 men admitted to the Nasonova Research Institute of Rheumatology were included. Results. HRCT revealed signs of ILD in 58 (73% cases. The patients with ILD were divided into three groups: 1 18 (31% patients with ground glass opacities; 2 34 (58.6% patients with fibrosis; 3 6 (10.4% patients with the honeycomb lung. Twenty-one (27% patients with ILD were included in Group 4. In the ILD patients with ground glass opacities, the levels of anti-cyclic citrullinated peptide (ACCP antibodies and rheumatoid factor (RF were much above those in the patients without ILD (240 [166; 410.5], 480 [140; 850.5] and 73 [31; 101], 330,5 [118.5; 604.8], respectively. In the patients with ILD, the concentration of C-reactive protein (CRP (46 [35; 91] was higher than that in those without ILD (24 [18; 31]; p < 0.05. In the ILD patients with ground glass opacities, DLC was considerably below that in those with ILD – 59.2±11.2 and 79.8±12.1% of the normal value, respectively (p < 0.001. Conclusion. The associations found between ACCP antibodies and DLC, DAS28 and DLC may suggest that ACCP antibodies are implicated in the pathogenesis of ILD and the lung is involved in the immunoinflammatory process. The high percent of smokers detected in our investigation confirms the considerable role of smoking in the pathogene- sis of RA-associated ILD. In the RA patients with ILD, ground glass opacities must be an indicator of the activity of an immunopathological process in the lung.

  12. Interstitial lung disease in anti-synthetase syndrome: Initial and follow-up CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Debray, Marie-Pierre, E-mail: marie-pierre.debray@bch.aphp.fr [AP-HP, Bichat-Claude Bernard Hospital, Department of Radiology, 46, rue Henri Huchard, 75877 Paris Cedex 18 (France); Borie, Raphael, E-mail: raphael.borie@bch.aphp.fr [AP-HP, Bichat-Claude Bernard Hospital, Department of Pneumology A and Centre de Compétence Maladies Pulmonaires rares, DHU Fire 46, rue Henri Huchard, 75877 Paris Cedex 18 (France); Inserm, U1152, Paris (France); Revel, Marie-Pierre, E-mail: marie-pierre.revel@htd.aphp.fr [AP-HP, Cochin Hospital, Department of Radiology, 27, Rue du Fg Saint Jacques, 75679 Paris Cedex 14 (France); Naccache, Jean-Marc, E-mail: jean-marc.naccache@tnn.aphp.fr [AP-HP, Avicenne Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, Bobigny (France); AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris (France); Khalil, Antoine, E-mail: antoine.khalil@tnn.aphp.fr [AP-HP, Tenon Hospital, Department of Radiology, 4, rue de la Chine, 75020 Paris (France); Toper, Cécile, E-mail: cecile.toper@gmail.com [AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris (France); Israel-Biet, Dominique, E-mail: dominique.israel-biet@egp.aphp.fr [Université Paris Descartes and AP-HP, Department of Pneumology, Georges Pompidou European Hospital, 20, rue Leblanc, 75015 Paris (France); and others

    2015-03-15

    Purpose: To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). Materials and methods: Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13–167 months) in 26 patients. Results: At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). Conclusion: CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients.

  13. Initial experience with lung-MRI at 3.0 T: Comparison with CT and clinical data in the evaluation of interstitial lung disease activity

    Energy Technology Data Exchange (ETDEWEB)

    Lutterbey, G. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany)]. E-mail: goetz.lutterbey@ukb.uni-bonn.de; Grohe, C. [Department of Internal Medicine, University of Bonn (Germany); Gieseke, J. [PHILIPS Medical Systems, Best (Netherlands); Falkenhausen, M. von [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Morakkabati, N. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Wattjes, M.P. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Manka, R. [Department of Internal Medicine, University of Bonn (Germany); Trog, D. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Schild, H.H. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany)

    2007-02-15

    Objectives: We evaluated the feasibility of highfield lung-MRI at 3.0 T. A comparison with Computed Tomography (CT) and clinical data regarding the assessment of inflammatory activity in patients with diffuse lung disease was performed. Material and methods: Prospective evaluation of 21 patients (15 males, 6 females, 43-80 y) with diffuse lung diseases who underwent clinical work-up inclusive laboratory tests, lung-function tests and transbronchial biopsy. After routine helical CT (additional 12 HRCT) a lung-MRI (3.0 Intera, Philips Medical Systems, Best, The Netherlands) using a T2-weighted, cardiac and respiratory triggered Fast-Spinecho-Sequence (TE/TR = 80/1500-2500 ms, 22 transverse slices, 7/2 mm slice-thickness/-gap) was performed. A pneumologist classified the cases into two groups: A = temporary acute interstitial disease or chronic interstitial lung disease with acute episode or superimposed infection/B = burned out interstitial lung disease without activity. Two blinded CT-radiologists graded the cases in active/inactive disease on the basis of nine morphological criteria each. A third radiologist rated the MRI-cases as active/inactive, depending on the signal-intensities of lung tissues. Results: The pneumologist classified 14 patients into group A and 7 patients into group B. Using CT, 6 cases were classified as active, 15 cases as inactive disease. With MRI 12 cases were classified as active and 9 cases as inactive. In the complete group of 21 patients MRI decisions and CT decisions respectively were false positive/false negative/correct in 2/4/15 respectively 0/8/13 cases. Correct diagnoses were obtained in 72% (MRI) respectively 62% (CT). In the subgroup of 12 cases including HRCT, MRI respectively CT were false positive/false negative/correct in 2/1/9 respectively 0/5/7 cases. Correct diagnoses were obtained in 75% (MRI) respectively 58% (CT). Conclusion: Highfield MRI of the lung is feasible and performed slightly better compared to CT in the

  14. Relationship between the level of interleukin-9 expression in serum of connective tissue disease patients with interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    欧阳涵

    2013-01-01

    Objective To investigate the level of interleukin (IL) -9 in patients with connective tissue disease (CTD) and connective tissue disease with interstitial lung disease (CTD-ILD) ,and explore the role of IL-9 in the pathogenesis of CTD and CTD-ILD.Methods Sixty-one hospitalized untreated CTD patients were recruited and 20healthy volunteers were enrolled as healthy controls.Patients in the CTD group included 19 systemic sclerosis (SSc) patients,15 rheumatoid arthritis (RA) patients,

  15. The importance of determining surgical indications in cases of lung cancer and interstitial pneumonia with multiple intrapulmonary lymph nodes

    Directory of Open Access Journals (Sweden)

    Yuki Nakajima

    2016-01-01

    Discussion and conclusions: The greatest problem in treating pulmonary cancer complicated with interstitial pneumonia is acute exacerbation, wherein, in the absence of any surgical indications, alternative treatment is limited. Thus, contra-indicating surgery for a patient due to a diagnosis of metastasis within the lungs, based only on nodule images, should be avoided. If nodules are noted in the area of the pleura, the possibility exists that these could be intrapulmonary lymph nodes, along with metastasis within the lung, and thoracoscopic surgery should be implemented proactively while keeping these in mind.

  16. Texture feature selection with relevance learning to classify interstitial lung disease patterns

    Science.gov (United States)

    Huber, Markus B.; Bunte, Kerstin; Nagarajan, Mahesh B.; Biehl, Michael; Ray, Lawrence A.; Wismueller, Axel

    2011-03-01

    The Generalized Matrix Learning Vector Quantization (GMLVQ) is used to estimate the relevance of texture features in their ability to classify interstitial lung disease patterns in high-resolution computed tomography (HRCT) images. After a stochastic gradient descent, the GMLVQ algorithm provides a discriminative distance measure of relevance factors, which can account for pairwise correlations between different texture features and their importance for the classification of healthy and diseased patterns. Texture features were extracted from gray-level co-occurrence matrices (GLCMs), and were ranked and selected according to their relevance obtained by GMLVQ and, for comparison, to a mutual information (MI) criteria. A k-nearest-neighbor (kNN) classifier and a Support Vector Machine with a radial basis function kernel (SVMrbf) were optimized in a 10-fold crossvalidation for different texture feature sets. In our experiment with real-world data, the feature sets selected by the GMLVQ approach had a significantly better classification performance compared with feature sets selected by a MI ranking.

  17. Therapeutic plasma exchange in antisynthetase syndrome with severe interstitial lung disease.

    Science.gov (United States)

    Omotoso, Bolanle A; Ogden, Melissa I; Balogun, Rasheed A

    2015-12-01

    Antisynthetase syndrome (ASS) is a rare condition characterized by interstitial lung disease (ILD), inflammatory myositis, fever, Raynaud phenomenon, mechanic's hand, and inflammatory polyarthritis in the setting of antibodies to amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. Prognosis is very poor especially when there is associated ILD. To date, there is no standardized treatment for ILD associated ASS. Therapy is based on the use of steroids alone or in combination with other immunosuppressive agents, especially in severe or refractory cases. The role of therapeutic plasma exchange (TPE) in the management of this rare condition has not been established. Here, we report a case of severe ILD associated ASS in a 41-year-old woman who did not show clinical or laboratory response after six doses of high dose steroids and a dose of IV cyclophosphamide. Because of the aggressive nature of her disease and poor prognostic indices present, a decision was made to add TPE to her treatment. She underwent five sessions of TPE. At the end of the 5th session, the anti-Jo-1 antibody levels dropped to 3.6 AI (antibody index) and her creatinine kinase (CK) level from 875 to 399 U L(-1) (Units per liter) with overall improvement in her respiratory status. This case suggests TPE may be a promising treatment option in patients with ILD associated ASS refractory to steroids and other immunosuppressive therapy, particularly those with severe disease.

  18. [Efficacy and safety of the use of levodropropizine in patients with chronic interstitial lung diseases].

    Science.gov (United States)

    Gunella, G; Zanasi, A; Massimo Vanasia, C B

    1991-02-28

    Efficacy and tolerability of an antitussive drug, levodropropizine, in 21 adult patients with interstitial lung disorders was evaluated in this study. Levodropropizine dosage was 60 mg t.i.d. for four days of therapy. The authors evaluated the effectiveness of the antitussive therapy and, on the other hand, the tolerability of levodropropizine in these patients, monitoring PaO2, PaCO2 and pH values. The changes in cough frequency (34.1 +/- 5.6 20.4 +/- 5.4 mean +/- d.s. before and after treatment) and the overall efficacy judgment as reported by the doctor indicate a decrease in cough as confirmed by the significance of Kruskal-Wallis test (p less than 0.05). Furthermore the tolerability has been excellent, because the PaO2, PaCo2 ad pH values before and after treatment were unchanged (PaO2 mmHg: basal 71 +/- 16.6, after treatment 73.8 +/- 14.2; PaCO2 mmHg: basal 36.6 +/- 5, after treatment 36.6 +/- 4.6; pH basal 7.4 +/- 0.03, after treatment 7.4 +/- 0.02).

  19. Interstitial lung disease associated with trastuzumab monotherapy: A report of 3 cases

    Science.gov (United States)

    Sugaya, Akinori; Ishiguro, Shingo; Mitsuhashi, Shoichi; Abe, Masahiro; Hashimoto, Ikuta; Kaburagi, Takayuki; Kojima, Hiroshi

    2017-01-01

    We herein report 3 cases of female patients with breast cancer who developed interstitial lung disease (ILD) during trastuzumab monotherapy in an adjuvant setting. Prior chemotherapy included 4 cycles of epirubicin and cyclophosphamide in patients 1 and 2, and 4 cycles of docetaxel, cyclophosphamide and trastuzumab in patient 3. Patient 1 presented with a cough and fever after the fourth cycle of trastuzumab. Patient 2 experienced rapid deterioration of oxygen saturation without subjective symptoms within 3 h of the first administration of trastuzumab. Patient 3 was unexpectedly diagnosed with organizing pneumonia in a scheduled computed tomography (CT) scan after the first course of trastuzumab. Based on clinical data, such as decreased PaO2 level, increased serum levels of KL-6 and/or lactate dehydrogenase, and findings on chest CT, these patients were diagnosed with drug-induced ILD. Considering the clinical course, trastuzumab was incriminated as the cause of ILD, particularly in patients 1 and 2. All 3 patients improved due to the timely diagnosis, discontinuation of trastuzumab and immediate administration of corticosteroid therapy. Although ILD is a rare adverse event associated with trastuzumab, it may cause rapid deterioration without preceding symptoms. Close observation and early diagnosis are required to avoid an unfavorable outcome.

  20. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    Science.gov (United States)

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-09-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862) while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

  1. Acute respiratory failure in critically ill patients with interstitial lung disease.

    Directory of Open Access Journals (Sweden)

    Lara Zafrani

    Full Text Available Patients with chronic known or unknown interstitial lung disease (ILD may present with severe respiratory flares that require intensive management. Outcome data in these patients are scarce.Clinical and radiological features were collected in 83 patients with ILD-associated acute respiratory failure (ARF. Determinants of hospital mortality and response to corticosteroid therapy were identified by logistic regression.Hospital and 1-year mortality rates were 41% and 54% respectively. Pulmonary hypertension, computed tomography (CT fibrosis and acute kidney injury were independently associated with mortality (odds ratio (OR 4.55; 95% confidence interval (95%CI (1.20-17.33; OR, 7.68; (1.78-33.22 and OR 10.60; (2.25-49.97 respectively. Response to steroids was higher in patients with shorter time from hospital admission to corticosteroid therapy. Patients with fibrosis on CT had lower response to steroids (OR, 0.03; (0.005-0.21. In mechanically ventilated patients, overdistension induced by high PEEP settings was associated with CT fibrosis and hospital mortality.Mortality is high in ILD-associated ARF. CT and echocardiography are valuable prognostic tools. Prompt corticosteroid therapy may improve survival.

  2. Correlation of spicule sign on computed tomography scans with peripheral lung cancers associated with interstitial lung disease and chronic obstructive pulmonary disease.

    Science.gov (United States)

    Chen, L; Gao, L; Wu, W L

    2015-03-27

    The objective of this study was to investigate the correlation between spicular signs on computed tomography (CT) scans and peripheral lung cancer (PLC) that is associated with interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD). We analyzed clinical data from 96 PLC cases and grouped patients based on whether they had interstitial pneumonia into either ILD/COPD group or non-ILD/COPD group. The occurrence rate of spicule sign was 90.3% in the ILD/COPD group and 61.8% in the non-ILD/COPD group, respectively. There was a significant difference between these groups (P < 0.05). There were no significant differences in the occurrence rate of spicular signs among patients with different pathological types of PLC. The severity of ILD affected the spicular morphology on CT scans directly. There was a significant correlation between the appearance of spicule sign on CT scans and PLC that was associated with ILD/COPD.

  3. A national internet-linked based database for pediatric interstitial lung diseases: the French network

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    Nathan Nadia

    2012-06-01

    Full Text Available Abstract Background Interstitial lung diseases (ILDs in children represent a heterogeneous group of rare respiratory disorders that affect the lung parenchyma. After the launch of the French Reference Centre for Rare Lung Diseases (RespiRare®, we created a national network and a web-linked database to collect data on pediatric ILD. Methods Since 2008, the database has been set up in all RespiRare® centres. After patient's parents' oral consent is obtained, physicians enter the data of children with ILD: identity, social data and environmental data; specific aetiological diagnosis of the ILD if known, genetics, patient visits to the centre, and all medical examinations and tests done for the diagnosis and/or during follow up. Each participating centre has a free access to his own patients' data only, and cross-centre studies require mutual agreement. Physicians may use the system as a daily aid for patient care through a web-linked medical file, backed on this database. Results Data was collected for 205 cases of ILD. The M/F sex ratio was 0.9. Median age at diagnosis was 1.5 years old [0–16.9]. A specific aetiology was identified in 149 (72.7% patients while 56 (27.3% cases remain undiagnosed. Surfactant deficiencies and alveolar proteinosis, haemosiderosis, and sarcoidosis represent almost half of the diagnoses. Median length of follow-up is 2.9 years [0–17.2]. Conclusions We introduce here the French network and the largest national database in pediatric ILDs. The diagnosis spectrum and the estimated incidence are consistent with other European databases. An important challenge will be to reduce the proportion of unclassified ILDs by a standardized diagnosis work-up. This database is a great opportunity to improve patient care and disease pathogenesis knowledge. A European network including physicians and European foundations is now emerging with the initial aim of devising a simplified European database/register as a first step to

  4. 间质性肺疾病治疗研究进展%Progress in treatment of interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    刘萍; 秦兴国

    2015-01-01

    Interstitial lung disease is a group of diseases which mainly affects the lung,alveolar and alveolar tissue.It is characterized by breathing difficulties after activity,double lung diffuse shadow in chest X-ray,restrictive ventilation barrier,decreased diffusion function and hypoxia.The incidence and mortality rates are increased year by year.It is caused by many reasons,and harmful to human health.At present,the exact pathogenesis of interstitial lung disease is not clear,and the ideal treatment method is relatively limited.In this paper,a hundred articles published in English and Chinese are analyzed in order to provide reference for clinical work and to develop new research ideas.%间质性肺疾病(interstitial lung disease,ILD)是一组主要累及肺间质、肺泡和肺泡周围组织的疾病,是以活动后呼吸困难、胸部X线可见双肺弥漫性阴影、限制性通气障碍、弥散功能降低和低氧血症为临床特征的异质性疾病群构成的临床-病理实体的总称.该病由多种原因引起,发病率及病死率呈逐年增高趋势,是危害人类健康的重要疾病.目前,ILD确切的发病机制尚未明确,且理想的治疗方法较为有限.本文针对近年来发表的百余篇中英文文献进行整理分析,旨在为临床工作提供参考,开拓新的研究思路.

  5. Percutaneous evacuation of diffuse pulmonary interstitial emphysema by lung puncture in a baby with extremely low birth weight: a case report

    Directory of Open Access Journals (Sweden)

    Watanabe Masahiro

    2012-09-01

    Full Text Available Abstract Introduction Pulmonary interstitial emphysema is a serious complication of mechanical ventilation and can become life-threatening if progression occurs. Therapeutic lung puncture is a treatment option for severe pulmonary interstitial emphysema but has a limited use in babies with extremely low birth weight. We present a case of pulmonary interstitial emphysema in a Japanese baby (1-day-old boy with extremely low birth weight. The emphysema was successfully decompressed by therapeutic lung puncture performed with a trocar catheter. Case presentation The baby was born with a weight of 420g, which, to the best of our knowledge, is the lowest reported birth weight among babies with pulmonary interstitial emphysema. A chest X-ray on postnatal day 2 revealed pulmonary interstitial emphysema, which gradually progressed to diffuse pseudocystic changes. His condition became life-threatening despite the use of high-frequency oscillatory ventilation and lateral decubitus positioning. We evacuated the pulmonary interstitial emphysema by lung puncture with a trocar catheter to avoid respiratory and cardiovascular collapse. This resulted in adequate evacuation of the emphysema and a dramatic improvement in his clinical condition. Conclusions Therapeutic lung puncture performed with a trocar catheter is beneficial in babies with extremely low birth weight and diffuse pulmonary interstitial emphysema. This treatment option may be broadly applicable, especially in an emergency situation.

  6. A Clinical Analysis of Risk Factors for Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myopathy

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    Xiaomin Cen

    2013-01-01

    Full Text Available Interstitial lung disease (ILD is a common and severe complication of idiopathic inflammatory myopathies (IIM. The aim of our study was to identify risk factors for ILD by evaluating both clinical and biochemical features in IIM patients with or without ILD. From January 2008 to December 2011, medical records of 134 IIM patients in our rheumatology unit were reviewed. The patients were divided into ILD group (83 patients and non-ILD group (51 patients. The clinical features and laboratory findings were compared. The univariable analyses indicated that arthritis/arthralgia (54.2% versus 17.6%, P<0.05, Mechanic’s hand (16.9% versus 2.0%, P<0.05, Raynaud’s phenomenon (36.1% versus 2.0%, P<0.05, heliotrope rash (44.6% versus 19.6%, P<0.05, fever (43.4% versus 21.6%, P<0.05, elevated ESR (60.2% versus 35.3%, P<0.05, elevated CRP (55.4% versus 31.4%, P<0.05, or anti-Jo-1 antibody (20.5% versus 5.9%, P<0.05 were risk factors for developing ILD in IIM. Multivariable unconditional logistic regression analysis that showed arthritis/arthralgia (OR 7.1, 95% CI 2.8–18.1, Raynaud’s phenomenon (OR 29.1, 95% CI 3.6–233.7, and amyopathic dermatomyositis (ADM (OR 20.2, 95% CI 2.4–171.2 were the independent risk factors for developing ILD in IIM.

  7. Usefulness of the second heart sound for predicting pulmonary hypertension in patients with interstitial lung disease

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    Sandra de Barros Cobra

    Full Text Available CONTEXT AND OBJECTIVE: P2 hyperphonesis is considered to be a valuable finding in semiological diagnoses of pulmonary hypertension (PH. The aim here was to evaluate the accuracy of the pulmonary component of second heart sounds for predicting PH in patients with interstitial lung disease. DESIGN AND SETTING: Cross-sectional study at the University of Brasilia and Hospital de Base do Distrito Federal. METHODS: Heart sounds were acquired using an electronic stethoscope and were analyzed using phonocardiography. Clinical signs suggestive of PH, such as second heart sound (S2 in pulmonary area louder than in aortic area; P2 > A2 in pulmonary area and P2 present in mitral area, were compared with Doppler echocardiographic parameters suggestive of PH. Sensitivity (S, specificity (Sp and positive (LR+ and negative (LR- likelihood ratios were evaluated. RESULTS: There was no significant correlation between S2 or P2 amplitude and PASP (pulmonary artery systolic pressure (P = 0.185 and 0.115; P= 0.13 and 0.34, respectively. Higher S2 in pulmonary area than in aortic area, compared with all the criteria suggestive of PH, showed S = 60%, Sp= 22%; LR+ = 0.7; LR- = 1.7; while P2> A2 showed S= 57%, Sp = 39%; LR+ = 0.9; LR- = 1.1; and P2 in mitral area showed: S= 68%, Sp = 41%; LR+ = 1.1; LR- = 0.7. All these signals together showed: S= 50%, Sp = 56%. CONCLUSIONS: The semiological signs indicative of PH presented low sensitivity and specificity levels for clinically diagnosing this comorbidity.

  8. Observer agreement in the diagnosis of interstitial lung diseases based on HRCT scans

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    Antunes, Viviane Baptista; Meirelles, Gustavo de Souza Portes; Jasinowodolinski, Dany; Verrastro, Carlos Gustavo Yuji; Torlai, Fabiola Goda, E-mail: antunes.viviane@gmail.co [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. of Diagnostic Imaging; Pereira, Carlos Alberto de Castro [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. of Internal Medicine; D' Ippolito, Giuseppe [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil)

    2010-01-15

    Objective: to determine the interobserver and intraobserver agreement in the diagnosis of interstitial lung diseases (ILDs) based on HRCT scans and the impact of observer expertise, clinical data and confidence level on such agreement. Methods: two thoracic radiologists and two general radiologists independently reviewed the HRCT images of 58 patients with ILDs on two distinct occasions: prior to and after the clinical anamnesis. The radiologists selected up to three diagnostic hypotheses for each patient and defined the confidence level for these hypotheses. One of the thoracic and one of the general radiologists re-evaluated the same images up to three months after the first readings. In the coefficient analyses, the kappa statistic was used. Results: the thoracic and general radiologists, respectively, agreed on at least one diagnosis for each patient in 91.4% and 82.8% of the patients. The thoracic radiologists agreed on the most likely diagnosis in 48.3% (kappa = 0.42) and 62.1% (kappa = 0.58) of the cases, respectively, prior to and after the clinical anamnesis; likewise, the general radiologists agreed on the most likely diagnosis in 37.9% (kappa 0.32) and 36.2% (kappa = 0.30) of the cases. For the thoracic radiologist, the intraobserver agreement on the most likely diagnosis was 0.73 and 0.63 prior to and after the clinical anamnesis, respectively. That for the general radiologist was 0.38 and 0.42.The thoracic radiologists presented almost perfect agreement for the diagnostic hypotheses defined with the high confidence level. Conclusions: Interobserver and intraobserver agreement in the diagnosis of ILDs based on HRCT scans ranged from fair to almost perfect and was influenced by radiologist expertise, clinical history and confidence level. (author)

  9. Survival benefit associated with early cyclosporine treatment for dermatomyositis-associated interstitial lung disease.

    Science.gov (United States)

    Go, Dong Jin; Park, Jin Kyun; Kang, Eun Ha; Kwon, Hyun Mi; Lee, Yun Jong; Song, Yeong Wook; Lee, Eun Bong

    2016-01-01

    Interstitial lung disease (ILD) is the most common cause of death in dermatomyositis (DM). Cyclosporine A (CsA) has shown to be effective in DM-associated ILD (DM-ILD). This study aimed to define the optimal time of CsA administration. A total of 47 patients with DM-ILD, who were treated with CsA at Seoul National University Hospital between January 1998 and June 2013, were enrolled. ILD was diagnosed based on typical chest high-resolution computed tomography (HRCT) findings. Patients with early and delayed CsA treatment were compared in regard to the mortality and ILD progression on HRCT. The early (n = 16) and the delayed treatment group (n = 31) did not differ in regard to baseline clinical characteristics including HRCT scores and pulmonary function. Patients with clinically amyopathic DM (CADM) were more common in the early treatment group. The mortality rate was significantly lower in the early treatment group than in the delayed treatment group (p = 0.009). The survival benefit of early CsA treatment remained significant even after adjusting for age, degree of dyspnea, CADM status, and the year of CsA treatment (hazard ratio 0.057, 95 % confidence interval 0.007-0.472). CsA stabilized disease progression on HRCT in the early treatment group (p = 0.738). Delay in CsA treatment is associated with a worse survival in patients with DM-ILD. Early CsA treatment should be considered at DM-ILD diagnosis especially in patients at a higher risk of developing a rapidly progressive ILD.

  10. Computer-assisted quantification of interstitial lung disease associated with rheumatoid arthritis: Preliminary technical validation

    Energy Technology Data Exchange (ETDEWEB)

    Marten, K. [Department of Radiology, Georg August University of Goettingen, Robert-Koch-Strasse 40, 37075 Goettingen (Germany); Dicken, V. [MeVis Research GmbH, Universitaetsallee 29, 28359 Bremen (Germany); Kneitz, C. [Department of Rheumatology and Clinical Immunology, Medizinische Klinik and Poliklinik, University Hospital of Wuerzburg, Klinikstrasse 6, 97070 Wuerzburg (Germany); Hoehmann, M.; Kenn, W.; Hahn, D. [Department of Radiology, University Hospital of Wuerzburg, Josef-Schneider-Strasse 2, 97080 Wuerzburg (Germany); Engelke, C. [Department of Radiology, Georg August University of Goettingen, Robert-Koch-Strasse 40, 37075 Goettingen (Germany)], E-mail: c.engelke@med.uni-goettingen.de

    2009-11-15

    Purpose: To validate a threshold-based prototype software application (MeVis PULMO 3D) for quantification of chronic interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) using variable threshold settings for segmentation of diseased lung areas. Methods: Twenty-two patients with rheumatoid arthritis were included and underwent thin-section CT (4 x 1.25 mm collimation). CT scans were assessed by two observers for extent of ILD (EoILD), and twice by MeVis PULMO 3D for each protocol. MeVis PULMO 3D used four segmentation threshold (ST) settings (ST = -740, -780, -800 and -840 HU). Pulmonary function tests were obtained in all patients. Statistical evaluation used 95% limits of agreement (LoA) and linear regression analysis. Results: There was total concordance between the software measurements. Interobserver agreement was good (LoA = -28.36 to 17.58%). EoILD by readers correlated strongly with DL{sub CO} (r = -0.702, p < 0.0001) and moderately with FVC (r = -0.523, p = 0.018). There was close correlation between readers and MeVis PULMO 3D with best results for ST <780 HU (EoILD vs. MeVis PULMO 3D: r = 0.650 for ST = -800 and -840 HU, respectively; p = 0.002). MeVis PULMO 3D correlated best with DL{sub CO} at ST of -800 HU (r = -0.44, -0.49, -0.58 and -0.57 for ST = -740, -780, -800 and -840, respectively; p = 0.007-0.05) and moderately with FVC (r = -0.44, -0.51, -0.59 and -0.45 for ST = -740, -780, -800 and -840), respectively; p = 0.007-0.05). Conclusion: The MeVis PULMO 3D system used holds promise to become a valuable instrument for quantification of chronic ILD in patients with RA when using the threshold value of -800 HU, with evidence of the closest correlations, both with human observers and physiologic impairment.

  11. Rituximab treatment in a case of antisynthetase syndrome with severe interstitial lung disease and acute respiratory failure

    Directory of Open Access Journals (Sweden)

    Zappa Maria

    2011-08-01

    Full Text Available Abstract We present a case of severe interstitial pneumonitis, mild polyarthritis and polymyositis, and Raynaud's syndrome with the presence of anti-Jo-1 antibodies, which had been diagnosed as anti-synthetase syndrome. The presence, however, of anti-Ro/SSA antibodies led us to understand that we were dealing here with a more severe form of interstitial lung disease. The patient was treated for acute respiratory failure but he showed resistance to glucocorticoids and cyclosporine. Thus, he was treated with infusions of anti-CD20 therapy (rituximab: his clinical conditions improved very rapidly and a significant decrease in the activity of pulmonary disease was detected using high-resolution computerized tomography (HRCT of the thorax and pulmonary function tests.

  12. Normal Expiratory Flow Rate and Lung Volumes in Patients with Combined Emphysema and Interstitial Lung Disease: A Case Series and Literature Review

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    Karen L Heathcote

    2011-01-01

    Full Text Available Pulmonary function tests in patients with idiopathic pulmonary fibrosis characteristically show a restrictive pattern including small lung volumes and increased expiratory flow rates resulting from a reduction in pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. When the diseases coexist, pulmonary volumes are compensated, and a smaller than expected reduction or even normal lung volumes can be found. The present report describes 10 patients with progressive breathlessness, three of whom experienced severe limitation in their quality of life. All patients showed lung interstitial involvement and emphysema on computed tomography scan of the chest. The 10 patients showed normal spirometry and lung volumes with severe compromise of gas exchange. Normal lung volumes do not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.

  13. Clinical analysis of interstitial lung disease associated with lung cancer%间质性肺病合并肺癌的临床分析

    Institute of Scientific and Technical Information of China (English)

    陈美芳; 施举红; 刘鸿瑞; 许文兵; 肖毅

    2013-01-01

    目的 探讨间质性肺病合并肺癌患者的临床特点,以提高临床诊治水平.方法 对8例经病理确诊的间质性肺病合并肺癌患者的临床表现、影像学、病理进行分析,治疗后随访.结果 8例患者中男性6例,女性2例,6例有吸烟史,中位年龄64岁.主要临床症状为咯血、刺激性于咳、咳大量泡沫痰、胸闷气短加重.3例有免疫学指标阳性.影像学表现为沿胸膜下分布为主的细网格及蜂窝肺,伴肺内结节.间质性肺病类型为非特异性间质性肺炎及寻常型间质性肺炎;肺癌以腺癌为主.2例ANCA阳性的患者给予糖皮质激素治疗;5例患者行肺癌根治术,其中3例患者无法耐受术后化疗,2例患者术后给予辅助化疗,1例行单纯化疗后出现重度骨髓抑制死亡.另2例患者放弃进一步治疗,分别于确诊后8个月及10个月死亡.结论 间质性肺炎合并肺癌起病隐匿,容易误诊、漏诊,临床应引起高度重视.%Objective To investigate the clinical features of interstitial lung disease associated with lung cancer.Methods The clinical manifestations,chest radiography,and pathology of eight interstitial lung disease associated with lung cancer patients were analyzed.All cases were followed up after treatment.Results The patients included six males and two females,six cases had history of smoking,three cases had positive immunological markers.The main clinical symptoms were hemoptysis,dry cough,frothy sputum,and shortness of breath.The pathological types of interstitial lung disease were usual interstitial pneumonia and nonspecific interstitial pneumonia.The pathological type of lung cancer was mainly adenocarcinoma.Five cases were treated with radical resection,three cases of them were unable to tolerate postoperative chemotherapy,two cases of them were given adjuvant chemotherapy.One case was died of severe bone marrow suppression after chemotherapy.The other two cases abandoned further treatment and died at

  14. β-thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up

    Directory of Open Access Journals (Sweden)

    Messana Irene

    2011-02-01

    Full Text Available Abstract Background β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of these factors with pulmonary functional and radiological parameters. Methods β-thymosins concentrations were determined by Reverse Phase-High Performance Liquid Chromatography-Electrospray-Mass Spectrometry in the bronchoalveolar lavage fluid of 46 scleroderma patients with lung involvement and of 15 controls. Results Thymosin β4, β4 sulfoxide, and β10 were detectable in bronchoalveolar lavage fluid of patients and controls. Thymosin β4 levels were significantly higher in scleroderma patients than in controls. In addition, analyzing the progression of scleroderma lung disease at one-year follow-up, we have found that higher thymosin β4 levels seem to have a protective role against lung tissue damage. Thymosin β4 sulfoxide levels were higher in the smokers and in the scleroderma patients with alveolitis. Conclusions We describe for the first time β-thymosins in bronchoalveolar lavage fluid and their possible involvement in the pathogenesis of scleroderma lung disease. Thymosin β4 seems to have a protective role against lung tissue damage, while its oxidation product mirrors an alveolar inflammatory status.

  15. Evaluation of chronic infectious interstitial pulmonary disease in children by low-dose CT-guided transthoracic lung biopsy

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    Heyer, Christoph M.; Lemburg, Stefan P.; Kagel, Thomas; Nicolas, Volkmar [Ruhr-University of Bochum, Institute of Diagnostic Radiology, Interventional Radiology and Nuclear Medicine, BG Clinics Bergmannsheil, Bochum (Germany); Mueller, Klaus-Michael [Ruhr-University of Bochum, Institute of Pathology, BG Clinics Bergmannsheil, Bochum (Germany); Nuesslein, Thomas G.; Rieger, Christian H.L. [Ruhr-University of Bochum, Pediatric Hospital, Bochum (Germany)

    2005-07-01

    Children with chronic infectious interstitial lung disease often have to undergo open lung biopsy to establish a final diagnosis. Open lung biopsy is an invasive procedure with major potential complications. Transthoracic lung biopsy (TLB) guided by computed tomography (CT) is a less-invasive well-established procedure in adults. Detailing the role of low-dose CT-guided TLB in the enhanced diagnosis of chronic lung diseases related to infection in children. A group of 11 children (age 8 months to 16 years) underwent CT-guided TLB with a 20-gauge biopsy device. All investigations were done under general anaesthesia on a multidetector CT scanner (SOMATOM Volume Zoom, Siemens, Erlangen, Germany) using a low-dose protocol (single slices, 120 kV, 20 mAs). Specimens were processed by histopathological, bacteriological, and virological techniques. All biopsies were performed without major complications; one child developed a small pneumothorax that resolved spontaneously. A diagnosis could be obtained in 10 of the 11 patients. Biopsy specimens revealed chronic interstitial alveolitis in ten patients. In five patients Chlamydia pneumoniae PCR was positive, in three Mycoplasma pneumoniae PCR was positive, and in two Cytomegalovirus PCR was positive. The average effective dose was 0.83 mSv. Low-dose CT-guided TLB can be a helpful tool in investigating chronic infectious inflammatory processes in children with minimal radiation exposure. It should be considered prior to any open surgical procedure performed for biopsy alone. In our patient group no significant complication occurred. A disadvantage of the method is that it does not allow smaller airways and vessels to be assessed. (orig.)

  16. INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

    NARCIS (Netherlands)

    GROEN, H; POSTMA, DS; KALLENBERG, CGM

    1993-01-01

    A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

  17. IMPORTANCE OF CYCLOPHOSPHANUM IN THE TREATMENT OF INTERSTITIAL LUNG LESION IN PATIENTS WITH SCLERODERMA SYSTEMATICA (A REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Olga Aleksandrova Koneva

    2010-01-01

    Full Text Available Interstitial lung diseases (ILD are a common manifestation of scleroderma systematica (SSD that along with pulmonary arterial hypertension remains the leading cause of death in this nosological entity. As of now, cyclophosphanum remains the only immunosuppressant recommended by the European League against Rheumatism for the treatment of ILD in SSD. The paper analyzes the papers providing evidence for the efficacy of cyclophosphanum in ILD in patients with SSD. It also considers the regimens and duration of treatment with cyclophosphanum, ways of evaluating its efficacy and effects on extrapulmonary manifestations of SSD. It is concluded that cyclophosphanum has a positive, predominantly stabilizing, effect on the course of ILD in SSD.

  18. Early pulmonary involvement of anti-CADM-140 autoantibody-positive rapidly progressive interstitial lung disease preceding typical cutaneous symptoms.

    Science.gov (United States)

    Tamai, Koji; Tachikawa, Ryo; Otsuka, Kyoko; Ueda, Hiroyuki; Hosono, Yuji; Tomii, Keisuke

    2014-01-01

    We herein report a patient with clinically amyopathic dermatomyositis (CADM) who developed anti-CADM-140 autoantibody in association with rapidly progressive interstitial lung disease (RP-ILD). Chest high-resolution computed tomography (HRCT) revealed early pulmonary involvement preceding typical cutaneous lesions. Primary lesions of patchy peribronchial opacity developed ground-glass opacity and consolidation with architectural distortion and traction bronchiectasis. The possibility of anti-CADM-140 autoantibody-associated RP-ILD should be considered when patchy peribronchial opacity of an unknown cause is visible on chest HRCT.

  19. 特发性间质性肺炎合并肺癌的研究进展%Research development of idiopathic interstitial pneumonia with lung cancer

    Institute of Scientific and Technical Information of China (English)

    易祥华

    2009-01-01

    @@ 特发性间质性肺炎(idiopathic interstitial pneumonia,IIP)是一组原因不明的间质性肺疾病(interstitial lung disease,ILD),以弥漫性肺泡炎和肺泡结构紊乱最终导致肺纤维化为特征,无特效治疗,严重威胁人类的健康[1].

  20. [Comparison of LCD and CRT monitors for detection of pulmonary nodules and interstitial lung diseases on digital chest radiographs by using receiver operating characteristic analysis].

    Science.gov (United States)

    Ikeda, Ryuji; Katsuragawa, Shigehiko; Shimonobou, Toshiaki; Hiai, Yasuhiro; Hashida, Masahiro; Awai, Kazuo; Yamashita, Yasuyuki; Doi, Kunio

    2006-05-20

    Soft copy reading of digital images has been practiced commonly in the PACS environment. In this study, we compared liquid-crystal display (LCD) and cathode-ray tube (CRT) monitors for detection of pulmonary nodules and interstitial lung diseases on digital chest radiographs by using receiver operating characteristic (ROC) analysis. Digital chest images with a 1000x1000 matrix size and a 8 bit grayscale were displayed on LCD/CRT monitor with 2M pixels in each observer test. Eight and ten radiologists participated in the observer tests for detection of nodules and interstitial diseases, respectively. In each observer test, radiologists marked their confidence levels for diagnosis of pulmonary nodules or interstitial diseases. The detection performance of radiologists was evaluated by ROC analyses. The average Az values (area under the ROC curve) in detecting pulmonary nodules with LCD and CRT monitors were 0.792 and 0.814, respectively. In addition, the average Az values in detecting interstitial diseases with LCD and CRT monitors were 0.951 and 0.953, respectively. There was no statistically significant difference between LCD and CRT for both detection of pulmonary nodules (P=0.522) and interstitial lung diseases (P=0.869). Therefore, we believe that the LCD monitor instead of the CRT monitor can be used for the diagnosis of pulmonary nodules and interstitial lung diseases in digital chest images.

  1. 关注结缔组织病相关问质性肺疾病%Focus on connective tissue disease-associated interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    孙耕耘

    2012-01-01

    @@ 结缔组织病(connective tissues disease,CTD)是风湿性疾病中的一大类,常累及全身多个系统,表现为慢性炎症性自身免疫病,当侵犯呼吸系统时,可出现间质性肺疾病(interstitial lung disease,ILD)、胸膜炎和肺动脉高压等,见表l.CTD与ILD同时存在时,常称为结缔组织病相关间质性肺疾病(connective tissues disease-interstitial lung disease,CTD-ILD).

  2. Computerized analysis of interstitial lung diseases on chest radiographs based on lung texture, geometric-pattern features, and artificial neural networks

    Science.gov (United States)

    Ishida, Takayuki; Katsuragawa, Shigehiko; Nakamura, Katsumi; Ashizawa, Kazuto; MacMahon, Heber; Doi, Kunio

    2002-05-01

    For computerized detection of interstitial lung disease on chest radiographs, we developed three different methods: texture analysis based on the Fourier transform, geometric- pattern feature analysis, and artificial neural network (ANN) analysis of image data. With these computer-aided diagnostic methods, quantitative measures can be obtained. To improve the diagnostic accuracy, we investigated combined classification schemes by using the results obtained with the three methods for distinction between normal and abnormal chest radiographs with interstitial opacities. The sensitivities of texture analysis, geometric analysis, and ANN analysis were 88.0+/- 1.6%, 91.0+/- 2.6%, and 87.5+/- 1.9%, respectively, at a specificity of 90.0%, whereas the sensitivity of a combined classification scheme with the logical OR operation was improved to 97.1%+/- 1.5% at the same specificity of 90.0%. The combined scheme can achieve higher accuracy than the individual methods for distinction between normal and abnormal cases with interstitial opacities.

  3. Altered monocyte and fibrocyte phenotype and function in scleroderma interstitial lung disease: reversal by caveolin-1 scaffolding domain peptide.

    Science.gov (United States)

    Tourkina, Elena; Bonner, Michael; Oates, James; Hofbauer, Ann; Richard, Mathieu; Znoyko, Sergei; Visconti, Richard P; Zhang, Jing; Hatfield, Corey M; Silver, Richard M; Hoffman, Stanley

    2011-07-01

    Interstitial lung disease (ILD) is a major cause of morbidity and mortality in scleroderma (systemic sclerosis, or SSc). Fibrocytes are a monocyte-derived cell population implicated in the pathogenesis of fibrosing disorders. Given the recently recognized importance of caveolin-1 in regulating function and signaling in SSc monocytes, in the present study we examined the role of caveolin-1 in the migration and/or trafficking and phenotype of monocytes and fibrocytes in fibrotic lung disease in human patients and an animal model. These studies fill a gap in our understanding of how monocytes and fibrocytes contribute to SSc-ILD pathology. We found that C-X-C chemokine receptor type 4-positive (CXCR4+)/collagen I-positive (ColI+), CD34+/ColI+ and CD45+/ColI+ cells are present in SSc-ILD lungs, but not in control lungs, with CXCR4+ cells being most prevalent. Expression of CXCR4 and its ligand, stromal cell-derived factor 1 (CXCL12), are also highly upregulated in SSc-ILD lung tissue. SSc monocytes, which lack caveolin-1 and therefore overexpress CXCR4, exhibit almost sevenfold increased migration toward CXCL12 compared to control monocytes. Restoration of caveolin-1 function by administering the caveolin scaffolding domain (CSD) peptide reverses this hypermigration. Similarly, transforming growth factor β-treated normal monocytes lose caveolin-1, overexpress CXCR4 and exhibit 15-fold increased monocyte migration that is CSD peptide-sensitive. SSc monocytes exhibit a different phenotype than normal monocytes, expressing high levels of ColI, CD14 and CD34. Because ColI+/CD14+ cells are prevalent in SSc blood, we looked for such cells in lung tissue and confirmed their presence in SSc-ILD lungs but not in normal lungs. Finally, in the bleomycin model of lung fibrosis, we show that CSD peptide diminishes fibrocyte accumulation in the lungs. Our results suggest that low caveolin-1 in SSc monocytes contributes to ILD via effects on cell migration and phenotype and that the

  4. Altered monocyte and fibrocyte phenotype and function in scleroderma interstitial lung disease: reversal by caveolin-1 scaffolding domain peptide

    Directory of Open Access Journals (Sweden)

    Tourkina Elena

    2011-07-01

    Full Text Available Abstract Interstitial lung disease (ILD is a major cause of morbidity and mortality in scleroderma (systemic sclerosis, or SSc. Fibrocytes are a monocyte-derived cell population implicated in the pathogenesis of fibrosing disorders. Given the recently recognized importance of caveolin-1 in regulating function and signaling in SSc monocytes, in the present study we examined the role of caveolin-1 in the migration and/or trafficking and phenotype of monocytes and fibrocytes in fibrotic lung disease in human patients and an animal model. These studies fill a gap in our understanding of how monocytes and fibrocytes contribute to SSc-ILD pathology. We found that C-X-C chemokine receptor type 4-positive (CXCR4+/collagen I-positive (ColI+, CD34+/ColI+ and CD45+/ColI+ cells are present in SSc-ILD lungs, but not in control lungs, with CXCR4+ cells being most prevalent. Expression of CXCR4 and its ligand, stromal cell-derived factor 1 (CXCL12, are also highly upregulated in SSc-ILD lung tissue. SSc monocytes, which lack caveolin-1 and therefore overexpress CXCR4, exhibit almost sevenfold increased migration toward CXCL12 compared to control monocytes. Restoration of caveolin-1 function by administering the caveolin scaffolding domain (CSD peptide reverses this hypermigration. Similarly, transforming growth factor β-treated normal monocytes lose caveolin-1, overexpress CXCR4 and exhibit 15-fold increased monocyte migration that is CSD peptide-sensitive. SSc monocytes exhibit a different phenotype than normal monocytes, expressing high levels of ColI, CD14 and CD34. Because ColI+/CD14+ cells are prevalent in SSc blood, we looked for such cells in lung tissue and confirmed their presence in SSc-ILD lungs but not in normal lungs. Finally, in the bleomycin model of lung fibrosis, we show that CSD peptide diminishes fibrocyte accumulation in the lungs. Our results suggest that low caveolin-1 in SSc monocytes contributes to ILD via effects on cell migration and

  5. 吴银根辨治间质性肺疾病经验%Professor Wu Yingen' s clinical experience in treating interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    喻晓; 方泓; 唐斌擎

    2011-01-01

    This article introduces Professor Wu Yingen' s clinical experience in treating interstitial lung disease by traditional Chinese medicine. Professor Wu thinks that obstruction of lung collaterals is the basic pathogenesis of interstitial lung disease, so tonifying and activating the lung collaterals is the basic treatment method. And one case is presented.%介绍吴银根教授运用中医药辨治间质性肺疾病的临床经验.认为肺络痹阻为间质性肺疾病的基本病机;其基本治法为通补肺络,以辛通络痹、搜络化痹为主;遣方用药,灵活变通,并根据患者不同病情,中西医结合,规范用药.并附验案1则.

  6. Efficacy and safety of cryobiopsy versus forceps biopsy for interstitial lung diseases and lung tumours: A systematic review and meta-analysis.

    Science.gov (United States)

    Ganganah, Oormila; Guo, Shu Liang; Chiniah, Manu; Li, Yi Shi

    2016-07-01

    Forceps biopsy (FB) is the most commonly used diagnostic tool for lung pathologies. FB is associated with a high diagnostic failure rate. Cryobiopsy (CB) is a novel technique providing a larger specimen size, few artefacts, more alveolar parts and superior diagnostic yield. CB, however, has drawbacks such as higher bleeding and pneumothorax rate. We conducted a meta-analysis to investigate the specimen area, diagnostic rate and bleeding severity in CB versus FB in interstitial lung diseases (ILDs) and lung tumours. A systematic literature search of PUBMED, BIOSIS PREVIEW and OVID databases was conducted using specific search terms. Eligible studies including RCTs and non-RCTs comparing cryobiopsy/cryotransbronchial biopsy (CB/CTBB) and forceps biopsy/forceps transbronchial biopsy (FB/FTBB) for specimen area, diagnostic rate and bleeding rate in ILDs and lung tumours were analysed. Two reviewers independently extracted data and evaluated the quality of the studies. Eight studies involving 916 patients were analysed. Specimen area (mm(2) ) was significantly larger in CB/CTBB than FB/FTBB (standard mean difference = 1.21, 95% confidence interval (0.94, 1.48), P FB/FTBB (Risk ratio 1.36, 95% confidence interval (1.16, 1.59), P = 0.0002). Three studies compared the bleeding severity with only one showing significantly more bleeding in CB. Cryobiopsy/cryotransbronchial shows superiority to FB/FTBB for specimen area and diagnostic rate. CB/CTBB has better efficacy over FB/FTBB.

  7. Differential cell reaction upon Toll-like receptor 4 and 9 activation in human alveolar and lung interstitial macrophages

    Directory of Open Access Journals (Sweden)

    Meyerhans Andreas

    2010-09-01

    Full Text Available Abstract Background Investigations on pulmonary macrophages (MΦ mostly focus on alveolar MΦ (AM as a well-defined cell population. Characteristics of MΦ in the interstitium, referred to as lung interstitial MΦ (IM, are rather ill-defined. In this study we therefore aimed to elucidate differences between AM and IM obtained from human lung tissue. Methods Human AM and IM were isolated from human non-tumor lung tissue from patients undergoing lung resection. Cell morphology was visualized using either light, electron or confocal microscopy. Phagocytic activity was analyzed by flow cytometry as well as confocal microscopy. Surface marker expression was measured by flow cytometry. Toll-like receptor (TLR expression patterns as well as cytokine expression upon TLR4 or TLR9 stimulation were assessed by real time RT-PCR and cytokine protein production was measured using a fluorescent bead-based immunoassay. Results IM were found to be smaller and morphologically more heterogeneous than AM, whereas phagocytic activity was similar in both cell types. HLA-DR expression was markedly higher in IM compared to AM. Although analysis of TLR expression profiles revealed no differences between the two cell populations, AM and IM clearly varied in cell reaction upon activation. Both MΦ populations were markedly activated by LPS as well as DNA isolated from attenuated mycobacterial strains (M. bovis H37Ra and BCG. Whereas AM expressed higher amounts of inflammatory cytokines upon activation, IM were more efficient in producing immunoregulatory cytokines, such as IL10, IL1ra, and IL6. Conclusion AM appear to be more effective as a non-specific first line of defence against inhaled pathogens, whereas IM show a more pronounced regulatory function. These dissimilarities should be taken into consideration in future studies on the role of human lung MΦ in the inflammatory response.

  8. Interstitial lung disease caused by TS-1: a case of long-term drug retention as a fatal adverse reaction.

    Science.gov (United States)

    Park, Joong-Min; Hwang, In Gyu; Suh, Suk-Won; Chi, Kyong-Choun

    2011-12-01

    TS-1 is an oral anti-cancer agent for gastric cancer with a high response rate and low toxicity. We report a case of long-term drug retention of TS-1 causing interstitial lung disease (ILD) as a fatal adverse reaction. A 65-year-old woman underwent a total gastrectomy with pathologic confirmation of gastric adenocarcinoma. She received 6 cycles of TS-1 and low-dose cisplatin for post-operative adjuvant chemotherapy followed by single-agent maintenance therapy with TS-1. After 8 months, the patient complained of a productive cough with sputum and mild dyspnea. A pulmonary evaluation revealed diffuse ILD in the lung fields, bilaterally. In spite of discontinuing chemotherapy and the administration of corticosteroids, the pulmonary symptoms did not improve, and the patient died of pulmonary failure. TS-1-induced ILD can be caused by long-term drug retention that alters the lung parenchyma irreversibly, the outcome of which can be life-threatening. Pulmonary evaluation for early detection of disease is recommended.

  9. [Use of pulmonary function tests and biomarkers studies to diagnose and follow-up interstitial lung disease in systemic sclerosis].

    Science.gov (United States)

    Hua-Huy, T; Rivière, S; Tiev, K P; Dinh-Xuan, A T

    2014-12-01

    Interstitial lung disease (ILD) is becoming one of the main causes of death of patients with systemic sclerosis (SSc). The prevalence of ILD associated with SSc (SSc-ILD) varies from 33% to 100% according to diagnostic methods. Clinical features such as dyspnea on exertion, dry cough, and chest pains are not specific and usually late-appearing, implying more specific tests in the diagnostic, prognosis, and follow-up of ILD in patients with SSc. High resolution thoracic CT scanner (HRCT) is more sensitive than chest X-ray in the detection of SSc-ILD. Pulmonary function tests (PFT) are non-invasive and periodically used to assess the impacts of SSc on respiratory function. Diagnostic values of bronchoalveolar lavage and histological examination on lung biopsy are controversial. However, these techniques are essential for studying cellular and molecular mechanisms underlying the pathophysiology of SSc-ILD. Several biomarkers such as surfactant-A (SP-A), -D (SP-D), mucin-like high molecular weight glycoprotein (KL-6), and chemokine CCL-18 have been implicated in SSc-PID. Serum levels of these proteins are correlated with the severity of SSc-ILD, as assessed by HRCT and/or PFT. Finally, alveolar concentration of exhaled nitric oxide can be used to screen SSc patients with high risk of deterioration of respiratory function, in whom immunosuppressant treatment could be useful in preventing the evolution to irreversible lung fibrosis.

  10. Pulmonary interstitial glycogenosis

    Energy Technology Data Exchange (ETDEWEB)

    Lanfranchi, Michael [Creighton University Medical School, Children' s Hospital and Medical Center, Omaha, NE (United States); Allbery, Sandra M.; Wheelock, Lisa [Children' s Hospital and Medical Center, Department of Radiology, Omaha, NE (United States); Perry, Deborah [Children' s Hospital and Medical Center, Department of Pathology, Omaha, NE (United States)

    2010-03-15

    Although bronchopulmonary dysplasia (BPD) is a common cause of interstitial lung disease in chronically intubated premature neonates, other interstitial lung disease in nonintubated infants is rare. We present a case of pulmonary interstitial glycogenosis that developed in a nonintubated, 31-week gestation infant in whom infectious etiologies had been excluded. The infant was well initially and then developed respiratory distress at 18 days of life. Radiographs at first day of life were normal, but CT and radiographic findings at 18 days of life showed severe interstitial lung disease, mimicking BPD. Lung biopsy showed pulmonary interstitial glycogenosis. This entity is not well described in the pediatric radiology literature and is important to consider, as the condition is responsive to a course of corticosteroids. (orig.)

  11. Effective Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease by B-Cell Targeted Therapy with Rituximab

    Directory of Open Access Journals (Sweden)

    Wolfgang Hartung

    2012-01-01

    Full Text Available Rheumatoid arthritis- (RA- associated interstitial lung disease (RA-ILD is the extra-articular complication with most adverse impact on the quality of life and survival in RA patients. However, treatment options are limited and controlled studies are lacking. Here, we present the case of a 66-year-old patient suffering from severe RA-ILD, which has been successfully treated with Rituximab (RTX. After failure of conventional DMARD therapy, our patient showed sustained improvement of clinical pulmonary parameters as well as joint inflammation following B-cell depletion with RTX. The six-minute-walk test improved from 380 meters to 536 meters and the forced vital capacity from 2.49 liters to 3.49. The disease activity score could be reduced from 7.7 to 2.8. Therefore, RTX might be considered as an alternative treatment for RA-ILD in patients not responding to conventional DMARD therapy.

  12. Successful experience of rituximab therapy for systemic sclerosis-associated interstitial lung disease with concomitant systemic lupus erythematosus.

    Science.gov (United States)

    Sumida, Hayakazu; Asano, Yoshihide; Tamaki, Zenshiro; Aozasa, Naohiko; Taniguchi, Takashi; Takahashi, Takehiro; Toyama, Tetsuo; Ichimura, Yohei; Noda, Shinji; Akamata, Kaname; Miyazaki, Miki; Kuwano, Yoshihiro; Yanaba, Koichi; Sato, Shinichi

    2014-05-01

    Previous studies have demonstrated that B cells play critical roles in autoimmune disorders including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). However, the effectiveness of rituximab (RTX), a chimeric anti-CD20 antibody, for SSc-associated interstitial lung disease (ILD) or SLE disease activity remains controversial. We herein report an SSc patient with severely progressed ILD and concomitant SLE treated by two cycles of RTX at baseline and half a year later. This treatment improved ILD and SLE activities, along with reduction of dermal sclerosis and serum anti-topoisomerase I antibody levels. In addition, our detailed time-course data indicate that half a year may be appropriate as an interval between each cycle of RTX therapy aimed at SSc-associated ILD or SLE. Overall, the current report could pave the way to establish RTX as a disease-modifying drug for patients with SSc and/or SLE showing resistance to other already approved medications.

  13. Management of a patient undergoing sitting position craniotomy for acoustic neuroma with co-existing interstitial lung disease

    Directory of Open Access Journals (Sweden)

    Deepa Suvarna

    2015-01-01

    Full Text Available A 38-year-old woman with acoustic neuroma associated with occupational interstitial lung disease (ILD was successfully managed for sitting position craniotomy using carefully titrated desflurane-based anaesthesia. The anaesthetic challenges included maintenance an adequate depth of anaesthesia, reducing perioperative airway events and ensuring smooth recovery. While dealing with ILD patient in sitting position, careful risk assessment is important because it will help us predict the course of the perioperative events. Balanced general anaesthesia using desflurane fulfilled the requirement of good depth and smooth recovery in this patient. Though there are reports of maintenance of anaesthesia with other inhalational agents, there are scanty reports of using desflurane in these cases.

  14. Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient

    Directory of Open Access Journals (Sweden)

    Tiphaine Goletto

    2015-01-01

    Full Text Available Werner syndrome (WS is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telomere attrition and decay are thought to play a causative role in the clinical and pathological manifestations of the disease. Although telomere length, with or without germline mutation, is known to be associated with interstitial lung disease, the latter is not associated with WS. To the best of our knowledge, we report the first case describing a WS patient with fatal ILD. This case suggests that older patients with WS could develop ILD. Clinical outcome of WS patients may thus be improved by counselling them regarding smoking cessation or other exposure and by proposing antifibrotic therapy.

  15. Heparin platelet factor 4 antibody positivity in pseudothrombocytopenia.

    Science.gov (United States)

    Balcik, Ozlem Sahin; Akdeniz, Derya; Cipil, Handan; Uysal, Sema; Isik, Ayse; Kosar, Ali

    2012-01-01

    Pseudothrombocytopenia (PTCP) is a laboratory event of platelet clustering related to drugs used for anticoagulation. This condition is engendered by autoantibodies against platelets in usually EDTA-anticoagulated blood. Pseudothrombocytopenia has no clinical significance but when evaluated as true thrombocytopenia, this misconception may lead to unnecessary diagnostic procedures. Heparin-induced thrombocytopenia with thrombosis (HITT) is a complication of heparin treatment caused by heparin platelet factor 4 (HPF-4) antibodies, leading to platelet activation and hypercoagulability. In our study, 48 patients with PTCP and 36 healthy volunteers were included. Heparin platelet factor 4 antibody positivity was detected in 12 patients from PTCP group; nobody from control group had. Citrated serum samples and peripheral blood smears showed normal platelet count. Of the 4 patients using heparin derivative, 1 (2.1%) had antibody positivity but without any bleeding symptoms. In conclusion, HPF-4 antibody positivity might be a risk factor for PTCP. Clinicians should be aware of this kind of condition.

  16. Physical activity in people with asbestos related pleural disease and dust-related interstitial lung disease: An observational study.

    Science.gov (United States)

    Dale, Marita T; McKeough, Zoe J; Munoz, Phillip A; Corte, Peter; Bye, Peter T P; Alison, Jennifer A

    2015-11-01

    This study aimed to measure the levels of physical activity (PA) in people with dust-related pleural and interstitial lung diseases and to compare these levels of PA to a healthy population. There is limited data on PA in this patient population and no previous studies have compared PA in people with dust-related respiratory diseases to a healthy control group. Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease (ARPD) and a healthy age- and gender-matched population wore the SenseWear(®) Pro3 armband for 9 days. Six-minute walk distance, Medical Outcomes Study 36-item short-form health survey and the Hospital Anxiety and Depression Scale were also measured. Fifty participants were recruited and 46 completed the study; 22 with ARPD, 10 with dust-related interstitial lung disease (ILD) and 14 healthy age-matched participants. The mean (standard deviation) steps/day were 6097 (1939) steps/day for dust-related ILD, 9150 (3392) steps/day for ARPD and 10,630 (3465) steps/day for healthy participants. Compared with the healthy participants, dust-related ILD participants were significantly less active as measured by steps/day ((mean difference 4533 steps/day (95% confidence interval (CI): 1888-7178)) and energy expenditure, ((mean difference 512 calories (95% CI: 196-827)) and spent significantly less time engaging in moderate, vigorous or very vigorous activities (i.e. >3 metabolic equivalents; mean difference 1.2 hours/day (95% CI: 0.4-2.0)). There were no differences in levels of PA between healthy participants and those with ARPD. PA was reduced in people with dust-related ILD but not those with ARPD when compared with healthy age and gender-matched individuals.

  17. 特发性肺间质纤维化合并肺癌%Idiopathic Pulmonary Interstitial Fibrosis Underlying Lung Cancer

    Institute of Scientific and Technical Information of China (English)

    张大力; 严春花

    2011-01-01

    Idiopathic pulmonary interstitial fibrosis is a common interstitial lung disease, its incidence rate is increasing every year, and in recent years there has been a great deal report about idiopathic pulmonary interstitial fibrosis underlying lung cancer. A number of studies confirm that the incidence rate of lung cancer for the patients with pulmonary fibrosis is high and its prognosis is poor. This paper reviews the recent relevant literature, conducts the literature review of the epidemiology, pathogenesis, clinical and imaging features, pathological features and prognosis of idiopathic pulmonary interstitial fibrosis underlying lung cancer, aiming to improving the disease understanding of clinical workers and vigilance, in order to achieve the early detection and treatment of idiopathic pulmonary interstitial fibrosis underlying lung cancer.%特发性肺纤维化是常见的肺问质性疾病,发病率逐年上升,且近年来出现了大量关于特发性肺纤维化合并肺癌肺的报道.多项研究证实,肺纤维化患者的肺癌发病率高而且预后差.本文复习了近期相关的文献,对于特发性肺纤维化合并肺癌的流行病学、发病机理、临床与影像学特点、病理学特点及预后等进行文献复习,旨在提高临床工作者对该病的认识,提高警觉,以期达到特发性肺纤维化合并肺癌肺的早期发现和诊疗.

  18. EFFECTS OF ALVEOLAR MACROPHAGE CONDITIONED MEDIA FROM INTERSTITIAL LUNG DISEASEPATIENTS ON THE PROCOLLAGEN mRNA EXPRESSION IN HUMAN LUNG FIBROBLASTS

    Institute of Scientific and Technical Information of China (English)

    郭子健; 朱元珏; 刘秉慈; 朱亚玲; 赵文理; 陈勇

    1996-01-01

    Progressive inflammation and fibrosis are the central processez in the pathogenesis of pulmonary fibrosis. It is believed that macrophages in areas of chronically inflamed lung play a key role in fibrotic response. Therefore, we investigated the effects of alveolar macrophage (Amφ) conditioned media from interstitial lung disease (ILD) patients on lung fibroblast proliferation and procollagen mRNA expression, After stimulating with Amφ conditioned media from ILD pasients, the fibroblast proliferation increased 71.4% compared with the control, but for media from bronchial carcinoma (BC) patients, it just increased 14.3%. There is a significant dffference between the two groups (P<0. 05). The procollagen αl(I) mRNA in fibroblasts stimulated with Amφ conditioned media from ILD patients was increased 21.3% α1(Ⅲ)was 37.2 higher than control (P<0. 05). It increased 6. 8% and 12.8% fof media from BC patients respectively, but there was no difference when compared to the control. We considered that Amφ from ILD patients might be in an activated state and could release some growth factors to stimulate fibroblast proliferation and promote collagen DNA expression,

  19. Successful erlotinib rechallenge for leptomeningeal metastases of lung adenocarcinoma after erlotinib-induced interstitial lung disease: a case report and review of the literature.

    Science.gov (United States)

    Togashi, Yosuke; Masago, Katsuhiro; Hamatani, Yasuhiro; Sakamori, Yuichi; Nagai, Hiroki; Kim, Young Hak; Mishima, Michiaki

    2012-08-01

    The most serious adverse reaction associated with treatment with epidermal growth factor receptor-tyrosine kinase inhibitors (EGFR-TKIs) is drug-induced interstitial lung disease (ILD). Because EGFR-TKIs are key drugs for patients with non-small cell lung cancer who have somatic activating mutations of the epidermal growth factor receptor gene (EGFR mutations), several cases of retreatment with EGFR-TKIs after ILD induced by these drugs have been reported. Here, we present a 68-year-old man with lung adenocarcinoma and leptomeningeal metastases having an EGFR mutation who was retreated with erlotinib after erlotinib-induced ILD. He suffered no ILD recurrence and his leptomeningeal metastases dramatically improved. In addition to the present case, reports of nine patients who were retreated with EGFR-TKIs after ILD were found in the literature. Only one patient had recurrence of ILD (although seven were retreated at a reduced dose of EGFR-TKIs, including the patient with recurrence). In contrast, three patients had no recurrence of ILD even without dose-reduction. These reports suggest that dose-reduction plays a limited role in preventing recurrence. Many patients received corticosteroids during retreatment, but not the one with recurrence of ILD. This may suggest that corticosteroids can prevent recurrence due to their antiinflammatory properties.

  20. Natural antioxidant betanin protects rats from paraquat-induced acute lung injury interstitial pneumonia.

    Science.gov (United States)

    Han, Junyan; Ma, Deshun; Zhang, Miao; Yang, Xuelian; Tan, Dehong

    2015-01-01

    The effect of betanin on a rat paraquat-induced acute lung injury (ALI) model was investigated. Paraquat was injected intraperitoneally at a single dose of 20 mg/kg body weight, and betanin (25 and 100 mg/kg/d) was orally administered 3 days before and 2 days after paraquat administration. Rats were sacrificed 24 hours after the last betanin dosage, and lung tissue and bronchoalveolar lavage fluid (BALF) were collected. In rats treated only with paraquat, extensive lung injury characteristic of ALI was observed, including histological changes, elevation of lung : body weight ratio, increased lung permeability, increased lung neutrophilia infiltration, increased malondialdehyde (MDA) and myeloperoxidase (MPO) activity, reduced superoxide dismutase (SOD) activity, reduced claudin-4 and zonula occluden-1 protein levels, increased BALF interleukin (IL-1) and tumor necrosis factor (TNF)-α levels, reduced BALF IL-10 levels, and increased lung nuclear factor kappa (NF-κB) activity. In rats treated with betanin, paraquat-induced ALI was attenuated in a dose-dependent manner. In conclusion, our results indicate that betanin attenuates paraquat-induced ALI possibly via antioxidant and anti-inflammatory mechanisms. Thus, the potential for using betanin as an auxilliary therapy for ALI should be explored further.

  1. Early aggressive intra-venous pulse cyclophosphamide therapy for interstitial lung disease in a patient with systemic sclerosis. A case report.

    LENUS (Irish Health Repository)

    Peshin, R

    2009-06-01

    Interstitial lung disease is an important cause of mortality and morbidity in patients with systemic sclerosis (SSc). There are currently no recommended guidelines for management of these patients. This is probably due to the rarity of this condition, as well as clinical trials with only a small number of cases. There are published case report and case series along with the two main trials, viz. Scleroderma Lung Study and the Fibrosing Alveolitis Study, but again, there is no consensus on treatment protocols. In this report, we present a case of aggressive interstitial lung disease in a patient with SSc, which improved dramatically on treatment with intra-venous cyclophosphamide and high dose prednisolone therapy.

  2. Interstitial lung disease in gefitinib-treated Japanese patients with non-small cell lung cancer – a retrospective analysis: JMTO LC03-02

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    Tada Harue

    2009-08-01

    Full Text Available Abstract Background In Japan, high incidences of interstitial lung disease (ILD and ILD-related deaths have been reported among gefitinib-treated patients with non-small cell lung cancer (NSCLC. We investigated the efficacy of gefitinib, the incidence of ILD and risk factors for ILD in these patients. Findings We obtained patient data retrospectively using questionnaires sent to 22 institutions. We asked for demographic and clinical data on NSCLC patients for whom gefitinib treatment had begun between July 2002 and February 2003. Data from a total of 526 patients were analyzed. The patient characteristics were as follows: 64% male, 69% with adenocarcinoma, 61% with a performance score of 0–1, and 5% with concurrent interstitial pneumonitis. The objective response proportion was 80/439 (18.2%; 95% CI: 14.7–22.0. ILD developed in 17 patients (3.2%; 95% CI 1.9–5.1%, of whom 7 died. According to multivariate analysis, female sex, history of prior chemotherapy, low absolute neutrophil count before gefitinib treatment, and adenocarcinoma histology were associated with response to gefitinib treatment. None of the factors we evaluated were associated with the development of ILD. Conclusion The results of this study are consistent with previously published values for treatment response proportions and incidence of ILD during gefitinib treatment in Japanese patients. Future studies should be aimed at identifying factors indicating that a patient has a high probability of receiving benefit from gefitinib and a low risk of developing ILD.

  3. A CLINICAL AND RADIOLOGICAL PROFILE OF PATIENTS OF INTERSTITIAL LUNG DISEASES, ATTENDING THE CHEST HOSPITAL OF MEDICAL COLLEGE FROM NORTH INDIA

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    Anand

    2016-05-01

    Full Text Available OBJECTIVE To know the clinical and radiological profile of patients of Interstitial Lung Diseases attending the Chest Hospital of Medical College from North India from February 2014 to December 2015. MATERIAL AND METHODS The study was conducted in Department of Tuberculosis and Respiratory Diseases, G.S.V.M. Medical College, Kanpur (India. The patients were subjected to complete clinical history (Including work environment and occupational history, physical examination, various haematological investigations, spirometry and high resolution computerized tomography thorax. RESULTS In present study a total of 80 patients with interstitial lung disease were analysed. There were 36 males and 44 females (mean age was 54.4±1.8 years. Of 80 patients of interstitial lung diseases examined, Idiopathic Pulmonary Fibrosis (IPF pattern was present in maximum number (38.8% followed by sarcoidosis (17.5%, Hypersensitivity pneumonitis (15% Non-specific interstitial pneumonias (10%, Connective Tissue Disorder (C.T.D. associated ILD (7.5% and Cryptogenic Organizing Pneumonias (C.O.P. in 5% of cases. CONCLUSION IPF was the most common subgroup among ILDs.

  4. Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency--histologic and immunohistochemical analyses of 16 cases.

    Science.gov (United States)

    Rao, Nagarjun; Mackinnon, A Craig; Routes, John M

    2015-09-01

    Common variable immunodeficiency is a primary immunodeficiency of unknown etiology characterized by low serum immunoglobulin G, a decreased ability to make specific antibodies, and variable T-cell defects. Approximately 10-30% of patients with common variable immunodeficiency develop clinical evidence of a diffuse parenchymal lung disease with a constellation of histopathologic findings termed granulomatous and lymphocytic interstitial lung disease. In this study, we characterized the histologic and immunohistochemical features in a series of 16 cases diagnosed by open lung biopsy. Peribronchiolar and interstitial lymphocytic infiltration, granulomatous inflammation, and organizing pneumonia were consistent features; interstitial fibrosis with architectural remodeling was also found in a subgroup of patients. By immunohistochemistry, a predominance of CD4+ T lymphocytes with variable numbers of CD8+ T cells and B cells was present, with a striking absence of FOXP3-positive T-regulatory cells. This heretofore unrecognized immunohistochemical finding needs further investigation for a potential role in the pathogenesis of the condition. The presence of interstitial fibrosis with or without architectural remodeling in a subset of patients also needs additional study, for effect on prognosis.

  5. Interstitial lung abnormalities in treatment-naïve advanced non-small-cell lung cancer patients are associated with shorter survival

    Energy Technology Data Exchange (ETDEWEB)

    Nishino, Mizuki, E-mail: Mizuki_Nishino@DFCI.HARVARD.EDU [Department of Radiology, Brigham and Women' s Hospital, 75 Francis St., Boston, MA 02115 (United States); Department of Imaging, Dana-Farber Cancer Institute, 450 Brookline Ave., Boston, MA 02215 (United States); Cardarella, Stephanie [Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave., Boston, MA 02215, (United States); Dahlberg, Suzanne E. [Department of Biostatistics and Computational Biology, Dana-Farber Cancer Institute, 450 Brookline Ave., Boston, MA 02215 (United States); Araki, Tetsuro [Department of Radiology, Brigham and Women' s Hospital, 75 Francis St., Boston, MA 02115 (United States); Lydon, Christine; Jackman, David M.; Rabin, Michael S. [Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave., Boston, MA 02215, (United States); Hatabu, Hiroto [Department of Radiology, Brigham and Women' s Hospital, 75 Francis St., Boston, MA 02115 (United States); Johnson, Bruce E. [Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave., Boston, MA 02215, (United States)

    2015-05-15

    Highlights: • Interstitial lung abnormalities were present in 14% of stage IV NSCLC patients. • ILA was more common in older patients with heavier smoking history. • ILA was associated with shorter survival after adjusting for smoking and therapy. • ILA could be an additional independent marker for survival in advanced NSCLC. - Abstract: Objective: Interstitial lung diseases are associated with increased risk of lung cancer. The prevalence of ILA at diagnosis of advanced non-small-cell lung cancer (NSCLC) and its impact on overall survival (OS) remain to be investigated. Materials and method: The study included 120 treatment-naïve stage IV NSCLC patients (53 males, 67 females). ILA was scored on CT prior to any systemic therapy using a 4-point scale [0 = no evidence of ILA, 1 = equivocal for ILA, 2 = suspicious for ILA, 3 = ILA] by a sequential reading method previously reported. ILA scores of 2 or 3 indicated the presence of ILA. Results: ILA was present in 17 patients (14%) with advanced NSCLC prior to any treatment (score3: n = 2, score2: n = 15). These 17 patients were significantly older (median age: 69 vs. 63, p = 0.04) and had a heavier smoking history (median: 40 vs. 15.5 pack-year, p = 0.003) than those with ILA score 0 or 1. Higher ILA scores were associated with shorter OS (p = 0.001). Median OS of the 17 patients with ILA was 7.2 months [95%CI: 2.9–9.4] compared to 14.8 months [95%CI: 11.1–18.4] in patients with ILA score 0 or 1 (p = 0.002). In a multivariate model, the presence of ILA remained significant for increased risk for death (HR = 2.09, p = 0.028) after adjusting for first-line systemic therapy (chemotherapy, p < 0.001; TKI, p < 0.001; each compared to no therapy) and pack years of smoking (p = 0.40). Conclusion: Radiographic ILA was present in 14% of treatment-naïve advanced NSCLC patients. Higher ILA scores were associated with shorter OS, indicating that ILA could be a marker of shorter survival in advanced NSCLC.

  6. How common is the lipid body-containing interstitial cell in the mammalian lung?

    Science.gov (United States)

    Tahedl, Daniel; Wirkes, André; Tschanz, Stefan A; Ochs, Matthias; Mühlfeld, Christian

    2014-09-01

    Pulmonary lipofibroblasts are thought to be involved in lung development, regeneration, vitamin A storage, and surfactant synthesis. Most of the evidence for these important functions relies on mouse or rat studies. Therefore, the present study was designed to investigate the presence of lipofibroblasts in a variety of early postnatal and adult mammalian species (including humans) to evaluate the ability to generalize functions of this cell type for other species. For this purpose, lung samples from 14 adult mammalian species as well as from postnatal mice, rats, and humans were investigated using light and electron microscopic stereology to obtain the volume fraction and the total volume of lipid bodies. In adult animals, lipid bodies were observed only, but not in all rodents. In all other species, no lipofibroblasts were observed. In rodents, lipid body volume scaled with body mass with an exponent b = 0.73 in the power law equation. Lipid bodies were not observed in postnatal human lungs but showed a characteristic postnatal increase in mice and rats and persisted at a lower level in the adult animals. Among 14 mammalian species, lipofibroblasts were only observed in rodents. The great increase in lipid body volume during early postnatal development of the mouse lung confirms the special role of lipofibroblasts during rodent lung development. It is evident that the cellular functions of pulmonary lipofibroblasts cannot be transferred easily from rodents to other species, in particular humans.

  7. Blocking TGFβ via Inhibition of the αvβ6 Integrin: A Possible Therapy for Systemic Sclerosis Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Tamiko R. Katsumoto

    2011-01-01

    Full Text Available Interstitial lung disease (ILD is a commonly encountered complication of systemic sclerosis (SSc and accounts for a significant proportion of SSc-associated morbidity and mortality. Its pathogenesis remains poorly understood, and therapies that treat SSc ILD are suboptimal, at best. SSc ILD pathogenesis may share some common mechanisms with other fibrotic lung diseases, in which dysregulation of lung epithelium can contribute to pathologic fibrosis via recruitment or in situ generation and activation of fibroblasts. TGFβ, a master regulator of fibrosis, is tightly regulated in the lung by the integrin αvβ6, which is expressed at low levels on healthy alveolar epithelial cells but is highly induced in the setting of lung injury or fibrosis. Here we discuss the biology of αvβ6 and present this integrin as a potentially attractive target for inhibition in the setting of SSc ILD.

  8. Production of Fibronectin by the Human Alveolar Macrophage: Mechanism for the Recruitment of Fibroblasts to Sites of Tissue Injury in Interstitial Lung Diseases

    Science.gov (United States)

    Rennard, Stephen I.; Hunninghake, Gary W.; Bitterman, Peter B.; Crystal, Ronald G.

    1981-11-01

    Because cells of the mononuclear phagocyte system are known to produce fibronectin and because alveolar macrophages are activated in many interstitial lung diseases, the present study was designed to evaluate a role for the alveolar macrophage as a source of the increased levels of fibronectin found in the lower respiratory tract in interstitial lung diseases and to determine if such fibronectin might contribute to the development of the fibrosis found in these disorders by being a chemoattractant for human lung fibroblasts. Production of fibronectin by human alveolar macrophages obtained by bronchoalveolar lavage and maintained in short-term culture in serum-free conditions was demonstrated; de novo synthesis was confirmed by the incorporation of [14C]proline. This fibronectin had a monomer molecular weight of 220,000 and was antigenically similar to plasma fibronectin. Macrophages from patients with idiopathic pulmonary fibrosis produced fibronectin at a rate 20 times higher than did normal macrophages; macrophages from patients with pulmonary sarcoidosis produced fibronectin at 10 times the normal rate. Macrophages from 6 of 10 patients with various other interstitial disorders produced fibronectin at rates greater than the rate of highest normal control. Human alveolar macrophage fibronectin was chemotactic for human lung fibroblasts, suggesting a functional role for this fibronectin in the derangement of the alveolar structures that is characteristic of these disorders.

  9. Fatal Interstitial Lung Disease after Addition of Sorafenib to a Patient with Lung Adenocarcinoma Who Had Failed to Improve with Erlotinib Alone

    Directory of Open Access Journals (Sweden)

    Yin Guan

    2014-04-01

    Full Text Available Interstitial lung disease (ILD induced by epidermal growth factor receptor tyrosine kinase inhibitors has been extensively documented with decreasing incidence after appropriate patient selection due to increasing awareness over the years. However, ILD induced by sorafenib was mentioned with lower frequency only in patients with hepatocellular and renal cell carcinoma living in Japan but not in patients with other carcinomas or living outside Japan, and it has been overlooked in clinical practice. In the present case, sorafenib was added to the treatment of a 60-year-old non-smoking patient with non-small cell lung cancer (NSCLC. After his failing to improve with erlotinib alone, erlotinib was continued to be given in combination with sorafenib as a salvage therapy. Although clinical signs of ILD were observed 2 weeks after the addition of sorafenib, the radiological diagnosis of ILD was only made 41 days after the initiation of the combination treatment, and the patient died 56 days after treatment onset. It was concluded that ILD was indeed induced by sorafenib. This is the first report of ILD induced by sorafenib in a patient with NSCLC living outside Japan. Oncologists should be aware of this fatal complication for its early detection in order to avoid a severe course of ILD leading to a decrease in the ILD mortality rate.

  10. A clinical study of dermatomyositis complicated with interstitial lung disease%皮肌炎合并肺间质病变的临床研究

    Institute of Scientific and Technical Information of China (English)

    夏秀娟

    2009-01-01

    Objective To study the clinical characteristics of dermatomyositis compliacted with interstitial lung disease. Methods The patient's clinical symptoms and the laboratory inspection results were analyzed. Results The incidence of dermatomyositis complicated with interstitial lung disease was 25%. The rate of intersti-tial lung disease complicated with myalgia, fever, joint pain was 63.33% (19 cases) , 63.3% (19 cases), 53.3% (16 cases), respectively. Interstitial lung disease-free group included 30 cases (33.3%), 16 cases (17.8% ), 23 cases (25.6%) respectively. Positive rate of anti-jo-1 antibody and patients combined with interstitial lung disease increased the ratio of ESR were 7/30 (23.3%) and 24/30 (80.0%), while those in patients without interstitial lung disease were 0/90 (0) and 45/90 (50.0 % ). Conclusion There's a higher incidence for dermatomyositis pa-tients complicated with interstitial lung disease. Combination of interstitial lung disease shoule be of considered when myalgia, arthralgia and fever occurrs, anti-Jo-1 antibody appears positive and erythrocyte sedimentation rate increases.%目的 探讨皮肌炎合并肺间质病变的临床特点.方法 对比分析皮肌炎伴肺间质病变组(30例)和无肺问质病变组(90例)患者临床症状和实验室检查的结果.结果 皮肌炎合并肺间质病变发病率为25%.合并肺间质病变组中肌痛、发热、关节痛的比例分别是19例(63.3%)、19例(63.3%)、16例(53.3%),无肺间质病变组分别是30例(33.3%)、16例(17.8%)、23例(25.6%),2组比较,差异有统计学意义(P<0.01).合并肺问质病变者抗Jo-1抗体阳性率和红细胞沉降率升高比例分别为7/30(23.3%)、24/30(80.0%).无肺间质病变组分别为0.45/90(50.0%),2组差异有统计学意义(P<0.0l或P<0.05).结论 皮肌炎患者的肺间质病变发生率高,对皮肌炎患者出现肌痛、关节痛、发热等症状,同时抗Jo-1抗体阳性、红细胞沉降率升高时,应警惕合并肺间质病变.

  11. Interstitial lung disease complicated by inflammatory myopathies%炎症性肌病合并的肺间质病变

    Institute of Scientific and Technical Information of China (English)

    郑小蕾; 王雪芬; 孙益兰

    2009-01-01

    肺间质病变是炎症性肌病(多发性肌炎和皮肌炎)常见且严重的合并症,是引起死亡的主要原因之一.本文就炎症性肌病合并的肺间质病变的发病机制、病理变化、临床表现、实验室检查、肺功能表现、影像学改变及药物治疗、预后进行综述.%Interstitial lung disease is a common and severe complication of inflammatory myopathies (polymyositis and dermatomyositis). It is one of the main causes of death in these patients. This article reviews the following aspects of interstitial lung disease complicated by inflammatory myopathies, such as pathogenesis, pathologies, clinical manifestations, experimental discoveries, pulmonary function tests, pulmonary radiology,drug therapy and prognosis.

  12. Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-10-01

    We would like to report our experience of using rituximab in cyclophosphamide refractory, rapidly progressive interstitial lung disease (ILD) in a patient with limited scleroderma. A 40-year-old man presented with 10-week history of inflammatory polyarthritis, which responded to a short course of oral corticosteroids. However, 3 weeks later, he developed new onset of exertional dyspnoea. High-resolution CT of the thorax was suggestive of early ILD. Surgical lung biopsy showed features of fibrotic non-specific interstitial pneumonia. He was diagnosed with scleroderma on the basis of: presence of anticentromere antibodies, Raynaud\\'s phenomenon, pulmonary fibrosis, digital oedema and hypomotility along with a dilated oesophagus. He was treated aggressively with pulse doses of corticosteroids and cyclophosphamide; however, his ILD continued to deteriorate. At this stage, he received rituximab (two pulses of 1 g each), which led to a gradual clinical improvement. Now, 12 months since his rituximab infusion, he walks 2 miles daily without any exertional dyspnoea.

  13. Systems biology of interstitial lung diseases: integration of mRNA and microRNA expression changes

    Directory of Open Access Journals (Sweden)

    Price Jennifer

    2011-01-01

    Full Text Available Abstract Background The molecular pathways involved in the interstitial lung diseases (ILDs are poorly understood. Systems biology approaches, with global expression data sets, were used to identify perturbed gene networks, to gain some understanding of the underlying mechanisms, and to develop specific hypotheses relevant to these chronic lung diseases. Methods Lung tissue samples from patients with different types of ILD were obtained from the Lung Tissue Research Consortium and total cell RNA was isolated. Global mRNA and microRNA were profiled by hybridization and amplification-based methods. Differentially expressed genes were compiled and used to identify critical signaling pathways and potential biomarkers. Modules of genes were identified that formed a regulatory network, and studies were performed on cultured cells in vitro for comparison with the in vivo results. Results By profiling mRNA and microRNA (miRNA expression levels, we found subsets of differentially expressed genes that distinguished patients with ILDs from controls and that correlated with different disease stages and subtypes of ILDs. Network analysis, based on pathway databases, revealed several disease-associated gene modules, involving genes from the TGF-β, Wnt, focal adhesion, and smooth muscle actin pathways that are implicated in advancing fibrosis, a critical pathological process in ILDs. A more comprehensive approach was also adapted to construct a putative global gene regulatory network based on the perturbation of key regulatory elements, transcription factors and microRNAs. Our data underscores the importance of TGF-β signaling and the persistence of smooth muscle actin-containing fibroblasts in these diseases. We present evidence that, downstream of TGF-β signaling, microRNAs of the miR-23a cluster and the transcription factor Zeb1 could have roles in mediating an epithelial to mesenchymal transition (EMT and the resultant persistence of mesenchymal cells

  14. Diagnosis and differential diagnosis on interstitial lung disease%弥漫性间质性肺疾病的诊断和鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    徐作军

    2008-01-01

    @@ 一、概述 弥漫性间质性肺疾病(interstitial lung disease)是一组以是以肺间质、肺泡壁和肺泡腔具有不同形式和程度的炎症和纤维化为主要特征性病理改变的异源性疾病.

  15. Anti-Cyclic Citrullinated Peptide Antibodies and Severity of Interstitial Lung Disease in Women with Rheumatoid Arthritis

    Science.gov (United States)

    Ponce-Guarneros, Manuel; Mejía, Mayra; Juárez-Contreras, Pablo; Corona-Sánchez, Esther Guadalupe; Rodríguez-Hernández, Tania Marlen; Salazar-Páramo, Mario; Cardona-Muñoz, Ernesto German; Celis, Alfredo; González-Lopez, Laura

    2015-01-01

    Objective. To evaluate whether serum titers of second-generation anticyclic citrullinated peptide antibodies (anti-CCP2) are associated with the severity and extent of interstitial lung disease in rheumatoid arthritis (RA-ILD). Methods. In across-sectional study, 39 RA-ILD patients confirmed by high-resolution computed tomography (HRCT) were compared with 42 RA without lung involvement (RA only). Characteristics related to RA-ILD were assessed in all of the patients and serum anti-CCP2 titers quantified. Results. Higher anti-CCP2 titers were found in RA-ILD compared with RA only (medians 77.9 versus 30.2 U/mL, P < 0.001). In the logistic regression analysis after adjustment for age, disease duration (DD), smoke exposure, disease activity, functioning, erythrocyte sedimentation rate, and methotrexate (MTX) treatment duration, the characteristics associated with RA-ILD were higher anti-CCP2 titers (P = 0.003) and + RF (P = 0.002). In multivariate linear regression, the variables associated with severity of ground-glass score were anti-CCP2 titers (P = 0.02) and with fibrosis score DD (P = 0.01), anti-CCP2 titers (P < 0.001), and MTX treatment duration (P < 0.001). Conclusions. Anti-CCP2 antibodies are markers of severity and extent of RA-ILD in HRCT. Further longitudinal studies are required to identify if higher anti-CCP2 titers are associated with worst prognosis in RA-ILD. PMID:26090479

  16. Anti-Cyclic Citrullinated Peptide Antibodies and Severity of Interstitial Lung Disease in Women with Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Alberto Daniel Rocha-Muñoz

    2015-01-01

    Full Text Available Objective. To evaluate whether serum titers of second-generation anticyclic citrullinated peptide antibodies (anti-CCP2 are associated with the severity and extent of interstitial lung disease in rheumatoid arthritis (RA-ILD. Methods. In across-sectional study, 39 RA-ILD patients confirmed by high-resolution computed tomography (HRCT were compared with 42 RA without lung involvement (RA only. Characteristics related to RA-ILD were assessed in all of the patients and serum anti-CCP2 titers quantified. Results. Higher anti-CCP2 titers were found in RA-ILD compared with RA only (medians 77.9 versus 30.2 U/mL, P<0.001. In the logistic regression analysis after adjustment for age, disease duration (DD, smoke exposure, disease activity, functioning, erythrocyte sedimentation rate, and methotrexate (MTX treatment duration, the characteristics associated with RA-ILD were higher anti-CCP2 titers (P=0.003 and + RF (P=0.002. In multivariate linear regression, the variables associated with severity of ground-glass score were anti-CCP2 titers (P=0.02 and with fibrosis score DD (P=0.01, anti-CCP2 titers (P<0.001, and MTX treatment duration (P<0.001. Conclusions. Anti-CCP2 antibodies are markers of severity and extent of RA-ILD in HRCT. Further longitudinal studies are required to identify if higher anti-CCP2 titers are associated with worst prognosis in RA-ILD.

  17. IgG4-related interstitial lung disease%IgG4相关间质性肺疾病

    Institute of Scientific and Technical Information of China (English)

    荣令; 李家树; 周新

    2010-01-01

    IgG4-related interstitial lung disease is proposed based on the findings of systemic IgG4-related sclerosing disease. The diagnosis of IgG4-related interstitial lung disease requires the combination of clinical-radiological-pathological features. The therapeutic response of IgG4-related interstitial lung disease to glucocorticoid is good,and follow-up is necessary for the detection of recurrence.%IgG4相关间质性肺疾病的提出是基于对系统性IgG4相关硬化性疾病的研究发现.IgG4相关间质性肺疾病的诊断需要临床医师结合临床-影像学-病理学特征疑及此病.IgG4相关间质性肺疾病对糖皮质激素治疗反应良好,监测随访对于发现复发是必要的.

  18. High-resolution computed tomography to differentiate chronic diffuse interstitial lung diseases with predominant ground-glass pattern using logical analysis of data

    Energy Technology Data Exchange (ETDEWEB)

    Martin, Sophie Grivaud; Brauner, Michel W.; Rety, Frederique [Universite Paris 13, Assistance Publique-Hopitaux de Paris, Hopital Avicenne, UPRES EA 2363, Department of Radiology, Bobigny (France); Kronek, Louis-Philippe; Brauner, Nadia [Universite Joseph Fourier, Laboratoire G-SCOP, Grenoble (France); Valeyre, Dominique; Nunes, Hilario [Universite Paris 13, Assistance Publique-Hopitaux de Paris, Hopital Avicenne, UPRES EA 2363, Department of Pneumology, Bobigny (France); Brillet, Pierre-Yves [Universite Paris 13, Assistance Publique-Hopitaux de Paris, Hopital Avicenne, UPRES EA 2363, Department of Radiology, Bobigny (France); Hopital Avicenne, Service de Radiologie, Bobigny Cedex (France)

    2010-06-15

    We evaluated the performance of high-resolution computed tomography (HRCT) to differentiate chronic diffuse interstitial lung diseases (CDILD) with predominant ground-glass pattern by using logical analysis of data (LAD). A total of 162 patients were classified into seven categories: sarcoidosis (n = 38), connective tissue disease (n = 32), hypersensitivity pneumonitis (n = 18), drug-induced lung disease (n = 15), alveolar proteinosis (n = 12), idiopathic non-specific interstitial pneumonia (n = 10) and miscellaneous (n = 37). First, 40 CT attributes were investigated by the LAD to build up patterns characterising a category. From the association of patterns, LAD determined models specific to each CDILD. Second, data were recomputed by adding eight clinical attributes to the analysis. The 20 x 5 cross-folding method was used for validation. Models could be individualised for sarcoidosis, hypersensitivity pneumonitis, connective tissue disease and alveolar proteinosis. An additional model was individualised for drug-induced lung disease by adding clinical data. No model was demonstrated for idiopathic non-specific interstitial pneumonia and the miscellaneous category. The results showed that HRCT had a good sensitivity ({>=}64%) and specificity ({>=}78%) and a high negative predictive value ({>=}93%) for diseases with a model. Higher sensitivity ({>=}78%) and specificity ({>=}89%) were achieved by adding clinical data. The diagnostic performance of HRCT is high and can be increased by adding clinical data. (orig.)

  19. Management of Rheumatoid Arthritis Patients with Interstitial Lung Disease: Safety of Biological Antirheumatic Drugs and Assessment of Pulmonary Fibrosis.

    Science.gov (United States)

    Mori, Shunsuke

    2015-01-01

    Interstitial lung disease (ILD) is one of the major causes of morbidity and mortality of patients with rheumatoid arthritis (RA). Accompanying the increased number of reports on the development or exacerbation of ILD in RA patients following therapy with biological disease-modifying antirheumatic drugs (DMARDs), RA-associated ILD (RA-ILD) has aroused renewed interest. Although such cases have been reported mainly in association with the use of tumor necrosis factor inhibitors, the use of other biological DMARDs has also become a matter of concern. Nevertheless, it is difficult to establish a causative relationship between the use of biological DMARDs and either the development or exacerbation of ILD. Such pulmonary complications may occur in the natural course of RA regardless of the use of biological DMARDs. Since rheumatologists currently aim to achieve remission in RA patients, the administration of biological DMARDs is increasing, even for those with RA-ILD. However, there are no reliable, evidence-based guidelines for deciding whether biological DMARDs can be safely introduced and continued in RA-ILD patients. A standardized staging system for pulmonary conditions of RA-ILD patients is needed when making therapeutic decisions at baseline and monitoring during biological DMARD therapy. Based on the available information regarding the safety of biological DMARDs and the predictive factors for a worse prognosis, this review discusses candidate parameters for risk evaluation of ILD in RA patients who are scheduled to receive biological antirheumatic therapy.

  20. Correlation between HRCT findings, pulmonary function tests and bronchoalveolar lavage cytology in interstitial lung disease associated with rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Biederer, J.; Muhle, C.; Heller, M.; Reuter, M. [Department of Diagnostic Radiology, Christian Albrechts University Kiel, Arnold-Heller-Strasse 9, 24105, Kiel (Germany); Schnabel, A.; Gross, W.L. [Department of Clinical Rheumatology, University of Luebeck, Luebeck (Germany); Rheumaklinik Bad Bramstedt, Bad Bramstedt (Germany)

    2004-02-01

    A prospective study correlating high-resolution computed tomography (HRCT), lung function tests (PFT) and bronchoalveolar lavage (BAL) cytology in patients with interstitial lung disease (ILD) associated with rheumatoid arthritis (RA). Fifty-three RA patients with suspected ILD (19 men, 34 women) underwent 71 HRCT (14 of 53 with sequential HRCT, mean follow-up 24.3 months). The HRCT evaluation by two observers on consensus included a semi-quantitative characterisation of lesion pattern and profusion on representative anatomical levels. Fifty-two HRCT were followed by PFT and BAL. Agreement or discordance of HRCT-, PFT- and BAL findings were analysed with Pearson's correlation, {kappa} score and McNemar's test. Tobacco-fume exposure was estimated in pack years. Smoking/non-smoking groups were compared with Student's t test. In 49 of 53 patients, HRCT was suggestive of ILD associated with RA (66 of 71 HRCT). Reticular lesions were found in 40 of 53 patients, in 15 of 40 presenting as mixed pattern with ground-glass opacities (GGO). Pure reticular patterns predominated in patients with long duration of ILD (p>0.01). Pure GGO were not observed. Lesion profusion was highly variable and correlated moderately negative with diffusion capacity (mean 88.2% (SD{+-}20.9%); r=-0.54; p<0.001) and very weak with vital capacity and FEV1 (mean values 92.2% (SD{+-}18.3%); r=-0.27; p<0.05 and 89.8% (SD{+-}17.5%); r=-0.31; p<0.01). In patients with GGO, BAL differentials tended towards neutrophilia ({kappa}=0.39; p=0.04; McNemar test p>0.2), but not towards lymphocytosis ({kappa}=0.10; p=0.23; McNemar test p>0.2). Differences in smoking history were not significant (p>0.1). The HRCT appears most appropriate for the detection and follow-up of ILD associated with RA. The PFT and BAL correlate only partially with lesion profusion or grading on HRCT, but they contribute valuable information about dynamic lung function and differential diagnoses (pneumonia, medication side

  1. Clinical study of rheumatoid interstitial lung disease evaluated by high resolution CT

    Energy Technology Data Exchange (ETDEWEB)

    Okuda, Yasuaki; Takasugi, Kiyoshi; Imai, Atsuko; Oyama, Tetsu; Oyama, Hiroko (Dohgo Spa Hospital, Matsuyama (Japan)); Kawamura, Sachiko

    1993-02-01

    High resolution computed tomographic (HRCT) scans were obtained in 215 patients with rheumatoid arthritis to assess pulmonary fibrosis (PF). We classified the HRCT appearances as five-point scale (0-4) based on the degrees of PF. We found 117 cases (54.4%) of PF on HRCT. Patients with PF (grade 1-4) showed significantly increased leucocyte cell counts and significantly worsened pulmonary function test than patients without PF (grade 0). Patients with advanced articular involvement had significantly higher prevalence of PF than others without them. Patients who were previously or currently receiving gold sodium thiomalate (GST) injection or administration of methotrexate had higher prevalence of PF than others. However, patients who were receiving long term GST therapy (1 year long or [Sigma]1000 mg) had slightly lower prevalence of PF than others. This finding suggests that dose-dependent lung injury is not related to GST therapy. Patients with advanced PF (grade 3, 4) had high prevalence of male sex, smoker, extraarticular manifestation. (author).

  2. Angiogenesis in Interstitial Lung Diseases: a pathogenetic hallmark or a bystander?

    Directory of Open Access Journals (Sweden)

    Anevlavis Stavros

    2006-05-01

    Full Text Available Abstract The past ten years parallels have been drawn between the biology of cancer and pulmonary fibrosis. The unremitting recruitment and maintenance of the altered fibroblast phenotype with generation and proliferation of immortal myofibroblasts is reminiscent with the transformation of cancer cells. A hallmark of tumorigenesis is the production of new blood vessels to facilitate tumor growth and mediate organ-specific metastases. On the other hand several chronic fibroproliferative disorders including fibrotic lung diseases are associated with aberrant angiogenesis. Angiogenesis, the process of new blood vessel formation is under strict regulation determined by a dual, yet opposing balance of angiogenic and angiostatic factors that promote or inhibit neovascularization, respectively. While numerous studies have examined so far the interplay between aberrant vascular and matrix remodeling the relative role of angiogenesis in the initiation and/or progression of the fibrotic cascade still remains elusive and controversial. The current article reviews data concerning the pathogenetic role of angiogenesis in the most prevalent and studied members of ILD disease-group such as IIPs and sarcoidosis, presents some of the future perspectives and formulates questions for potential further research.

  3. 吸烟相关性间质性肺疾病研究进展%Recent advances in research on smoking-related interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    刘寅; 张德平

    2008-01-01

    吸烟可能与间质性肺疾病的发生与发展密切相关,包括脱屑性间质性肺炎、呼吸性细支气管炎伴间质性肺疾病、肺朗格汉斯细胞组织细胞增生症和特发性肺纤维化等,这类疾病统称为吸烟相关性间质性肺疾病,了解这些疾病的特征,对问质性肺疾病的诊治有重要意义.%Cigarette smoking may be related tO development ot several interstitial lung diseases(ILD).Smoking-related ILD is a term including desquamative interstitial pneumonia,respiratory bronchiolitisassociated interstitial lung disease,pulmonary Langerhana cell histiocytosis and idiopathic pulmonary fibrosis.To know the features of ILD is significant for diagnosis and treatment of ILD.

  4. 111例弥漫性肺间质疾病的临床研究%Clinical study of 111 patients with diffuse interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    龙雄; 郭树彬; 邓春涛; 杜铁宽

    2015-01-01

    目的:分析弥漫性间质性肺疾病( diffuse interstitial lung disease, DILD,简称ILD)的疾病谱分布情况及临床特征,以提高诊疗水平。方法收集111例2006~2013年在北京协和医院住院的弥漫性肺间质性疾病患者的临床资料,回顾性分析其病因、临床表现、肺功能、血气分析及病理特征等。结果患者以咳嗽、呼吸困难为主要临床表现,近一半患者存在低氧血症。弥漫性间质性肺疾病与继发性结缔组织疾病相关性弥漫性肺间质疾病( CTD-ILD)相比杵状指的发生率较高,而后者有特殊接触史者较多( P<0.05)。83.9%的患者存在限制性通气功能障碍。高分辨率计算机断层扫描影像主要表现为斑片、淋巴结肿大、毛玻璃样改变、胸膜病变、结节和网格影。特发性间质肺炎(IIP)与继发性ILD相比,后者结节影发生率更高(P<0.05)。 ILD主要病理类型为非特异性间质肺炎(NSIP)(19.8%)、外源性过敏性肺泡炎(18.9%)、CTD -ILD (11.7%)、结节病(8.1%)。结论明确不同弥漫性肺间质疾病的临床特征可减少漏诊、误诊及误治,提高诊疗水平。%Objective This study aimed to analyze the disease spectrum distribution of diffuse interstitial lung disease and increase diagnostic and therapeutic level of this kind of disease.Methods Clinical materials of one hundred and eleven patients with diffuse interstitial lung disease hospitalized into Peking Union Medical College Hospital from 2006 to 2013 were collected.Causes, symptoms, lung function, blood gas and pathological characters were analyzed retrospectively.Results Cough, dyspnea were the major symptoms and nearly half of the patients had hypoxemia.The incidence of acropachia of patients with interstitial lung disease was higher than that of the patients with interstitial lung disease associated with connective tissue disease, but the

  5. The influence of liquid crystal display monitors on observer performance for the detection of interstitial lung markings on both storage phosphor and flat-panel-detector chest radiography

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Yon Mi [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-dong, Kangnam-ku, Seoul 135-710 (Korea, Republic of); Department of Radiology, Gachon University Gil Hospital, 1198, Guwol-dong, Namdong-gu, Incheon 405-760 (Korea, Republic of); Chung, Myung Jin [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-dong, Kangnam-ku, Seoul 135-710 (Korea, Republic of)], E-mail: mj1.chung@samsung.com; Lee, Kyung Soo [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-dong, Kangnam-ku, Seoul 135-710 (Korea, Republic of); Choe, Bong-Keun [Department of Preventive Medicine, School of Medicine, Kyunghee University, Seoul (Korea, Republic of)

    2010-04-15

    Purpose: To compare observer performance with a flat-panel liquid crystal display (LCD) monitor and with a high-resolution gray-scale cathode ray tube (CRT) monitor in the detection of interstitial lung markings using a silicon flat-panel-detector direct radiography (DR) and storage phosphor computed radiography (CR) in a clinical setting. Materials and methods: We displayed 39 sets of posteroanterior chest radiographs from the patients who were suspected of interstitial lung disease. Each sets consisted of DR, CR and thin-section CT as the reference standard. Image identities were masked, randomly sorted, and displayed on both five mega pixel (2048 x 2560 x 8 bits) LCD and CRT monitors. Ten radiologists independently rated their confidence in detection for the presence of linear opacities in the four fields of the lungs; right upper, left upper, right lower, and left lower quadrant. Performance of a total 6240 (39 sets x 2 detector systems x 2 monitor system x 4 fields x 10 observers) observations was analyzed by multi-reader multi-case receiver operating characteristic (ROC) analysis. Differences between monitor systems in combinations of detector systems were compared using ANOVA and paired-samples t-test. Results: Area under curves (AUC) for the presence of linear opacities measured by ROC analysis was higher on the LCDs than CRTs without statistical significance (p = 0.082). AUC was significantly higher on the DR systems than CR systems (p = 0.006). AUC was significantly higher on the LCDs than CRTs for DR systems (p = 0.039) but not different for CR systems (p = 0.301). Conclusion: In clinical conditions, performance of the LCD monitor appears to be better for detecting interstitial lung markings when interfaced with DR systems.

  6. Differentiation of several interstitial lung disease patterns in HRCT images using support vector machine: role of databases on performance

    Science.gov (United States)

    Kale, Mandar; Mukhopadhyay, Sudipta; Dash, Jatindra K.; Garg, Mandeep; Khandelwal, Niranjan

    2016-03-01

    Interstitial lung disease (ILD) is complicated group of pulmonary disorders. High Resolution Computed Tomography (HRCT) considered to be best imaging technique for analysis of different pulmonary disorders. HRCT findings can be categorised in several patterns viz. Consolidation, Emphysema, Ground Glass Opacity, Nodular, Normal etc. based on their texture like appearance. Clinician often find it difficult to diagnosis these pattern because of their complex nature. In such scenario computer-aided diagnosis system could help clinician to identify patterns. Several approaches had been proposed for classification of ILD patterns. This includes computation of textural feature and training /testing of classifier such as artificial neural network (ANN), support vector machine (SVM) etc. In this paper, wavelet features are calculated from two different ILD database, publically available MedGIFT ILD database and private ILD database, followed by performance evaluation of ANN and SVM classifiers in terms of average accuracy. It is found that average classification accuracy by SVM is greater than ANN where trained and tested on same database. Investigation continued further to test variation in accuracy of classifier when training and testing is performed with alternate database and training and testing of classifier with database formed by merging samples from same class from two individual databases. The average classification accuracy drops when two independent databases used for training and testing respectively. There is significant improvement in average accuracy when classifiers are trained and tested with merged database. It infers dependency of classification accuracy on training data. It is observed that SVM outperforms ANN when same database is used for training and testing.

  7. Interstitial lung disease associated with collagen vascular disorders: disease quantification using a computer-aided diagnosis tool

    Energy Technology Data Exchange (ETDEWEB)

    Marten, K.; Engelke, C. [University Hospital of Goettingen, Department of Radiology, Goettingen (Germany); Dicken, V. [MeVis Research GmbH, Bremen (Germany); Kneitz, C. [University Hospital of Wuerzburg, Dept. of Rheumatology and Clinical Immunology, Medizinische Klinik and Poliklinik, Wuerzburg (Germany); Hoehmann, M.; Kenn, W.; Hahn, D. [University Hospital of Wuerzburg, Department of Radiology, Wuerzburg (Germany)

    2009-02-15

    The purpose of this study was to evaluate a computer-aided diagnosis (CAD) tool compared to human observers in quantification of interstitial lung disease (ILD) in patients with collagen-vascular disorders. A total of 52 patients with rheumatoid arthritis (n=24), scleroderma (n=14) and systemic lupus erythematosus (n=14) underwent thin-section CT. Two independent observers assessed the extent of ILD (EoILD), reticulation (EoRet) and ground-glass opacity (EoGGO). CAD assessed EoILD twice. Pulmonary function tests were obtained. Statistical evaluation used 95% limits of agreement and linear regression analysis. CAD correlated well with diffusing capacity (DL{sub CO}) (R=-0.531, P<0.0001) and moderately with forced vital capacity (FVC) (R=-0.483, P=0.0008). There was close correlation between CAD and the readers (EoILD vs. CAD: R=0.716, P<0.0001; EoRet vs. CAD: R=0.69, P<0.0001). Subgroup analysis including patients with minimal EoGGO (<15%) strengthened the correlations between CAD and the readers, readers and PFT, and CAD and PFT. EoILD by readers correlated strongly with DL{sub CO} (R=-0.705, P<0.0001) and moderately with FVC (R=-0.559, P=0.0002). EoRet correlated closely with DL{sub CO} and moderately with FVC (DL{sub CO}: R=-0.663; FVC: R=-0.436; P{<=}0.005). The CAD system is a promising tool for ILD quantification, showing close correlation with human observers and physiologic impairment. (orig.)

  8. mTOR inhibitor-induced interstitial lung disease in cancer patients: Comprehensive review and a practical management algorithm.

    Science.gov (United States)

    Willemsen, Annelieke E C A B; Grutters, Jan C; Gerritsen, Winald R; van Erp, Nielka P; van Herpen, Carla M L; Tol, Jolien

    2016-05-15

    Mammalian target of rapamycin inhibitors (mTORi) have clinically significant activity against various malignancies, such as renal cell carcinoma and breast cancer, but their use can be complicated by several toxicities. Interstitial lung disease (ILD) is an adverse event of particular importance. Mostly, mTORi-induced ILD remains asymptomatic or mildly symptomatic, but it can also lead to severe morbidity and even mortality. Therefore, careful diagnosis and management of ILD is warranted. The reported incidence of mTORi-induced ILD varies widely because of a lack of uniform diagnostic criteria and active surveillance. Because of the nonspecific clinical features, a broad differential diagnosis that includes (opportunistic) infections should be considered in case of suspicion of mTORi-induced ILD. The exact mechanism or interplay of mechanisms leading to the development of ILD remains to be defined. Suggested mechanisms are either a direct toxic effect or immune-mediated mechanisms, considering mTOR inhibitors have several effects on the immune system. The clinical course of ILD varies widely and is difficult to predict. Consequently, the discrimination between when mTOR inhibitors can be continued safely and when discontinuation is indicated is challenging. In this review, we give a comprehensive review of the incidence, clinical presentation and pathophysiology of mTORi-induced ILD in cancer patients. We present newly developed diagnostic criteria for ILD, which include clinical symptoms as well as basic pulmonary function tests and radiological abnormalities. In conjunction with these diagnostic criteria, we provide a detailed and easily applicable clinical management algorithm.

  9. Elevated Erythrocyte Sedimentation Rate Is Predictive of Interstitial Lung Disease and Mortality in Dermatomyositis: a Korean Retrospective Cohort Study.

    Science.gov (United States)

    Go, Dong Jin; Lee, Eun Young; Lee, Eun Bong; Song, Yeong Wook; Konig, Maximilian Ferdinand; Park, Jin Kyun

    2016-03-01

    Interstitial lung disease (ILD) is a major cause of death in patients with dermatomyositis (DM). This study was aimed to examine the utility of the erythrocyte sedimentation rate (ESR) as a predictor of ILD and prognostic marker of mortality in patients with DM. One hundred-and-fourteen patients with DM were examined, including 28 with clinically amyopathic DM (CADM). A diagnosis of ILD was made based on high resolution computed tomography (HRCT) scans. The association between elevated ESR and pulmonary impairment and mortality was then examined. ILD was diagnosed in 53 (46.5%) of 114 DM patients. Cancer was diagnosed in 2 (3.8%) of 53 DM patients with ILD and in 24 (92.3%) of those without ILD (P < 0.001). The median ESR (50.0 mm/hour) in patients with ILD was significantly higher than that in patients without ILD (29.0 mm/hour; P < 0.001). ESR was inversely correlated with forced vital capacity (Spearman ρ = - 0.303; P = 0.007) and carbon monoxide diffusing capacity (ρ = - 0.319; P = 0.006). DM patients with baseline ESR ≥ 30 mm/hour had significantly higher mortality than those with ESR < 30 mm/hour (P = 0.002, log-rank test). Patients with a persistently high ESR despite immunosuppressive therapy was associated with higher mortality than those with a normalized ESR (P = 0.039, log-rank test). Elevated ESR is associated with increased mortality in patients with DM due to respiratory failure. Thus, monitoring ESR should be an integral part of the clinical care of DM patients.

  10. Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Hugh J Freeman; Helen R Gillett; Peter M Gillett; Joel Oger

    2009-01-01

    Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a glutenfree diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.

  11. 婴幼儿弥漫性肺实质疾病/肺间质疾病概述%Diffuse parenchymal lung disease/interstitial lung disease in infancy

    Institute of Scientific and Technical Information of China (English)

    农光民

    2014-01-01

    婴幼儿时期弥漫性肺实质疾病/肺间质疾病(diffuse parenchymal lung disease/interstitial lung disease in infancy,DPLD/ILD),是一组在婴幼儿时期发生的和(或)特有的弥漫性肺疾病,病变累及肺间质和(或)肺实质.本文对婴幼儿时期弥漫性肺实质疾病/肺间质疾病的分类、诊断、治疗及其预后等进行简单论述.

  12. Diagnosis and treatment of rheumatosis-related interstitial lung disease%风湿病相关间质性肺疾病的诊断治疗

    Institute of Scientific and Technical Information of China (English)

    王勇; 马玉琛

    2010-01-01

    风湿病肺间质性肺疾病(Rheumatic Disease Interstitial Lung Disease RD-ILD)早期症状体征不明显,随着医学水平的发展,其检出率不断增高.我们总结近十几年来国内外RD-ILD的发病率、发病机制、诊断、治疗相关情况,以期提高对该病的认识;早期发现,早期治疗,以达到最佳临床治疗效果.

  13. 儿童弥漫性间质性肺疾病的高分辨率CT表现%High-resolution CT findings of diffused interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    袁新宇; 叶滨宾; 陈慧中

    2010-01-01

    @@ 弥漫性间质性肺疾病(diffused interstitial lung disease,DILD)是指可导致瘢痕和纤维化的肺间质炎性病变.在临床上,儿童DILD诊断标准应包括缺氧或呼吸困难,以及特征性影像学表现.DILD疾病谱因儿童成长和发育的阶段不同而有所不同.所以,不能应用成人诊断标准对其进行分类.

  14. Trisomy 21 syndrome associated interstitial lung disease: a case report%21-三体综合征相关肺间质疾病一例

    Institute of Scientific and Technical Information of China (English)

    陈杰华; 马红玲; 郑跃杰; 曹娟; 曾洪武; 张青

    2015-01-01

    目的 探讨21-三体综合征相关肺间质疾病的肺组织病理、影像和临床特点.方法 报道1例经影像及病理证实的21-三体综合征相关肺间质疾病,并复习文献总结其肺组织病理、影像及临床特点.结果 该例临床表现为重症肺炎,感染反复;病情稳定期无明显气促、发绀,胸部CT仍表现为磨玻璃样影、区域性肺气肿、肺实质带等肺间质病变征象.胸腔镜可见双肺各叶充气不均,表面呈弥漫性小气泡样改变.病理可见肺泡、肺泡管、肺泡囊扩张,肺泡间隔增宽.复习文献,国内未见报道.国外分别报道7例和24例21-三体综合征病理资料,86% ~ 88%病例可见肺泡生长异常.影像学特征性表现为胸膜下肺囊肿.临床上21-三体综合征高发呼吸道感染并容易加重引起呼吸衰竭;术后可引起持续低氧血症,需长时间呼吸支持.结论 21-三体综合征存在染色体相关的肺泡生长异常,病理表现肺泡简单化,影像表现肺间质疾病,临床应警惕感染、手术导致其呼吸衰竭的风险.%Objective To study the pathology,imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.Method Data of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected,analyzed and the related reports in literature were reviewed.Result The patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay.When his disease was stable,he did not have shortness of breath and cyanosis,but a chest computed tomography (CT) showed ground-glass opacity,regional emphysema,band-like change in lung parenchyma,which indicated interstitial lung diseases.Unequal air inflation in bilateral lungs and diffuse overdistension of peripheral air spaces in lung surface were seen through thoracoscope.Pathological examination indicated that alveolar

  15. Clinical analysis of connective tissue diseases with interstitial lung disease%结缔组织病肺间质病变临床分析

    Institute of Scientific and Technical Information of China (English)

    张佳林; 叶宝国; 陈宏浦

    2013-01-01

    目的 提高对结缔组织病肺间质病变的认识及诊断.方法 回顾性分析124例结缔组织病肺间质病变患者呼吸系统的临床表现、肺功能检查、肺部影像学特点、有无肺动脉高压、肺功能与肺部影像学关系.结果 124例结缔组织病肺间质病变患者呼吸系统临床表现为咳嗽、咳痰、气短、胸闷憋气,严重时表现为活动耐量下降、呼吸闲难.64例行肺功能检查均有弥散功能减低,其中26例为单纯弥散功能减低,32例为限制性通气功能障碍伴弥散功能减低,4例为混合性通气功能障碍伴弥散功能减低,2例为阻塞性通气功能障碍伴弥散功能减低.22例合并有肺动脉高压.肺间质病变在影像学上可有结节影、索条影、斑片影、网格影、磨玻璃影、肺大泡等多种改变.32例限制性通气功能障碍伴弥散功能减低患者中有15例表现为网格样、纤维化样改变,26例单纯弥散功能减低患者中有8例表现为网格样、纤维化样改变.结论 结缔组织病肺间质病变患者呼吸系统表现多种多样,肺功能检查主要表现为限制性通气功能障碍伴弥散功能减低.混合性结缔组织病、重叠综合征、系统性红斑狼疮、系统性硬化症是较易出现肺间质病变合并肺动脉高压的结缔组织病.结缔组织病肺间质病变在影像学上可有多种多样改变,影像学上表现为网格样、纤维化样改变的患者肺功能易出现限制性通气伴弥散功能障碍.%Objective To observe the diagnosis of connective tissue diseases with interstitial lung disease.Methods The clinical manifestations of respiratory,pulmonary function tests,lung imaging characteristics,the presence or absence of pulmonary hypertension,pulmonary function in 124 patients with interstitial lung disease associated with connective tissue disease were retrospective analyzed.Results Cough,expectoration,shortness of breath,chest stuffiness and choking were

  16. Interstitial Lung Disease

    Science.gov (United States)

    ... Health. Dr. Henson is in the Department of Academic Affairs. View Full Profile Make an Appointment Michael P. Mohning, MD + × Michael P. Mohning, MD Instructor Department of Medicine Division of Pulmonary, Critical Care and Sleep Medicine ...

  17. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    920673 The role of oxygen radicals in BLM-induced pulmonary fibrosis. WANGXiaozhi(王晓芝), et al. Respir Dis Res Lab,Union Hosp, Tongji Med Univ, Wuhan, 430022.Chin J Tuberc & Respir Dis 1992; 15(3): 158-160.

  18. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    2005206 The pivotal role of CXCR3 in the patho-genesis of bleomycin-induced pulmonary fibrosis. GAO Jin-ming(高金明), Dept Respir Med, PUMC Hosp, PUMC & CAMS, Beijing 100730. Chin J Tu-berc Respir Dis, 2005; 28 (1): 28-32. Objective: To investigate the contribution of chemokine receptor-CXCR3 to the fibrotic disease process induced by bleomycin in CXCR3 gene defi-

  19. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008051 Effects of noninvasive positive pressure ventilation on respiratory muscle fatigue in patients with acute exacerbation of chronic obstructive pulmonary disease:a randomized controlled trial. SUN Lihua(孙丽华),et al.Dept Pulm, Nanjing 1st Hosp, Nanjing Med Univ, Nanjing 210006. Chin J Intern Med 2007;46(12):992-995. Objective To study the effects of noninvasive positive pressure ventilation (NPPV) on respiratory muscle fatigue in patients

  20. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008290 Effects of vitamin A on the proliferation and apoptosis of alveolar cells of experimental emphysema in rats. HOU Gang(侯刚), et al. Instit Respir Dis, China Med Univ, Shengyang 110001. Chin J Tuberc Respir Dis 2008;31(3):209-212.Objective To study the effectof vitamin Aon experimental emphysema in rats,and on the proliferation and apoptosis of the alveolar cells.Methods Twenty-four Wistar rats were randomly divided into 3 groups:a control group(C),an emphysema model group(M)and a

  1. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    2009189 Therapeutic effects of aerosolized signal transducer and activator of transcription 1 antisense oligonucleotide administered at different time points on bleomycin-induced pulmonary fibrosis:experiment with rats. LJ Jing(李晶),et al.Dept Respir Med,Affili Hosp,Luzhou Med Coll,Luzhou 646000.Natl Med J China,2009;89(12):831-835.

  2. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    2010334 Morphological analysis and pathological basis of the fine pulmonary reticulation at high-resolution CT. GUAN Chunshuang(关春爽),et al. Dept Radiol,Beijing Friendship Hosp,Capital Med Univ,Beijing 100050.Chin J Radiol 2010;44(4):374-378. Objective To study the morphological appearance and pathological basis of the fine pulmonary reticulation at HRCT.

  3. Interstitial Lung Disease

    Science.gov (United States)

    ... toxins. If you work in mining, farming or construction or for any reason are exposed to environmental ... shield Mayo Clinic logo are trademarks of Mayo Foundation for Medical Education and Research. © 1998-2017 Mayo ...

  4. Interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970328 Expression FGF-b and C-myc in mouse lungtissue affected by hypoxia OUYANG Nengtai(欧阳能太), et al. Guangzhou Respir Instit,Guangzhou, 510120, Chin J Tuberc Respir Dis 1997; 20(1): 22-24. Objective: To study FGF’S roles in the reconstruc-tion of vascular walls owing to chronic hypoxia. Meth-

  5. The clinical significance of HRCT in evaluation of patients with rheumatoid arthritis-associated interstitial lung disease: a report from China.

    Science.gov (United States)

    Zou, Yu-Qiong; Li, Ya-Song; Ding, Xiao-Nan; Ying, Zhen-Hua

    2012-03-01

    The objective of this study is to describe the interstitial lung disease (ILD) in rheumatoid arthritis (RA) patients of China, and to study clinical significance of high-resolution computed tomography (HRCT) in evaluation and treatment. One hundred and ten Chinese patients (79 women and 31 man) diagnosed with RA between December 2008 to November 2009 were analyzed. According to the HRCT, 47 (42.73%) RA patients were diagnosed as ILD. Old age, smoking and pulmonary rales were closely related to ILD (P pulmonary nodules (11.82%), emphysema (9.09%), bronchiectasis (3.64%), subpleural nodules (11.82%) and pleural thickening (24.55%). In treatment, honeycombing and subpleural nodules were more common in patients with methotrexate (MTX) and/or leflunomide treatment than without (P 0.05). Pulmonary involvement is common in RA patients, and it is suggested that HRCT could be a sensitive and useful way in evaluating the lung of RA patients.

  6. Leflunomide and rheumatoid interstitial lung disease%来氟米特与类风湿肺间质病变

    Institute of Scientific and Technical Information of China (English)

    刘蕊; 刘湘源

    2011-01-01

    肺间质病变(ILD)是类风湿关节炎(RA)常见的关节外表现之一,发生率为7.7%,预后较差,近年来备受关注.而作为治疗RA的常用药物,来氟米特引起间质性肺炎的报道逐年增加,那么,RA合并ILD能否使用来氟米特呢?有文献指出来氟米特可以有效地治疗已经存在肺部疾病(包括甲氨蝶呤导致的间质性肺病)的RA患者,且未发现药物不良反应及肺部症状加重,因此指出当出现甲氨蝶呤相关ILD时,来氟米特可作为替代治疗用药.来氟米特在治疗中国RA患者的安全性是相对较好的,而近年来国外对于来氟米特引起ILD的报道却屡见不鲜.加拿大从一项治疗RA患者的队列研究中发现,应用来氟米特治疗的RA患者中ILD发病风险增高(校正RR 1.9),但其中无甲氨蝶呤用药史和ILD史的患者经来氟米特治疗后出现ILD的风险并未增高(校正RR 1.2).日本学者提出对于已有ILD或吸烟、高龄的男性RA患者应禁用或慎用来氟米特.文献报道不同种族RA患者对来氟米特并发的肺部不良反应存在差异,基因学检测将是寻找答案的关键.总之,虽然来氟米特有导致ILD的潜在隐患,但荟萃分析仍提示来氟米特治疗RA有很好的疗效及安全性,所以,对于已存在肺部疾患的RA患者,应分析具体病因慎重应用来氟米特,并定期监测肺CT.%Interstitial lung disease(ILD) is one of the common extra-articular manifestations of rheumatoid arthritis(RA). Its incidence in RA is 7.7%, and the prognosis is poor. Leflunomide is one of the commonly used drugs for RA, but there are more and more reports about leflunomide caused interstitial pneumonia. Then, should leflunomide be used when RA is complicated with ILD? However, It has been reported that leflunomide can be an effective treatment for RA patient who already sufferred from lung disease [including ILD caused by methotrexate (MTX)], and no adverse drug reaction or pulmonary exacerbation was

  7. The application of High-resolution CT scanning in the diagnosis of interstitial lung disease%高分辨CT扫描技术在肺间质性病变中的应用价值

    Institute of Scientific and Technical Information of China (English)

    苏毅; 倪傲; 李源

    2012-01-01

    目的 探讨高分辨CT扫描技术,对肺间质性病变的诊断价值.方法 采用Philips MX8000 Dual 螺旋CT机对30例肺间质性病变患者,行高分辨CT技术扫描.结果 高分辨CT可清晰显示肺间质性病变的各种征象,肺间质性病变的高分辨CT主要表现为:(1)磨玻璃样密度影;(2)肺小叶间隔增厚;(3)小叶内间质增生;(4)网格状影;(5)胸膜下弧线影;(6)蜂窝肺;(7)牵拉支气管扩张;(8)胸膜增厚.病变分布以两肺中外带、胸膜下为主,病灶自肺尖向肺底逐渐加重,两肺基底部病变明显.结论 高分辨CT扫描具有良好的空间分辨率,能细致、准确地反映肺间质病变的影像特征,对肺间质性病变具有重要的诊断价值.%Objective To study the value of HRCT scanning technology in improving the diagnosis of interstitial lung disease. Methods 30 cases of interstitial lung disease were scanned throng high-resolution CT with Philips MX8000 Dual CT machine. Results High-resolution CT can clearly show various signs of interstitial lung disease. The Interstitial lung disease of High-resolution CT Perform-ance:( 1 ) ground-glass opacity;( 2 ) pulmonary interlobular septal thickening; ( 3 ) intralobular interstitial thickening;( 4 ) grid-like shadow ;( 5 ) pleural shadow off the assembly line;( 6 ) cell lung;( 7 ) traction bronchiectasis; ( 8 ) pleural thickening. The lesion mainly distributes in lateral and under pleural of two lungs. The infection aggravates gradually from lung' s point to lung' s bottom. Conclusion The application of high-resolution CT scan in the interstitial lung disease has important diagnostic value. As the high-resolution CT scan with a good spatial resolution, can detail, accurately reflect the images of interstitial lung disease characterized.

  8. Humidifier disinfectant-associated children's interstitial lung disease: Computed tomographic features, histopathologic correlation and comparison between survivors and non-survivors

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hee Mang; Lee, Jin Seong; Do, Kyung-Hyun; Jung, Ah Young; Yoon, Chong Hyun; Cho, Young Ah [Asan Medical Center, University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Songpa-gu, Seoul (Korea, Republic of); Lee, Eun; Hong, Soo-Jong [Asan Medical Center, University of Ulsan College of Medicine, Department of Pediatrics, Songpa-gu, Seoul (Korea, Republic of); Kim, Seon-Ok [Asan Medical Center, University of Ulsan College of Medicine, Department of Clinical Epidemiology and Biostatistics, Songpa-gu, Seoul (Korea, Republic of); Jang, Se-Jin [Asan Medical Center, University of Ulsan College of Medicine, Departments of Pathology, Songpa-gu, Seoul (Korea, Republic of)

    2016-01-15

    To report radiologic findings with histopathologic correlations of humidifier disinfectant-associated children's interstitial lung disease (HD-chILD) and to compare computed tomography (CT) findings between survivors and non-survivors. Forty-seven children with HD-chILD (27.4 ± 12.4 months old) were categorized as survivors (n = 25) and non-survivors (n = 22). The patterns, distributions, and chronological changes in lung lesions at follow-up CT were investigated. Histopathologic correlations were performed in 23 patients. CT features were characterized by chronological changes, from consolidation to centrilobular opacities, and lesions eventually became faint centrilobular nodules. Histopathologic features were bronchocentric-distributed fibro-inflammatory lesions, which were more profound in the advanced stage than the early stage. Consolidation ≥30 % [hazard ratio (HR), 2.932], centrilobular opacities ≥60 % of the total lung volume (TLV; HR, 0.206) and spontaneous air leaks (HR, 3.457) were significant factors associated with patient survival, as per univariate analysis. Consolidation ≥30 % (HR, 3.519), centrilobular opacities ≥60 % (HR, 0.205) and diffuse ground glass opacity (GGO) ≥70 % of the TLV (HR, 3.521) were significant factors associated with patient survival, as determined via multivariate analysis. Distinctive chronological CT features were observed in the HD-chILD images. Spontaneous air leaks, consolidation, GGO, and centrilobular opacities were prognostic factors. (orig.)

  9. Pulmonary structure and function analysis in systemic sclerosis : clinical assessment of complicating interstitial lung disease and pulmonary vasculopathy

    NARCIS (Netherlands)

    Ninaber, Maarten

    2015-01-01

    In SSc, we evaluated the optimal percentile density score in SSc by quantitative CT densitometry, against pulmonary function. Lung volumes and the nth percentile density (between 1 and 99%) of the entire lungs were calculated from CT histograms. The nth percentile density is defined as the threshold

  10. Autoimmune thyroiditis in antinuclear antibody positive children without rheumatologic disease

    Directory of Open Access Journals (Sweden)

    Arkachaisri Thaschawee

    2010-05-01

    Full Text Available Abstract Background Children are commonly referred to a pediatric rheumatology center for the laboratory finding of an Anti-nuclear antibody (ANA of undetermined significance. Previous studies regarding adult rheumatology patients have supported an association between ANA and anti-thyroid antibodies, with the prevalence of thyroid antibodies being significantly higher in patients referred to a rheumatology center for an ANA without evidence of connective tissue disease compared to the general population. The purpose of the present study was to determine the frequency of thyroid antibodies in children referred to a pediatric rheumatology center for a positive ANA without evidence of a connective tissue disease. Methods A retrospective chart review was performed on children who were referred to our pediatric rheumatology center between August 2003 and March 2007 for positive ANA with concurrent thyroid antibody and thyroid function tests performed who did not fulfill criteria for a specific connective tissue disease. Laboratory and clinical features were recorded and analyzed. Mean and standard deviation were used to describe continuous data. Chi-square or Fisher's exact tests were used to compare proportions between variables. Results One-hundred and four ANA-positive patients with concurrent thyroid studies were evaluated (88% female, 93% Caucasian, mean age 11.9 ± 4.0 years. Half of patients had an ANA titer ≥ 1:320. The ANA pattern was speckled in 60% of the patients. Thyroid antibodies were detected in 30% of the patients. Anti-Thyroglobulin (ATG was detected in 29% and Anti-thyroid peroxidase (ATPO in 21% of the patients; of these children, 14% had hypothyroidism. ANA pattern and titer were not associated with anti-thyroid antibody positivity. Conclusion Thyroid antibodies associated with chronic lymphocytic thyroiditis, ATG and ATPO, were detected significantly higher in ANA-positive children without a rheumatologic condition (30% as

  11. [Idiopathic interstitial pneumonias in 2016].

    Science.gov (United States)

    Debray, M-P; Borie, R; Danel, C; Khalil, A; Majlath, M; Crestani, B

    2017-02-01

    Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.

  12. 肺泡生长异常所致婴幼儿肺间质疾病%Alveolar growth abnormalities interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    邹映雪

    2014-01-01

    虽然间质性肺疾患可见于儿童生长发育的各个时期,但婴幼儿时期有其特殊类型的弥漫性肺间质疾病(diffuse interstitial lung disease,ILD),这种与肺发育直接相关的肺疾患与成人ILD不同.肺泡生长异常(alveolar growth abnormalities,AGA)是常见的导致婴幼儿ILD的原因,本文对此类婴幼儿特殊类型ILD的临床表现、诊断评估、治疗和临床转归进行探讨.

  13. Progress in the clinical diagnosis of interstitial lung diseases%间质性肺疾病的临床诊断进展

    Institute of Scientific and Technical Information of China (English)

    李惠萍

    2005-01-01

    随着工业的发展,空气污染日益严重;加上吸烟、室内装饰材料的大量使用、烹调油烟及不良的卫生习惯等因素,使间质性肺疾病(interstitial lung disease,ILD)发病率呈逐年上升趋势。但目前医学界对ILD研究的重视仍显不足、认识水平尚有限。现将近些年来国内外关于ILD的研究进展和本人的一些学习体会在此与读者进行交流。

  14. Progress in the clinical treatment of interstitial lung diseases%间质性肺疾病的临床治疗进展

    Institute of Scientific and Technical Information of China (English)

    蔡后荣

    2005-01-01

    间质性肺疾病(Interstitial lung disease,ILD)是由一组异质性疾病组成的弥漫性、主要侵犯肺泡单位和肺泡周围组织、累及肺间质为主的疾病。尽管其病因各异,但临床表现、影像学特点、肺功能测定、发病机制及病理学改变等均有某些相似之处或共性。ILD的治疗并没有统一的治疗方案。大多数ILD目前所采用的治疗方法通常是综合性而非特异性治疗。

  15. Role of α1 and α2 chains of type IV collagen in early fibrotic lesions of idiopathic interstitial pneumonias and migration of lung fibroblasts.

    Science.gov (United States)

    Urushiyama, Hirokazu; Terasaki, Yasuhiro; Nagasaka, Shinya; Terasaki, Mika; Kunugi, Shinobu; Nagase, Takahide; Fukuda, Yuh; Shimizu, Akira

    2015-08-01

    Early fibrotic lesions are thought to be the initial findings of fibrogenesis in idiopathic interstitial pneumonias, but little is known about their properties. Type IV collagen comprises six gene products, α1-α6, and although it is known as a major basement membrane component, its abnormal deposition is seen in fibrotic lesions of certain organs. We studied the expression of type I and III collagen and all α chains of type IV collagen in lung specimens from patients with usual interstitial pneumonia (UIP) or organizing pneumonia (OP) via immunohistochemistry. With cultured lung fibroblasts, we analyzed the expression and function of all α chains of type IV collagen via immunohistochemistry, western blotting, real-time quantitative PCR, and a Boyden chamber migration assay after the knockdown of α1 and α2 chains. Although we observed type I and III collagens in early fibrotic lesions of both UIP and OP, we found type IV collagen, especially α1 and α2 chains, in early fibrotic lesions of UIP but not OP. Fibroblasts enhanced the expression of α1 and α2 chains of type IV collagen after transforming growth factor-β1 stimulation. Small interfering RNA against α1 and α2 chains increased fibroblast migration, with upregulated phosphorylation of focal adhesion kinase (FAK), and adding medium containing fibroblast-produced α1 and α2 chains reduced the increased levels of fibroblast migration and phosphorylation of FAK. Fibroblasts in OP were positive for phosphorylated FAK but fibroblasts in UIP were not. These results suggest that fibroblasts in UIP with type IV collagen deposition, especially α1 and α2 chains, have less ability to migrate from early fibrotic lesions than fibroblasts in OP without type IV collagen deposition. Thus, type IV collagen deposition in early fibrotic lesions of UIP may be implicated in refractory pathophysiology including migration of lesion fibroblasts via a FAK pathway.

  16. Increased Risks of Spontaneous Bacterial Peritonitis and Interstitial Lung Disease in Primary Biliary Cirrhosis Patients With Concomitant Sjögren Syndrome.

    Science.gov (United States)

    Chen, Chun-Ting; Tseng, Yu-Chen; Yang, Chih-Wei; Lin, Hsuan-Hwai; Chen, Peng-Jen; Huang, Tien-Yu; Shih, Yu-Lueng; Chang, Wei-Kuo; Hsieh, Tsai-Yuan; Chu, Heng-Cheng

    2016-01-01

    The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients.The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant.Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients.

  17. Effect of mixing scanner types and reconstruction kernels on the characterization of lung parenchymal pathologies: emphysema, interstitial pulmonary fibrosis and normal non-smokers

    Science.gov (United States)

    Xu, Ye; van Beek, Edwin J.; McLennan, Geoffrey; Guo, Junfeng; Sonka, Milan; Hoffman, Eric

    2006-03-01

    In this study we utilize our texture characterization software (3-D AMFM) to characterize interstitial lung diseases (including emphysema) based on MDCT generated volumetric data using 3-dimensional texture features. We have sought to test whether the scanner and reconstruction filter (kernel) type affect the classification of lung diseases using the 3-D AMFM. We collected MDCT images in three subject groups: emphysema (n=9), interstitial pulmonary fibrosis (IPF) (n=10), and normal non-smokers (n=9). In each group, images were scanned either on a Siemens Sensation 16 or 64-slice scanner, (B50f or B30 recon. kernel) or a Philips 4-slice scanner (B recon. kernel). A total of 1516 volumes of interest (VOIs; 21x21 pixels in plane) were marked by two chest imaging experts using the Iowa Pulmonary Analysis Software Suite (PASS). We calculated 24 volumetric features. Bayesian methods were used for classification. Images from different scanners/kernels were combined in all possible combinations to test how robust the tissue classification was relative to the differences in image characteristics. We used 10-fold cross validation for testing the result. Sensitivity, specificity and accuracy were calculated. One-way Analysis of Variances (ANOVA) was used to compare the classification result between the various combinations of scanner and reconstruction kernel types. This study yielded a sensitivity of 94%, 91%, 97%, and 93% for emphysema, ground-glass, honeycombing, and normal non-smoker patterns respectively using a mixture of all three subject groups. The specificity for these characterizations was 97%, 99%, 99%, and 98%, respectively. The F test result of ANOVA shows there is no significant difference (p <0.05) between different combinations of data with respect to scanner and convolution kernel type. Since different MDCT and reconstruction kernel types did not show significant differences in regards to the classification result, this study suggests that the 3-D AMFM can

  18. Rare Association of Anti-Hu Antibody Positive Paraneoplastic Neurological Syndrome and Transitional Cell Bladder Carcinoma

    Directory of Open Access Journals (Sweden)

    S. Lukacs

    2012-01-01

    Full Text Available Introduction. Paraneoplastic encephalomyelitis (PEM and subacute sensory neuronopathy (SSN are remote effects of cancer, usually associated with small-cell lung carcinoma and positive anti-Hu antibody. We describe the rare association of bladder transitional cell carcinoma (TCC with anti-Hu antibody positivity resulting in this paraneoplastic neurological syndrome. Patient. A 76-year-old female presented with bilateral muscle weakness and paraesthesia of the upper and lower limbs in a length-dependent “glove and stocking” distribution. Central nervous system symptoms included cognitive problems, personality change, and truncal ataxia. Case notes and the literature were reviewed. Result. Autoantibody screening was positive for anti-Hu antibody (recently renamed antineuronal nuclear antibody 1, ANNA-1. The diagnosis of PEM and SSN was supported by MRI and lumbar puncture results. A superficial bladder TCC was demonstrated on CT and subsequently confirmed on histology. No other primary neoplasm was found on full-body imaging. The neurological symptoms were considered to be an antibody-mediated paraneoplastic neurological syndrome and improved after resection of the tumour. Discussion. The association of anti-Hu positive paraneoplastic neurological syndrome and TCC has not been described in the literature previously. We emphasize the need for detailed clinical examination and the importance of a multidisciplinary thought process and encourage further awareness of this rare association.

  19. Comprometimento do interstício pulmonar em portadores de esclerose sistêmica progressiva: estudo de uma série de 58 casos Interstitial lung disease in patients with progressive systemic sclerosis: a study of 58 cases

    Directory of Open Access Journals (Sweden)

    Sergio Fernandes de Oliveira Jezler

    2005-08-01

    Full Text Available OBJETIVO: Estimar a freqüência de doença intersticial pulmonar em um grupo de indivíduos com esclerose sistêmica progressiva e descrever suas características clínicas, funcionais e radiológicas. MÉTODOS: Após confirmação diagnóstica, 58 pacientes com esclerose sistêmica progressiva foram estudados com tomografia computadorizada de alta resolução, provas de função pulmonar, bem como pesquisa do anti-Scl 70. Foram feitas comparações entre os pacientes com e sem comprometimento intersticial pulmonar e pesquisados possíveis fatores preditivos deste acometimento através de análise multivariada. RESULTADOS: Do total, 51,7% apresentaram evidências de doença intersticial pulmonar na tomografia computadorizada de alta resolução. Dispnéia e tosse foram os sintomas mais relatados, (65,5% e 39,7%, respectivamente. Bronquiolectasias e faveolamento foram as anormalidades tomográficas mais comuns (83,3% e 80,0% respectivamente. Quando comparados com os indivíduos sem doença intersticial pulmonar, os pacientes com este acometimento apresentaram freqüência semelhante de sintomas pulmonares e extrapulmonares, porém apresentaram esclerose sistêmica progressiva de maior duração, estertores crepitantes mais freqüentes, maior positividade de anti-Scl 70, e capacidade vital forçada e pulmonar total reduzidas. Somente uma capacidade vital forçada OBJECTIVE: To estimate the frequency of interstitial lung disease in a group of patients with progressive systemic sclerosis, and to describe the clinical, functional and radiological characteristics of the patients studied. METHODS: Fifty-eight patients diagnosed with progressive systemic sclerosis were submitted to high-resolution computed tomography of the chest, pulmonary function tests and a blood test for anti-Scl 70 antibodies. Comparisons were drawn between patients with interstitial lung disease and those without. Logistic regression with multivariate analysis was used to

  20. High-resolution computed tomography versus chest radiography in the diagnosis of interstitial lung disease in systemic sclerosis; Avaliacao da tomografia de alta resolucao versus radiografia de torax na doenca intersticial pulmonar na esclerose sistemica

    Energy Technology Data Exchange (ETDEWEB)

    Azevedo, Ana Beatriz Cordeiro de; Calderaro, Debora; Moreira, Caio [Minas Gerais Univ., Belo Horizonte, MG (Brazil). Hospital das Clinicas. Servico de Reumatologia; Guimaraes, Silvana Mangeon Meirelles [Gerais Univ., Belo Horizonte, MG (Brazil). Hospital das Clinicas. Secao de Tomografia Computadorizada; Tavares Junior, Wilson Campos; Leao Filho, Hilton Muniz; Andrade, Diego Correa de [Minas Gerais Univ., Belo Horizonte, MG (Brazil). Hospital das Clinicas. Servico de Radiologia e Diagnostico por Imagem]. E-mail: wilsontavaresjrmd@yahho.fr; Ferreira, Cid Sergio; Vieira, Jose Nelson Mendes [Minas Gerais Univ., Belo Horizonte, MG (Brazil). Faculdade de Medicina. Dept. de Radiologia

    2005-04-01

    Objective: To compare the accuracy of high-resolution computed tomography (HRCT) with chest radiography in the diagnosis of interstitial lung disease in systemic sclerosis (SSc). Materials And Methods: HRCT scans and chest radiographs in postero-anterior and lateral views were performed in 34 patients with systemic sclerosis, according to the American College of Rheumatology preliminary criteria for the diagnosis of SSc. The prevalence of radiological findings suggestive of interstitial lung disease in SSc seen on both imaging methods was compared. Results: Interstitial disease was observed on HRCT images of 31 patients (91%) and in the chest radiographs of 16 patients (47%). The most frequent findings observed on HRCT were septal lines (74%), honeycombing (56%) and parenchymal bands (26%). Chest radiographs showed reticular areas of attenuation in 11 patients (32%) and parenchymal distortion in 12% of the patients. In 18 patients (53%) with normal chest radiographs HRCT showed septal lines in 55%, ground glass in 44%, honeycombing in 38.5% and cysts in 33%. Conclusion: HRCT is more sensitive than chest radiography in the evaluation of incipient interstitial lung involvement in patients with SSc and can provide a justification for immunosuppressive therapy in patients with early disease. (author)

  1. Doença pulmonar intersticial crônica na criança Chronic interstitial lung disease in children

    Directory of Open Access Journals (Sweden)

    Maria Aparecida S. S. Paiva

    2007-06-01

    conduct in a group of pediatric patients with chronic interstitial lung disease. METHODS: A retrospective study of 25 immunocompetent patients, aged 2 months to 17 years, with chronic interstitial lung disease, admitted to the Pediatric Pulmonary Section, Department of Pediatrics, Hospital dos Servidores do Estado, over a 20-year period (1984-2004. A routine protocol for persistent chronic pneumonias was used and the patients with interstitial lung disease were selected. Clinical, laboratory and imaging data were analyzed. RESULTS: Twenty-five patients were diagnosed with chronic interstitial lung disease, 13 were aged less than 2 years and 17 were male. Diagnoses were made based on history, physical examination and routine tests in one case, based on more complex tests in three cases and based on the results of invasive tests in 21 cases (20 by lung biopsy and one by bronchoalveolar lavage. Except for one patient with pulmonary lymphangiectasia, the long-term treatment (1 to 7 years consisted of corticosteroid, in six cases associated with hydroxychloroquine. Four patients required home oxygen therapy. The authors followed the patients in the outpatient department (6 a 8 visits/year. Patient outcome was: good (15; regular, with mild sequelae (4; and poor, with severe sequelae (3. One patient was lost in the follow-up period and two died. CONCLUSIONS: Chronic interstitial lung diseases in children are a group of rare pulmonary disorders, but a relevant one because of the possible progression to pulmonary fibrosis. Early diagnosis and a long-term, specialized treatment and follow-up are important for the patient outcome. Pediatricians should be aware of these diseases because in many cases diagnosis and treatment are overlooked.

  2. Clearance of /sup 99m/-technetium-labelled DTPA in asbestos-exposed subjects without clinical or radiological evidence of interstitial lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Gellert, A.R.; Langford, J.A.; Winter, R.J.; Lewis, C.A.; Tolfree, S.E.; Rudd, R.M.

    1985-01-01

    The half-time clearance of an inhaled aerosol of /sup 99m/-technetium-labelled diethylene triamine pentacetate from lung to blood (T1/2LB) was measured using a gamma camera in 20 non-smoking subjects (mean age 54, range 40-69 years) with previous occupational asbestos exposure, but no clinical or radiological evidence of asbestosis, and 20 non-smoking normal subjects (mean age 54, range 40-62). Mean T1/2LB was 44.7 minutes (range 12-102) in exposed subjects, significantly less than 57.2 minutes (range 30.5-109) in normal subjects (P less than 0.05). There was no correlation between age and T1/2LB in either group. In exposed subjects T1/2LB showed a weak rank correlation with the membrane component of DLco (Dm) (r = 0.40, P less than 0.05) but no significant correlation with FVC, TLC, DLco, Kco, resting Pao2 or change in Pao2 on exercise. In six exposed subjects T1/2LB was shorter than in any of the normal subjects. These six did not differ from the other 14 exposed subjects in any physiological variables. T1/2LB is abnormal in some asbestos-exposed subjects without clinical, radiological or physiological evidence of asbestosis. Follow-up will show whether it is an early indicator of development of interstitial lung disease.

  3. Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Karina Portillo

    2012-01-01

    Full Text Available Combined pulmonary fibrosis and emphysema (CPFE is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.

  4. CLINICAL EVALUATION OF THE MANIFESTATIONS OF INTERSTITIAL LUNG INJURYIN SYSTEMIC SCLERODERMA FROM HIGH-RESOLUTION COMPUTER T OMOGRAPHY DATA

    Directory of Open Access Journals (Sweden)

    L P Anan'eva

    2011-01-01

    Conclusion. Chest HRCT reveals the characteristic symptoms of ILI and reflects different phases of a fibrosing process in the lung. It is essential to make an in-depth examination using HRCT in all patients with SDD, irrespective of its clinical form in the earliest periods for the timely detection and treatment of ILI.

  5. Clinical analysis of 305 patients with connective tissue disease associated interstitial lung disease%305例结缔组织病相关间质性肺病临床特点分析

    Institute of Scientific and Technical Information of China (English)

    谢荣华; 吴振彪; 贾俊峰; 韩青; 朱平

    2016-01-01

    目的:分析结缔组织病相关间质性肺病( Connective tissue disease associated interstitial lung dis-ease,CTD-ILD)临床特征。方法回顾性分析305例西京医院临床免疫科CTD-ILD的临床特征及类风湿关节炎间质性肺病( Rheumatoid arthritis associated interstitial lung disease, RA-ILD )和干燥综合征间质性肺病( Sjogren's syndrome associated interstitial lung disease,pSS-ILD)患者胸部高分辨CT特点。结果 CTD患者ILD的发病率为11.78%,其中多发性肌炎相关ILD的发病率最高(53.13%),其次为抗中性粒细胞胞浆抗体相关性血管炎(40.74%)、混合结缔组织病(35.14%)和硬皮病(29.73%)。 pSS-ILD和RA-ILD在CTD-ILD中所占比例最大,分别为24.92%和23.61%,两者约占所有CTD-ILD的一半。结论 CTD患者ILD的发病率高, RA-ILD和pSS-ILD是CTD-ILD的主要疾病,加强对CTD-ILD的早期筛查和早期治疗非常重要。%Objective To analyze the clinical features of connective tissue disease associated interstitial lung disease (CTD-ILD). Methods The clinical manifestation of 305 patients diagnosed CTD-ILD and chest high resolu-tion computed tomography features of rheumatoid arthritis associated interstitial lung disease ( RA-ILD) and Sjogren's syndrome associated interstitial lung disease ( pSS-ILD) were retrospectively analyzed. Results The total incidence of ILD in CTD was 11. 78%. The highest incidence of ILD was polymyositis ( 53. 13%) , then was anti-neutrophil cytoplasmic antibody associated vasculitis (40. 74%), mixed connective tissue disease (35. 14%) and scleroderma (29. 73%). pSS-ILD and RA-ILD were the major part of the total CTD-ILD with the proportion of 24. 92% and 23. 61% respectively and almost occupied the half of all CTD-ILD patients. Conclusion The incidence of CTD-ILD is very high. pSS-ILD and RA-ILD are the main diseases of CTD-ILD, and the early screening and treatment is very important.

  6. Discussion on TCM Syndrome Differentiation and Treatment of Interstitial Lung Disease from the Deficiency of Pectoral Qi%从“宗气亏虚”探讨间质性肺疾病的中医辨治

    Institute of Scientific and Technical Information of China (English)

    景海卿; 付义

    2015-01-01

    间质性肺疾病是临床多发病及常见病,其发病机理不明,西医临床缺乏有效的治疗手段。而中医通过临床观察,发现本病与“宗气亏虚”有着密切关系,故可采用升补宗气治疗间质性肺疾病,且临床疗效显著。%Interstitial lung disease is a common disease.Its pathogenic mechanism is unclear, and the clinical Western medicine lacks of effective method.The pectoral qi assembles in chest, and is the pivot of respiratory, which promotes the movement of the lung.Through clinical observation, we found that the interstitial lung disease had a close relationship with the deficiency of pectoral qi, and the deficiency of pectoral qi throughout the disease.We treated interstitial lung disease with promoting and invigorating pectoral qi.Using the modified Shengxian decoction of Zhang Xichun, promoting and invigorating pectoral qi as well as invigorating and ascending yang qi, which can effectively alleviate symptom.The quality of life was improved.

  7. Interstitial lung disease in children: a report of two cases and review of literature%儿童间质性肺疾病2例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    车大钿; 陆权; 陆敏; 董晓艳; 张慧燕; 吴蓓蓉

    2012-01-01

    目的 总结间质性肺疾病的诊断,旨在引起临床重视.方法 回顾性分析2例按临床-放射-病理学(CRP)模式确诊的儿童间质性肺疾病病例,探讨其临床特征、胸部影像学特征及病理学表现.结果 2例患儿均有咳嗽、气促,但均无杵状指趾.病原学检查无感染证据,也无结缔组织疾病证据.胸部高分辨CT均见磨玻璃影和囊泡影.经开胸肺活检,1例诊断为类脂性肺炎,1例为脱屑性间质性肺炎.结论 儿童间质性肺疾病无特征性临床表现,对病情迁延、常规治疗效果不明显的患儿,应警惕间质性肺疾病.%Objective To explore the importance of lung biopsy in diagnosis of interstitial lung disease. Methods Two cases of interstitial lung disease in children, diagnosed by pathological examination, were reviewed and analyzed retrospectively. Their clinical features, chest radiology and pathology were studied. The final diagnosis was made based on the principle of clinico-radiologic-pathologic diagnosis (C-R-P). Results Two patients presented with cough and shortness of breath, no clubbing of fingers and toes observed. Ground-glass opacities and vesicles shadow were presented under high resolution CT. Neither the evidence of microbial infection nor connective tissue disease was found. In this case, lung biopsies were needed to identify the causes of the infection. The final diagnoses were made after lung biopsy that one child was lipid pneumonia and another one was desquamative interstitial pneumonia. Conclusions The respiratory symptoms of interstitial lung disease in children include cough, wheezing and shortness of breath. For those children who do not show obvious effect after conventional treatment physician should consider the possibility of interstitial lung disease.

  8. Clinical analysis of 12 cases of lung cancer associated with interstitial lung diseases%12例间质性肺疾病合并肺癌临床分析

    Institute of Scientific and Technical Information of China (English)

    孙志宏; 许文兵; 冯瑞娥; 张晓彤; 田欣伦; 朱元珏

    2011-01-01

    目的 了解间质性肺疾病合并肺癌的临床特点和治疗.方法 回顾性分析12例北京协和医院住院患者中诊断间质性肺疾病合并肺癌患者的临床症状、影像学、病理、治疗及预后.结果 12例患者,平均年龄(61±8)岁,男女比例11:1,平均吸烟指数(26±5)包·年,8例为特发性肺纤维化.临床表现包括干咳(10/12)、活动后气短(9/12),肺癌发生后可出现咯血(1/12).肺癌病理类型以腺癌(4/12)和鳞癌(5/12)为主,还包括复合癌(2/12)、小细胞肺癌(1/12).胸部影像学表现为原有间质性疾病基础上新出现的结节团块影,主要在下叶外带(7/12).肺癌的治疗出现的并发症包括间质性肺疾病急性加重(1/12)、肺部感染(3/12).特发性肺纤维化合并肺癌占同期全部特发性肺纤维化住院患者15%(8/54),特发性肺纤维化合并肺癌组住院病死率(38%)高于单纯特发性肺纤维化组(15%).结论 间质性肺疾病合并肺癌发生率高,临床表现不特异,影像学检查有助于早期诊断,肺癌治疗易引起间质性肺疾病急性加重,慎重选择治疗方案,并密切监测治疗过程.%Objective To understand the clinical features and treatment of lung cancer associated with interstitial lung disease(ILD-Ca).Methods Twelve patients with ILD-Ca were retrospectively studied in Peking Union Medical College Hospital.Their clinical features,chest radiography,pathology,therapy and prognosis were analyzed.Results The mean age at presentation was(61±-8)years,11 were man.Lung cancer was found in(3± 2)years after the diagnosis of ILD.The average smoking index was (26±5)packet · year.There were eight cases of idiopathic pulmonary fibrosis(IPF),three cases of rheumatoid arthritis and one case of nonspecific interstitial lung disease(NSIP).The clinical presentations included dry cough(10/12),shortness of breath(10/12),inspiratory crepitus(10/12)and acropachy (10/12).Those ILD patients had exacerbation of the above(3

  9. High-resolution computed tomography interpretation model for differential diagnosis of chronic interstitial lung diseases; Modelo de interpretacao da tomografia computadorizada de alta resolucao do diagnostico diferencial das doencas intersticiais cronicas

    Energy Technology Data Exchange (ETDEWEB)

    Silva, C. Isabela S.; Mueller, Nestor L. [Vancouver General Hospital, BC (Canada). Dept. of Radiology]. E-mail: nmuller@vanhosp.bc.ca

    2005-04-01

    The recognition and differential diagnosis of the various patterns of abnormality seen on high-resolution computed tomography (HRCT) are important in the evaluation of patients with chronic interstitial lung diseases. Various patterns of abnormality have been described in the literature, which sometimes are overlapped. Distinction between the various patterns can be difficult particularly for residents and radiologists who are not familiarized with these images. The authors illustrate the characteristic patterns of abnormality seen in chronic interstitial lung diseases and propose a simple interpretation algorithm based on the pattern and distribution of the findings. The algorithm includes the six main patterns of abnormality seen on HRCT: septal lines, reticular pattern, cystic pattern, nodular pattern, ground-glass opacities and consolidation. (author)

  10. 高分辨率CT在儿童间质性肺疾病诊断中的应用%High resolution CT in the diagnosis of pediatric interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    彭芸

    2012-01-01

    儿童间质性肺疾病与成人有不同的特点,正确诊断需要依靠临床、放射和病理相结合,其中高分辨CT(high-resolution CT,HRCT)在诊断中发挥了重要的作用.文章就HRCT在儿童间质性肺疾病临床诊断中的应用作一概述.%Pediatric interstitial lung disease presents different characteristics versus that in adult. Diagnosis needs to be based on comprehensive understanding of clinical presentation, imaging and pathology. High-resolution computed tomography plays an important role in diagnosis. This article summarizes the application of this technology in the diagnosis of pediatric interstitial lung disease.

  11. Effects of long-term low-dose oxygen supplementation on the epithelial function, collagen metabolism and interstitial fibrogenesis in the guinea pig lung

    Directory of Open Access Journals (Sweden)

    Ishizaka Akitoshi

    2008-04-01

    Full Text Available Abstract Background The patient population receiving long-term oxygen therapy has increased with the rising morbidity of COPD. Although high-dose oxygen induces pulmonary edema and interstitial fibrosis, potential lung injury caused by long-term exposure to low-dose oxygen has not been fully analyzed. This study was designed to clarify the effects of long-term low-dose oxygen inhalation on pulmonary epithelial function, edema formation, collagen metabolism, and alveolar fibrosis. Methods Guinea pigs (n = 159 were exposed to either 21% or 40% oxygen for a maximum of 16 weeks, and to 90% oxygen for a maximum of 120 hours. Clearance of inhaled technetium-labeled diethylene triamine pentaacetate (Tc-DTPA and bronchoalveolar lavage fluid-to-serum ratio (BAL/Serum of albumin (ALB were used as markers of epithelial permeability. Lung wet-to-dry weight ratio (W/D was measured to evaluate pulmonary edema, and types I and III collagenolytic activities and hydroxyproline content in the lung were analyzed as indices of collagen metabolism. Pulmonary fibrotic state was evaluated by histological quantification of fibrous tissue area stained with aniline blue. Results The clearance of Tc-DTPA was higher with 2 week exposure to 40% oxygen, while BAL/Serum Alb and W/D did not differ between the 40% and 21% groups. In the 40% oxygen group, type I collagenolytic activities at 2 and 4 weeks and type III collagenolytic activity at 2 weeks were increased. Hydroxyproline and fibrous tissue area were also increased at 2 weeks. No discernible injury was histologically observed in the 40% group, while progressive alveolar damage was observed in the 90% group. Conclusion These results indicate that epithelial function is damaged, collagen metabolism is affected, and both breakdown of collagen fibrils and fibrogenesis are transiently induced even with low-dose 40% oxygen exposure. However, these changes are successfully compensated even with continuous exposure to low

  12. 99mTc-MIBI Lung Scintigraphy in the Assessment of Pulmonary Involvement in Interstitial Lung Disease and Its Comparison With Pulmonary Function Tests and High-Resolution Computed Tomography: A Preliminary Study.

    Science.gov (United States)

    Bahtouee, Mehrzad; Saberifard, Jamshid; Javadi, Hamid; Nabipour, Iraj; Raeisi, Alireza; Assadi, Majid; Eftekhari, Mohammad

    2015-11-01

    The differentiation of active inflammatory processes from an inactive form of the disease is of great value in the management of interstitial lung disease (ILD). The aim of this investigation was to assess the efficacy of 99mTc-methoxy-isobutyl-isonitrile (99mTc-MIBI) scans in distinguishing the severity of the disease compared to radiological and clinical parameters.In total, 19 known cases of ILD were included in this study and were followed up for 1 year. Five patients without lung disease were considered as the control group. The patients underwent pulmonary function tests (PFTs) and high-resolution computed tomography scans, followed by 99mTc-MIBI scanning. The 99mTc-MIBI scans were analyzed either qualitatively (subjectively) or semiquantitatively.All 19 ILD patients demonstrated a strong increase in 99mTc-MIBI uptake in the lungs compared to the control group. The 99mTc-MIBI scan scores were higher in the patient group in both the early phase (0.24[0.19-0.31] vs 0.11[0.10-0.15], P 0.14). The 99mTc-MIBI scan scores were not significantly correlated with the PFT findings (P > 0.05). In total, 5 patients died and 14 patients were still alive over the 1-year follow-up period. There was also a significant difference between the uptake intensity of 99mTc-MIBI and the outcome in the early phase (dead: 0.32[0.29-0.43] vs alive: 0.21[0.18-0.24], P pulmonary involvement in early views, which were well correlated with HRCT findings. These results also revealed that 99mTc-MIBI lung scans might be used as a complement to other diagnostic and clinical examinations in terms of functional information in ILD; however, further investigations are strongly required.

  13. 间质性肺疾病的临床特点影像学特征及病理类型分析%The clinical characteristics of interstitial lung disease,imaging characteristics and pathologic types of analysis

    Institute of Scientific and Technical Information of China (English)

    温杰冉; 唐雪玲; 谢永平; 黎国梅; 巫嘉雯

    2013-01-01

      目的了解间质性肺疾病的临床特点、影像学特征和病理类型。方法对18例间质性肺疾病患者的临床表现、胸CT或高分辨率CT(HRCT)、肺组织病理特点进行分析。结果18例患者均有咳嗽,其中12例伴有呼吸困难,4例肺内可闻及湿啰音,3例有杵状指趾。胸CT或HRCT示:15例以磨玻璃影为主,2例弥散性细小结节影,1例多发实变影。据病理改变,18例间质性肺炎包括非特异性6例,急性3例,淋巴细胞性1例,未分化型1例,隐源性机化性肺炎2例,呼吸性细支气管炎伴间质性肺炎1例,弥散性泛细支气管炎2例,肺泡微石症、类脂性肺炎各1例。结论间质性肺疾病是一组异质性疾病,临床表现以咳嗽、呼吸困难为主,胸部影像学以磨玻璃影为主,不同的间质性肺疾病病理表现各异。%Objective To understand the clinical characteristics of interstitial lung disease, imaging characteristics and pathologic types. Methods To diffuse pulmonary disease in 18 patients clinical manifestations, Chest CT and high resolution CT (HRCT) and lung tissue pathology characteristic carries on the analysis.Results Clinical manifestations:18 cases were cough, 12 cases with have difficulty breathing, audible and wet rale in the 4 cases of lungs, 3 cases with finger clubbing toe. Chest CT and HRCT shows:15 cases with ground glass shadow is given priority to,2 cases of diffuse small nodular shadows, 1 case of multiple consolidation shadows. According to the pathological changes, 18 cases of interstitial pneumonia included in 6 cases of nonspecific interstitial pneumonia (NSIP) and acute interstitial pneumonia (AIP) in 3 patients, hidden source sex machine pneumonia in 2 cases, 1 lymphocytic interstitial pneumonia, respiratory bronchiolitis associated with interstitial pneumonia (RBILD) in 1 case, undifferentiated type, 1 case of interstitial pneumonia, 4 cases including diffuse extensive

  14. Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study.

    Science.gov (United States)

    Oguz, Ekin Oktay; Kucuksahin, Orhan; Turgay, Murat; Yildizgoren, Mustafa Turgut; Ates, Askin; Demir, Nalan; Kumbasar, Ozlem Ozdemir; Kinikli, Gulay; Duzgun, Nursen

    2016-03-01

    It was aimed to evaluate KL-6 glycoprotein levels to determine if it may be a diagnostic marker for the connective tissue diseases (CTDs) predicting CTD-related interstitial lung diseases (ILDs) (CTD-ILD) development and to examine if there was a difference between patients and healthy controls. The study included 113 patients with CTD (45 CTD without lung involvement, 68 CTD-ILD) and 45 healthy control subjects. KL-6 glycoprotein levels were analyzed with ELISA in patients and the control group. The relationship between KL-6 glycoprotein levels and CTD-ILD was assessed. In the comparison of all the groups in the study, significantly higher levels of KL-6 were determined in the CTD-ILD group than in either the CTD without pulmonary involvement group or the healthy control group (p connective tissue diseases in the diagnostic groups (systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, mixed connective tissue disease, scleroderma, polymyositis/ dermatomyositis). In the healthy control group, there was a statistically significant difference between KL-6 levels in smokers and non-smokers. Smokers had significantly higher serum KL-6 levels compared with non-smokers (p < 0.05). There was no statistically significant difference between smoking status (pack-year) and serum KL-6 levels. There was no statistically significant correlation between serum KL-6 levels and time since diagnosis of CTD and CTD-ILD. The level of KL-6 as a predictive factor could be used to identify the clinical development of ILD before it is detected on imaging modality. Further prospective clinical studies are needed to define whether levels of KL-6 might have prognostic value or might predict progressive ILD.

  15. Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase Myositis-related interstitial lung disease and antisynthetase syndrome

    Directory of Open Access Journals (Sweden)

    Joshua Solomon

    2011-02-01

    Full Text Available Em pacientes com miosite, é comum o comprometimento pulmonar, e a presença de anticorpos anti-aminoacil-RNAt sintetase (anti-ARS é preditora da presença ou do desenvolvimento de doença pulmonar intersticial (DPI. Uma entidade clínica distinta - a síndrome antissintetase - é caracterizada pela presença de anticorpos anti-ARS, miosite, DPI, artrite, fenômeno de Raynaud e "mãos de mecânico". O mais comum anticorpo anti-ARS é o anti-Jo-1. Anticorpos anti-ARS mais recentemente descritos podem conferir um fenótipo que é distinto daquele de pacientes com positividade para anti-Jo-1, sendo caracterizado por uma menor incidência de miosite e uma maior incidência de DPI. Nos pacientes com DPI relacionada à síndrome antissintetase, a resposta a medicações imunossupressoras é em geral favorável.In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS antibodies marks the presence or predicts the development of interstitial lung disease (ILD. A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

  16. [Roentgenographic pattern of interstitial pneumonia and allergic alveolitis (author's transl)].

    Science.gov (United States)

    Stender, H S

    1977-01-01

    Roentgenographic examination of the lungs permits diagnosis of inflammatory and allergic pulmonary disease with predominantly interstitial and less alveolar involvement in which pulmonary fibrosis may develop. Reaction of the sensitised lung to allergic exposure causes typical roentgenological patterns. Development of pulmonary fibrosis in interstitial lung disease can be prevented be early cortison therapy.

  17. Reconciling healthcare professional and patient perspectives in the development of disease activity and response criteria in connective tissue disease-related interstitial lung diseases.

    Science.gov (United States)

    Saketkoo, Lesley Ann; Mittoo, Shikha; Frankel, Sid; LeSage, Daphne; Sarver, Catherine; Phillips, Kristine; Strand, Vibeke; Matteson, Eric L

    2014-04-01

    Interstitial lung diseases (ILD), including those related to connective tissue disease (CTD), and idiopathic pulmonary fibrosis (IPF) carry high morbidity and mortality. Great efforts are under way to develop and investigate meaningful treatments in the context of clinical trials. However, efforts have been challenged by a lack of validated outcome measures and by inconsistent use of measures in clinical trials. Lack of consensus has fragmented effective use of strategies in CTD-ILD and IPF, with a history of resultant difficulties in obtaining agency approval of treatment interventions. Until recently, the patient perspective to determine domains and outcome measures in CTD-ILD and IPF had never been applied. Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. Regarding CTD-ILD, this is the first OMERACT working group to directly address a manifestation/comorbidity of a rheumatic disease (ILD) as well as a disease not considered rheumatic (IPF). The OMERACT 11 proceedings of the CTD-ILD Working Group describe the forward and lateral process to include both the medical and patient perspectives in the urgently needed identification of a core set of preliminary domains and outcome measures in CTD-ILD and IPF.

  18. Successful polymyxin B hemoperfusion treatment associated with serial reduction of serum anti-CADM-140/MDA5 antibody levels in rapidly progressive interstitial lung disease with amyopathic dermatomyositis.

    Science.gov (United States)

    Teruya, Aoi; Kawamura, Kodai; Ichikado, Kazuya; Sato, Shinji; Yasuda, Yuko; Yoshioka, Masakazu

    2013-12-01

    Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.

  19. NT-proBNP <95 ng/l can exclude pulmonary hypertension on echocardiography at diagnostic workup in patients with interstitial lung disease

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    Charlotte Andersen

    2016-07-01

    Full Text Available Background: Pulmonary hypertension (PH is a serious complication to interstitial lung disease (ILD and has a poor prognosis. PH is often diagnosed by screening with echocardiography followed by right heart catheterisation. A previous study has shown that a value of NT-pro-brain natriuretic peptide (NT-proBNP 95 ng/l for signs of PH on echocardiography were calculated. The study was approved by the Danish Health Authority. Results: In 118 patients, data from both echocardiography and measurements of NT-proBNP were available. Eleven of these were screened positive for PH on echocardiography. Sensitivity, specificity, NPV and PPV of NT-proBNP <95 ng/l for PH were 100, 44, 16 and 100%, respectively. Furthermore, no patients with left heart failure as the cause of dyspnoea were missed using this cut-off value. Conclusion: NT-proBNP <95 ng/l precludes a positive echocardiographic screen for PH in ILD patients at referral for diagnostic workup.

  20. Perfusion- and pattern-based quantitative CT indexes using contrast-enhanced dual-energy computed tomography in diffuse interstitial lung disease: relationships with physiologic impairment and prediction of prognosis

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Jung Won [Sungkyunkwan University School of Medicine, Department of Radiology, Kangbuk Samsung Hospital, Seoul (Korea, Republic of); Bae, Jang Pyo; Kim, Namkug; Chang, Yongjun; Seo, Joon Beom [University of Ulsan College of Medicine, Department of Radiology, Seoul (Korea, Republic of); Lee, Ho Yun; Lee, Kyung Soo [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Chung, Man Pyo; Park, Hye Yun [Sungkyunkwan University School of Medicine, Department of Pulmonology, Samsung Medical Center, Seoul (Korea, Republic of)

    2016-05-15

    To evaluate automated texture-based segmentation of dual-energy CT (DECT) images in diffuse interstitial lung disease (DILD) patients and prognostic stratification by overlapping morphologic and perfusion information of total lung. Suspected DILD patients scheduled for surgical biopsy were prospectively included. Texture patterns included ground-glass opacity (GGO), reticulation and consolidation. Pattern- and perfusion-based CT measurements were assessed to extract quantitative parameters. Accuracy of texture-based segmentation was analysed. Correlations between CT measurements and pulmonary function test or 6-minute walk test (6MWT) were calculated. Parameters of idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) and non-IPF/UIP were compared. Survival analysis was performed. Overall accuracy was 90.47 % for whole lung segmentation. Correlations between mean iodine values of total lung, 50-97.5th (%) attenuation and forced vital capacity or 6MWT were significant. Volume of GGO, reticulation and consolidation had significant correlation with DLco or SpO{sub 2} on 6MWT. Significant differences were noted between IPF/UIP and non-IPF/UIP in 6MWT distance, mean iodine value of total lung, 25-75th (%) attenuation and entropy. IPF/UIP diagnosis, GGO ratio, DILD extent, 25-75th (%) attenuation and SpO{sub 2} on 6MWT showed significant correlations with survival. DECT combined with pattern analysis is useful for analysing DILD and predicting survival by provision of morphology and enhancement. (orig.)

  1. 吴银根辨治间质性肺疾病验案分析%Effective Cases Analysis of Professor WU Yingen in Differentiating and Treating Interstitial Lung Disease

    Institute of Scientific and Technical Information of China (English)

    石克华; 熊必丹; 吴银根

    2013-01-01

    通过验案分析,总结吴银根教授辨治间质性肺疾病的临床经验.其辨治要点是:认为本病的病位在肺络,病机为肺络痹阻,痰瘀互结.虚、痰、瘀是其主要病理特点,以通补肺络为大法,主张络虚宜通补,采用祛痰通络,活血化瘀,温阳益气,滋阴补肾等治法,善用峻烈、有毒和虫类药物.%This article introduced the clinical experience of Professor WU Yingen who differentiated and treated interstitial lung disease by analyzing effective cases. The important point of syndrome differentiation and treatment is that lung collaterals is the position of the disease, obstructing lung collaterals and intermingled phlegm and blood stasis are the basis pathogenesis of interstitial lung disease. Deficiency,phlegm and blood stasis are the main pathological features. And tonifying the lung collaterals is the method of treatment. Professor WU thinks that collaterals deficiency must be tonified,and the disease can be treated by using therapeutic principles and methods like reducing phlegm and vein relaxing, promoting blood circulation for removing blood stasis, wanning yang and benefiting qi, replenishing vital essence to tonify the kidney, and so on. Professor WU makes full use of drastic, toxic and insects medicine of TCM.

  2. 经支气管镜肺活检术对儿童间质性肺疾病的诊断价值%Diagnostic value of transbronchial lung biopsy in pediatric interstitial lung diseases

    Institute of Scientific and Technical Information of China (English)

    杨海明; 李惠民; 唐晓蕾; 李干; 赵顺英; 刘玺诚

    2015-01-01

    Objective To evaluate the diagnostic value of transbronchial lung biopsy (TBLB) in pediatric interstitial lung diseases.Methods Retrospective data evaluation the efficacy and application of 10 undiagnosed pediatric interstitial lung diseases patients by No-X-ray guided TBLB between December 2013 and December 2014 were investigated.Results Specimens from all cases were successfully obtained,6 patients were confirmed by pathological diagnoses,and4 patients were confirmed by pathology and clinical manifestations.Among 10 patients,3 of them had allergic alveolitis,2 cases had bronchiolitis obliterans organizing pneumonia (BOOP),1 had pulmonary vasculitis,2 cases had nonspecific interstitial pneumonia,and the other 2 had bronchiolitis obliterans.The complications include 2 pneumothorax,1 case was cured with thoracic cavity closed drainage,and the other case case did not receive special treatment and alleviated himself.No bleeding and other complications occurred.Conclusion The results suggest that TBLB is a safe and effective minimally invasive modality for the diagnosis of pediatric diffuse lung disease.%目的 评价非影像直视下经支气管镜肺活检术在儿童间质性肺部疾病中的诊断价值.方法 对2013年12月至2014年12月未能明确诊断、可耐受支气管镜检查的10例间质性肺部疾病患儿进行非影像直视下经支气管镜肺活检术,对其效果和相关并发症进行回顾性分析.结果 10例患儿均成功留取肺组织样本,6例经病理确诊,包括过敏性肺泡炎3例、闭塞性细支气管炎伴机化性肺炎(BOOP)2例、嗜酸细胞性多血管炎1例;其余4例病理结合临床确诊,包括非特异性间质性肺炎2例、闭塞性细支气管炎(BO)2例.术后发生气胸2例,1例经过胸腔穿刺引流缓解,1例未处理自行缓解,无一例发生明显出血和其他并发症.结论 经支气管镜肺活检术是儿童间质性肺疾病较为安全有效的诊断方法.

  3. 99mTc-IgG-Lung Scintigraphy in the Assessment of Pulmonary Involvement in Interstitial Lung Disease and Its Comparison With Pulmonary Function Tests and High-Resolution Computed Tomography: A Preliminary Study

    Directory of Open Access Journals (Sweden)

    Bahtouee

    2015-08-01

    Full Text Available Background The discrimination of inactive inflammatory processes from the active form of the disease is of great importance in the management of interstitial lung disease (ILD. Objectives The aim of this study was to determine the efficacy of 99mTc-IgG scan for the detection of severity of disease compared to high-resolution computed tomography (HRCT and pulmonary function test (PFT. Patients and Methods Eight known cases of ILD including four cases of Mustard gas (MG intoxication and four patients with ILD of unknown cause were included in this study. A population of six patients without lung disease was considered as the control group. The patients underwent PFT and high-resolution computed tomography, followed by 99mTc-IgG scan. They were followed up for one year. 99mTc-IgG scan assessment of IgG uptake was accomplished both qualitatively (subjectively and semiquantitatively. Results All eight ILD patients demonstrated a strong increase in 99mTc-IgG uptake in the lungs, compared to the control patients. The 99mTc-IgG scan scores were higher in the patient group (0.64[95% confidence interval(CI=0.61-0.69] than the control group (0.35 (0.35[95% CI=0.28-0.40], (P 0.05. There were no significant correlations between 99mTc-IgG score and HRCT patterns including ground glass opacity, reticular fibrosis and honeycombing (P value > 0.05. Conclusion The present results confirmed that 99mTc-IgG scan could be applied to detect the severity of pulmonary involvement, which was well correlated with HRCT findings. This data also showed that the 99mTc-IgG scan might be used as a complement to HRCT in the functional evaluation of the clinical status in ILD; however, further studies are recommended.

  4. 参与类风湿关节炎间质性肺疾病发病的细胞因子%Cytokines involved in rheumatoid arthritis interstitial lung diseases

    Institute of Scientific and Technical Information of China (English)

    孙宝旗; 宋宁; 张瑞芳; 吴建玲

    2013-01-01

    类风湿关节炎(rheumatoid arthritis,RA)易于合并间质性肺疾病(interstitial lung disease,ILD),其缺乏有效治疗手段,病死率高,危害大.RA-ILD发病机制不明,有假说推测初始激活因素激活肺部居留细胞释放促炎性细胞因子和趋化因子浸润肺组织,导致肺泡炎,炎细胞产生促纤维化介质,促进细胞外基质沉积并破坏肺泡及间质正常解剖结构,最终导致严重的结构和功能紊乱.近年来,人们陆续发现了多种细胞因子参与RA-ILD发病的直接证据,证实了细胞因子参与了RA-ILD的发病过程,本文对此进行综述.%Interstitial lung disease (ILD) is commonly accompanied with rheumatoid arthritis (RA),and lack of effective treatment,so the mortality of the disease is high.The pathogenesis of RAILD is unclear,it has been hypothesized that the initial activation factors may activate the resident cells in lung tissue to release pro-inflammatory cytokines and cause alveolitis,then,the inflammatory cells produce pro-fibrotic media to promote extracellular matrix deposition and alveolar and interstitial cells damage,the normal lung anatomic structure disappear,eventually lead to severe structural and functional disorders.In recent years,researchers have been discovered some direct evidences to conform that a variety of cytokines are involved in the pathogenesis of RA-ILD,the advanced researches of cytokines and RA-ILD are discussed in this article.

  5. Can Serum Surfactant Protein D or CC-Chemokine Ligand 18 Predict Outcome of Interstitial Lung Disease in Patients with Early Systemic Sclerosis?

    Science.gov (United States)

    Elhaj, Mona; Charles, Julio; Pedroza, Claudia; Liu, Xiaochun; Zhou, Xiaodong; Estrada-Y-Martin, Rosa M.; Gonzalez, Emilio B.; Lewis, Dorothy E.; Draeger, Hilda T.; Kim, Sarah; Arnett, Frank C.; Mayes, Maureen D.; Assassi, Shervin

    2013-01-01

    Objective To examine the predictive significance of 2 pneumoproteins, surfactant protein D (SP-D) and CC-chemokine ligand 18 (CCL18), for the course of systemic sclerosis (SSc)-related interstitial lung disease. Methods The pneumoproteins were determined in the baseline plasma samples of 266 patients with early SSc enrolled in the GENISOS observational cohort. They also were measured in 83 followup patient samples. Pulmonary function tests were obtained annually. The primary outcome was decline in forced vital capacity (FVC percentage predicted) over time. The predictive significance for longterm change in FVC was investigated by a joint analysis of longitudinal measurements (sequentially obtained FVC percentage predicted) and survival data. Results SP-D and CCL18 levels were both higher in patients with SSc than in matched controls (p < 0.001 and p = 0.015, respectively). Baseline SP-D levels correlated with lower concomitantly obtained FVC (r = −0.27, p < 0.001), but did not predict the short-term decline in FVC at 1 year followup visit or its longterm decline rate. CCL18 showed a significant correlation with steeper short-term decline in FVC (p = 0.049), but was not a predictor of its longterm decline rate. Similarly, a composite score of SP-D and CCL18 was a significant predictor of short-term decline in FVC but did not predict its longterm decline rate. Further, the longitudinal change in these 2 pneumoproteins did not correlate with the concomitant percentage change in FVC. Conclusion SP-D correlated with concomitantly obtained FVC, while CCL18 was a predictor of short-term decline in FVC. However, neither SP-D nor CCL18 was a longterm predictor of FVC course in patients with early SSc. PMID:23588945

  6. Serum IL8 and mRNA level of CD11b in circulating neutrophils are increased in clinically amyopathic dermatomyositis with active interstitial lung disease.

    Science.gov (United States)

    Zou, Jing; Chen, Jie; Yan, Qingran; Guo, Qiang; Bao, Chunde

    2016-01-01

    The objective of this study is to assess serum IL8 and the potential activity of circulating neutrophils on relative messenger RNA (mRNA) levels and their relationship with disease activity in clinically amyopathic dermatomyositis (CADM) associated with interstitial lung disease (ILD). We studied 18 CADM patients and compared them with 18 classic dermatomyositis (DM) patients and 18 healthy control subjects. Serum IL8 level and mRNA expressions of neutrophils (chemokine (C-X-C motif) receptor 1 (CXCR1), cluster of differentiation molecule 11b (CD11b), cluster of differentiation 64 (CD64), myeloid cell leukemia 1 (MCL1), interleukin-18 (IL18)) were detected. The overproduction of serum IL8 level was most significant in the CADM group with active period. The mRNA expressions of CD11b, IL18, and MCL1 were greatly increased in the neutrophils in patients with CADM compared with DM or healthy controls. Up-expressions of CD11b, IL18, and MCL1 were detected in the neutrophils in CADM patients of active period compared with remission period. A positive correlation was found between CD11b mRNA level and high-resolution computed tomography (HRCT) score, in CADM associated with ILD. Serum IL8 level and mRNA levels of CD11b, MCL1, and IL18 in circulating neutrophils are related with the disease activity of CADM-ILD. The mRNA level of CD11b is positively correlated with HRCT score in CADM-ILD.

  7. Clinical, radiological and pathological features of 29 pediatric patients with interstitial lung disease%29例小儿间质性肺疾病临床、放射及病理分析

    Institute of Scientific and Technical Information of China (English)

    刘秀云; 周春菊; 彭芸; 江载芳

    2012-01-01

    目的 提高对小儿间质性肺疾病的认识.方法 收集2001年3月至2009年12月住院肺活检的弥漫性肺疾病患儿29例,对其临床表现、肺CT或高分辨CT(HRCT)、肺组织病理特点进行回顾性分析.结果 临床表现为咳嗽29例,呼吸困难15例,肺部湿啰音7例,杵状指趾9例.胸CT或HRCT显示:19例以磨玻璃影为主,5例为结节影,5例以多发实变影为主.根据肺病理的改变,结合临床和影像学特点,确诊特发性间质性肺炎18例,包括非特异性间质性肺炎(NSIP)8例、急性间质性肺炎(AIP)3例、隐源性机化性肺炎(COP)3例、淋巴细胞性间质性肺炎(LIP)2例、呼吸性细支气管炎伴间质性肺炎(RBILD)1例、未分型特发性间质性肺炎1例;其他间质性肺疾病11例,包括弥漫性泛细支气管炎2例、过敏性肺炎2例、类脂性肺炎2例、肺泡蛋白沉着症2例、肺泡微石症1例、特发性肺含铁血黄素沉着症1例、结节病1例.其中21例患儿应用糖皮质激素治疗,16例有效.结论 小儿间质性肺疾病的主要临床表现为咳嗽和呼吸困难,肺CT表现主要为磨玻璃影、结节影,肺组织病理为小儿间质性肺疾病诊断的主要依据.%Objective To understand clinical features of pediatric interstitial lung disease. Methods Twenty-nine in-patients with lung biopsy of the diffuse pulmonary disease were collected from March 2001 to December 2009. Their clinical, CT or HRCT of lung and pathological features were studied retrospectively. Results All the cases were with cough, 15 with dyspnea, 7 with crackles and 9 with clubbing fingers or toes. CT and HRCT showed 19 cases with ground-glass opacity, 5 with nodules, the other 5 with patchy consolidation. According to the pathological changes of the lung, combined with clinical and radiological features, 18 cases were diagnosed as idiopathic interstitial pneumonia, including 8 non-specific interstitial pneumonia (NSIP) , 3 acute interstitial pneumonia (AIP

  8. Idiopathic airway-centered interstitial fibrosis: report of two cases

    Institute of Scientific and Technical Information of China (English)

    YI Xiang-hua; CHU Hai-qing; CHENG Xiao-ming; LUO Ben-fang; LI Hui-ping

    2007-01-01

    @@ Airway-centered interstitial fibrosis (ACIF), a novel form of diffuse interstitial lung disease (ILD) of unknown cause, was recently presented.1 There is no final conclusion on its property and denomination, and it might be a new type of idiopathic interstitial pneumonia (ⅡP).

  9. Lymphocytic Interstitial Pneumonia.

    Science.gov (United States)

    Panchabhai, Tanmay S; Farver, Carol; Highland, Kristin B

    2016-09-01

    Lymphocytic interstitial pneumonia (LIP) is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. LIP is frequently associated with connective tissue diseases or infections. Idiopathic LIP is rare; every attempt must be made to diagnose underlying conditions when LIP is diagnosed. Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts. Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma. The 5-year mortality remains between 33% and 50% and is likely to vary based on the underlying disease process.

  10. 小儿间质性肺疾病14例临床-影像-病理诊断分析%Clinical, radiologic, pathological features and diagnosis of 14 cases with interstitial lung disease in children

    Institute of Scientific and Technical Information of China (English)

    中华医学会儿科学分会呼吸学组儿童弥漫性实质性肺疾病

    2011-01-01

    Objective The pediatric interstitial lung disease is a group of poorly understood disease entities. This study aimed to better understand the clinical features, radiological manifestations and pathological patterns of pediatric interstitial lung disease. Method Patients with diffuse lung disease seen in the year 2009 in 7 hospitals were studied by the Pediatric Interstitial Lung Disease Cooperative Group.Nineteen patients underwent lung biopsy, 11 cases were male, 8 were female and their age ranged from 1 year and 4 months to 13 years. Respiratory tract secretions were obtained for bacterial culture, respiratory virus antigen examination, mycoplasma antibody, EB virus, cytomegalovirus, and herpes simplex viruses antibody detection were performed. The CT or HRCT of the lung and blood-gas analysis and lung biopsy were performed for all the patients. One case underwent open lung biopsy, two cases received percutaneous biopsy, and other 16 cases were experienced video-assisted thoracoscopic biopsy. Result Five cases had been excluded, for one case had fungal infection, one had abnormal pneumoangiogram, one had sclerosing hemangioma, and two had no sufficient data. The remaining 14 cases were included into the analysis. All the 14 cases had cough, 12 of them also had tachuyppoea, four cases had rales and five had clubbing. High resolution CT showed that 12 cases had ground-glass opacification, 1 had diffuse micronodular opacities, the pathological pattern of this case was pulmonary alveolar microlithiasis, and in the case of diffuse reticulonodular opacities and cysts, the pathology of the lung was NSIP. All the 14 cases had the proof of the diagnosis or the type of the pathology. Four cases were diagnosed by pathology of the lung, incuding 1 case of pulmonary alveolar microlithiasis, 2 cases of pulmonary alveolar proteinosis, 1 case of lipoid pneumonia.Clinical-radiologic-pathologic (C-R-P) diagnosis of the other 10 cases were as follows: 4 cases had secondary

  11. Values of surfactant protein-A and surfactant protein-D in Interstitial lung disease%血清肺表面活性蛋白A、D在间质性肺疾病中的价值

    Institute of Scientific and Technical Information of China (English)

    刘晓立; 吕长俊; 王晓芝

    2008-01-01

    The pathological diagnosis of interstitial lung disease (ILD) by surgical lung biopsy is important for clinical decision. There is a need, however, to use serum markers which is non-invasive for differentiating ILD. Surfactant protein(SP)-A, SP-D are useful markers for the diagnosis and evaluation of activity of ILD. More studies have appeared on the proteins' usefulness in recent years and they are reviewed as follows.%开胸肺活检取得病理诊断是对间质性肺疾病(interstitial lung disease,ILD)的诊断起决定作用的.然而,需要一种非侵入性的血清标志物来区别ILD,尤其是对于最常见、预后最差的寻常型间质性肺炎.肺表面活性蛋白A、D在ILD鉴别诊断及评定疾病活动性上有提示意义.近年来这类蛋白的研究进展显著,现对ILD的价值作一综述.

  12. [DIP (desquamative interstitial pneumonia): as a tobacco-associated disease -- case report].

    Science.gov (United States)

    Sousa, Vitor; Carvalho, Lina

    2004-01-01

    DIP (desquamative interstitial pneumonia) is an interstitial lung disease with diffuse and uniform accumulation of alveolar macrophages. There is a strong association with tobacco since 90% of the patients are smokers. The interstitial lung diseases related to tobacco are diverse and include tumours, emphysema, chronic bronchitis, RBILD (Respiratory Bronchilites associated Interstitial Lung Disease), DIP and Langerhans Cell Histiocitosis. The authors present a case of DIP. A brief theorycal revision and discussion of a case is made facing the association with tobacco.

  13. 硬皮病相关间质性肺疾病发病机制的研究进展%Advances in the pathogenesis of scleroderma-related interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    罗鸯鸯; 肖嵘; 吴尚洁

    2012-01-01

    Interstitial lung disease is a prevalent complication of scleroderma, the leading cause of bad prognosis for this disease. Nowadays, though the pathogenesis of the disease remains unclear, some achievements have been made. Most observers suggest that microvascular damage and immunologically-mediated inflammation interactively contribute to fibrosis, which is the typical characteristic of scleroderma-related interstitial lung disease. Other possible mechanisms include gastroesophageal reflux and some newbiomarkes ( SP-D,KL-6,caveolin-l, and others) .In this review, we have summarized progress in understanding the possible mechanisms of scleroderma-related interstitial lung disease.%间质性肺疾病是硬皮病的一种常见肺损伤,是影响硬皮病预后的主要原因.目前,尽管其发病机制不十分明确,但已取得一定的研究进展.许多研究者提出微血管损伤和免疫介导的炎症反应共同参与肺纤维化的形成,从而导致硬皮病相关间质性肺疾病的发生.其他可能机制包括胃食管反流和一些新型标志物SP-D,KL-6,小窝蛋白-1等.本文就上述方面的有关研究进展进行综述.

  14. Anti-MDA5 antibody is associated with acute/subacute interstitial pneumonia and predicts poor prognosis for interstitial lung diseases in patients with dermatomyositis%血清抗黑色素瘤分化相关基因抗体检测在多发性肌炎/皮肌炎患者中的意义

    Institute of Scientific and Technical Information of China (English)

    陈芳; 王冬雪; 舒晓明; Ran Nakashima; Tsuneyo Mimori; 王国春

    2012-01-01

    肌炎患者人群,血清抗MDA5抗体主要存在于皮肌炎患者,是皮肌炎合并A/SIP的敏感及特异性血清学指标,也是皮肌炎合并ILD死亡的独立危险因素.%Objective To determine the serum MDA5 levels and their clinical associations in patients with polymyositis/dermatomyositis (PM/DM).Methods Serum anti-MDA5 antibody was detected by ELISA in 119 adult PM/DM patients,30 patients with systemic lupus erythematosus (SLE),30 patients with rheumatoid arthritis (RA),15 patients with primary Sj(O)gren's syndrome (SS),21 patients with pulmonary infection and 50 healthy controls.t-test,Mann-Whitney U test or chi-square test or Fisher exact test as well as Logistic multivariate regression analysis were carried out to compare the results of this study.Results Serum antiMDA5 antibody positive rate in DM patients (22.6%) were significantly higher compared with that of patients with PM (0,P<0.01),patients with SLE (3.3%,X2=5.68,P<0.05),RA (3.3%,X2=5.68,P<0.05),pSS (0,P<0.05) and pulmonary infection(0,P<0.05) and healthy controls (0,P<0.01).In the DM subgroup,CADM patients presented a higher positive anti-MDA5 antibody rate than classic DM patients.The incidence of arthritis,fever,vrash raised CEA and CA153 level,and serum concentration of GGT and ferritin were significantly higher in the anti-MDA5 positive DM group than anti-MDA5 negative DM group (X2=4.08,8.06,6.357,32.4,4.867; Z=-2.86,-2.44; P value of all these tests were less than 0.05 ),while the rate of serum positive ANA,serum level of CK and T,NK cell counts in the peripheral blood were much lower than those in anti-MDA5 negative DM group (X2=4.08; Z=-2.072,-2.013,-2.907; all P<0.05).Moreover,the incidence of acute/subacute interstitial pneumonia (A/SIP) was significantly higher in anti-MDA5 positive DM patients than anti-MDA5 negative DM patients.The sensitivity and specificity of anti-MDA5 antibody for diagnosing A/SIP in DM patients were 88.2% and 94% respectively.Additionally,logistic multivariate

  15. Interstitial hyperthermia.

    Science.gov (United States)

    Milligan, A J; Dobelbower, R R

    1984-01-01

    The effectiveness of hyperthermia as a treatment modality for cancer continues to gain popularity in the medical community. One of the disappointing findings has been the inability to deliver uniform thermal doses to tumor volumes. This inability to heat certain tumors is due to a variety of physical and physiologic phenomena. To increase the ability of heating tumors, local interstitial techniques have been developed that are proving to be safe and effective. These techniques employ implanted microwave or radiofrequency antennae for the delivery of local thermal doses. Recently, investigations into the placement of interstitially located ferromagnetic seeds for local hyperthermia have also been conducted. The seeds can be heated by delivery of a high-wattage RF magnetic field to the implanted volume by an external source after implantation. The tissue surrounding the ferromagnetic implant is heated by conduction of heat away from the implanted seeds. While these techniques have been effective, further development of the instrumentation for interstitial therapies is continuing. These developments will include the application of specific control circuitry for delivery of accurate thermal doses.

  16. Relationship between IgG4 and Connective Tissue Disease with Interstitial Lung Disease%IgG4与结缔组织病合并间质性肺病的相关性研究

    Institute of Scientific and Technical Information of China (English)

    鲁芙爱; 刘媛; 安燕; 王永福

    2013-01-01

    Objective To discuss the significance of IgG4 in the pathogenesis of connective tissue disease with interstitial lung disease and its relationship with activity of the disease. Methods Totally 205 patients with connective tissue diseases were divided into two groups, with 93 with interstitial lung disease and 112 without interstitial lung disease. Serum IgG4 levels of all patients were detected by immune nephelometry quantitative method, and IgG4 level changes between the two groups were compared. At the same time, the levels of ESR, C - reactive protein ( CRP ), IgA, IgG and IgM were detected in 93 patients with interstitial lung disease, and the correlation of the activity of these indices with IgG4 was analyzed. Results No significant difference in serum IgG4 level was found between the connective tissue disease patients with interstitial lung disease and the patients without interstitial lung disease ( P >0. 05 ). And in the patients with interstitial lung disease there was no significant correlation between the level of serum IgG4 and the levels of ESR, CRP, IgM, IgA and IgG ( P >0. 05 ) . Conclusion The IgG4 level is not correlated with connective tissue disease complicated by interstitial lung disease, suggesting that IgG4 - related diseases and connective tissue disease are two independent immune system diseases, and serum IgG4 level cannot be used to determine the activity of connective tissue disease with interstitial lung disease.%目的 探讨IgG4在结缔组织病合并间质性肺病发病中的意义及与其病情活动的关系.方法 选取205例结缔组织病患者,分为两组,其中合并间质性肺病组的患者93例,无间质性肺病组的患者112例.采用动态免疫散射比浊法定量检测两组患者血清中IgG4的水平,比较两组之间IgG4水平的变化;对合并间质性肺病的93例患者同时进行了红细胞沉降率(ESR)、C反应蛋白(CRP)、免疫球蛋白A(IgA)、免疫球蛋白G(IgG)、免疫球蛋白M(IgM)的

  17. 许建中教授治疗肺间质性疾病的经验%The Experience of Professor XU Jian-zhong on Treating Interstitial Lung Disease

    Institute of Scientific and Technical Information of China (English)

    许宗伟

    2011-01-01

    从中医病名病机分析入手,对许建中教授治疗肺间质性疾病的经验进行总结,以期为临床治疗此类疾病提供借鉴.%This article summarized the experience of professor XU Jian-zhong on treating interstitial lung disease from analyzing the name and pathogenesis of disease in TCM,in order to provide a reference for clinical treatment of these diseases.

  18. 类风湿性关节炎相关间质性肺疾病的研究进展%Advances in studies of rheumatoid arthritis-associated interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    张玲

    2010-01-01

    间质性肺疾病(interstitial lung disease,ILD)是类风湿性关节炎(rheumatoid arthritis,RA)常见的关节外病变和主要的死亡原因之一,早期诊治类风湿性关节炎相关间质性肺疾病(RA-ILD)直接影响RA患者预后.本文对近年RA-ILD的研究进展作简要概述,以期提高对该病的认识.

  19. Association of cross-reactive antibodies targeting peptidyl-arginine deiminase 3 and 4 with rheumatoid arthritis-associated interstitial lung disease.

    Directory of Open Access Journals (Sweden)

    Jon T Giles

    Full Text Available BACKGROUND: A subset of rheumatoid arthritis (RA patients have detectable antibodies directed against the peptidyl-arginine deiminase (PAD enzyme isoforms 3 and 4. Anti-PAD3/4 cross-reactive antibodies (anti-PAD3/4XR have been shown to lower the calcium threshold required for PAD4 activation, an effect potentially relevant to the pathogenesis of RA-associated interstitial lung disease (ILD. METHODS: RA patients underwent multi-detector computed tomography (MDCT of the chest with interpretation by a pulmonary radiologist for ILD features. A semi-quantitative ILD Score (range 0-32 was calculated. Concurrent serum samples were assessed for antibodies against PAD by immunoprecipitation with radiolabeled PAD3 and PAD4. RESULTS: Among the 176 RA patients studied, any ILD was observed in 58 (33% and anti-PAD3/4XR was detected in 19 (11%. The frequency of any ILD among those with anti-PAD3/4XR was 68% vs. 29% among those with no anti-PAD (crude OR = 5.39; p = 0.002 and vs. 27% among those with anti-PAD4 that was not cross-reactive with PAD3 (crude OR = 5.74; p = 0.001. Both associations were stronger after adjustment for relevant confounders (adjusted ORs = 7.22 and 6.61, respectively; both p-values<0.01. Among ever smokers with anti-PAD3/4XR, the adjusted frequency of any ILD was 93% vs. 17% for never smokers without the antibody (adjusted OR = 61.4; p = 0.001, p-value for the interaction of smoking with anti-PAD3/4XR<0.05. CONCLUSIONS: The prevalence and extent of ILD was markedly higher among RA patients with anti-PAD3/4 cross-reactive antibodies, even after accounting for relevant confounders, particularly among ever smokers. These findings may suggest etiopathologic mechanisms of RA-ILD, and their clinical utility for predicting ILD warrants additional study.

  20. Pay great effort to improve the skills of diagnosing pediatric interstitial lung diseases%努力提高儿童间质性肺疾病的诊断水平

    Institute of Scientific and Technical Information of China (English)

    陈慧中

    2011-01-01

    @@ 一直以来,我国儿童弥漫性实质性肺疾病/间质性肺疾病(pediatric diffuse parenchymal lung disease/pediatric interstitial lung disease,pDPLD/pILD)的诊断大多仅基于临床表现和胸部X线征象,诊断名称单一,缺乏统一分类,治疗方案杂乱,难以达成共识.中华医学会儿科学分会呼吸学组儿童弥漫性肺疾病/间质性肺疾病协作组(简称儿童pDPLD/pILD协作组)近年来以国际化诊断模式为样板,取得了阶段性的进展.

  1. Interstitial Lung Disease and Respiratory Failure Causes Mechanical Ventilation Treatment Effect Observation%间质性肺疾病并呼吸衰竭的原因及机械通气治疗效果观察

    Institute of Scientific and Technical Information of China (English)

    王济德; 庞运兰

    2015-01-01

    ObjectiveTo explore the combination of respiratory failure cause interstitial lung disease and the clinical effect of application of mechanical ventilation treatment.MethodsSelected from our hospital in 2011-2013 were the combination of respiratory failure in patients with interstitial lung disease of 50 cases, depending on the type of mechanical ventilation therapy is divided into invasive group and noninvasive group, 10 patients with invasive mechanical treatment, 40 patients with noninvasive treatment. On the clinical data analyzed by retrospective method, inductive result in interstitial lung disease related factors, and the application of mechanical ventilation.ResultsThe combination of respiratory failure cause interstitial lung disease mainly include: the cause of interstitial lung disease exacerbations, pure infection, pulmonary embolism, heart failure and respiratory tract infection; Patients receiving mechanical ventilation therapy, the mortality in patients with invasive mechanical ventilation group was 80.0%(8/10); noninvasive mechanical ventilation patients case fatality rate was 55.0%(22/40), 2 groups of patients with clinical effect contrast was statistically signiifcant(P<0.05). In total hospital mortality of 60.0% (30/50).ConclusionIn patients with interstitial lung disease between lead to respiratory failure are the major factors of respiratory infection, the severity of interstitial lung disease, pulmonary embolism, and heart failure, etc. on the combination of respiratory failure in patients with interstitial lung disease application of noninvasive mechanical ventilation treatment, the clinical curative effect than in patients with invasive mechanical ventilation is much good, worthy of clinical promotion.%目的:探究导致间质性肺疾病合并呼吸衰竭的原因以及应用机械通气治疗的临床效果。方法选自本院2011年~2013年收治的间质性肺疾病合并呼吸衰竭患者50例,根据接

  2. Current status of the application of pulmonary biopsy to interstitial lung diseases in children%肺活检用于儿童肺间质疾病诊断现状

    Institute of Scientific and Technical Information of China (English)

    郑跃杰

    2010-01-01

    @@ 肺间质疾病(interstitial lung diseases,ILDs)是指肺间质损伤而产生的一类疾病,由于肺间质包括肺实质的大部分,由位于肺泡之间的组织所组成,故现在也称为弥漫性肺实质疾病(diffuse parenchymal lung disease,DPLD).DPLD是一组病因不同,但临床、影像学和组织病理学类似的异质性疾病,包括已知病因(如结缔组织病、环境、职业或药物相关肺病、微生物感染等)和未知病因两大类,后者包括特发性间质性肺炎(idiopathic interstitial pneumonias,IIP)、肉芽肿性肺病(例如结节病)及其他多种少见疾病[如淋巴管平滑肌肉瘤病(LAM)、组织细胞增生症X、嗜酸粒细胞肺炎等[1

  3. 具有间质性肺病及慢性阻塞性肺病肺背景的周围型肺癌毛刺征的CT表现%CT manifestations of spicular sign in peripheral lung cancer under background complicated by interstitial lung disease and chronic obstructive pulmonary disease

    Institute of Scientific and Technical Information of China (English)

    侯书法; 马大庆

    2012-01-01

    目的 探讨具有间质性肺病及慢性阻塞性肺病(COPD)的肺背景与周围型肺癌(PLC)毛刺征CT表现之间的联系.方法 收集经病理证实的100例PLC,将其分为2组,其中间质性肺病及COPD组67例,无间质性肺病及COPD组33例,比较2组病灶CT毛刺征表现的差异.结果 间质性肺病及COPD组毛刺出现率明显较无间质性肺病及COPD组高,间质性肺病及COPD组为88%(59/67),无间质性肺病及COPD组为70%(23/33),具有明显统计学差异(P<0.05).具有间质性肺病肺背景的PLC的毛刺形态与肺间质疾病的线状影表现一致,周围间质改变越明显,毛刺越多;具有COPD肺背景的PLC毛刺形态多样,为小叶中心肺气肿的壁构成,形态与病灶周围肺气肿严重程度有关.结论 具有间质性肺病及COPD背景的PLC毛刺出现率较高,具有间质性肺病及COPD的肺背景对PLC毛刺征CT表现有影响.%Objective To study the relationship between CT manifestations of spicular sign in peripheral lung cancer (PLC) under background complicated by interstitial lung disease and chronic obstructive pulmonary disease (COPD). Methods 100 cases with PLC confirmed by pathological diagnosis were collected and divided into two groups:PLC complicated by interstitial lung disease andf COPD groupfgroup A,67 cases) ,PLC without the complications of interstitial lung disease and COPD group (group B,33 cases). The differences of CT manifestations of spicular sign between two groups were compared. Results The frequency of spicular sign in group A( 88%, 59/67) was much higher than that in group B( 70%, 23/33) , there was significant difference between two groups (P <0. 05). CT manifestations of spicular sign as linear opacity in PLC complicated by interstitial lung disease were agreement with that in interstitial lung disease. The more alterations were in peripheral intersitium, the more spiculations were in PLC. Spicular appearances of PLC complicated by COPD were multiplicity, which

  4. Research progress of rheumatoid arthritis-associated interstitial lung disease%类风湿关节炎相关性间质性肺疾病的研究进展

    Institute of Scientific and Technical Information of China (English)

    张艳敏; 姜莉

    2011-01-01

    类风湿关节炎相关性间质性肺疾病发病率高,是类风湿关节炎患者的主要死因之一.其发病机制尚不明确,病史不清,以慢性咳嗽和缓慢进展的呼吸困难为主要症状,肺功能显示限制性通气功能障碍,影像学改变以磨玻璃样阴影、网格状阴影、蜂窝肺最为常见,支气管肺泡灌洗提示存在肺泡炎,病理类型以普通型间质性肺炎最为多见,激素治疗效果差,预后不佳.现就类风湿关节炎相关性间质性肺疾病作一综述.%The incidence of rheumatoid arthritis-associated interstitial lung disease is high,it is one of the main causes of death in patients with rheumatoid arthritis. The pathogenesis is not well defined and the natural history is unclear. The main symptoms are chronic cough and slowly progressive dyspnea.Pulmonary function tests show restrictive ventilation dysfunction. The most common image changes are ground-glass opacity, grid-like shadows, and honeycombing. Bronchoalveolar lavage shows the presence of alveolitis. The most common pathological type is usual interstitial pneumonia. The therapeutic efficacy of hormone and prognosis are poor. This article reviews the latest research progress of rheumatoid arthritisassociated interstitial lung disease.

  5. 弥漫性肺间质疾病中医证素分布规律的临床文献研究%Literature study on TCM syndrome dlements distribution law of diffuse interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    赵栋梁; 白云苹; 王明航; 李建生

    2011-01-01

    Objective: To probe distribution regularity of TCM syndromes of diffuse interstitial lung disease. Methods:Literatures about diffuse interstitial lung disease in 15 years were collected from the Chinese biological medicine on disc, China national knowledge infrastructure and TCM database. The name of the disease was filtered. The distribution and association law of the nature and location pattern elements were analyzed. Results: Deficiency of vital energy, blood stasis, yin-deficiency and phlegm were the main nature elements of pattern of diffuse interstitial lung disease. Lung and kidney were the main location elements of pattern. in combination with single nature element and location element, the deficiency of lung qi was the most common one; in combination with two nature element the deficiency of vital energy and blood stasis the most common one, the location elements of pattern were the lung and kidney; in combination of three nature elements, the qi and yin deficiency with blood stasis and phlegm and blood stasis with qi deficiency was the more common one, the mainly location elements of pattern were the lung and kidney; in combination of four nature elements, the qi and yin deficiency and the phlegm and blood stasis were the more common one, the location element were lung and kidney. Conclusion: Lung and kidney were the main locations of the disease, while deficiency of vital energy, blood stasis, yin-deficiency and phlegm were the main nature of disease, can providing the scientific basis for study of pathogenesis and the establishing of standardization of TCM syndrome.%目的:探讨弥漫性肺间质疾病中医证素分布规律.方法:从中国生物医学文献数据库、中国学术期刊全文数据库和中国中医药文献检索系统数据库,检索近15年弥漫性肺间质疾病文献,筛选整理规范名称.对病性证素与病位证素的分布组合规律进行分析.结果:弥漫性肺间质疾病的病性证素以气虚、血瘀、阴

  6. Doenças pulmonares intersticiais crônicas na criança Chronic interstitial lung diseases in children

    Directory of Open Access Journals (Sweden)

    Maria Aparecida Soares de Souza Paiva

    2009-08-01

    Full Text Available As doenças pulmonares intersticiais (DPIs da criança constituem um grupo heterogêneo de doenças raras que têm sido definidas e classificadas de acordo com as experiências e as pesquisas em adultos. Entretanto, os pneumologistas pediátricos vêm observando que o espectro clínico é mais amplo nas crianças, e que muitas destas doenças evoluem e respondem ao tratamento de forma diferente. Além disso, provavelmente devido a estágios diferentes de desenvolvimento e maturação pulmonares, novas formas clínicas têm sido descritas, principalmente em lactentes, ampliando a classificação nessa faixa etária. A compreensão de que nem a definição nem as classificações estabelecidas se aplicam inteiramente ao grupo pediátrico tem motivado a realização de estudos multicêntricos com o objetivo de estudá-las melhor, unificando as estratégias diagnósticas e terapêuticas. Fizemos a revisão atualizando a conceituação das DPIs no grupo pediátrico, considerando as particularidades desse grupo na utilização do esquema de classificação dessas doenças para adultos e revendo o histórico dos esforços para uma melhor compreensão do problema com os estudos multicêntricos. Foram ressaltadas as diferenças na apresentação clínica, procurando realçar os novos conhecimentos sobre as doenças recém descritas nas crianças pequenas. Alertamos também para a necessidade de ser seguida uma rotina padronizada de investigação laboratorial, radiológica e de processamento das biópsias à luz desses conhecimentos. É importante lembrar que, do grupo das novas doenças descritas, as alterações genéticas do surfactante devem constar também do diagnóstico diferencial das DPIs dos adultos, podendo se apresentar nesse grupo como uma das doenças classificadas como idiopáticas.Interstitial lung diseases (ILDs in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences

  7. Clinical characteristics analysis of interstitial lung disease associated with connective tissue disease%结缔组织病相关的肺间质病变临床特点分析

    Institute of Scientific and Technical Information of China (English)

    周明韬

    2014-01-01

    Objective To improve the understanding of clinical characteristics of interstitial lung dis-ease associated with connective tissue disease.Methods Clinical manifestations , imaging changes , treatment and prognosis in 12 patients with connective tissue disease associated with interstitial lung disease were retro -spective analyzed.Results There were 5 cases of patients with rheumatoid arthritis , 2 cases of mixed connec-tive tissue disease, 2 cases of Sjogren's syndrome, 1 case of systemic lupus erythematosus , 1 case of dermatomy-ositis and 1 case of polymyositis in 12 patients.Respiratory symptoms included cough , expectoration and short-ness of breath after activity; Main pulmonary physical signs were Velcro rales; pulmonary high-resolution CT ( HRCT) examination showed ground-glass opacities , cord-like shadow , reticular opacities , patchy shadow , pulmonary bulla and honeycomb -like shadow;full chest X-ray showed streak shadow and patchy shadow ;one case of Sjogren ’ s syndrome and one case of systemic lupus erythematosus patients with interstitial lung disease were discovered earlier and their symptoms decreased with glucocorticoids and cyclophosphamide treatment .Pa-tients whose pulmonary interstitial lesions found later , with glucocorticoids and immunosuppressant therapy ,had no obvious improvement of symptoms and imaging findings.Their prognosis were poor.Conclusion Early symptoms of respiratory system are not obvious in patients with interstitial lung disease of connective tissue dis -ease.Pulmonary HRCT examination is helpful for early diagnosis and early treatment of glucocorticoid and cy -clophosphamide to improve their condition and prognosis.%目的:提高对结缔组织病相关的肺间质病变临床特点的认识。方法回顾分析12例结缔组织病合并肺间质病变患者的临床表现、影像学改变、治疗与转归。结果12例患者类风湿关节炎5例,混合性结缔组织病2例,干燥综合征2

  8. Subclinical interstitial lung involvement in rheumatic diseases. Correlations of high-resolution Computed Tomography patterns with functional and cytologic findings. L'interessamento polmonare interstiziale subclinico nelle malattie reumatiche. Correlazione fra Tomografia Computerizzata con alta risoluzione e i reperti funzionali e citologici

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    Salaffi, F. (Ancona Univ. (Italy). Clinica Reumatologica. Cattedra di Reumatologia); Baldelli, S. (Ancona Univ. (Italy). Ist. di Semeiologia, Diagnostica e Terapia Strumentale. Cattedra di Radiologia) (and others)

    The aims of this study were to quantify the severity and extent of subclinical interstitial lung disease as depicted on HRCT and to study the relationship between the patterns of lung disease quantified by HRCT and the functional parameters and bronchoalveolar lavage findings in patients with rheumatic diseases. The results confirm that HRCT is a sensitive tool in detecting interstitial lung disease in patients with rheumatic diseases with no signs and symptoms of pulmonary involvement. The relationship between the different HRCT patterns and bronchoalveolar lavage cell profiles can identify patients at higher risk of developing irreversible lung fibrosis. A long-term, prospective follow-up study is needed to determine whether these patients will develop over pulmonary disease.

  9. Analysis of risk factors for rheumatoid arthritis-related interstitial lung disease%类风湿关节炎相关肺间质病变的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    黄义鸿; 许赤多; 吴南辉

    2015-01-01

    目的:通过分析与探讨类风湿关节炎相关肺间质病变的危险因素,为临床上预防该种疾病提供相关依据。方法97例类风湿关节炎患者作为研究对象,其中单纯类风湿关节炎患者(单纯组)53例,类风湿关节炎相关肺间质病变患者(合并组)44例,对两组患者的临床资料进行对比分析。结果合并组患者的发病年龄、病程、抗环瓜氨酸肽(CCP)抗体值、吸烟率、类风湿因子(RF)、疾病活动性评分均明显高于单纯组,差异均有统计学意义(P<0.05)。结论类风湿关节炎患者年龄越大、病程越长、有吸烟史、疾病活动率越高、抗CCP抗体值越高越容易发生肺间质病变,应早期检查、早期诊治,以降低死亡率,促进患者早日康复。%Objective To analyze risk factors for rheumatoid arthritis-related interstitial lung disease, in order to provide related reference for clinical prevention.Methods Among the 97 patients with rheumatoid arthritis as study subjects, there were 53 cases with single rheumatoid arthritis (single group) and 44 cases with rheumatoid arthritis-related interstitial lung disease (complicated group). Clinical data of the two groups were comparative analyzed.Results The complicated group had much higher onset age, course of disease, anti-cyclic citrullinated peptide (CCP) antibody value, smoking rate, rheumatoid factors (RF), and disease activity score than the single group, and their differences all had statistical significance (P<0.05).Conclusion Interstitial lung disease occurs easily in rheumatoid arthritis patients with old onset age, long course of disease, smoking history, high disease activity and anti-CCP antibody value. Early examination and diagnosis are necessary for reducing mortality and accelerating rehabilitation of patients.

  10. Spectrum of fibrosing diffuse parenchymal lung disease.

    Science.gov (United States)

    Morgenthau, Adam S; Padilla, Maria L

    2009-02-01

    The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky-Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end-stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed.

  11. Comparison between chest imaging and detection of pulmonary function and alveolar lavage fluid in interstitial lung disease%间质性肺疾病胸部影像与肺功能、肺泡灌洗液检查对比观察

    Institute of Scientific and Technical Information of China (English)

    魏路清; 董彦; 韩文杰; 陈萍

    2000-01-01

    @@ 间质性肺疾病(interstitial lung disease,ILD)是引人注目的呼吸系统疾病,作者对ILD患者进行了胸部影像、肺功能及肺泡灌洗液的对比观察,综合评价其临床意义.

  12. Clinical Report and Literature Review of Bronchioloalveolar Carcinoma Misdiagnosed as Interstitial Lung Disease%误诊为间质性肺纤维化的肺泡细胞癌临床报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    何彦侠; 薛兵; 邸丽娟

    2015-01-01

    Objective To highlight the interstitial changes of bronchioloalveolar carcinoma in radiology and clinical manifestations in order to prevent misdiagnosis. Methods The clinical data of a case of bronchioloalveolar carcinoma misdi-agnosed as lung fibrosis for 3 years in other hospitals were respectively reviewed and relevant literature was reviewed. Results A patient with a history of wood dust inhalation admitted to the hospital complied of progressive dry cough and dyspnea at exertion for 3 years. A physical examination revealed bilateral crackles mainly on the left lung, and radiology showed intersti-tial changes mainly on the left lung. A presumptive diagnosis of lung fibrosis was made in several hospitals and corresponding treatment was prescribed, but with no significant clinical improvement. Surgical lung biopsy revealed bronchioloalveolar carci-noma. The patient refused further treatment, and therefore was discharged. The patient died of respiratory failure 3 months lat-er. Conclusion Bronchioloalveolar carcinoma may mimic benign interstitial disease. PET, bronchoscopy and pneumocentesis may possibly be falsely negative,therefore surgical lung biopsy should be performed as soon as possible.%目的:提高对肺泡细胞癌间质样肺部病变影像学及临床表现的认识,以减少误诊。方法对3年来在多家医院就诊均误诊为间质性肺纤维化的肺泡细胞癌1例的临床资料进行回顾性分析,并复习相关文献。结果本例因咳嗽、呼吸困难3年,加重1个月入院。有长期木屑吸入史;双侧中下肺均可闻及吸气相爆裂音,胸部CT检查示以左肺为主的间质样改变,曾在多家医院就诊,均诊断为间质性肺纤维化,予相应治疗效果不佳。入我院后经胸腔镜肺活组织病理检查确诊肺泡细胞癌。患者拒绝进一步治疗出院。出院3个月后患者因呼吸困难迅速加重、呼吸衰竭死亡。结论肺泡细胞癌临床表现及肺部病变影像学特征与

  13. Interstitial lung disease needs more attention from different disciplines%间质性肺疾病——一个需要多学科共同关注的领域

    Institute of Scientific and Technical Information of China (English)

    李惠萍; 鲍春德

    2009-01-01

    @@ 间质性肺疾病(interstitiallung disease,ILD)也称"弥漫性实质性肺疾病(diffuse parenchymal lung disease,DPLD)",指主要累及肺间质、肺泡和(或)细支气管的一组肺部弥漫性疾病.其病因复杂,包括200余种疾病.通常将ILD分为特发性和继发性两大.类.原因不明的ILD被称为"特发性间质性肺炎(idiopathic interstitial pneumonias,IIPs)",而由其他疾病和病因引起者为继发性ILD.

  14. 过氧化物酶增殖物激活受体γ在间质性肺疾病中的作用%The Effect of Peroxisome Proliferators Activated Receptor γ on Interstitial Lung Disease

    Institute of Scientific and Technical Information of China (English)

    潘晓娟; 张晓

    2011-01-01

    Peroxisome proliferators activated receptor-γ (PPARγ) is a nuclear hormone receptor activated by iigand. It has been realized that PPAR-γ played an important role in the lipogenesis, metabolism and homeostasis. The research work of recent years enabled scientists to understand that PPAR are important in anti-inflammatory , modulating cell proliferation and differentiation, apoptosis and anti-fibrosis processes. In particular, the down- regulation effect of PPAR-γ on the interstitial lung disease attracted the researcher' s attention. Further research on PPAR-γ in interstitial lung disease is needed to clarify whether it can be a good therapeutic target of pulmonary fi-brosis.%过氧化物酶增殖物激活受体γ(PPARγ)是配体激活的核激素受体,以往只认识到其在脂肪生成、代谢及维持内环境稳态的作用.近年来的体内外实验使研究者逐渐认识到过氧化物酶增殖物激活受体在抗炎、调节细胞增殖、分化、凋亡及抗纤维化中均起到重要作用.特别是PPARγ在间质性肺疾病中的负性调节作用逐渐受到重视,期待进一步对PPARγ抗纤维化作用的研究,使其成为治疗肺纤维化的新靶点.

  15. Circulating thymus and activation-regulated chemokine/CC chemokine ligand 17 is a strong candidate diagnostic marker for interstitial lung disease in patients with malignant tumors: a result from a pilot study

    Directory of Open Access Journals (Sweden)

    Yamane H

    2015-06-01

    Full Text Available Hiromichi Yamane, Nobuaki Ochi, Tomoko Yamagishi, Yoshihiro Honda, Masami Takeyama, Nagio TakigawaDepartment of General Internal Medicine 4, Kawasaki Medical School, Kita-ku, Okayama, JapanIntroduction: Serum Krebs von den Lungen-6 (KL-6 level is an established diagnostic marker of interstitial lung disease (ILD. However, it is also elevated in patients with non-small cell lung cancer (NSCLC. The significance of circulating thymus and activation-regulated chemokine (TARC/CC chemokine ligand 17 (CCL17 in malignant diseases remains unknown.Methods: We measured circulating TARC/CCL17 and KL-6 using enzyme-linked immunosorbent assay and electrochemiluminescence immunoassay, respectively, in 26 patients with malignant disease and six patients with benign lung disease (BLD. The cutoff levels were 500 U/mL for KL-6 and 450 pg/mL for TARC/CCL17. The significance of the markers was evaluated in relationship to the presence of ILD (n=10. The statistical significance was set at P<0.05.Results: The KL-6 positive ratio was significantly higher in the patients with NSCLC (n=17 than in those with BLD. There was a significant difference in the KL-6 positive ratio between the patients with NSCLC without ILD and those with BLD without ILD. However, there were no significant differences in the TARC/CCL17 positive ratio between the patients with NSCLC and BLD or between those with NSCLC without ILD and those with BLD without ILD. The TARC/CCL17 positive ratio was significantly higher in the patients with malignancy and ILD than in those without ILD. There was also a significant difference in the TARC/CCL17 positive ratio between the patients with NSCLC without ILD and those with ILD.Conclusion: TARC/CCL17 may be useful for the diagnosis of ILD in patients with malignancies. Confirmation of the results is warranted through a large-scale study.Keywords: thymus and activation-regulated chemokine/CC chemokine ligand 17, Krebs von den Lungen-6, interstitial lung

  16. 呼出气冷凝液在COPD、哮喘、肺癌及间质性肺疾病中应用的研究进展%Advances of Application of Exhaled Breath Condensate in COPD, Asthma, Lung Cancer and Interstitial Lung Disease

    Institute of Scientific and Technical Information of China (English)

    杨伟煌; 黄平

    2015-01-01

    Exhaled breath condensate( EBC) is a new method for the detection of respiratory system diseases. Detecting real-time biomarkers in EBC evaluates acute and chronic airway inflammation and oxidative stress level.Since the EBC has non-invasive, real-time monitoring, simple, repeatable advantages, it can be used as diagnosis of lung disease, severity assessment, evaluation of drug efficacy and prognostic marker,and has good application prospects. EBC has extensive application in COPD, asthma, lung cancer and interstitial lung disease.In this paper, we review the advances of EBC in COPD, asthma, lung cancer and interstitial lung disease.%呼出气冷凝液(EBC)是一种新兴的呼吸道系统疾病检测方法。通过检测EBC中的实时生物标记物,以评价气道急慢性炎症、氧化应激水平。由于EBC具有无创、实时监测、简便、可重复等优点,具有良好的发展应用前景,因而可作为肺部疾病的诊断、疾病严重程度、药物疗效及预后评估,在COPD、哮喘、肺癌及间质性肺病得到广泛应用。本文对近年来EBC在COPD、哮喘、肺癌及间质性肺病中的研究做简要综述。

  17. Autopsy-proven causes of death in lungs of patients immunocompromised by secondary interstitial pneumonia Causas de óbito por pneumonia intersticial secundária em autópsias pulmonares de pacientes imunocomprometidos

    Directory of Open Access Journals (Sweden)

    Alberto Antonio Terrabuio Junior

    2007-02-01

    Full Text Available PURPOSE: To present the more frequent associations found in autopsies of immunocompromised patients who developed secondary interstitial pneumonia as well as the risk of death (odds ratio in having specific secondary interstitial pneumonia according to the cause of immunocompromise. METHOD: From January 1994 to March 2004, 17,000 autopsies were performed at Hospital das Clínicas, São Paulo University Medical School. After examining the pathology report review, we selected 558 of these autopsies (3.28% from patients aged 15 years or more with primary underlying diseases who developed radiologically diffuse infiltrates of the lung during their hospital course and died after secondary interstitial pneumonia (bronchopneumonia, lobar pneumonia, interstitial pneumonia, diffuse alveolar damage, pulmonary recurrence of underlying disease, drug-induced lung disease, cardiogenic pulmonary edema, or pulmonary embolism. Histology slides were reviewed by experienced pathologists to confirm or not the presence of secondary interstitial pneumonia. Statistical analysis included the Fisher exact test to verify any association between histopathology and the cause of immunocompromise; a logistic regression was used to predict the risk of death for specific histological findings for each of the independent variables in the model. RESULTS: Secondary interstitial pneumonia was histologically represented by diffuse interstitial pneumonitis ranging from mild nonspecific findings (n = 213 to a pattern of diffuse alveolar damage (n = 273. The principal causes of immunocompromise in patients with diffuse alveolar damage were sepsis (136 cases, neoplasia (113 cases, diabetes mellitus (37 cases, and transplantation (48 cases. A high risk of death by pulmonary edema was found for patients with carcinoma of colon. Similarly, in patients with lung cancer or cachexia, A high risk of death by bronchopneumonia (OR = 3.6; OR = 2.6, respectively was found. Pulmonary

  18. Desquamative interstitial pneumonia: A case report

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    Lovrenski Aleksandra

    2014-01-01

    Full Text Available Introduction. Desquamative interstitial pneumonia is one of the rarest idiopathic interstitial pneumonias and the rarest form of smoking-related interstitial lung diseases. It was first described by Liebow in 1965. Histologically, it is characterized by the presence of eosinophilic macrophages uniformly filling airspaces which often contain a finely granular light-brown pigment that does not stain for hemosiderin. The alveolar walls are usually mildly thickened by fibrous tissue and infiltrated by a moderate number of lymphocytes. Case Outline. Our patient was a 56-year-old male, heavy smoker, with bilateral lung infiltrations of unknown etiology and several months of discomfort in the form of dry cough and shortness of breath. Lung function tests showed a moderate restrictive ventilation disorder and a severe reduction of diffusing capacity. Since bronchoscopic specimens did not reveal lung lesion etiology, an open lung biopsy of the lower left pulmonary lobe was performed, and based on the obtained surgical material the pathohistologically diagnosis of desquamative interstitial pneumonia was established. The patient was started on corticosteroid and immunosuppressive therapy, and he ceased smoking. At the last control examination, two years after the onset of symptoms, the patient was feeling well, and high-resolution computed tomography (HRCT scan of the thorax showed regression of pathological changes. Conclusion. Although, as in our case, the majority of DIP patients improve on treatment, some patients still develop progressive irreversible fibrosis despite therapy.

  19. Primary Sjogren’s syndrome associated rapidly progressive interstitial lung disease(1 case report and literature review)%原发性干燥综合征相关急进性肺间质病变(1例报告并文献复习)

    Institute of Scientific and Technical Information of China (English)

    史悦; 王婉瑜; 史永红

    2014-01-01

    Objective To promote the understanding of the clinical characteristics of primary Sjogren’s syndrome associated rapidly progressive interstitial lung disease. Methods Literature was reviewed combining the clinical data of primary Sjogren’s syndrome associated with rapidly progressive interstitial lung disease. Primary Sjogren’s syndrome associated with rapidly progressive interstitial lung disease was a rare and severe complication and chest HRCT played an important role on diagnosing and evaluating therapy response of rapidly progressive interstitial lung disease. Prognosis can be improved by diagnosis in early stage and active immunosuppressive therapy. Results A 62-year old female patient was admitted for cough and dyspnea. 1. rapidly progressive interstitial lung disease. 2. primary Sjogren’s syndrome;prednisone therapy was effective. Conclusion Primary Sjogren’s syndrome associated rapidly progressive interstitial lung disease is a rare serious complications, chest HRCT plays an important role inf accelerated pulmonary interstitial disease diagnosis and curative effect evaluation. Early diagnosis and active immunosuppressive therapy can improve prognosis.%目的:提高对原发性干燥综合征相关急进性肺间质病变临床特征认识。方法结合1例原发性干燥综合征相关性肺间质病变临床资料进行文献复习。结果患者,女,62岁,因咳嗽气喘5 d入院,诊断:①急进性肺间质病变,呼吸衰竭。②原发性干燥综合征。强的松治疗有效。结论原发性干燥综合征相关急进性肺间质病变是一种少见严重并发症,胸部HRCT对急进性肺间质病变诊断和疗效评估起重要作用。早期诊断,积极免疫抑制治疗能改善预后。

  20. HRCT technique with low-dose protocols for interstitial lung diseases; HRCT-Technik mit Low-dose-Protokollen bei interstitiellen Lungenerkrankungen

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    Ley-Zaporozhan, J. [Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer Klinische Radiologie, Muenchen (Germany); Ley, S. [Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer Klinische Radiologie, Muenchen (Germany); Chirurgische Klinik Dr. Rinecker, Diagnostische und Interventionelle Radiologie, Muenchen (Germany)

    2014-12-15

    For optimal visualization of the lung parenchyma a dedicated computed tomography (CT) examination protocol has to be selected. High resolution CT (HRCT) is the best technique for non-invasive evaluation of the lung parenchyma. Given the possibility to examine the complete lungs within one breath hold, assessment of disease severity and distribution is easily possible. Various parameters have to be considered to generate optimal image quality and best possible clinical results. This review article discusses the various image acquisition parameters for HRCT as well as the issue of dose reduction. (orig.) [German] Die Wahl des optimalen Untersuchungsprotokolls fuer eine CT der Lunge ist relevant fuer die diagnostische Aussagekraft. Die hochaufloesende Computertomographie (HRCT) der Lunge ist das beste Verfahren zur nichtinvasiven Beurteilung des Lungenparenchyms. Durch die Moeglichkeit der Untersuchung der gesamten Lunge in einer Atemstillstandsphase ist die Abschaetzung der Erkrankungsschwere problemlos moeglich. Verschiedene Aspekte sind zu beachten, um eine optimale Bildqualitaet zu erreichen und eine bestmoegliche Aussagekraft der Untersuchung zu gewaehrleisten. In dieser Uebersicht werden die verschiedenen Aufnahmeparameter erlaeutert sowie das Thema der Dosisreduktion diskutiert. (orig.)

  1. [Serological survey of feline leukemia virus infection and the outcome of antibody-positive cats].

    Science.gov (United States)

    Higashihara, T; Tajima, M; Ishiguro, T; Tamura, H; Maejima, K

    1988-04-01

    A serological survey was carried out to examine the presence of antibodies against feline leukemia virus (FeLV) and feline oncornavirus-associated cell membrane antigen (FOCMA) in 208 cat sera collected at Teikyo University School of Medicine. Seven cats (3.4%) were positive for FeLV antibodies by enzyme-linked immunosorbent assay whereas no cat was positive for FOCMA antibody by indirect membrane immunofluorescent test. Anemia, leukemia and/or lymphoma formation were not observed in these FeLV antibody-positive cats. But among these seven cats, three were positive for toxoplasma antibodies. One of them was also positive for Chlamydia psittaci antibody and it died in pneumonia. Among the four toxoplasma antibody negative cats, one was died in eosinophilic granuloma. Furthermore, two of three cats, which were used for experiments, had cold and took therapy.

  2. Remarkably increased resistin levels in anti-AChR antibody-positive myasthenia gravis.

    Science.gov (United States)

    Zhang, Da-Qi; Wang, Rong; Li, Ting; Li, Xin; Qi, Yuan; Wang, Jing; Yang, Li

    2015-06-15

    Resistin is a pro-inflammatory cytokine involved in the pathogenesis of autoimmune diseases. To investigate serum resistin levels in patients with myasthenia gravis (MG) and determine if there are associations between resistin levels and disease severity, we measured serum resistin levels in 102 patients with anti-acetylcholine receptor antibody-positive MG (AChR-MG). We further analyzed associations between serum resistin levels and clinical variables in patients with MG. Our findings demonstrate that serum resistin levels are elevated in patients with AChR-generalized MG and AChR-MG with thymoma and are correlated with disease severity. Resistin has potential as a useful serum biomarker for inflammation in AChR-MG.

  3. Interstitial pneumonitis following intrapleural chemotherapy

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    Humphries Gary N

    2009-02-01

    Full Text Available Abstract Background Mucinous neoplasms within the abdomen may disseminate by direct extension through the diaphragm to involve the pleural space. Treatment of this condition is by parietal and visceral pleurectomy followed by hyperthermic intrapleural chemotherapy. Case presentation In this case report a patient developed persistent right upper lobe interstitial pneumonitis and progressive parenchymal fibrosis following intrapleural chemotherapy treatment with mitomycin C and doxrubicin. The condition persisted until death 28 months later. Death was from progressive intraabdominal disease with intestinal obstruction and sepsis associated with progressive pulmonary parenchymal disease. The right pleural space disease did not recur. Conclusion This manuscript is the first case report describing interstitial pneumonitis and lung fibrosis following intrapleural chemotherapy. Since pulmonary toxicity from chemotherapy is a dose-dependent phenomenon, dose reduction of intrapleural as compared to intraperitoneal hyperthermic chemotherapy may be necessary.

  4. Research advance on interstitial lung disease in patient with rheumatoid arthritis and serum surfactant proteins%类风湿关节炎合并间质性肺病及血清表面活性蛋白研究进展

    Institute of Scientific and Technical Information of China (English)

    宋庆珍; 高惠英

    2012-01-01

    The interstitial lung disease can usually be discovered in rheumatoid arthritis,and it has high incidence rate and case fatality.It can be misdiagnosis and missed diagnosis in early period,and appearance pulmonary interstitial fibrosis in end,which is very difficult to treat in clinic.Recent years,there are more research of serum surfactant protein in interstitial lung disease.This articles will review the diagnosis and treatment progress of interstitial lung disease complicated by rheumatoid arthritis,and research advance of serum surfactant proteins on interstitial lung disease,so that provide new approach of early diagnosis and therapeutic efficacy observation of this disease.%类风湿关节炎合并间质性肺病发病率、病死率高,早期易误诊、漏诊,尤其后期出现肺间质纤维化,临床治疗很困难.近年来,血清表面活性蛋白在间质性肺病研究较多.本文基于对类风湿关节炎合并间质性肺病的诊断和治疗进展方面的现代认识及血清表面活性蛋白在间质性肺病的研究进展进行综述,为该疾病早期诊断和治疗效果观察提供新的方法和手段.

  5. 类风湿关节炎相关性间质性肺疾病的临床研究%Clinical study of rheumatoid arthritis-associated interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    张艳敏; 姜莉

    2011-01-01

    Objective To analyse the clinical feature and risk factors of rheumatoid arthritisassociated interstitial lung disease (RA-ILD). Methods The data of 135 patients with rheumatoid arthritis (RA) hospitalized in the hospital in 2009 were retrospectively analyzed. The study factors included gender, age, disease duration, clinical manifestations (including arthritis, rheumatoid nodules, fever, dry mouth and dry eyes, Raynaud's phenomenon, skin rash), biochemical and immunological indexes, and treatment measures. The date were analyzed with Logistic regression analysis. Results In 135 RA patients, 48 cases (35.6%) had interstitial lung disease (ILD), in which 40 cases (83.3%) were diagnosed combined ILD (85±75) months after the diagnosis of RA, and 29 cases (60.4%) had no respiratory symptoms in the detection of ILD. Univariate analysis showed that age, duration, incidence of cough, dyspnea and Velcro tone, positive rate of rheumatoid factor (RF), C3 and γ-globulin in RA-ILD group were higher than those in RA without ILD group ( P <0.05 or P <0.01). Multivariate analysis showed that cough (OR =4.387,95%CI :1.143-16.831, P <0.05), Velcro tone (OR =6.727,95% CI :2. 220-20. 378, P <0.01),and RF ( OR =3. 522,95% CI :1. 304-9. 512, P <0.05) were risk factors of RA-ILD. Multivariate analysis identified that the sensitivity of RF was highest (79.2%), and the positive predictive value of cough was highest (73.3%) in three risk factors. Conclusions RA is diagnosed before ILD in most of patients. The interstitial pulmonary damages of some RA patients are subclinical. Cough,Velcro tone and RF are interrelated factors of interstitial pulmonary damages in RA patients. When patients have these symptoms, signs or abnormal laboratory examination, interstitial lung disease should be highly alerted, the relevant checks should be consummated in time, and rational treatment should be carried out to improve prognosis.%目的 探讨类风湿关节炎

  6. Expression of TRAIL and its receptors in interstitial lung tissue of autoimmune myositis in rats%TRAIL及其受体在自身免疫性炎性肌病大鼠肺组织中的表达

    Institute of Scientific and Technical Information of China (English)

    赵华; 王国春

    2011-01-01

    目的 探讨自身免疫性炎性肌病肺间质病变的病因及发病机制.方法 用异种动物骨骼肌匀浆免疫诱导建立自身免疫性炎性肌病大鼠模型,HE染色和Masson染色观察肌肉和肺组织病理改变,免疫组织化学染色结合计算机图像分析方法检测TRAIL及其受体在病变肺组织中的表达.结果 45例自身免疫性炎性肌病大鼠中有16只出现不同程度的肺间质病变(P<0.05);病变肺组织中TRAIL表达由对照组的2 035±657升高至8 934±741(P<0.05),DCR1表达由对照组的6 985±497降低至1 996±401(P<0.05),局部炎性细胞浸润高表达DCH2;肺泡炎性反应程度与肺组织中CD8+T淋巴细胞浸润相关(P<0.01),肺纤维化程度与肺组织中TRAIL表达升高相关(P<0.05).结论 首次证实自身免疫性炎性肌病大鼠模型出现与特发性炎性肌病患者相似肺间质病变,且与局部异常活跃的免疫炎性反应有关,TRAIL及其受体可能参与其中.%Objective To investigate the pathogenesis of interstitial lung disease (ILD) in experimental autoimmune myositis (EAM) models.Methods EAM mode was established by injecting the homogenate of rabbit skeletal muscle with Freund's complete adjuvant.The histopathological features of the muscle and lung from the EAM model were investigated by HE staining and Masson's trichrome.The expression of TRAIL and TRAIL receptors (TRAILR1, TRAIL-R2, TRAIL-R3, and TRAIL-R4) in lung tissues was measured by immunohistochemical method and computer image analysis.Results Sixteen of 45 rats with autoimmune inflammatory myopathies had varying degrees of interstitial lung disease ( P < 0.05 ); TRAIL expression was increased in lung tissue (2 035 ± 657 ) and that of DCR1 was decreased (6 985 ±497) (P <0.05); Inflammatory cells infiltrated locally were found with high expression of DCR2; Alveolitis was related with CD8 + T lymphocytes ( P < 0.001 ) and pulmonary fibrosis was related with TRAIL expression in lung tissues (P

  7. Retrospective analysis for TCM symptoms characteristic of interstitial lung disease associated with rheumatoid arthritis%类风湿关节炎相关肺间质病变中医证候特征回顾性分析

    Institute of Scientific and Technical Information of China (English)

    李松伟; 李素云; 郭洪涛; 黄云台; 李桓; 王济华

    2013-01-01

    目的:分析类风湿关节炎相关肺间质病变(RA-ILD)患者的中医证候特征.方法:运用回顾性研究的方法,总结68例RA-ILD患者,分析合并疾病、肺损伤程度、中医证候特征.结果:RA-ILD常合并贫血、干燥综合征、肺部感染等;有呼吸道症状43例;肺部HRCT表现以磨玻璃样变、网格状影及蜂窝样改变为主;肺功能主要表现为通气功能或弥散功能障碍.证候方面:以肺、脾、肾为主,主要以气虚及阴虚为主.标实证多见痰瘀证、痰热证、痰湿证.结论:RA-ILD以本虚标实为主,肺肾气阴两虚证合并痰瘀证为主要证候类型.%Objective: To investigate the syndrome rules of rheumatoid arthritis-interstitial lung disease(RA-ILD). Methods: A retrospective study of 68 patients with RA-ILD were performed. The patients informations including the clinical symptoms, complications, lung HRCT, pulmonary function, experiments results, et al. Results: The complications of RA-ILD mainly included anemia, sjogren's syndrome, lung infection. In addition to, we found that pulmonary HRCT, pulmonary function, ESR and CRP show a significant positive correlations with the disease. The common syndrome types of RA-ILD included arthritis, fatigue, shortness of breath, cough, dry mouth, weigh loss, and et al. The main syndrome types of deficiency of the root included qi deficiency, yin deficiency, lung-kidney qi-yin deficiency. The main syndrome types of excess in superficiality included phlegm and blood, phlegm and heat, phlegm and wet Conclusion: We believe that the basic pathology of RA-ILD is deficiency in origin and excess in superficiality, and the main syndrome type is lung-kidney qi-yin deficiency and stagnation of phlegm and blood.

  8. Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders

    Science.gov (United States)

    2016-04-19

    Lung Transplant; Lung Resection; Lung Cancer; Asthma; Cystic Fibrosis; Chronic Obstructive Pulmonary Disease; Emphysema; Mesothelioma; Asbestosis; Pulmonary Embolism; Interstitial Lung Disease; Pulmonary Fibrosis; Bronchiectasis; Seasonal Allergies; Cold Virus; Lung Infection; Pulmonary Hypertension; Pulmonary Dysplasia; Obstructive Sleep Apnea

  9. 原发性干燥综合征发生肺间质病变后的临床特点分析%Clinical features of interstitial lung involvement in primary Sj(o)gren's syndrome

    Institute of Scientific and Technical Information of China (English)

    安媛; 张学武; 何菁; 栗占国

    2009-01-01

    Objective To analyse the clinical features and risk factors of interstitial lung disease in primary SjSgren's syndrome (pSS).Methods A retrospective study was employed.All 130 pSS patients who were hospitalized during 2004 to 2006 were enrolled into this study.Thirty-seven clinical and laboratory variables were used as the research factors and interstitial lung involvement as the related factor.The data were analyzed with the Logistic regression model.Results of 130 patients,22(16.9%) had interstitial lung involvement.Twelve patients were asymptomic when pulmonary involvement was detected.Variables in the univariate analysis which were significantly associated with lung fibrosis were age,exocrine glands(including parotid,sublingual gland and submandibular gland) swelling,oral ulceration and fever.Positive rate of antiU1RNP antibody in group of lung damage was significantly higher than non-lung damage group [5/22(22.7%)vs 6/108 (5.6%),P=0.021].In the Logistic regression model.variables which were significantly associated with pulmonary involvement were exocrine glands swelling (OR=3.739,95%CI 1.069~16.079,P<0.05).oral ulceration (OR=3.739,95%CI 1.069~16.079,P<0.05)and fever (OR=3.067,95%CI 1.198~20.067,P<0.05).Conclusions This study indicates that some of the pulmonary damages of pSS are subclinical.Exocrine glands swelling,oral ulceration and fever are the risk factors for interstitial pulmonary involvements in pSS.%目的 探讨原发性干燥综合征(pSS)发生肺间质病变后的临床特点和相关因素.方法 回顾性分析2004-2006年我院风湿免疫科住院的130例pSS病例.以患者首次出现症状为研究起点,以胸部高分辨CT检查发现肺间质病变为研究终点.研究因素包括:年龄,病程,临床表现包括口干、眼干、外分泌腺肿大(腮腺、下颌下腺、舌下腺)、雷诺现象、口腔溃疡等,免疫指标,唇黏膜活检及治疗措施等37个变量.建立Logestic回归分析模型,计算OR值及95

  10. Normal Lung Quantification in Usual Interstitial Pneumonia Pattern: The Impact of Threshold-based Volumetric CT Analysis for the Staging of Idiopathic Pulmonary Fibrosis.

    Directory of Open Access Journals (Sweden)

    Hirotsugu Ohkubo

    Full Text Available Although several computer-aided computed tomography (CT analysis methods have been reported to objectively assess the disease severity and progression of idiopathic pulmonary fibrosis (IPF, it is unclear which method is most practical. A universal severity classification system has not yet been adopted for IPF.The purpose of this study was to test the correlation between quantitative-CT indices and lung physiology variables and to determine the ability of such indices to predict disease severity in IPF.A total of 27 IPF patients showing radiological UIP pattern on high-resolution (HR CT were retrospectively enrolled. Staging of IPF was performed according to two classification systems: the Japanese and GAP (gender, age, and physiology staging systems. CT images were assessed using a commercially available CT imaging analysis workstation, and the whole-lung mean CT value (MCT, the normally attenuated lung volume as defined from -950 HU to -701 Hounsfield unit (NL, the volume of the whole lung (WL, and the percentage of NL to WL (NL%, were calculated.CT indices (MCT, WL, and NL closely correlated with lung physiology variables. Among them, NL strongly correlated with forced vital capacity (FVC (r = 0.92, P <0.0001. NL% showed a large area under the receiver operating characteristic curve for detecting patients in the moderate or advanced stages of IPF. Multivariable logistic regression analyses showed that NL% is significantly more useful than the percentages of predicted FVC and predicted diffusing capacity of the lungs for carbon monoxide (Japanese stage II/III/IV [odds ratio, 0.73; 95% confidence intervals (CI, 0.48 to 0.92; P < 0.01]; III/IV [odds ratio. 0.80; 95% CI 0.59 to 0.96; P < 0.01]; GAP stage II/III [odds ratio, 0.79; 95% CI, 0.56 to 0.97; P < 0.05].The measurement of NL% by threshold-based volumetric CT analysis may help improve IPF staging.

  11. Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.

    Science.gov (United States)

    Watanabe, Ryu; Shirai, Tsuyoshi; Tajima, Yumi; Ohguchi, Hiroto; Onishi, Yasushi; Fujii, Hiroshi; Takasawa, Naruhiko; Ishii, Tomonori; Harigae, Hideo

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.

  12. ILO尘肺胸片分类标准在间质性肺疾病中的应用%The Radiographic Description of Pneumoconiosis with International Labor Office Classification in Diffused Interstitial Lung Disease

    Institute of Scientific and Technical Information of China (English)

    王洪武; 李庆棣; 朱元珏

    2001-01-01

    To analyze the changes of Sarc and idiopathic pulmornary fibrosis(IPF)with ILO (International Labor Office)pneumocniosis classification method. Methods: The types and degrees of Sanc and IPF were analyzed according to their chest X-ray changes compared with the standard ILO pneumconiosis X-ray in 78 Sarc(stage 2,3)and 25 IPF patients. Resuits:The main X-ray change was linear, and the second one was complex. This change deteriorated from upper to lower lung,and there was a significant difference between them. The lesion of upper and middle lung in IPF was more severe than that in Sarc. The density of lower lung at stage 3 was higher than that at stage 2 in Sarc. Conclusion: ILO pneumoconiosis classification method is useful in qualitatively and quantitatively analyzing diffused interstitial lung disease.%目的:采用ILO尘肺胸片分类方法分析胸内结节病(Sarc)及特发性肺间质纤维化(IPF)的肺内病变情况。方法:78例Sarc(Ⅱ、Ⅲ期)及25例IPF的普通X线胸片,对照ILO尘肺标准胸片分析Sarc及IPF肺内间质病变的类型和程度。结果:两种疾病肺内病变均以线状影为主,次为混合影。上、中、下肺野病变依次加重,彼此间有明显差异。IPF上、中肺野病变重于Sanc相应肺野。SarcⅢ期两下肺野病变密集度也高于Ⅱ期。同种疾病左、右两侧病变的类型和程度相似。结论:ILO尘肺胸片分类方法有助于间质性肺疾病肺内病变的定性和定量分析。

  13. Computed Tomography–Guided Interstitial High-Dose-Rate Brachytherapy in Combination With Regional Positive Lymph Node Intensity-Modulated Radiation Therapy in Locally Advanced Peripheral Non–Small Cell Lung Cancer: A Phase 1 Clinical Trial

    Energy Technology Data Exchange (ETDEWEB)

    Xiang, Li; Zhang, Jian-wen; Lin, Sheng; Luo, Hui-Qun; Wen, Qing-Lian; He, Li-Jia; Shang, Chang-Ling; Ren, Pei-Rong; Yang, Hong-Ru; Pang, Hao-Wen; Yang, Bo; He, Huai-Lin [Department of Oncology, Affiliated Hospital of Luzhou Medical College, Luzhou (China); Chen, Yue, E-mail: chenyue5523@126.com [Department of Nuclear Medicine, Affiliated Hospital of Luzhou Medical College, Luzhou (China); Wu, Jing-Bo, E-mail: wjb6147@163.com [Department of Oncology, Affiliated Hospital of Luzhou Medical College, Luzhou (China)

    2015-08-01

    Purpose: To assess the technical safety, adverse events, and efficacy of computed tomography (CT)-guided interstitial high-dose-rate (HDR) brachytherapy in combination with regional positive lymph node intensity modulated radiation therapy in patients with locally advanced peripheral non–small cell lung cancer (NSCLC). Methods and Materials: Twenty-six patients with histologically confirmed NSCLC were enrolled in a prospective, officially approved phase 1 trial. Primary tumors were treated with HDR brachytherapy. A single 30-Gy dose was delivered to the 90% isodose line of the gross lung tumor volume. A total dose of at least 70 Gy was administered to the 95% isodose line of the planning target volume of malignant lymph nodes using 6-MV X-rays. The patients received concurrent or sequential chemotherapy. We assessed treatment efficacy, adverse events, and radiation toxicity. Results: The median follow-up time was 28 months (range, 7-44 months). There were 3 cases of mild pneumothorax but no cases of hemothorax, dyspnea, or pyothorax after the procedure. Grade 3 or 4 acute hematologic toxicity was observed in 5 patients. During follow-up, mild fibrosis around the puncture point was observed on the CT scans of 2 patients, but both patients were asymptomatic. The overall response rates (complete and partial) for the primary mass and positive lymph nodes were 100% and 92.3%, respectively. The 1-year and 2-year overall survival (OS) rates were 90.9% and 67%, respectively, with a median OS of 22.5 months. Conclusion: Our findings suggest that HDR brachytherapy is safe and feasible for peripheral locally advanced NSCLC, justifying a phase 2 clinical trial.

  14. A Special Electroencephalography Pattern Might Help in the Diagnosis of Antibody-positive Encephalitis

    Institute of Scientific and Technical Information of China (English)

    Li-Ping Mei; Li-Ping Li; Jing Ye; Yu-Ping Wang; Jun Zhao; Tong Zhang

    2015-01-01

    Background:Patterns observed with electroencephalography (EEG) for patients who have encephalitis are usually known as generalized nonspecific cerebral abnormalities.The aim of this study was to investigate the presence of a special EEG pattern for patients with encephalitis and to explore features related to this special and uncommon pattern.Methods:EEG monitoring was performed for every patient aged > 15 years with encephalitis who was hospitalized between December 2011 and March 2014.Clinical characteristics and EEG recordings were collected and evaluated.Results:Fifty-two patients with encephalitis were enrolled in our study with a 2-h median EEG recording time,and extreme beta brushes (EBBs) occurred in 17 patients (32.7%).Its presence was not significant regarding gender,age,psychiatric medication use,EEG rhythmic disorganization (P > 0.05).Nevertheless,among the patients with EBBs,nine patients (52.9%) had epileptic seizures that had a significant detection rate (P < 0.05); moreover,the cerebrospinal fluid (CSF) or serum of 15 patients (88.2%) with EBBs was positive for antibodies (P < 0.05).Four patients (23.5%) who had EBB had corresponding regional distributions on neuroimaging scans.The EBBs completely correlated with the regional distributions of spike discharges for four patients.Conclusion:EBB is a special EEG pattern for patients with encephalitis,especially those with epileptic seizures or who have antibody-positive CSF/serum,and should be considered in clinical practice.

  15. Interstitial pulmonary fibrosis in an automobile body shop worker

    Energy Technology Data Exchange (ETDEWEB)

    Fischbein, A.; Rohl, A.N.; Suzuki, Y.; Bigman, O.

    1985-09-01

    Interstitial pulmonary fibrosis (IPF) is often of uncertain etiology and is therefore named idiopathic pulmonary fibrosis. Some occupational exposures, however, are known to cause interstitial fibrosis, asbestos and silica being well-known examples. The authors present clinical and pathological findings of a case with IPF and the results of microchemical analysis of inorganic particulate matter in the lung tissue. A very high lung burden of inorganic contaminants was found, including silica and metallic compounds. Emphasis is given to the importance of obtaining detailed occupational histories and conducting microchemical analysis of lung tissue in order to clarify etiological factors in cases with idiopathic pulmonary fibrosis.

  16. 肿瘤标志物在类风湿性关节炎伴间质性肺病中的临床意义%Clinical value of tumor markers in rheumatoid arthritis with interstitial lung diseases

    Institute of Scientific and Technical Information of China (English)

    夏俊波; 严秀娟; 任振义; 陈灏; 王娇莉

    2012-01-01

    目的 探讨肿瘤标志物在类风湿性关节炎伴间质性肺病(RA-ILD)中的临床意义.方法 收集2008年1月至2011年1月住院的82例类风湿关节炎患者.根据是否伴有间质性肺病,将其分为RA-ILD组(38例)和RA组(44例),比较两组血清肿瘤标志物癌胚抗原(CEA)、糖蛋白抗原15-3(CA15-3)、糖蛋白抗原19-9(CA19-9)水平、抗环瓜氨酸肽(CCP)抗体水平和肺功能包括用力肺活量(FVC)、第1秒用力呼气量(FEV1)、一氧化碳弥散量(DL)占预计值百分比,分析两组患者肿瘤标志物表达差异以及RA-ILD组肿瘤标志物与肺功能、抗CCP抗体相关性.结果 RA-ILD组外周血CEA、CA15-3、CA19-9水平均明显高于RA组(均P<0.01);RA-ILD组FVC、FEV1和DLco占预计值百分比均明显低于RA组(均P<0.01),但RA-ILD组抗CCP抗体水平与RA组比较差异无统计学意义(P >0.05).RA-ILD组CEA水平与FVC、FEV1和DLco呈负相关(r=-0.499、-0.576、-0.512,均P<0.01),但CEA、CA15-3和CA19-9均与抗CCP抗体无相关性(r=-0.192、-0.264、-0.268,均P >0.05).结论 肿瘤标志物CEA、CA15-3和CA19-9在RA-ILD中表达升高,CEA可能反应RA-ILD肺间质受损的严重程度,而不是关节损害的严重程度.%Objective To assess the clinical significance of tumor markers (TMs) in rheumatoid arthritis (RA) with interstitial lung diseases (ILD). Methods Eighty-two RA inpatients were admitted in Hangzhou First People's Hospital from January 2008 to January 2011; among whom 38 were complicated by interstitial lung diseases (RA-ILD). Tumor markers (CEA, CA15-3, CA19-9), pulmonary function indexes (FVC, FEVi, DLco) and anti-cyclic citrullinated peptide antibody (anti-CCP) levels were measured in all patients. Correlation between TMs and pulmonary function indexes, level of anti-CCP antibody was analyzed by Spearman rank correlation. Results Serum levels of CEA, CA15-3 and CA19-9 in RA-ILD group were significantly higher than those in RA group (P0.05). Serum CEA level was

  17. Interstitial fluid flow of alveolar primary septa after pneumonectomy.

    Science.gov (United States)

    Haber, Shimon; Weisbord, Michal; Mishima, Michiaki; Mentzer, Steve J; Tsuda, Akira

    2016-07-01

    Neoalveolation is known to occur in the remaining lung after pneumonectomy. While compensatory lung growth is a complex process, stretching of the lung tissue appears to be crucial for tissue remodeling. Even a minute shear stress exerted on fibroblasts in the interstitial space is known to trigger cell differentiation into myofibroblast that are essential to building new tissues. We hypothesize that the non-uniform motion of the primary septa due to their heterogeneous mechanical properties under tidal breathing induces a spatially unique interstitial flow and shear stress distribution in the interstitial space. This may in turn trigger pulmonary fibroblast differentiation and neoalveolation. In this study, we developed a theoretical basis for how cyclic motion of the primary septal walls with heterogeneous mechanical properties affects the interstitial flow and shear stress distribution. The velocity field of the interstitial flow was expressed by a Fourier (complex) series and its leading term was considered to induce the basic structure of stress distribution as long as the dominant length scale of heterogeneity is the size of collapsed alveoli. We conclude that the alteration of mechanical properties of the primary septa caused by pneumonectomy can develop a new interstitial flow field, which alters the shear stress distribution. This may trigger the differentiation of resident fibroblasts, which may in turn induce spatially unique neoalveolation in the remaining lung. Our example illustrates that the initial forming of new alveoli about half the size of the original ones.

  18. Characteristics of anti-hepatitis C virus antibody-positive patients in a hospital setting in Douala, Cameroon

    Directory of Open Access Journals (Sweden)

    Henry Namme Luma

    2016-04-01

    Conclusions: In the gastroenterology clinic of Douala General Hospital, while almost 40% of patients who were anti-HCV antibody-positive were asymptomatic and diagnosed fortuitously, some already presented complications, including cirrhosis and hepatocellular carcinoma. There is an urgent need to put in place programs to increase awareness and diagnosis of HCV infection and to develop extensive and targeted anti-HCV treatment guidelines to improve the management of these patients in Cameroon.

  19. The clinical study on classification and diagnostic procedures of diffuse parenchymal lung disease/interstitial lung disease in 349 children%儿童弥漫性实质性肺疾病349例分类及诊断程序研究

    Institute of Scientific and Technical Information of China (English)

    张晶莹; 刘秀云; 彭芸; 周春菊

    2013-01-01

    Objective To better understand the spectrum of the disease and to explore the clinical significance of diagnostic procedures in clinical application. Method The medical records of 349 children with diffuse parenchymal lung disease /interstitial lung disease admitted to Beijing Children's hospital from July 2001 to September 2011 were studied retrospectively. The diagnostic approaches were applied, including the history, physical examination, non-invasive examination and invasive examination. Result The 321 cases were made specific diagnosis through the diagnostic procedures and 28 cases were unclassified. The classification of the 349 cases were as follows:146 cases were with primary lung diseases, 14 cases were with lung disease secondary to systemic disease, 161 cases had the lung disease of known causes, and the 28 cases were unclassified. The 160/349(45.85%) cases were diagnosed based on the noninvasive examination, including all 147 cases of bronchiolitis obliterans, 3 cases of hypersensitive pneumonitis, 2 cases secondary to aspiration, 2 cases of systemic lupus erythematosus, 2 cases of juvenile rheumatoid arthritis, 1 case infected by CMV, 1 case of Langerhans cell histocytosis, 1 case of undifferentiated connective tissue disease and 1 case of Wegener's granulo-matosis. The others were diagnosed by invasive examination, including 2 cases of Langerhans cell histocytosis and 1 case of dermatomyositis by skin biopsies, 1 case of Langerhans cell histocytosis by lymphaden biopsy, all 113 cases of idiopathic pulmonary hemosiderosis, 2 cases of eosinophilic pneumonia and 2 cases of hypersensitive pneumonitis by bronchoalveolar lavage fluid; 40 cases were diagnosied by the lung biopsy, including 23 cases of idiopathic interstitial pneumonia. Conclusion Diffuse parenchymal lung disease in children is a heterogeneous group of respiratory disorders. Bronchiolitis obliterans, idiopathic pulmonary hemosiderosis and idiopathic interstitial pneumonia are the most common

  20. Acute and subacute idiopathic interstitial pneumonias.

    Science.gov (United States)

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.

  1. Computer-Aided Tomographic Analysis of Interstitial Lung Disease (ILD in Patients with Systemic Sclerosis (SSc. Correlation with Pulmonary Physiologic Tests and Patient-Centred Measures of Perceived Dyspnea and Functional Disability.

    Directory of Open Access Journals (Sweden)

    Fausto Salaffi

    Full Text Available This study was designed (a to evaluate an improved quantitative lung fibrosis score based on a computer-aided diagnosis (CaM system in patients with systemic sclerosis (SSc,-related interstitial lung disease (SSc-ILD, (b to investigate the relationship between physiologic parameters (forced vital capacity [FVC] and single-breath diffusing capacity for carbon monoxide [DLCO], patient-centred measures of dyspnea and functional disability and CaM and visual reader-based (CoVR methods, and (c to identify potential surrogate measures from quantitative and visual HRCT measurement.126 patients with SSc underwent chest radiography, HRCT and PFTs. The following patient-centred measures were obtained: modified Borg Dyspnea Index (Borg score, VAS for breathing, and Health Assessment Questionnaire-Disability Index (HAQ-DI. HRCT abnormalities were scored according to the conventional visual reader-based score (CoVR and by a CaM. The relationships among the HRCT scores, physiologic parameters (FVC and DLCO, % predicted results and patient-centred measures, were calculated using linear regression analysis and Pearson's correlation. Multivariate regression models were performed to identify the predictor variables on severity of pulmonary fibrosis.Subjects with limited cutaneous SSc had lower HAQ-DI scores than subjects with diffuse cutaneous SSc (p <0.001. CaM and CoVR scores were similar in the 2 groups. In univariate analysis, a strong correlation between CaM and CoVR was observed (p <0.0001. In multivariate analysis the CaM and CoVR scores were predicted by DLco, FVC, Borg score and HAQ-DI. Age, sex, disease duration, anti-topoisomerase antibodies and mRSS were not significantly associated with severity of pulmonary fibrosis on CaM- and CoVR methods.Although a close correlation between CaM score results and CoVR total score was found, CaM analysis showed a more significant correlation with DLco (more so than the FVC, patient-centred measures of perceived

  2. The idiopathic interstitial pneumonias: understanding key radiological features

    Energy Technology Data Exchange (ETDEWEB)

    Dixon, S. [Department of Radiology, Churchill Hospital, Old Road, Oxford OX3 7LJ (United Kingdom); Benamore, R., E-mail: Rachel.Benamore@orh.nhs.u [Department of Radiology, Churchill Hospital, Old Road, Oxford OX3 7LJ (United Kingdom)

    2010-10-15

    Many radiologists find it challenging to distinguish between the different interstitial idiopathic pneumonias (IIPs). The British Thoracic Society guidelines on interstitial lung disease (2008) recommend the formation of multidisciplinary meetings, with diagnoses made by combined radiological, pathological, and clinical findings. This review focuses on understanding typical and atypical radiological features on high-resolution computed tomography between the different IIPs, to help the radiologist determine when a confident diagnosis can be made and how to deal with uncertainty.

  3. Clinical Characteristics of Rheumatic Disease -related Interstitial Lung Disease%风湿性疾病相关性间质性肺病患者的临床特点分析

    Institute of Scientific and Technical Information of China (English)

    唐开奖; 宋星慧; 蒋茵; 韦阳妙; 覃玉花

    2016-01-01

    目的 分析风湿性疾病相关性间质性肺病(RD-ILD)患者的临床特点,提高临床对该病的认识.方法 选择2013年1月—2014年6月在广西医科大学第四附属医院风湿免疫科确诊的风湿性疾病(RD)患者513例,统计其间质性肺病(ILD)发生情况,回顾性分析RD-ILD患者原发病、胸部高分辨率CT检查结果、肺功能检查结果及血气分析结果.结果 本组513例RD患者发生ILD 87例(占17.0%).原发病:系统性硬化34例(占39.0%)、类风湿关节炎21例(占24.1%)、干燥综合征16例(占18.4%)、系统性红斑狼疮8例(占9.2%)、肌炎/皮肌炎5例(占5.7%)、其他3例(占3.4%).胸部高分辨率CT检查结果显示,25例(28.7%)为普通型间质性肺炎(UIP),62例(71.3%)为非UIP,其中类风湿关节炎患者UIP发生率最高,为42.9%.肺功能检查结果显示,肺功能正常16例(占18.4%),限制性通气功能障碍47例(占54.0%),阻塞性通气功能障碍2例(占2.3%),混合性通气功能障碍4例(占4.6%),弥散功能障碍60例(占69.0%).血气分析结果显示,正常42例(占48.2%)、低氧血症27例(占31.3%)、Ⅰ型呼吸衰竭15例(占17.2%)、Ⅱ型呼吸衰竭3例(占3.4%).结论 RD患者ILD发生率较高,其中以系统性硬化、类风湿关节炎及干燥综合征患者多见,RD-ILD患者肺功能障碍主要表现为弥散功能障碍和限制性通气功能障碍,常伴有低氧血症及Ⅰ型呼吸衰竭.%Objective To analyze the clinical characteristics of rheumatic disease - related interstitial lung disease (RD-ILD),to improve the understanding of clinicians. Methods From January 2013 to June 2014,a total of 513 patients with rheumatic disease was collected in the Department of Rheumatology, the Fourth Affiliated Hospital of Guangxi Medical University,the incidence of RD-ILD was recorded,and primary illness,chest high - resolution CT examination results, pulmonary function examination results and blood - gas analysis examination results of patients with RD

  4. Interstitial lung disease as an initial manifestation of dermatomyositis%以间质性肺疾病起病的皮肌炎临床特点分析

    Institute of Scientific and Technical Information of China (English)

    沈敏; 龚瑜林; 曾小峰; 张奉春; 唐福林

    2014-01-01

    目的 探讨以间质性肺疾病(ILD)起病的皮肌炎患者的临床特点和预后.方法 北京协和医院1999年1月至2013年1月皮肌炎并ILD住院患者资料完整者共184例,回顾性分析其临床表现、生化指标、抗体谱、影像学、肺功能、肺活体组织检查学、治疗及转归等,并对ILD起病组及非ILD起病组皮肌炎患者进行上述指标的比较.结果 皮肌炎住院患者并发ILD的发生率为17%,平均年龄(48±12)岁,男∶女=63∶121.ILD起病组88例(47.8%),包括ILD与皮肌炎同时发生(1个月内)42例(22.8%),以及ILD先于皮肌炎发生46例(25.0%),后者ILD平均先于皮肌炎(11±3)个月发生.ILD起病组活动后气短、咳嗽和肺部爆裂音的发生率高于非ILD起病组,而向阳疹、前胸Ⅴ区皮疹、披肩征和关节受累的发生率较后者低(P<0.05).ILD起病组与非ILD起病组相比,肌酶水平、抗核抗体和抗SSA阳性率更低(P<0.05),抗Jo-1抗体阳性率(13.6%)差异无统计学意义(P>0.05).ILD起病的皮肌炎主要表现为弥散功能及限制性通气功能障碍,一氧化碳弥散率显著低于非ILD起病组(P<0.01).ILD起病的皮肌炎临床病理类型主要是机化性肺炎和非特异性间质性肺炎.ILD起病组病死率19.3%,两组差异无统计学意义(P>0.05).死亡原因主要是ILD进展所致呼吸衰竭(13例,76.5%).结论 以ILD起病的皮肌炎临床常见,是皮肌炎住院患者主要死亡原因.其主要临床病理类型是机化性肺炎和非特异性间质性肺炎.%Objective To explore the clinical features and prognosis of dermatomyositis patients with interstitial lung disease (ILD) as an initial manifestation.Methods Medical records of 184 dermatomyositis inpatients complicated with ILD,admitted into Peking Union Medical College Hospital from January 1999 to January 2013,were retrospectively analyzed.The clinical features,biochemical parameters,positive rates of autoantibodies

  5. Irreversible Respiratory Failure in a Full-Term Infant with Features of Pulmonary Interstitial Glycogenosis as Well as Bronchopulmonary Dysplasia

    NARCIS (Netherlands)

    Jiskoot-Ermers, M.E.C.; Antonius, T.A.J.; Looijen, M.G.; Wijnen, M.H.W.A.; Loza, B.F.; Heijst, A.F.J. van

    2015-01-01

    Pulmonary interstitial glycogenosis (PIG) is a rare interstitial lung disease in the newborns. We report on the clinical presentation and pathological findings of a full-term male infant with pulmonary hypertension requiring extracorporeal membrane oxygenation (ECMO). An open lung biopsy demonstrate

  6. Heterophile antibody positive, acute cytomegaloviral infection in an immunocompetent pre-teen: an atypical presentation of an atypical infection.

    Science.gov (United States)

    Raja, Junaid; de Quesada, Gonzalo

    2015-01-01

    Mononucleosis and mononucleosis-like illnesses comprise a significant proportion of pediatric and adolescent infectious illnesses. By far, the most common cause of these illnesses is Epstein-Barr virus, which causes mononucleosis, and a distant second is cytomegalovirus, which is the most common cause of mononucleosis-like illnesses. This case provides an interesting juxtaposition of laboratory findings of an adolescent who was heterophile antibody positive but acute Epstein-Barr virus antigen-antibody negative. A subsequent immunologic assay resulted in a final diagnosis of an acute cytomegaloviral infection. This is, to our knowledge, the first such report in the literature.

  7. Interstitial pulmonary emphysema in ventilated neonates. Long-term observations

    Energy Technology Data Exchange (ETDEWEB)

    Schneider, K.; Fendel, H.

    1986-06-01

    106 (15.7%) of 675 artificially ventilated newborn developed interstitial pulmonary emphysema (PIE). Basic lung diseases were: IRDS, neonatal pneumonia, shock lung, meconium aspiration, hypoplasia of the lungs and other miscellaneous disorders of the chest. PIE developed in 68% of patients within 8 hours following artificial respiration. At the beginning of PIE both lungs were concerned in 41.5% of patients, one lobe of both lungs was affected in 32.1%. PIE was located in one lung in 8.5% and in only one lobe in 17.9%. Maximum of PIE was seen within 5 days after initiating respiration in 76.7% of the patients. Persistent PIE developed in 28.7% of the patients. Persistent PIE of both lungs was seen in 11 cases, PIE of one lung in 8 cases and persistent lobar emphysema in another 8. Pulmonary pseudocysts developed in 22 (20.8%) of the patients.

  8. 儿童慢性肺间质病变相关表面活性物质蛋白基因突变研究进展%Research progress in genic mutation of surfactant protein related to chronic interstitial lung diseases in children

    Institute of Scientific and Technical Information of China (English)

    徐秀娟; 刘恩梅

    2010-01-01

    @@ 慢性间质性肺疾病(chronic interstitial lung diseases,CILD)是一组主要累及肺间质、肺泡和(或)细支气管的肺部弥漫性疾病,具有以下特点:两肺野或大部分肺野布满粟粒状、大小不等的结节状、小片状、粗细不等的线条或网格状、蜂窝状阴影.

  9. Associated risk factors of patients with rheumatoid arthritis complicated with interstitial lung disease%类风湿关节炎合并肺间质病变的发病相关因素

    Institute of Scientific and Technical Information of China (English)

    孙文文; 朱芸; 李明; 刘芹; 周卉

    2013-01-01

    目的 分析类风湿关节炎(RA)合并肺间质病变(ILD)的临床及实验室特点,探讨其发病相关因素.方法 分析76例RA患者的临床资料,分为单纯RA组(n=40)和RA-ILD组(n=36),比较两组患者的一般情况、临床表现以及实验室指标.结果 两组在年龄、关节肿痛数目、血管炎及发热阳性率、CRP、RF、IgM、白细胞计数比较差异具有统计学意义(P<0.05).结论 年龄大,RF高滴度表达,RA病情重,伴有关节外表现的RA患者易合并ILD,应尽早行高分辨CT等检查早期诊断及治疗.%Objective To analyze the clinical characteristics and laboratory data of rheumatoid arthritis ( RA ) patients complicated with interstitial lung disease ( ILD ) and to investigate the associated risk factors causing HA-ILD. Methods 76 RA patients were divided into the RA group ( n = 40 ) and the RA-ILD group ( n = 36 ), and their clinical data were analyzed retrospectively. The general state of health, clinical manifestations, and levels of serum markers were compared between the two groups. Results The ages, the counts of involved joints, the positive rate of vasculitis and fever, the serum levels of CRP, RF, IgM and the number of white blood cells were obviously higher in the RA-ILD group than in the RA group with statistical significance ( P < 0. 05 ). Conclusions Chest HRCT should be taken to get early diagnosis and treatment of ILD in patients with RA, especially in the elder patients with high titer of RF, severer disease of RA and extra articular manifestations.

  10. Giant-cell interstitial pneumonia and hard-metal pneumoconiosis. A clinicopathologic study of four cases and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Ohori, N.P.; Sciurba, F.C.; Owens, G.R.; Hodgson, M.J.; Yousem, S.A.

    1989-07-01

    We report four cases of giant-cell interstitial pneumonia that occurred in association with exposure to hard metals. All patients presented with chronic interstitial lung disease and had open-lung biopsies that revealed marked interstitial fibrosis, cellular interstitial infiltrates, and prominent intraalveolar macrophages as well as giant cells displaying cellular cannibalism. We also review the literature to determine the sensitivity and specificity of giant-cell interstitial pneumonia for hard-metal pneumoconiosis. Although hard-metal pneumoconiosis may take the form of usual interstitial pneumonia, desquamative interstitial pneumonia, and giant-cell interstitial pneumonia, the finding of giant-cell interstitial pneumonia is almost pathognomonic of hard-metal disease and should provoke an investigation of occupational exposure. 25 references.

  11. The clinical analysis on rheumatoid arthritis-associated interstitial lung disease in the elderly patients%老年类风湿关节炎合并间质性肺疾病临床分析

    Institute of Scientific and Technical Information of China (English)

    陈丽明; 刘波; 李萍

    2013-01-01

    Objective To analyze the incidence, clinical characteristics, image features and pulmonary function tests of rheumatoid arthritis-interstitial lung disease (RA-ILD) in the elderly patients. Methods The clinical characteristics, image features and pulmonary function tests of 44 patients with RA-ILD among 520 patients with RA were retrospectively analyzed. The RA-ILD patients were divided into 2 groups by age: elderly group (group A, age ≥60 years old) and non-elderly group (group B, age 0.05); (2) The differences in the clinical manifestations of fever, cough, dyspnea, smoking and infection between the 2 groups were not statistically significant (P>0.05). The moist crackles were more frequently found in group A than in group B (P0.05); (4) The pulmonary function tests were obviously lower in group A than that in group B (P0.05). Conclusions The incidence of RA-ILD in the elderly patients with RA is higher than that in the non-elderly patients with RA. More attention should be paid to the elderly RA patients with moist crackles in case of RA-ILD. The HRCT of the lung and pulmonary function tests should be carried out in the elderly patients with RA in the early stage by clinicians. The small airway dysfunction and restrictive ventilation function abnormality occur more frequently in the elderly patients with RA-ILD than in the non-elderly patients with RA-ILD. The pulmonary function of the elderly patients with RA-ILD should be improved as early as possible.%  目的探讨老年类风湿关节炎(RA)合并间质性肺疾病(ILD)的发生率、临床特点、影像改变及肺功能的特点.方法回顾性分析520例 RA 患者中44例合并 ILD 患者(RA-ILD)的临床特点、影像学改变、肺功能情况.将 RA-ILD 患者按年龄分为老年组(A组,年龄≥60岁)和非老年组(B组,年龄0.05);(2)两组间发热、咳嗽、呼吸困难、吸烟、感染差异无统计学意义(P>0.05);老年组湿音发现率较非老年组高(P0.05);(4)

  12. Clinical characteristics analysis of 65 patients with the elderly interstitial lung diseases%65例老年间质性肺疾病临床特征分析

    Institute of Scientific and Technical Information of China (English)

    刘萍; 庄伟荣; 张雅娟; 周瑛; 秦兴国

    2011-01-01

    目的 探讨我院和市东医院老年患者间质性肺疾病(ILDs)的临床特点,以提高对ILDs的认识,加强对ILDs病因和基础疾病的分析,更好地治疗ILDs患者.方法 对我院和市东医院自2008年9月~2010年12月临床诊断为ILDs的病例进行回顾性分析,对比分析其病程、症状、体征及影像学(胸片、胸部高分辨CT或HRCT)、肺功能及血气分析结果,从中发现共性及各自的特点.结果 (1)65例老年ILDs患者中,特发性肺间质纤维化(IPF)发病率最高(43.08%),其余发病率较多的依次为:结缔组织病、慢性阻塞性肺气肿(COPD)、肺癌合并ILDs和充血性心力衰竭,分别占21.54%、12.31%、9.23%和7.69%.(2)不同病因的ILDs老年患者有ILDs的共同表现,但其临床症状、体征、影像学表现和肺功能等方面又有各自的特点.结论认识ILDs的共性和个性对ILDs的病因诊断、针对性治疗及判断疾病进展和预后具有重要意义.%Objective To discuss the clinical characteristics of the elderly interstitial lung diseases (ILDs) in our hospital and Shidong Hospital. This may help us know ILDs well, search their etiological factors, and treat them properly. Methods A total of 65 ILD patients in the past 27 months were included in this study. Their clinical courses, symptoms, physical signs, chest radiography features,computed tomographs (CT) or high resolution computed tomographs (HRCT) of the chest, pulmonary function, and arterial blood gas were analyzed and compared, so discovered general character and separately characteristic. Results ( 1 ) Among 65 patients, the ratio of idiopathic pulmonary fibrosis (IPF) was the highest (43.08%), followed by connective tissue diseases, chronic obstructive pulmonary disease ( COPD), lung cancer complicating ILD and congestive heart failure, with rations of 21.54%, 12. 31%, 9. 23% and 7. 69%.(2) ILD patients with different etiological factors had similar clinical manifestations, but

  13. 如何早期确诊间质性肺疾病疑似病例的临床诊断思路与程序%The clinical diagnosis ideas and procedures of how to confirm the suspected cases of interstitial lung disease in the early stage

    Institute of Scientific and Technical Information of China (English)

    吴亚梅; 陈亚娟

    2010-01-01

    @@ 间质性肺疾病(interstitial lung disease,ILD)又称为弥慢性实质性肺疾病(diffuse parenchymal lung disease,DPLD)是一大类疾病,包括200多个病种,肺间质纤维化是ILD一个结局性或终末期病理改变,此期激素治疗效果差,而目前尚无有效逆转纤维化的药物.并且,多数ILD起病隐匿,早期不易发现,一经确诊,纤维化已形成,往往表现为右心衰竭、呼吸衰竭,严重威胁生命[1].

  14. 类风湿性关节炎伴间质性肺病中肿瘤标志物与肺功能的相关性分析%Correlation of tumor markers with lung function in rheumatoid arthritis accompanied with interstitial lung diseases

    Institute of Scientific and Technical Information of China (English)

    夏俊波; 严秀娟; 任振义; 陈灏; 王娇莉

    2012-01-01

    Objective To explore the correlation of tumor markers with lung function in rheumatoid arthritis (RA) accompanied with interstitial lung diseases. Methods Eighty-two RA inpatients in our hospital from January 2009 to January 2011 were recruited in this study. Among these RA patients, 38 were accompanied with interstitial lung diseases, and the left 44 were not. Tumor markers , such as CEA, CA15-3, CA19-9, and pulmonary function indexes, including forced expiratory volume ( FVC) , first second forced expiratory volume (FEV, ) , carbon monoxide diffusion capacity ( DLC0), and anti-CCP antibody were detected in all of patients. Correlation between the expression of these markers and pulmonary function indexes, level of anti-CCP antibody was analyzed by Spearman Rank Correlation. Results Serum levels of CEA, CA15-3 and CA19-9 were significantly higher in RA-ILD group than in RA group ( P 0.05). Serum CEA level was inversely correlated with FVC ( r= -0.499, P 0. 05). Conclusion The expression of tumor markers, including CEA, CA15-3 and CA19-9 is increased in RA-ILD. CEA level may reflect the degree of lung function impairment in RA-ILD, but have no relation with the joint lesion severity.%目的 探讨肿瘤标志物在类风湿性关节炎伴间质性肺病(rheumatoid arthritis with interstitial lung disease,RA-ILD)中的表达及其临床意义.方法 收集本院呼吸科2008年1月至2011年1月住院的82例类风湿关节炎患者.根据是否伴有间质性肺病,将其分为RA-ILD组(38例)和RA组(44例),检测两组患者血清肿瘤标志物癌胚抗原( carcinoembryonic antigen,CEA)、糖蛋白抗原15-3( carbohydrate antigen 15-3,CA15-3)、糖蛋白抗原19-9( carbohydrate antigen 19-9,CA19-9)水平,抗环瓜氨酸肽(CCP)抗体水平和肺功能包括用力肺活量(FVC)、第1秒用力呼气量(FEV1)、一氧化碳弥散量( DLco)占预计值百分比,分析两组患者肿瘤标志物表达差异以及RA-ILD组肿瘤标志物与肺功

  15. Two novel mutations in surfactant protein-C, lung function and obstructive lung disease

    DEFF Research Database (Denmark)

    Baekvad-Hansen, Marie; Nordestgaard, Børge G; Tybjaerg-Hansen, Anne;

    2010-01-01

    Dominant mutations in the surfactant protein-C(SFTPC) gene have been linked with interstitial lung disease. The frequency of lung disease due to SFTPC mutations in the general population is unknown. The aim of this study was to identify novel SFTPC mutations that are associated with lung function...... pulmonary disease or interstitial lung disease. No Y106X heterozygotes suffered from asthma, chronic obstructive pulmonary disease (COPD), or interstitial lung disease. We identified two novel mutations in highly conserved areas of the SFTPC gene, and show that heterozygotes for the mutations have normal...... lung function and are unaffected by COPD and interstitial lung disease. A53T heterozygotes had increased asthma risk, but further research is required to conclusively determine whether this mutation is associated with asthma....

  16. 结缔组织病相关间质性肺疾病继发肺动脉高压的临床分析%Clinical analysis of connective tissue disease with interstitial lung disease complicating pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    温海艳; 姜莉

    2015-01-01

    Objective To study the clinical features of connective tissue disease (CTD) with interstitial lung disease (ILD) complicating pulmonary hypertension (PH).Methods The clinical data of 557 cases of CTD-ILD were retrospectively analyzed,the clinical characteristics of CTD-ILD-PH group and CTD-ILD group were compared.Results ① The incidence rate of ILD secondary to CTD was 27.65%,the prevalence rate of CTD-ILD-induced PH was 13.11%.②The primary diseases in PH secondary to CTD-ILD according to prevalence rate from high to low were:overlap syndrome,mixed connective tissue disease,systemic sclerosis,systemic lupus erythematosus,primary desiccation syndrome,polymyositis/dermatomyositis,and rheumatoid arthritis.③ The incidence rates of expectoration,breathless,dyspnea,Raynaud's phenomenon,skin hardens,and resting heart rate of CTD-ILD-PH group were higher than those of CTD-ILD group (all P <0.05).④The incidence rates of ground-glass opacity,grid shadow,interlobular septal thickening,thickening of pulmonary artery,heart enlargement and pleural effusion in pulmonary CT/HRCT of CTD-ILD-PH group were higher than those of CTD-ILD group (all P < 0.05).⑤FVC%pred,DLCO%pred,and PaO2 in CTD-ILD-PH group were lower than those in CTD-ILD group (all P < 0.05).⑥ The echocardiography showed that the incidence rates of right ventricular diameter,right ventricular outflow tract diameter,pulmonary artery diameter and three tricuspid regurgitation velocity in CTD-ILD-PH group were higher than those in CTD-ILD group (all P <0.05).⑦ The positive ANA and SM antibodies were prone to secondary PH.Conclusions ①The incidence rate of ILD secondary to CTD is 27.65%,the prevalence rate of CTD-ILD-induced PH is 13.11%.②Overlap syndrome or mixed connective tissue disease combined with ILD is more likely to complicating PH than other CTD.③When the CTD-ILD patients have expectoration,breathless,dyspnea,Raynaud' s phenomenon,skin hardens and increased heart rate

  17. Tumor interstitial fluid

    DEFF Research Database (Denmark)

    Gromov, Pavel; Gromova, Irina; Olsen, Charlotta J.

    2013-01-01

    secretion, non-classical secretion, secretion via exosomes and membrane protein shedding. Consequently, the interstitial aqueous phase of solid tumors is a highly promising resource for the discovery of molecules associated with pathological changes in tissues. Firstly, it allows one to delve deeper...

  18. [Persistent tachypnea in infants: possibly due to interstitial pneumonitis

    NARCIS (Netherlands)

    Kingma, M.L.; Nef, J.J.E.M. de; Krieken, J.H.J.M. van; Merth, I.T.

    2002-01-01

    In three male infants aged 3, 4.5 and 11 months with tachypnea and feeding problems, the initial supplementary examination revealed no possible cause. The tissue obtained by open lung biopsy showed interstitial pneumonia/pneumonitis. The two youngest patients were treated with hydrochloroquine and p

  19. Effects of Dragons Blood on Interstitial Over -repairing and TGFβ1 mRNA Expression in the Lung Tissue of BLM -lung- injury rat%龙血竭对大鼠肺损伤间质过度修复及其TGFβ1 mRNA表达的影响

    Institute of Scientific and Technical Information of China (English)

    杨礼腾; 刘欣; 程德云; 方洵; 穆茂; 胡晓波; 聂莉

    2012-01-01

    目的:探讨龙血竭(LXJ)对博莱霉素肺损伤大鼠肺间质过度修复及其TGFβ1mRNA表达的调控作用.方法:60只健康SD大鼠,随机分为生理盐水组(NS)、博莱霉素肺损伤组(BLM)及龙血竭组(LXJ),每组20只,再分成两亚组,每亚组10只,通过博莱霉素一次性气管内滴入复制肺损伤修复大鼠模型,并在肺损伤修复过程中予龙血竭灌胃干预,分别于14和28天取大鼠肺组织,运用组织芯片,进行HE、胶原纤维染色及免疫组化分别定量肺内炎性细胞数、胶原蛋白、Ⅰ型胶原,用实时荧光定量RT -PCR技术定量转化生长因子beta( TGFβ1)mRNA的表达.结果:龙血竭对两时相肺组织的炎症程度和胶原(IOD分别从0.5492±0.2105和0.9578±0.3756降至0.2749±0.1592和0.3181±0.1210)及28天的Ⅰ型胶原过度沉积(IOD从0.9348±0.3774降至0.5045±0.2796)皆有明显抑制作用(P<0.05),对两时相TGFβ1 mRNA表达(从0.0219±0.0110和0.0170±0.0157分别1降至0.0083±0.0024和0.0062±0.0051)有明显下调的作用(P<0.05).结论:龙血竭具有抑制炎症性肺损伤促进修复,并可通过抑制胶原尤其是Ⅰ型胶原的过度沉积而调控肺损伤肺间质过度修复之作用,后者可能主要与下调肺组织TGFβ1mRNA的表达有关.%Objectives:To investigate the effects of the Dragons Blood (LXJ) on interstitial over - repairing and transform growth factor betal(TGFβ1 )mRNA expression in the lung tissue of bleomycin( BLM) - lung - injury rat. Methods; 60 healthy Sprague - Dawley rats were randomly divided into the NS group ( NS) ,the BLM - lung - injury group ( BLM) and the Dragons Blood group ( LXJ) ,every group had 20 rats and were suhdivided into two subgroups by 14 days and 28 days courses,each subgroup had 10 rats. BLM -lung-injury rat models were established by blemycin -induced by way of once -dripping into trachea and prevented and treated with the Dragons Blood by primed -stomach,at day 14 and day 28 respectively,the rats

  20. RA-ILD 与临床和实验室指标相关性研究%Clinical and laboratory correlation study of rheumatoid arthritis combined with interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    张路路; 武丽君

    2016-01-01

    目的:收集类风湿关节炎合并肺间质病变(RA-ILD)患者的相关临床指标、实验室指标和肺部影像学表现等临床资料,分析这些因素与 RA-ILD 的发生和发展的关系,为 RA-ILD 的诊疗提供一定的临床依据。方法回顾性收集2014年1月-2015年1月在新疆维吾尔自治区人民医院风湿免疫科住院的 RA 患者,一般资料、临床表现、实验室指标和影像学检查。结果①204例 RA 患者中44例发生 ILD,占21.6%。6例胸闷、气短;6例有咳嗽、咳痰;6例听诊有 Velcro 音。比较发现 RA-ILD 组咳嗽咳痰、胸闷气短、Velcro 音的发生率高于单纯 RA 组(P 0.05)。④ RA-ILD 组抗 CCP 抗体滴度显著大于单纯 RA组(P 0.05)。结论 RA-ILD 患者年龄大、平均病程长,大部分临床表现不明显,需及时完善 HRCT,早期诊断和治疗。高抗 CCP 抗体滴度、ANA 阳性可能与 RA-ILD 的发生相关。%Objective To analyze the relationship between these factors and occurrence and development of RA-ILD by collecting the relevant clinical and laboratory data of rheumatoid arthritis with interstitial lung disease (RA-ILD)patients. Methods The clinical data of hospitalized patients with RA from January 2014 to January 2015 were retrospectively analyzed,including its population information,clinical manifestation,laboratory and imaging ex-amination. Results (1)44 cases occurred with RA-ILD in 204 patients,accounting for 21. 6% . There were 6 ca-ses of chest tightness and shortness of breath,6 cases of cough and sputum,and 6 cases with Velcro rales. Compared the RA-ILD group,the incidence of Velcro sound was higher than patients without ILD(P 0. 05).(4)The level of anti-CCP was significantly higher in the RA-ILD group than in the RA group(P 0. 05). Conclusion RA patients with ILD are more common with elderly age and longer average duration,but most of the clinical symptoms is not obvious. HRCT can provide a useful tool for

  1. Clinical Features and Risk Factors in Patients with Rheumatoid Arthritis Combined with Interstitial Lung Disease%类风湿关节炎合并肺间质病变的临床特点及相关因素

    Institute of Scientific and Technical Information of China (English)

    姜帆; 刘荣清

    2012-01-01

    目的 分析类风湿关节炎合并肺间质病变(RA-ILD)患者的临床特点及相关因素.方法 回顾性分析2006年9月-2011年5月我院住院及门诊病人93例RA-ILD及93例未合并ILD的类风湿关节炎(RA)患者的一般情况、临床特点及类风湿因子(RF)、血沉(ESR)、C反应蛋白(CRP)、抗核抗体(ANA)及抗角蛋白抗体(AKA)、抗环瓜氨酸肽(CCP)抗体、抗SSA/SSB抗体、红细胞计数(RBC)、血红蛋白(HGB)、血小板计数(PLT)进行比较.结果 RA-ILD组患者呼吸系统症状多数以咳嗽为首发症状.RA-ILD组患者年龄、RF滴度、吸烟构成比均显著高于无ILD的RA组,有统计学意义(P均<0.05).RA-ILD组病程、性别、ESR、CRP、ANA、抗SSA/SSB抗体,以及服用甲氨蝶呤与无ILD的RA组比较差异无统计学意义(P均>0.05).经过后退法建立Logistic回归分析模型,多因素分析显示年龄(OR=1.093,95%CI 1.053~1.136,P<0.05),年龄每增大1岁,危险性增加0.093倍.结论 年龄增大是RA发生ILD的相关因素,高龄的RA患者应警惕ILD的发生.%Objective To analyze the clinical features and risk factors of rheumatoid arthritis combined with interstitial lung disease (RA - ILD). Methods 93 cases with RA - ILDand 93 cases with RA without ILD from September,2006 to May,2011 were retrospectively analyzed. General,Clinical,rheumatoid factor (RF) , erythrocyte sedimentation rate (ESR) ,C -reactive protein (CRP) ,antinuclear antibody (ANA) ,anti -keratin antibody (AKA) ,anti -cyclic citrullinated peptide antibody (CCP) ,anti - SSA/anti -SSB,red blood cell (ESR) ,hemoglobin (HGB) and platelets (PLT) were compared between two groups. Results First symptoms of RA - ILD patients was cough. The age, RF level, prevalences of smoking in RA - ILD group were significantly higher than those in control group (P 0.05). The Logistic regression model showed that onset age were significantly associated with pulmonary disease ( OR = 1. 093 ,95% CI 1. 053 ~ 1. 136 , P < 0. 05

  2. 结缔组织病肺间质病变的临床特点及治疗效果分析%Interstitial lung disease in patients with connective tissue diseases

    Institute of Scientific and Technical Information of China (English)

    李杨; 桂明; 李英; 张浩; 季迎; 易斌

    2011-01-01

    Objective To investigate the prevalence and clinical characters of connective tissue diseases- interstitial lung disease (CTD-ILD). Methods Eight-two patients with CTD-ILD were studied. Every patients had radiography and high-resolution computed tomography (HRCT). Results ( 1 ) ILD positive rate in SSc is 70. 0%, more than other CTDs. SLE-ILD patients are yungest ( 26. 2±7. 8 ). RA-ILD and SSc-ILD have longer course of disease than pSS-ILD, SLE-ILD and PM-ILD. RA-ILD espiratory symptoms are very light. 85.7% SSc-ILD accompany with Raynaud's phenomenon. (2) Honey combing and mediastinal lymphadenectasis are the most imagings on the HRCT of pSS-ILD. Ground glass opacity was commonly seen in SLE-ILD. (3) compared images of HRCT, 56. 7% cases responded to treatment, 28.4% cases had no change, and 14.9% cases grew worse. Conclusion Patients with CTD-ILD need to begin treatment once they are diagnosed.%目的 探讨不同结缔组织病肺间质病变(CTD-ILD)的临床特点和治疗效果.方法 分析82例CTD-ILD患者的临床特征及胸部高分辨CT(HRCT)特点并观察治疗后HRCT的变化.结果 (1) SSc-ILD的发生率最高,为70.0%.其次为pSS-ILD、PM/DM-ILD、RA-ILD、SLE-ILD,系统性红斑狼疮继发的ILD(SLE-ILD)发病年龄(26.2±7.8)较其他CTD-ILD早(P<0.05).RA-ILD、SSc-ILD病程较长,pSS-ILD、SLE-ILD和PM-ILD病程较短.RA-ILD出现活动后气促少见(P<0.05).pSS-ILD出现呼吸道症状(40%)和体征(50%)最多见.85.7% SSc-ILD出现雷诺现象,支持雷诺现象与ILD相关.(2)SLE-ILD以磨玻璃影多见,而蜂窝状改变较少;pSS-ILD以蜂窝状改变和纵隔淋巴结肿大相对较多.(3)大部分患者治疗后肺部间质病变吸收好转(56.7%),其中88.2% SLE-ILD吸收好转,28.4%患者肺部病变处于静止,14.9%患者间质病变较前进展.结论 CTD-ILD患者一旦确诊,应尽早用激素联合免疫抑制剂治疗.

  3. 肺表面活性蛋白基因突变相关性婴幼儿肺间质疾病一例报告并文献复习%Pulmonary surfactant protein gene mutation associated with pediatric interstitial lung disease: a case study and the review of related literature

    Institute of Scientific and Technical Information of China (English)

    朱春梅; 曹玲; 黄荣妍; 王亚军; 邹继珍; 袁新宇; 宋昉; 陈慧中

    2013-01-01

    目的 报告1例肺表面活性蛋白(SP)基因突变相关性婴幼儿肺间质疾病的临床诊断过程,复习文献,了解SP基因突变与婴幼儿肺间质疾病的关系.方法 总结、分析病例的临床、胸部影像学、肺活检病理资料及基因检测资料并进行相关文献复习.结果 (1)病例资料:2岁女童因呼吸急促,咳嗽、咳痰,进行性加重3月余入院,既往体健.入院体检:呼吸60次/min,口周略紫绀,三凹征(+),两肺底可闻及细湿啰音,未见杵状指、趾.病原学检查均阴性,多次血气分析提示低氧血症,胸部高分辨CT提示双肺野磨玻璃密度影,双肺野内见弥漫斑片、索条影.入院后经抗感染、抗炎治疗,临床无好转,胸部CT无改变,行胸腔镜下肺活检术,肺组织病理符合慢性间质性肺炎伴部分纤维化.SP相关基因检测提示:SP-A1基因R219W突变,SP-C基因S186N突变.(2)复习文献:1篇文献报道成人特发性肺纤维化患者携带SP-A1基因R219W突变,无相关儿童病例报告;17例SP-C基因突变婴幼儿肺间质疾病患儿资料显示:临床表现主要为呼吸急促,呼吸困难,影像学多表现为弥漫性肺部浸润,肺组织病理改变主要为非特异性间质性肺炎,检测到17种SP-C基因突变.所报病例SP-A1基因突变与成年人特发性肺纤维化患者SP-A1基因突变一致;SP-C基因S186N突变,在已发表婴幼儿SP-C突变病例中尚未见报告.结论 (1)初步诊断1例与SP-C基因S186N突变相关性婴幼儿肺间质疾病.(2) SP-C基因突变与部分婴幼儿肺间质疾病发病密切相关.%Objective To report a case of pulmonary surfactant protein (SP) gene mutation associated with pediatric interstitial lung disease,and study the clinical diagnosis process and review of related literature,to understand the relationship between interstitial lung disease and SP gene mutation in infants and children.Method The clinical,radiological,histological,and genetic testing information

  4. Clinical characteristics and correlative factors analysis of ANCA-associated vasculitis with interstitial lung disease%抗中性粒细胞胞质抗体相关性血管炎合并肺间质病变的临床特点和相关因素分析

    Institute of Scientific and Technical Information of China (English)

    张娟; 胡大伟; 王丽萍; 王春燕; 王晓元

    2014-01-01

    Objective To understand the clinical characteristics and correlative factors of ANCA-associated systemic vasculitis with interstitial lung disease.Methods Seventy-five AASV patients in Shanghai Renji Hospital from January 2010 to June 2013 were selected.The clinical data of those cases with interstitial lung disease were analyzed retrospectively and logistic regression model was applied to analyze the correlative factors.Results Of the 75 AASV patients,52% were male,48% were female,and the average age was (56±19) years.Forty-four cases were defined as interstitial lung disease.Among patients with interstitial lung dis-ease,31 (70%) cases were chronic onset,32(72%) cases had pulmonary infection and 8 (18%) cases died.The main chnical manifestations were dry cough,shortness of breath after labour and fever,the proportion was 68%,36% and 50% respectively.Irregular linear shadow was the main imagine changes,the rate was 55%.Pulmonary function changes varied in degrees with diffusion capacity reduction.Multivariate logistic regres-sion analysis showed that those who were older than 60 years (OR=3.44,95%CI:1.05-11.28),positive for MPO-ANCA (OR=3.7,95%CI:1.10-12.65) and Birmingham vasculitis activitv score higher than15 (OR=3.67,95%CI:1.21-11.15) were more likely to have interstitial lung disease.Conclusion Interstitial lung disease is one of the common pulmonary damages of ANCA-associated systemic vasculitis and it will lead to death because of respiratory failure or secondary pulmonary infection.Those who are elders,with positive MPO-ANCA,and with active disease are at high risk for interstitial lung disease among AASV patients.The results of this study sugest that clinicians should pay more attention to patients with these high risk factors.%目的 了解ANCA相关性血管炎(AASV)合并肺间质病变(ILD)的临床特点和相关危险因素.方法 选择上海交通大学医学院附属仁济医院风湿科2010年1月至2013年6月确诊75

  5. Interventional MR: interstitial therapy

    Energy Technology Data Exchange (ETDEWEB)

    Vogl, T.J.; Mack, M.G.; Straub, R.; Engelmann, K.; Eichler, K. [Dept. of Diagnostic and Interventional Radiology, Johann Wolfgang Goethe University, Frankfurt am Main (Germany); Mueller, P.K. [Department of Radiology, Virchow, Humboldt Univ. of Berlin (Germany)

    1999-10-01

    The rationale and results for interstitial therapies via interventional MRI in the treatment of tumors in various regions are presented. Different interstitial treatment techniques are presented based on varying technologies both for tumor ablation and treatment monitoring. Data are presented based on 335 patients, 29-84 years of age (mean age 59 years, 196 men and 139 women) with a total of 932 liver tumors, 16 head and neck tumors and 14 abdominal recurrent pelvic and lymphatic tumors. All lesions had been treated with MR-guided laser-induced interstitial thermotherapy (LITT) via 2516 laser applications and 1856 cannulations. Data in the literature are extremely varying depending on author experience, treatment technique, and the included patient material. In our patient material we were able to achieve a local tumor control of 96.7 % depending on the size of the tumorous lesion, the topographical relationship, and the applied laser parameters. The overall cumulative survival rate of patients with liver metastases was 45.74 months (median 40.97 months, 95 % confidence interval 31.42-50.52). The cumulative survival rate of the patient group with hepatic metastases of colorectal carcinoma was 42.71 months (median 39.33 months, 95 % confidence interval 33.26-45.37). In patients with head and neck tumors a relevant reduction in clinically relevant symptoms such as pain, swallowing disorders, or nervous compression was achieved in 11 of 15 patients treated with LITT. In 14 soft tissue tumors, such as pelvic tumor recurrence and lymph node metastases, a local tumor control was obtained in 68 % of lesions. Interstitial therapies under interventional MRI guidance, such as LITT, results in a high local tumor control with an improved survival rate. (orig.) With 7 figs., 28 refs.

  6. Interstitial pulmonary disorders in indium-processing workers.

    Science.gov (United States)

    Chonan, T; Taguchi, O; Omae, K

    2007-02-01

    The production of indium-tin oxide has increased, owing to the increased manufacture of liquid-crystal panels. It has been reported that interstitial pneumonia occurred in two indium-processing workers; therefore, the present study aimed to evaluate whether interstitial pulmonary disorders were prevalent among indium workers. The study was carried out in 108 male workers in the indium plant where the two interstitial pneumonia patients mentioned above were employed, and included high-resolution computed tomography (HRCT) of the lungs, pulmonary function tests and analysis of serum sialylated carbohydrate antigen KL-6 and the serum indium concentration. Significant interstitial changes were observed in 23 indium workers on HRCT and serum KL-6 was abnormally high (>500 U x mL(-1)) in 40 workers. Workers with serum indium concentrations in the highest quartile had significantly longer exposure periods, greater HRCT changes, lower diffusing capacity of the lung for carbon monoxide and higher KL-6 levels compared with those in the lowest quartile. The serum indium concentration was positively correlated with the KL-6 level and with the degree of HRCT changes. In conclusion, the results of the present study indicated that serum KL-6 and high-resolution computed tomography abnormalities were prevalent among indium workers and that these abnormalities increased with the indium burden, suggesting that inhaled indium could be a potential cause of occupational lung disease.

  7. 皮肌炎/多发性肌炎合并肺间质性病变患者的护理%Nursing for dermatomyositis or polymyositis patients when combined with interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    金妙娟; 杜红卫

    2010-01-01

    @@ 皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)是以横纹肌和/或皮肤受累为主的系统性自身免疫性疾病,病变可同时累及全身各组织器官,其中肺问质病变(interstitial lungdisease,ILD)是其常见的并发症,发生率为5%~30%~([1]).

  8. Interstitial pneumonia with autoimmune features (IPAF) and radiological findings suggestive of lymphocytic interstitial pneumonia (LIP) - case report.

    Science.gov (United States)

    Płóciniczak, Alicja; Goździk-Spychalska, Joanna; Batura-Gabryel, Halina Batura-Gabryel

    2017-01-01

    Interstitial pneumonia with autoimmune features (IPAF) is a term to describe individuals with both interstitial lung disease (ILD) and combinations of other clinical, serologic, and/or pulmonary morphologic features, which presumably originate from an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a defined connective tissue disease (CTD). Predominantly, interstitial pneumonia arises in the course of an established CTD, but it is not so rare for the ILD to be the first, and possibly the one and only manifestation of a latent CTD. Lymphocytic Interstitial Pneumonia (LIP) is an uncommon disease, characterized by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells and other lymphoreticular elements. The cause of LIP is still unknown but it could be also a manifestation of CTD. Clinically, it is highly variable, from spontaneous resolution to progressive respiratory failure and death despite glucocorticoid treatment. Since there are no recent standards for the management of LIP, the disease is treated empirically. We report a case of a HIV-negative 54-year-old woman, who was suspected of LIP according to clinical features and radiological findings. Positive laboratory results were highly suggestive of underlying autoimmune process, but did not fulfil the criteria of any particular CTD. Because of severe general condition of the patient, immunosuppressive treatment was started immediately, without further invasive diagnostics including lung biopsy, which is required for a definitive diagnosis. We present two-year observation of the patient with all our doubts concerning clinical proceedings.

  9. Comparative evaluation of antibody positive titer by ELISA and IFA in Theileria annulata vaccinated cattle in Iran

    Directory of Open Access Journals (Sweden)

    Hashemi-Fesharki R.

    2006-03-01

    Full Text Available An enzyme linked immunosorbent assay (ELISA was used to evaluate antibody positive titer in vaccinated and non-vaccinated cattle using schizont infected myeloid cells as an antigen. The result was compared with indirect fluorescent antibody level in the same animals. For this study 116 milking cows, 95 vaccinated and 21 non-vaccinated, were bleeded in order to prepare sera. They were tested with both ELISA and IFA tests. 94 sera had positive antibody titer and 22 sera were negative through ELISA test but, with IFA test, only 89 sera showed positive antibody titer and 27 were negative. Thereby, it was concluded that the sensitivity and specificity of ELISA test in comparison with IFA test was 95.5 % and 66.6 % respectively. This study generally indicated that ELISA could be an effective test for seroepidemiological investigations of bovine tropical theileriosis, and it is considered to be valid as an additional test to distinguish the vaccinated from the non vaccinated cattle in order to schedule vaccination programs.

  10. Idiopathic non-specific interstitial pneumonia.

    Science.gov (United States)

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.

  11. Clinical Characteristics of Rheumatoid Arthritis Associated with Interstitial Lung Disease%类风湿关节炎合并肺间质疾病患者临床特点

    Institute of Scientific and Technical Information of China (English)

    宋良月; 左大辰; 张榕; 肖卫国

    2015-01-01

    目的:探讨类风湿关节炎(rheumatoid arthritis,RA)伴间质性肺病(interstitial lung disease,ILD)患者临床表现、肺部高分辨率 CT 及实验室指标的特点,分析单纯 RA 患者与 RA 合并 ILD 患者临床特点的差异,为临床诊治提供依据。方法回顾性分析中国医科大学附属第一医院风湿免疫科住院 RA 患者470例,根据肺部高分辨率 CT 结果分为 RA-ILD 组和单纯 RA 组。比较两组患者的一般情况、临床表现以及实验室指标。结果共纳入 RA 患者470例,RA-ILD 组163例,单纯 RA 组307例。RA-ILD 组患者在年龄[(63±12)岁比(55±13)岁]、发病年龄[(55±14)岁比(48±14)岁]、病程[(88±92)个月比(78±86)个月]均高于单纯 RA 组,差异有统计学意义(均 P <0.05)。RA-ILD 组患者的关节肿痛(关节数)(85.9%比63.6%)、咳嗽咯痰胸痛(11.0%比1.9%)、活动后气短(16.6%比5.5%)发生率均高于单纯 RA 组。RA-ILD组限制性通气功能障碍(22.9%比11.1%)、弥散功能减低(35.4%比16.7%)发生率显著高于单纯 RA-ILD 组。RA-ILD 组抗核抗体阳性率(38.2%比45.1%)高于单纯 RA 组,差异有统计学意义(均 P <0.05)。RA-ILD 组纤维蛋白原[(5.59±3.04)g/L 比(5.14±2.50)g/L)]、类风湿因子[(704.90±1326.64)U/ml 比(389.42±669.15)U/ml]、C-反应蛋白[(56.70±56.07)mg/L 比(45.71±52.64)mg/L]、免疫球蛋白 A 水平[(3.62±1.74)g/L 比(3.21±1.50)g/L]均高于单纯RA 组;而血清白蛋白水平[(42.35±5.17)g/L 比(43.89±6.03)g/L]低于单纯 RA 组,差异有统计学意义(均 P <0.05)。结论年龄较大、病程较长、关节炎性明显的 RA 患者易合并 ILD,而且纤维蛋白原、C 反应蛋白、血尿素氮、C-胱抑素肽、血清白蛋白、外周血白细胞

  12. The pathogenesis of interstitial lung disease (ILD) associated with polymyositis (PM) and dermatomyositis (DM)%多发性肌炎(PM)/皮肌炎(DM)相关的肺间质病变发(ILD)病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    张立

    2015-01-01

    肺间质病变(interstitial lung disease,ILD)是多发性肌炎(polymyositis,PM)/皮肌炎(dermatomyositis,DM)的常见并发症,预后不良且死亡率高,是PM/DM患者住院和死亡的重要原因.PM/DM相关ILD发病机制目前仍不清楚;治疗上仍以激素为主,尚缺乏有效的治疗方法,近年来,对ILD的发病机制和治疗已成为一个研究热点.本文就参与发病的自身抗体、免疫细胞、细胞因子、组织蛋白酶、遗传因素学等方面对PM/DM相关的ILD的发病机制研究作一简要综述.

  13. Interstitial pregnancy: role of MRI

    Energy Technology Data Exchange (ETDEWEB)

    Filhastre, M.; Lesnik, A. [Lapeyronie Hospital, Department of Radiology, Montpellier Cedex 5 (France); Dechaud, H.; Taourel, P. [Arnaud de Villeneuve Hospital, Department of Gynecology, Montpellier (France)

    2005-01-01

    We report the MRI features of two cases of interstitial pregnancy. In both cases, MRI was able to localize the ectopic pregnancy by showing a gestational structure surrounded by a thick wall in the upper part of the uterine wall separated from the endometrium by an uninterrupted junctional zone. Because US may confuse angular and interstitial pregnancies and because interstitial pregnancy has a particular evolutive course, MR imaging may play a key role in the diagnosis and management of women with interstitial pregnancy. (orig.)

  14. Lymphocytic interstitial pneumonia as a manifestation of SLE and secondary Sjogren's syndrome.

    Science.gov (United States)

    Garcia, Daniel; Young, Lary

    2013-08-02

    A 47-year-old woman with systemic lupus erythematosus (SLE) diagnosed at age of 35 years was admitted for dyspnoea, substernal chest pain, dry mucosas and difficulty in swallowing. Physical examination revealed vesicular breath sounds bilaterally. Laboratory work showed antinuclear antibody (ANA) (speckled pattern, 1:40), positive anti-Sjogren's syndrome antigen (SSA) and antisingle side band (SSB) and negative double-strand DNA (dsDNA), with normal C3,C4,C50. A high-resolution chest CT scan demonstrated multiple bronchial cysts and diffuse interstitial infiltrates. Surgical lung biopsy revealed emphysematous changes and mild lymphocytic infiltrate around the bronchioles compatible with lymphocytic interstitial pneumonia diagnosis. This case illustrates a patient with primary SLE overlapped by initial manifestations of secondary Sjogren's syndrome (SS) presenting with associated autoimmune interstitial lung disease. Antibody markers, high-resolution chest CT scan and surgical lung biopsy were essential in evaluating this patient, confirming the interstitial lymphocytic infiltration of the lung. Primary SS (pSS) is the most commonly associated disease to lung interstitial pneumonia (LIP) (25%). High-resolution chest CT scan demonstrates areas of ground-glass attenuation, suggestive of interstitial disease. Surgical lung biopsy shows pathologic increase of mature lymphocyte cells and histiocytes. Most of the cases have a benign presentation and shortly relapse. Superimposed infection, pulmonary fibrosis and lymphoma develop in less than 20% of cases. Corticosteroids are the primary therapy. While pSS is commonly associated with interstitial lung involvement, secondary Sjogren's syndrome (sSS) is only rare. It has been described the initial sSS presentation by Sica symptoms development only, and our case is the first report of LIP presentation as initial manifestation of sSS. Our patient remained stable after corticosteroids and hydoxychloroquine therapy and no

  15. Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.

    Science.gov (United States)

    Ito, Kotaro; Imafuku, Shinichi; Hamaguchi, Yasuhito; Fujimoto, Manabu; Nakayama, Juichiro

    2013-07-01

    Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.

  16. Patterns of human papillomavirus DNA and antibody positivity in young males and females, suggesting a site-specific natural course of infection

    NARCIS (Netherlands)

    Vriend, Henrike J; Bogaards, Johannes A; van der Klis, Fiona R M; Scherpenisse, Mirte; Boot, Hein J; King, Audrey J; van der Sande, Marianne A B; Rossen, John

    2013-01-01

    BACKGROUND: To monitor the impact of human papillomavirus types 16 and 18 vaccine on HPV infection dynamics in the Netherlands, we started an ongoing study in sexually transmitted infection (STI) clinics in 2009. Here, we analyze baseline type-specific HPV DNA and HPV-specific antibody positivity ra

  17. 多发性肌炎/皮肌炎合并间质性肺疾病预测因素的Meta分析%Meta-analysis of predictive factors of interstitial lung disease in patients with polymyositis and dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    陈芳; 王国春

    2011-01-01

    Objective To systematically review and evaluate the predictive factors of interstitial lung disease (ILD) in patients with polymyositis and dermatomyositis (PM/DM), and provide evidence for early clinical diagnosis. Methods The predictive factors of PM/DM-related ILD from 23 published papers were analyzed by Meta-analysis. Analysis of heterogeneity and sensitivity were performed and pooled odds ratio (OR) with 95% confidence interval (95%CI) were calculated using either fixed or random effects models by Rev-Man 4.2 software. Stata 10.0 software was used to identify publication bias. Results Meta-analysis was performed to analyze seven factors including anti-Jo-1 antibody, fever, arthritis/arthralgia, Gottron's sign,antinuclear antibody (ANA), and dysphagia Raynaud's phenomenon. Factors correlated with ILD in patients with PM/DM and their OR values and 95%CI were as follows: the presence of anti-Jo-1 antibody 6.94 (4.74~10.16), fever 4.90(3.82~6.29), arthritis/arthralgia 3.93(3.21~4.80), Gottron's sign 2.52(1.24~5.14), and the presence of ANA 1.59( 1.02~2.47 ). Raynaud's phenomenon and dysphagia were not correlated with ILD in patients with PM/DM with OR values and 95%CI as 1.40 (0.97~2.01), and 1.21 (0.94~1.56) respectively.Conclusion The presence of anti-Jo-1 antibody, fever, arthritis/arthralgia, Gottron's sign and ANA are major predictive factors for ILD in patients with PM/DM.%目的 系统分析和评价多发性肌炎/皮肌炎合并间质性肺疾病的预测因素,为临床早期诊治提供循证依据。方法 利用Meta分析方法分析国内外23篇关于多发性肌炎(PM)/皮肌炎合并间质性肺疾病( ILD)预测因素的文献,应用Rev-Man 4.2分析软件进行异质性和敏感性分析,并根据异质性检验结果,采用固定效应或随机效应模型计算合并比值比(OR值)和95%可信区间(95%CI)。应用Stata 10.0软件进行发表偏倚识别。结果 对关节炎/关节痛、发热、抗Jo-1

  18. Clinical features of primary Sj(o)gren syndrome associated with interstitial lung disease%原发性干燥综合征合并间质性肺疾病的临床特征分析

    Institute of Scientific and Technical Information of China (English)

    冯石军; 陈燕; 石志辉

    2011-01-01

    目的 探讨原发性干燥综合征(pSS)合并间质性肺疾病(ILD)患者的临床特征,以提高临床诊断水平.方法 收集2006年3月至2009年3月收治的pSS患者58例,其中合并ILD患者27例(ILD组),不合并ILD患者34例(非ILD组).采用免疫比浊法检测类风湿因子、C反应蛋白(CRP)、血蛋白电泳、补体C3、补体C4;间接免疫荧光法和免疫印迹法检测抗核抗体及亚类;酶联免疫吸附法检测癌抗原125(CA125);魏氏法检测红细胞沉降率(ESR);肺功能仪测定肺通气功能和一氧化碳弥散功能(DLCO),放射学检查包括胸部正侧位X线片和肺部高分辨CT扫描.结果 ILD组口干、眼干、猖撅齿、气促、Velcro哕音较非ILD组更常见,ILD组较非ILD组更常出现ESR快、CRP高、CA125高及高γ球蛋白血症,ILD组肺功能主要表现为弥散功能障碍和限制性通气功能障碍,弥散功能障碍者18例(66.7%),限制性通气功能障碍者14例(51.9%),阻塞性通气功能障碍者6例(22.2%),混合性通气功能障碍者8例(29.6%),用力肺活量、第1秒用力呼气容积、最大呼气中段流量及DLCO占预计值百分比与非ILD组比较差异有统计学意义(P<0.01),肺部高分辨CT在发现pSS合并ILD时敏感性优于普通胸部X线片.结论 pSS合并ILD的发生与原发病的活动性明显相关,肺弥散功能、肺部高分辨CT对早期诊断pSS合并ILD具有重要价值.%Objective To investigate the clinical features of primary Sj(o)gren syndrome (pSS) with interstitial lung disease (pSS-ILD) so as to raise the clinical diagnosis Level. Methods The clinical data were collected from 58 patients with pSS,who were admitted from March 2006 to March 2009. The patients were divided into ILD group (27 cases) and non-ILD group (31 cases). Rheumatoid factor(RF),C-reactive protein (CRP), protein electrophoresis and complement C3, C4 in serum were measured by immunoturbidimetric methods. Antinuclear antibodies (ANA) and anti-ENA antibodies

  19. 泼尼松联合环磷酰胺治疗系统性硬化病继发间质性肺疾病的疗效观察%Curative effect observation of Hormone combined with cyclophosphamide therapy for interstitial lung disease secondary to systemic sclerosis

    Institute of Scientific and Technical Information of China (English)

    陈悦

    2016-01-01

    Objective To observe curative effect of Hormone combined with cyclophosphamide therapy for interstitial lung disease secondary to systemic sclerosis.Methods Selection of 30 patients with SSc-ILD, early metaphase group 18 cases, advanced group of 12 cases, to observe HRCT lung function score difference and the related indicators of the two groups before and after the treatment.Results After the treatment, the lung function index of FEV1, FVC, FVC% and DLCO, DLCO% in early metaphase group were more apparent improvement than those in advanced group, the differences were statistically significant(P0.05); two groups of SGRQ activity points are reduced, but the advanced group reduced more obvious(P<0.05). Conclusion Though the hormone combined with cyclophosphamide therapy doesn’t work in advanced interstitial lung disease secondary to systemic sclerosis, it is good for the disease in early metaphase stage.%目的:探究对系统性硬化病继发间质性肺疾病(SSc-ILD)患者以泼尼松联合环磷酰胺进行治疗的临床疗效。方法选取30例SSc-ILD患者,包括早中期组18例与晚期组12例,采用泼尼松联合环磷酰胺进行治疗,观察治疗前后两组肺功能、HRCT评分及相关指标差异。结果治疗后早中期组比晚期组 FEV1、FVC、FVC%、DLCO、DLCO%等肺功能指标改善更为明显,差异均有统计学意义(P<0.05),且治疗后早中期组HRCT 评分明显减少(P<0.05),但晚期组基本不变(P>0.05),两组SGRQ活动分均有所降低,但晚期组活动分降低更明显(P<0.05)。结论激素联合环磷酰胺治疗对早中期系统性硬化病继发间质性肺疾病效果显著,但对晚期患者效果不明显。

  20. Chronic interstitial pneumonia with honeycombing in coal workers

    Energy Technology Data Exchange (ETDEWEB)

    Brichet, A.; Tonnel, A.B.; Brambilla, E.; Devouassoux, G.; Remy-Jardin, M.; Copin, M.C.; Wallaert, B. [A. Calmette Hospital, Lille (France)

    2002-10-01

    Coal worker's pneumoconiosis (CWP) results from coal mine dust inhalation. The paper reports the presence of a chronic interstitial pneumonia (CIP) with honeycombing in 38 cases of coal miners, with or without CWP. The 38 patients were selected on the basis of clinical criteria which are unusual in CWP, i.e. fine inspiratory crackles and severe dyspnea. There were 37 men and one woman; mean age was 67.5 {+-} 9.1 years. Thirty-two were smokers. Duration of exposure was 26.7 {+-} 9.9 years. All the patients had clinical examination, chest radiography, computed tomography (CT), lung function, laboratory investigations, wedged fiberoptic bronchoscopy with bronchoalveolar lavage (BAL). In eight cases, lung specimens were obtained. Seventeen out of 38 had finger clubbing. 17 had radiological signs of CWP limited to the upper lobes or diffusely distributed. CT showed honeycombing (36 cases), and/or ground glass opacities (30 cases) with traction bronchiectasis (8 cases) predominant in the lower lobes. BAL analysis demonstrated an increased percentage of neutrophils (9.4% {+-} 6). Lung function showed a restrictive pattern associated with a decreased DLCO and hypoxemia. Lung specimens demonstrated in 2 cases a homogenous interstitial fibrosis of intra-alveolar septum with an accumulation of immune and inflammatory cells without temporal variation and with obvious honeycombing. The 6 other cases showed features of usual interstitial pneumonia. These cases, should alert other clinicians to a possible association between CIP with honeycombing and coal dust exposure, with or without associated CWP.

  1. Idiopathic pleuroparenchymal fibroelastosis, a rare entity of interstitial pneumonia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jong Chun; Chae, Eun Jin; Song, Joon Seon [University of Ulsan College of Medicine, Asan Medical Center, Seou (Korea, Republic of)

    2014-04-15

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

  2. Clinical Management of Acute Interstitial Pneumonia: A Case Report

    Directory of Open Access Journals (Sweden)

    Yang Xia

    2012-01-01

    Full Text Available We describe a 51-year-old woman who was admitted to hospital because of cough and expectoration accompanied with general fatigue and progressive dyspnea. Chest HRCT scan showed areas of ground glass attenuation, consolidation, and traction bronchiectasis in bilateral bases of lungs. BAL fluid test and transbronchial lung biopsy failed to offer insightful evidence for diagnosis. She was clinically diagnosed with acute interstitial pneumonia (AIP. Treatment with mechanical ventilation and intravenous application of methylprednisolone (80 mg/day showed poor clinical response and thus was followed by steroid pulse therapy (500 mg/day, 3 days. However, she died of respiratory dysfunction eventually. Autopsy showed diffuse alveolar damage associated with hyaline membrane formation, pulmonary interstitial, immature collagen edema, and focal type II pneumocyte hyperplasia.

  3. Interstitial pneumonitis after acetylene welding: a case report.

    Science.gov (United States)

    Brvar, Miran

    2014-01-01

    Acetylene is a colorless gas commonly used for welding. It acts mainly as a simple asphyxiant. In this paper, however, we present a patient who developed a severe interstitial pneumonitis after acetylene exposure during aluminum welding. A 44-year old man was welding with acetylene, argon and aluminum electrode sticks in a non-ventilated aluminum tank for 2 h. Four hours after welding dyspnea appeared and 22 h later he was admitted at the Emergency Department due to severe respiratory insufficiency with pO2 = 6.7 kPa. Chest X-ray showed diffuse interstitial infiltration. Pulmonary function and gas diffusion tests revealed a severe restriction (55% of predictive volume) and impaired diffusion capacity (47% of predicted capacity). Toxic interstitial pneumonitis was diagnosed and high-dose systemic corticosteroid methylprednisolone and inhalatory corticosteroid fluticasone therapy was started. Computed Tomography (CT) of the lungs showed a diffuse patchy ground-glass opacity with no signs of small airway disease associated with interstitial pneumonitis. Corticosteroid therapy was continued for the next 8 weeks gradually reducing the doses. The patient's follow-up did not show any deterioration of respiratory function. In conclusion, acetylene welding might result in severe toxic interstitial pneumonitis that improves after an early systemic and inhalatory corticosteroid therapy.

  4. Intercellular adhesion molecule-1 in patients with idiopathic interstitial pneumonia.

    Directory of Open Access Journals (Sweden)

    Takehara H

    2001-08-01

    Full Text Available This study focuses on a possible role of intercellular adhesion molecule-1 (ICAM-1 in interstitial pulmonary diseases. We determined a soluble form of ICAM-1 in serum and bronchoalveolar lavage fluid (BALF using ELISA in patients with usual interstitial pneumonia (UIP, bronchiolitis obliterance organizing pneumonia (BOOP, or nonspecific interstitial pneumonia (NSIP. In addition, we investigated the expression of ICAM-1 in the lung tissues of these patients by means of immunohistochemical staining. Serum levels of soluble ICAM-1 were significantly higher in patients with UIP or NSIP than in healthy subjects, and were also high in patients with BOOP. The soluble ICAM-1 in BALF tended to be higher in patients with UIP, BOOP, or NSIP than in normal subjects. A significant correlation was seen between soluble levels of ICAM-1 in serum and BALF. In the immunostaining of ICAM-1 of the lung tissues, ICAM-1 expression was more pronounced in patients with UIP than in those with BOOP or NSIP. The increased expression of ICAM-1 was seen in type II alveolar epithelium and vascular endothelium in patients with interstitial pneumonia. A positive correlation was observed between the degree of ICAM-1 expression in the lung tissues and the BALF levels of soluble ICAM-1. The expression of ICAM-1 in type II alveolar epithelium suggests that ICAM-1 plays a specific role in the fibrotic process of the lung, and that the measurement of soluble ICAM-1 in sera and BALF could be a useful marker for evaluating the progression of fibrosis.

  5. [Humidifier lung].

    Science.gov (United States)

    Gerber, P; de Haller, R; Pyrozynski, W J; Sturzenegger, E R; Brändli, O

    1981-02-07

    Breathing air from a humidifier or an air conditioning unit contaminated by various microorganisms can cause an acute lung disease involving fever, cough and dyspnea, termed "humidifier fever". This type of hypersensitivity pneumonitis was first described in 1959 by PESTALOZZI in the Swiss literature and subsequently by BANASZAK et al. in the Anglo-American. Here a chronic form of this disease which led to pulmonary fibrosis is described: A 37-year-old woman who works in a cheese shop presented with dyspnea which had been progressive over two years, weight loss, a diffuse reticular pattern radiographically and a severe restrictive defect in lung function tests. Open lung biopsy revealed chronic interstitial and alveolar inflammation with non-caseating granulomas and fibrotic changes. Circulating immune complexes and precipitins against the contaminated humidifier water and cheese mites were found, but no antibodies suggesting legionnaires' disease. Two out of five otherwise healthy employees of this cheese shop, where a new humidifying system had been installed 7 years earlier, also had precipitins against the contaminated water from the humidifier and the cheese mites. Despite ending of exposure and longterm steroid and immunosuppressive therapy, the signs and symptoms of pulmonary fibrosis persisted. Contrary to the acute disease, this chronic form is termed "humidifier lung". The importance is stressed of investigating the possibility of exposure to contaminated humidifiers or air conditioning units in all cases of newly detected pulmonary fibrosis.

  6. Hepatitis C virus (HCV)-specific in vitro antibody secretion by peripheral blood lymphocytes: correlation with progression of disease and HCV RNA in HCV antibody-positive patients.

    OpenAIRE

    Ducos, J.; Bianchi-Mondain, A M; Francois, M.; Boisset, M; Vendrell, J P; Barin, F; Serre, A; Larrey, D

    1994-01-01

    Hepatitis C virus-specific in vitro antibody production (HCV IVAP) by peripheral blood lymphocytes in 53 HCV antibody-positive patients was investigated in comparison with alanine aminotransferase (ALT) levels and HCV RNA in serum samples. All 29 HCV IVAP-positive patients were HCV RNA positive; 26 had elevated ALT levels. Among the 24 HCV IVAP-negative patients, 16 were HCV RNA negative, with 12 presenting normal ALT values. These data indicate that HCV IVAP results are highly correlated (P ...

  7. Interstitial Pulmonary Edema Following Bromocarbamide Intoxication

    Science.gov (United States)

    Sugihara, H.; Hagedorn, M.; Bōttcher, D.; Neuhof, H.; Mittermayer, Ch.

    1974-01-01

    Bromocarbamides are sleep-inducing drugs which can lead, in man, to intoxication and death due to respiratory failure. To prove whether hemodynamic factors or the changed endothelial permeability induce pulmonary edema, animal experiments were performed. The fine structural changes in pulmonary edema in rabbits were observed at 60, 90 and 120 minutes after oral administration. The major findings were a) large blebs between capillary endothelium and alveolar epithelium and b) interstitial edema of the vessel wall. The bleb contents were much less electron dense than the blood contents in the capillary. Colloidal carbon did not enter the bleb or the edematous interstitial tissue. Exogenous peroxidase uptake in pinocytotie vesicles increased in pathologic cases. The hemodynamic measurements in animal receiving artificial respiration which maintained the blood pO2 at a steady state showed similar blebs in the pulmonary vessels, indicating that anoxia is not the major cause of the vascular lesion. Moreover, pulmonary arterial pressure and pulmonary vascular resistance could be held in the normal range in artificially respirated animals under bromocarbamide intoxication. Thus, hemodynamic factors are not likely to play a pathogenetic role in bringing about pulmonary edema. The chief, early factor is the increased endothelial permeability due to increased cytoplasmic transport. From this a practical suggestion for treating patients with bromocarbamide intoxication is derived: the usual fluid replacement in shock patients should be handled with great care to avoid fluid overload of the lung. ImagesFig 1Fig 2Fig 3Fig 4Fig 5Fig 6 PMID:4835993

  8. Increased Detection of Sin Nombre Hantavirus RNA in Antibody-Positive Deer Mice from Montana, USA: Evidence of Male Bias in RNA Viremia

    Directory of Open Access Journals (Sweden)

    James N. Mills

    2013-09-01

    Full Text Available Hantaviruses are widespread emergent zoonotic agents that cause unapparent or limited disease in their rodent hosts, yet cause acute, often fatal pulmonary or renal infections in humans. Previous laboratory experiments with rodent reservoir hosts indicate that hantaviruses can be cleared from host blood early in the infection cycle, while sequestered long term in various host organs. Field studies of North American deer mice (Peromyscus maniculatus, the natural reservoir of Sin Nombre hantavirus, have shown that viral RNA can be transiently detected well past the early acute infection stage, but only in the minority of infected mice. Here, using a non-degenerate RT-PCR assay optimized for SNV strains known to circulate in Montana, USA, we show that viral RNA can be repeatedly detected on a monthly basis in up to 75% of antibody positive deer mice for periods up to 3–6 months. More importantly, our data show that antibody positive male deer mice are more than twice as likely to have detectable SNV RNA in their blood as antibody positive females, suggesting that SNV-infected male deer mice are more likely to shed virus and for longer periods of time.

  9. SIV Infection of Lung Macrophages.

    Directory of Open Access Journals (Sweden)

    Yue Li

    Full Text Available HIV-1 depletes CD4+ T cells in the blood, lymphatic tissues, gut and lungs. Here we investigated the relationship between depletion and infection of CD4+ T cells in the lung parenchyma. The lungs of 38 Indian rhesus macaques in early to later stages of SIVmac251 infection were examined, and the numbers of CD4+ T cells and macrophages plus the frequency of SIV RNA+ cells were quantified. We showed that SIV infected macrophages in the lung parenchyma, but only in small numbers except in the setting of interstitial inflammation where large numbers of SIV RNA+ macrophages were detected. However, even in this setting, the number of macrophages was not decreased. By contrast, there were few infected CD4+ T cells in lung parenchyma, but CD4+ T cells were nonetheless depleted by unknown mechanisms. The CD4+ T cells in lung parenchyma were depleted even though they were not productively infected, whereas SIV can infect large numbers of macrophages in the setting of interstitial inflammation without depleting them. These observations point to the need for future investigations into mechanisms of CD4+ T cell depletion at this mucosal site, and into mechanisms by which macrophage populations are maintained despite high levels of infection. The large numbers of SIV RNA+ macrophages in lungs in the setting of interstitial inflammation indicates that lung macrophages can be an important source for SIV persistent infection.

  10. [Interstitial Pneumonia and Emphysema].

    Science.gov (United States)

    Sawa, Teiji; Kato, Yuko; Ishii, Sachiyo

    2015-09-01

    Interstitial pneumonia (IP) and chronic obstructive pulmonary disease (COPD) are representative diseases of restrictive pulmonary dysfunction and obstructive pulmonary dysfunction, respectively. In the preoperative anesthesia clinic, anesthesiologists are frequently asked to assess the anesthesia management of patients with these diseases. In respiratory function tests, IP is detected as a decrease in % vital capacity (< 80%), and COPD as a decrease in % FEV1.0 (< 70%). Other key factors which affect the assessment are; 1) severity assessment that affects the safety of anesthesia management, 2) prognostic evaluation including the acute exacerbation in the postoperative period, and 3) patient-related factors (age, life degree of autonomy, other comorbidities, surgery-related factors, and anesthesia method). In the patients in the disease stage I or II, anesthesia management is relatively safe. On the other hand, the patients in the disease stage IV have no surgical indication except life-saving emergent situation. In another words, anesthesiologists are required to make the judgment for the anesthesia management of the patient in the disease stage III, based on the assessment of patient-related factors, surgery-related factors, and prognosis.

  11. 类风湿性关节炎合并肺间质病变的 CT 诊断价值研究%Diagnostic Value of CT in interstitial lung disease of Rheumatoid Arthritis

    Institute of Scientific and Technical Information of China (English)

    黄振强

    2012-01-01

      Objective: To explore the diagnostic value of CT in ininterstitial lung disease of Rheumatoid Arthritis(RA).Methods: Retrospectively analyzed the 60 cases of patients in ininterstitial lung disease of Rheumatoid Arthritis(RA) in my hospital from 2009 July to 2012 July.Grouped and compared the clinical manifestations and detection of lung lesions of Chest x-ray film and CT examination. Results: Among 30 cases, the most manifestations of chest X-ray wer thest pulmonary markings were in-creased but the changes of pulmonary interstitium and pleura were not obvious.The detection rate of lung disease is 56.67%. Among other 30 cases,the manifestations of CT examination changed of interstitium and pleura pathological.The detection rate of lung disease is 96.66%.There is significant differences Between two groups(P<0.05).Conclusion:The application of CT examination can significantly improve the detection rate of Rheumatoid Arthritis(RA) lung disease and help to determine the stage of RA and to estimate the prognosis in patients with RA.%  目的:探讨 CT 对类风湿性关节炎(RA)合并肺间质病变的诊断价值.方法:对我院2009年7月至2012年7月的60例 RA 合并肺间质病变患者进行回顾性分析.分组比较 X 线胸片和 CT 检查的临床表现及对肺部病变的检出率.结果:30例患者 X 线胸片主要表现为:两肺纹理增粗,肺间质及胸膜改变不明显,对肺部病变检出率为56.67%;另30例患者 CT 扫描检查表现为:部分肺间质改变及胸膜病变,对肺部病变检出率为96.66%,两者具显著性差异(P<0.05).结论:应用 CT 检查可以明显提高类风湿性关节炎(RA)肺部病变的检出率,以早期了解 RA 浸润肺部的程度及估计预后.

  12. Application of comprehensive nursing intervention on nosocomial infections in interstitial lung disease patients%综合护理干预在结缔组织病相关肺间质病变患者院内感染中的应用

    Institute of Scientific and Technical Information of China (English)

    黄宏伟

    2015-01-01

    ABSTRACT:Objective To study and analyze the significance of comprehensive nursing in-tervention on the prevention of nosocomial infections in interstitial lung disease patients.Methods A total of 162 hospitalized elderly patients in our hospital were randomly divided into experimen-tal group(84 cases)and control group(78 cases),the experimental group was taken comprehensive nursing intervention,and the control group was taken routine nursing intervention.The nosocomial infection rates of the two groups were observed and compared,and the quality of life of the two groups after care were evaluated.Results The infection rate in the experimental group was lower than that in the control group.And quality of life in the experimental group was significantly high-er than that in the control group.Conclusion The comprehensive nursing intervention can effec-tively prevent the incidence of nosocomial infections in interstitial lung disease patients.%目的:研究分析综合护理干预对预防肺间质病变患者院内感染的意义。方法将结缔组织病合并肺间质病变162例,随机分为干预组(84例)和对照组(78例),干预组患者实行综合护理干预,对照组患者应用常规护理干预,观察比较2组患者并发院内感染率,并评价护理后2组患者生活质量。结果与对照组相比,干预组感染率明显降低,各部位感染发生率明显下降,血沉、C 反应蛋白及降钙素原等炎症指标明显下降,生活质量显著提高。结论综合护理干预能够有效预防肺间质病变患者院内感染。

  13. 丙基硫氧嘧啶致儿童继发性抗中性粒细胞胞质抗体相关性小血管炎1例%Propylthioouracil-associated antineutrophil cytoplasmic antibody-positive vasculitis:1 case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    史卓; 孙涛; 夏正坤

    2015-01-01

    目的:分析丙基硫氧嘧啶(PTU)致抗中性粒细胞质抗体(ANCA)相关性小血管炎的机制。方法采用1例长期服用PTU患儿所致ANCA相关性小血管炎的临床资料。结果患儿因贫血、蛋白尿、肾功能不全,伴有咯血而就诊,追问病史发现其发病前曾服用PTU 3年,经免疫抑制、血浆置换等治疗后症状缓解,肾功能改善。结论 PTU可引起ANCA相关性小血管炎,及时停用相关药物以及应用免疫抑制剂、血浆置换等治疗,可使病情缓解。%Objective To analyze pathogenesis of antineutrophil cytoplasmic antibody-positive vasculitis induced by propylthiouracil (PTU).Method By reporting a case of Antineutrophil Cytoplasmic Antibody-positive Vasculitis induced by PTU.ResultsThe patient (who had been treated with PTU for 3 years because of hyperthyreosis) presented with hypohemia, proteinuria, renal function damage, injury of lungs, positive Antineutrophil Cytoplasmic Antibody.After immunosuppressive therapy, plasma exchange and other treatment, the symptoms of the patients were relieved and the renal function was restored.Conclusions PTU may induce Antineutrophil Cytoplasmic Antibody-positive Vasculitis. Early diagnosis, withdraw of ptu, plasma exchange and administration of glucocortocoid can improve its prognosis.

  14. 振动反应成像系统在间质性肺疾病患者肺功能评价中的应用%A new lung imaging: vibration response imaging applied to evaluate pulmonary function of interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    张钰旋; 张苑; 徐金富; 李霞; 周瑛; 卫平; 金晓凤; 蒋幸; 李惠萍

    2012-01-01

    Objective To discuss the value of vibration response imagine (VRI) on evaluating the pulmonary function of interstitial lung disease (ILD).Methods 80 patients who were diagnosed as ILD from June 2010 to September 2011 enrolled in this study.Then,all the patients took the VRIxp pulmonary function test so as to make a comparison with the traditional pulmonary function test.This paper analyzed the advantage and disadvantage of VRIxp and the traditional pulmonary function teat in evaluating the pulmonary function of ILD by observing the changes of patients' condition after the test respectively.Results The changes and significance of the main VRIxp parameters:①By observing airflow distribution map and maximum vibration energy diagram,a variety of abnormities were discovered such as the out-sync of the images and the size difference in all patients.②It is found that 69 patients had abnormal energy distribution.According to grouping of the various disease types and CT scan,the energy values were compared which indicated that the abnormities had no statistical significance.③ The energy curve had some abnormities such as the platform and curve separation.④The study showed that VRIxp could qualitatively,quantificationally detect the abnormal breath sounds and VRIxp was more sensitive than that of traditional auscultation (the positive rate were 90.00% vs 82.50%,respectively).⑤VRIxp could locate the lesion directly by combining air distribution figure with regional energy distribution.The accuracy rate was as high as 91.25% compared with CT scan.Comparing with traditional pulmonary function technology,there were some advantages of VRI.①For some patients,their ventilatory functions were detected as normal by traditional pulmonary function technology,while through VRI test,the results were abnormal.And the lesions were consistent with HRCT.② By evaluating conditions of patients before and after pulmonary function test,VRIxp was less secondary effects than

  15. Surgical lung biopsy for the management of diffuse interstitial lung disease%胸腔镜及开胸肺活检在弥漫性间质性肺疾病诊断中的临床价值分析

    Institute of Scientific and Technical Information of China (English)

    黄慧; 李珊; 张婷婷; 王艳勋; 黄诚; 李立; 李单青; 冯瑞娥; 刘鸿瑞

    2014-01-01

    疗方案;术后并发症以发热、伤口延迟愈合为主要表现,手术相关病死率低;对于诊断不明的弥漫性间质性肺疾病患者建议接受外科肺活检以助于明确诊断、指导治疗.%Objective To evaluate the role of surgical lung biopsy (SLB) in the management of diffuse interstitial lung disease (DILD) with no specific diagnosis.Methods We conducted a retrospective analysis of 179 cases with DILD of non-specific diagnosis after non-invasive methods and minimally invasive processes,including bronchoscopy and CT-guided lung biopsy,were performed SLB at Peking Union Medical College Hospital between 2006 and 2012.Patient demographics,surgical approach,number and site of biopsies,post-operative complications and postoperative pathological diagnosis and treatment were analyzed.Results Of 179 cases,there were 91 males (50.8%) and 88 females (49.2%),with a median age of 47.3 years (range 16-76).There were 6 cases(3.4%) who were younger than 20 years and 25 cases(14%) older than 60 years.From 2006 to 2012,every year there were 16 case (8.9%),17 cases (9.5%),19 cases(10.6%),44 cases(24.6%),33 cases(18.4%),31 cases(17.3%) and 19 cases (10.6%) respectively.The total median hospital stay was 33.4 days (range 6-76) and the mean postoperative stay was 18.6 days (range 2-56).The mean duration for chest drainage was 3.2 days (range 2-18).Among them,150 cases were arranged with video-assisted thoracoscopy surgery (VATS) and 29 cases were arranged with minithoracotomy.The number of biopsies taken was ranged from one to four and there were 126 cases (70.4%) had more than one biopsy.The biopsy was performed in left lingular lobe (54 cases/30.2%),left natural upper lobe (16 cases/8.9%),left inferior basal segments (56 cases/ 31.3%),right upper lobe (30 cases/16.7%),right meddle lobe (40case/22.3%) and right inferior basal segments (56 case/31.3%) respectively.Definitive pathological diagnosis was reached in 103 cases

  16. Giant cell interstitial pneumonia in patients without hard metal exposure: analysis of 3 cases and review of the literature.

    Science.gov (United States)

    Khoor, Andras; Roden, Anja C; Colby, Thomas V; Roggli, Victor L; Elrefaei, Mohamed; Alvarez, Francisco; Erasmus, David B; Mallea, Jorge M; Murray, David L; Keller, Cesar A

    2016-04-01

    Giant cell interstitial pneumonia is a rare lung disease and is considered pathognomonic for hard metal lung disease, although some cases with no apparent hard metal (tungsten carbide cobalt) exposure have been reported. We aimed to explore the association between giant cell interstitial pneumonia and hard metal exposure. Surgical pathology files from 2001 to 2004 were searched for explanted lungs with the histopathologic diagnosis of giant cell interstitial pneumonia, and we reviewed the associated clinical histories. Mass spectrometry, energy-dispersive x-ray analysis, and human leukocyte antigen typing data were evaluated. Of the 455 lung transplants, 3 met the histologic criteria for giant cell interstitial pneumonia. Patient 1 was a 36-year-old firefighter, patient 2 was a 58-year-old welder, and patient 3 was a 45-year-old environmental inspector. None reported exposure to hard metal or cobalt dust. Patients 1 and 2 received double lung transplants; patient 3 received a left single-lung transplant. Histologically, giant cell interstitial pneumonia presented as chronic interstitial pneumonia with fibrosis, alveolar macrophage accumulation, and multinucleated giant cells of both alveolar macrophage and type 2 cell origin. Energy-dispersive x-ray analysis revealed no cobalt or tungsten particles in samples from the explanted lungs. None of the samples had detectable tungsten levels, and only patient 2 had elevated cobalt levels. The lack of appropriate inhalation history and negative analytical findings in the tissue from 2 of the 3 patients suggests that giant cell interstitial pneumonia is not limited to individuals with hard metal exposure, and other environmental factors may elicit the same histologic reaction.

  17. Interstitial Functionalization in elemental Si

    Science.gov (United States)

    Kiefer, Boris; Fohtung, Edwin

    Societies in the 21st century will face many challenges. Materials science and materials design will be essential to address and master some if not all of these challenges. Semiconductors are among the most important technological material classes. Properties such as electrical transport are strongly affected by defects and a central goal continues to be the reduction of defect densities as much as possible in these compounds. Here we present results of interstitial Fe doping in elemental Si using first-principles DFT calculations. The preliminary results show that Fe will only occupy octahedral interstitial sites. The analysis of the electronic structure shows that the compounds are ferromagnetic and that a bandgap opens as interstitial Fe concentrations decrease, with a possible intermittent semi-metallic phase. The formation energy for interstitial Fe is unfavorable, as expected, by ~1.5 eV but becomes favorable as the chemical potential of Fe increases. Therefore, we expect that biasing the system with an external electrical field will lead to the formation of these materials. Thus, our results show that interstitial defects can be beneficial for the design of functionalities that differ significantly from those of the host material.

  18. Diagnosis and treatment of cystic lung disease

    Science.gov (United States)

    Park, Sanghoon; Lee, Eun Joo

    2017-01-01

    Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually Hogg-Dube syndrome, lymphocytic interstitial pneumonia/follicular bronchiolitis, and amyloidosis. PMID:28264540

  19. Clinical analysis of diffusive interstitial lung diseases with positive anti-neutrophil cytoplasmic antibody%抗中性粒细胞胞浆抗体阳性的弥漫性间质性肺病的临床分析

    Institute of Scientific and Technical Information of China (English)

    丁艳芩; 朱红; 姚婉贞; 赵鸣武

    2011-01-01

    目的:探讨弥漫性间质性肺病(diffuse interstitial lung diseases,DILD)中抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)阳性患者的肺间质病变特点,以及ANCA阳性的非原发小血管炎疾病与原发性系统性小血管炎的临床异同.方法:回顾性分析1995年10月至2008年9月收治的具有完整ANCA检查资料的DILD病例122例,比较ANCA阳性的非原发小血管炎组(A组)、ANCA阳性的原发性系统性血管炎组(B组)与ANCA阴性组(C组)的临床症状体征、影像学、肺功能、纤维支气管镜下表现、支气管肺泡灌洗液(bronchoalveolar lavage fluid,BALF)细胞学分析及其他实验室检查的差异.结果:具有完整ANCA资料的DILD患者122例,ANCA阳性者36例,占29.51%;A、B、C组的患者数分别为7例、29例、86例.A、B组与C组相比,肺总量(total lung capacity,TLC)降低较少,胸膜病变多见.A组患者发生少尿、血尿、蛋白尿、贫血、肾功能不全者与C组相似,均较B组少见.纤维支气管镜下改变、BALF的细胞学、抗核抗体(antinuclear antibody,ANA)等项检查3组患者之间差异均无统计学意义.结论:DILD患者中,ANCA阳性患者的肺间质病变伴有胸膜受累较ANCA阴性者相对多见,TLC降低相对较少.ANCA阳性的非原发小血管炎疾病与原发性系统性小血管炎相比,临床表现相似,但合并肾脏损害、贫血相对少见.%Objective:To explore the features of pulmonary interstitial pathological changes in diffuse interstitial lung disease (DILD) patients with positive anti-neutrophil cytoplasmic antibody (ANCA), and the similarities as well as differences between ANCA positive patients with non-primary vasculitis and primary systematic vasculitis. Methods: Clinical data of 122 patients with DILD having ANCA examined from October 1995 to September 2008, were reviewed. Among the ANCA positive patients with non-primary vasculitis (Group A), those with primary systematic vasculitis

  20. 慢性阻塞性肺疾病合并肺纤维化的肺动脉压力变化及其与肺功能和生活质量关系的研究%Clinical study on pulmonary arterial pressure, and the correlation between it and lung function and life quality of chronic obstructive pulmonary disease with pulmonary interstitial fiberosis

    Institute of Scientific and Technical Information of China (English)

    宋欢欢; 曹洁

    2011-01-01

    目的探究慢性阻塞性肺疾病(COPD)合并肺纤维化患者的肺动脉压力变化及其与肺功能、生活质量的关系。方法 回顾性对比分析天津医科大学总医院2009年7月至2011年3月诊断的20例COPD合并肺纤维化及随机抽取同期30例单纯COPD患者的肺动脉收缩压、肺功能、Medieal Research Council dyspnea scale(MRC)评分和COPD Assessment Test(CAT)评分,及COPD合并肺纤维化患者的肺动脉收缩压与肺功能指标、MRC和CAT评分的相关性。结果COPD合并肺纤维化组肺动脉收缩压较单纯COPD组升高(P<0.05),MRC、CAT评分较COPD组高(P<0.05),COPD合并肺纤维化组的肺动脉收缩压与FVC% pred、FEV1% pred、DLCO% pred、MRC评分、CAT评分呈负相关,与FEV1/FVC无相关性。结论COPD合并肺纤维化组与COPD组相比,肺动脉收缩压更高,生活质量进一步下降,COPD合并肺纤维化患者的肺动脉收缩压与FVC% pred、FEV1% pred、DLCO% pred、MRC评分、CAT评分呈负相关。%Objective To explore pulmonary arterial pressure, and the correlation between it and lung function and life quality in chronic obstructive pulmonary disease(COPD) with pulmonary interstitial fiberosis. Methods 20 COPD patients following with pulmonary interstitial fiberosis and 30 patients with COPD were retrospectively compared, which came from the General Hospitat of Tianjin Medical University from July 2009 to March 2011. Pulmonary artery systolic pressure, lung function, Medieal Research Council dyspnea scale(MRC) score and COPD Assessment Test (CAT) score, and the correlation between pulmonary artery systolic pressure and lung function, MRC score and CAT score were analized.Results COPD with pulmonary interstitial fiberosis had higher pulmonary arterial systolic pressure ( P <0.05), more MRC score and more CAT score( P <0.05). Pulmonary arterial systolic pressure of COPD with pulmonary interstitial fiberosis had positive correlation with

  1. Unusual progression and subsequent improvement in cystic lung disease in a child with radiation-induced lung injury

    Energy Technology Data Exchange (ETDEWEB)

    Wolf, Michael S. [Monroe Carell Jr. Children' s Hospital at Vanderbilt, Department of Pediatrics, Nashville, TN (United States); Chadha, Ashley D. [Vanderbilt University School of Medicine, Division of Pulmonary Medicine, Department of Pediatrics, Nashville, TN (United States); Carroll, Clinton M.; Borinstein, Scott C. [Vanderbilt University School of Medicine, Division of Hematology and Oncology, Department of Pediatrics, Nashville, TN (United States); Young, Lisa R. [Vanderbilt University School of Medicine, Division of Pulmonary Medicine, Department of Pediatrics, Nashville, TN (United States); Vanderbilt University School of Medicine, Division of Allergy, Pulmonary and Critical Care, Department of Medicine, Nashville, TN (United States); Vanderbilt University School of Medicine, Division of Pulmonary Medicine, Nashville, TN (United States)

    2015-07-15

    Radiation-induced lung disease is a known complication of therapeutic lung irradiation, but the features have not been well described in children. We report the clinical, radiologic and histologic features of interstitial lung disease (ILD) in a 4-year-old child who had previously received lung irradiation as part of successful treatment for metastatic Wilms tumor. Her radiologic abnormalities and clinical symptoms developed in an indolent manner. Clinical improvement gradually occurred with corticosteroid therapy. However, the observed radiologic progression from interstitial and reticulonodular opacities to diffuse cystic lung disease, with subsequent improvement, is striking and has not been previously described in children. (orig.)

  2. Interstitial brines in playa sediments

    Science.gov (United States)

    Jones, B.F.; Van Denburgh, A.S.; Truesdell, A.H.; Rettig, S.L.

    1969-01-01

    Study of several closed drainages in the Great Basin has shown that the interstitial solutions of shallow, fine-grained playa deposits store a large quantity of dissolved solids and are often more concentrated than associated lakes and ponds, except in peripheral zones of stream or ground-water inflow. These interstitial fluids, when compared with local runoff, impoundments, or spring waters, commonly have a distinctive ionic composition which sometimes cannot be explained by either simple mixing of surface and subsurface inflow or by evaporative concentration. At Abert Lake, Oregon, the interstitial solute concentrations increased with depth to values as much as five times greater than the lake, except where springs indicate significant ground-water input. Where Na+, Cl, and CO2 species constitute more than 90% of the solutes, Na+ Cl- ratios in the lake water are lower than in interstitial solutions of bottom cores and higher than in playa fluids. At the same time, Na+ K+ ratios are highest in the fluids of lake bottom muds and lowest in playa interstitials. In deeper playa profiles, interstitial Na+ Cl- tended to decrease with depth (5 ft. maximum). In the Abert Lake area, as in other parts of the western Great Basin, Na+ Cl- ratios are indicative of total CO2 in solution and the effects of organic decay in surficial sediments. These ratios, coupled with data on silica and bulk density, show that higher PCO2 accompanying decay promotes silicate dissolution and hydrogen ion exchange, stripping alkalis from sediment which had preferentially adsorbed K+ when entering the lake. On subsequent loss of pore fluid in the playa regime, silica initially released to solution in the lake environment is readsorbed on dissolution products. ?? 1969.

  3. Acute respiratory failure secondary to mesalamine-induced interstitial pneumonitis.

    Science.gov (United States)

    Abraham, Albin; Karakurum, Ali

    2013-08-20

    Interstitial pneumonitis as an adverse effect of mesalamine therapy is a rare but potentially serious complication. Patients typically have a mild disease course with no documented cases of respiratory failure in published literature. Given its variable latent period and non-specific signs and symptoms, it may be difficult to diagnose. We present the case of a 65-year-old man who presented with symptoms of fever, shortness of breath and a non-productive cough, 2 weeks after initiation of therapy with mesalamine. His hospital course was complicated by acute respiratory failure requiring intubation and mechanical ventilation. Radiographic studies revealed bilateral lower lobe infiltrates and bronchosopy with bronchoalveolar lavage and transbronchial biopsy were consistent with a diagnosis of drug-induced interstitial pneumonitis. The aim of this paper is to highlight the importance of considering a diagnosis of mesalamine-induced lung injury in patients presenting with respiratory symptoms while on mesalamine therapy and to review relevant literature.

  4. [Lung infiltrations in Hodgkin lymphoma].

    Science.gov (United States)

    Ciurea-Löchel, A; Ciurea, A; Stey, C; Pestalozzi, B

    2001-08-02

    We report the case of a young patient presenting with cervical lymphadenopathy and interstitial pulmonary infiltrates due to Hodgkin's Disease. Although lung involvement regressed under chemotherapy, we observed new alveolar infiltrates during treatment. Steroid administration after exclusion of an infectious cause was followed by rapid clinical and radiological improvement, indicating the probable presence of pulmonary bleomycine toxicity.

  5. Successful Management of Graft Reinfection of HCV Genotype 2 in Living Donor Liver Transplantation from a Hepatitis B Core Antibody-Positive Donor with Sofosbuvir and Ribavirin

    Directory of Open Access Journals (Sweden)

    Reina Sasaki

    2016-07-01

    Full Text Available Direct-acting antivirals (DAAs are relatively safe and highly effective for the eradication of hepatitis C virus (HCV in liver transplant recipients. In this case study, we present a female with a graft reinfected with HCV genotype 2 who was treated with a combination of sofosbuvir and ribavirin after living donor liver transplantation (LDLT. Because the graft was from a hepatitis B core antibody-positive donor, passive immunization with hyperimmune hepatitis B immunoglobulin (HBIG and entecavir were also provided to prevent hepatitis B virus (HBV reactivation. It became clear that the combination of sofosbuvir and ribavirin promptly led to a sustained virologic response and that this combination was safe to treat graft reinfection with HCV genotype 2 after LDLT. Adverse events caused by DAAs were not observed, except for slight anemia. HBIG and entecavir were useful in the prevention of HBV reactivation. In conclusion, the present case indicated that DAA treatment for graft reinfection with HCV is safe and effective in LDLT from hepatitis B core antibody-positive donors.

  6. Successful Management of Graft Reinfection of HCV Genotype 2 in Living Donor Liver Transplantation from a Hepatitis B Core Antibody-Positive Donor with Sofosbuvir and Ribavirin

    Science.gov (United States)

    Sasaki, Reina; Kanda, Tatsuo; Ohtsuka, Masayuki; Yasui, Shin; Haga, Yuki; Nakamura, Masato; Yokoyama, Masayuki; Wu, Shuang; Nakamoto, Shingo; Arai, Makoto; Maruyama, Hitoshi; Miyazaki, Masaru; Yokosuka, Osamu

    2016-01-01

    Direct-acting antivirals (DAAs) are relatively safe and highly effective for the eradication of hepatitis C virus (HCV) in liver transplant recipients. In this case study, we present a female with a graft reinfected with HCV genotype 2 who was treated with a combination of sofosbuvir and ribavirin after living donor liver transplantation (LDLT). Because the graft was from a hepatitis B core antibody-positive donor, passive immunization with hyperimmune hepatitis B immunoglobulin (HBIG) and entecavir were also provided to prevent hepatitis B virus (HBV) reactivation. It became clear that the combination of sofosbuvir and ribavirin promptly led to a sustained virologic response and that this combination was safe to treat graft reinfection with HCV genotype 2 after LDLT. Adverse events caused by DAAs were not observed, except for slight anemia. HBIG and entecavir were useful in the prevention of HBV reactivation. In conclusion, the present case indicated that DAA treatment for graft reinfection with HCV is safe and effective in LDLT from hepatitis B core antibody-positive donors. PMID:27721720

  7. [A case of an anti-Ma2 antibody-positive patient presenting with variable CNS symptoms mimicking multiple system atrophy with a partial response to immunotherapy].

    Science.gov (United States)

    Shiraishi, Wataru; Iwanaga, Yasutaka; Yamamoto, Akifumi

    2015-01-01

    A 70-year-old man with a 5-month history of progressive bradykinesia of the bilateral lower extremities was admitted to our hospital. At the age of 64, he underwent proximal gastrectomy for gastric cancer. He also had a history of subacute combined degeneration of the spinal cord since the age of 67, which was successfully treated with vitamin B12 therapy. Four weeks before admission to our hospital, he admitted himself to his former hospital complaining of walking difficulty. Two weeks later, however, his symptoms progressed rapidly; he was immobilized for two weeks and did not respond to the vitamin therapy. On admission to our hospital, he showed moderate paralysis of the lower extremities, cog-wheel rigidity of the four extremities, and dystonic posture of his left hand. He also showed orthostatic hypotension and vesicorectal disorders. Blood examination and cerebrospinal fluid analysis revealed no remarkable abnormalities. Electroencephalography showed frontal dominant, high voltage, sharp waves. His brain and spinal MRI revealed no notable abnormalities. We suspected autoimmune disease and commenced one course of intravenous methylprednisolone therapy, resulting in improvement of the parkinsonism and orthostatic hypotension. Based on these results, we investigated possible neural antigens and detected anti-Ma2 antibody. In addition to limbic encephalitis, anti-Ma2 antibody-positive neural disorders are characterized by rapid eye movement sleep behavior disorders or parkinsonism. Here, we report an anti-Ma2 antibody positive patient presenting variable CNS symptoms mimicking multiple system atrophy, who responded to immunotherapy.

  8. Pulmonary interstitial fibrosis following near-drowning and exposure to short-term high oxygen concentrations.

    Science.gov (United States)

    Glauser, F L; Smith, W R

    1975-09-01

    Following near-drowning in fresh water, a 19-year-old man experienced severe adult respiratory distress syndrome, necessitating ventilatory support with positive end-expiratory pressure and high oxygen concentrations. Post-extubation, his course was highlighted by persistent hypoxemia and interrupted by a lung abscess which responded promptly to antibiotics. Pulmonary function tests were consistent with severe restrictive disease and chest radiograph revealed persistent bilateral alveolar and interstitial infiltrates. An open lung biopsy on the 26th hospital day showed interstitial fibrosis. Over the ensuing two months, the chest radiograph and pulmonary function tests returned towards normal. We attribute the pulmonary fibrosis to incomplete resolution of the alveolar interstitial pathology secondary to the near-drowning and exposure to high oxygen mixtures.

  9. The Serum Levels and Clinical Significance of HGF and KL-6 in Patients of Interstitial Lung Disease with Reumatoid Arthritis%HGF 与 KL-6在 RA-ILD 患者血清中的表达水平及意义

    Institute of Scientific and Technical Information of China (English)

    王树刚; 王晓东; 慈春增; 于杰; 李向东

    2015-01-01

    目的:通过检测肝细胞生长因子( HGF)和人Ⅱ型肺泡细胞表面抗原( KL-6)在类风湿关节炎合并肺间质病变( RA-ILD)患者血清中的表达水平,探讨其在RA-ILD中的意义。方法采用ELISA法检测60例RA(包括28例RA-ILD)和20例健康体检者血清HGF,KL-6的表达水平。结果 RA-ILD组中HGF的含量明显低于单纯RA组及对照组,差异有统计学意义(P<0.05);RA-ILD组中KL-6的含量明显高于单纯RA组及对照组,差异有统计学意义(P<0.05);且HGF与KL-6呈负相关关系;在RA-ILD组,HGF与疾病活动性指标DAS28评分呈负相关关系,KL-6与疾病活动性指标DAS28评分呈正相关关系。结论 HGF和KL-6可能参与RA-ILD的发生发展过程,HGF和KL-6可作为判断RA-ILD活动的指标。%Objective To analyze the hepatocyte growth factor( HGF) and human typeⅡalveolar cell sur-face antigen( KL-6 ) serum levels and their clinical significance in the patients of interstitial lung disease with rheumatoid arthritis( RA-ILD) .Methods The serum levels of HGF and KL-6 were detected in 60 patients with rheumatoid arthritis ( including 28 patients with interstitial lung disease) and 20 healthy individuals by means of enzyme linked immunosor-bent assay( ELISA) .Results In patients of RA-ILD,the serum levels of HGF was lower than simple RA group and the control group,and the serum levels of KL-6 was higher than simple RA group and the control group,there were signifi-cantly statistical difference(P<0.05).The levels of HGF and KL-6 were negatively related;In group RA-ILD,HGF was negatively correlated with disease activity index DAS28 score,KL-6 was positively correlated with disease activity index DAS28 score.Conclusion The cytokines of HGF and KL-6 may play important roles in the development of RA-ILD;perhaps they can be seen as indexes of RA-ILD activities.

  10. 原发性干燥综合征间质性肺疾病与临床免疫学指标的关系%Relationship between clinic and index of commune and interstitial lung involvement in patients with primary sjogrens syndrome

    Institute of Scientific and Technical Information of China (English)

    吴晓丹; 龙武彬

    2009-01-01

    目的 探讨原发性干燥综合征患者间质性肺疾病与临床及类风湿因子(RF)、血沉(SR)、C反应蛋白(CRP)、补体及免疫球蛋白、ANA、抗SSA、抗SSB的关系.方法 原发性干燥综合征患者79例分为两组,一组是伴有间质性肺病变23例,另一组是不伴有间质性肺病变56例.所有患者均测定类风湿因子、血沉、C反应蛋白、补体C3及免疫球蛋白、ANA、抗SSA、抗SSB.结果 原发性干燥综合征发生间质性肺疾病的发生率为24%.原发性干燥综合征发生间质性肺疾病组的年龄、类风湿因子滴定度、血沉、C反应蛋白、免疫球蛋白均明显高于无间质性肺疾病组(P0.05). Conclusion The rising of Age, RF titer, and immunoglobulin,the low of C3 in patients suggest interstitial lung involvement, these patients with primary sjogrens syndrome were treated and controlled early.

  11. Dermatomyositis and clinically amyopathic dermatomyositis complicated with interstitial lung disease: clinical analysis of 43 cases%43例皮肌炎和临床无肌病型皮肌炎合并肺间质病变的临床分析

    Institute of Scientific and Technical Information of China (English)

    周琳; 曹华; 郑捷

    2014-01-01

    目的:探讨皮肌炎(dermatomyositis,DM)和临床无肌病性皮肌炎(clinically amyopathic dermatomyositis,CADM)合并肺间质病变(interstitial lung disease,ILD)患者的胸部影像学表现与临床特征、实验室检查及预后的关系.方法:收集43例DM/CADM合并ILD患者的临床资料,根据肺高分辨率CT结果将患者分为2组,G1组患者的肺高分辨率CT影像主要表现为网格影及纤维条索影,G2组则主要表现为磨玻璃样、蜂窝状病变.比较2组间的临床特征、实验室检查及预后.结果:G2组患者发热、低氧血症的发生率、铁蛋白、乳酸脱氢酶水平、死亡率显著高于G1组:而G2组患者血清肌酸激酶水平、肌电图及肌肉活检阳性率均显著低于G1组.结论:在合并ILD的DM/CADM患者中,肺高分辨率CT影像特征与ILD病情密切相关,以网格影及纤维条索影为主的患者ILD病情轻,预后好,而磨玻璃样、蜂窝状病变为主的患者ILD病情重、预后差.

  12. Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome.

    Science.gov (United States)

    Kim, Ji-Hye; Yee, Cheonga; Kuk, Jin-Yi; Choi, Suk-Joo; Oh, Soo-Young; Roh, Cheong-Rae; Kim, Jong-Hwa

    2016-09-01

    Pregnant women with antiphospholipid syndrome (APS) carry a high risk of arterial or venous thrombosis. Such thrombotic conditions occur more frequently in patients with triple positivity to antiphospholipid antibodies or with high antibody titers. Hepatic infarction is a rare complication in pregnant women with APS, and it sometimes mimics HELLP syndrome. This report describes a preeclamptic pregnant woman with APS who had high titers of three antiphospholipid antibodies. She experienced severe epigastric pain with elevated liver enzymes; in addition, she had tachycardia and tachypnea. The clinical findings suggested hepatic infarction and pulmonary thromboembolism, a partial manifestation of catastrophic APS. Therefore, she underwent emergent cesarean section at 25+2 weeks of gestation. After the delivery, her laboratory test indicated HELLP-like features, and computed tomography confirmed hepatic infarction and pulmonary micro-thromboembolism. Here, we report a case of a partial manifestation of catastrophic APS in a pregnant woman with triple antibody positivity, including a brief literature review.

  13. Bacterial Interstitial Nephritis in Children

    OpenAIRE

    Bobadilla Chang, Fernando; Departamento de Ciencias Dinámicas Facultad de Medicina Universidad Nacional Mayor de San Marcos Lima, Perú; Villanueva, Dolores; Departamento de Ciencias Dinámicas Facultad de Medicina Universidad Nacional Mayor de San Marcos Lima, Perú

    2014-01-01

    OBJECTIVE: To assess the diagnosis approach to urinary tract infections in children. MATERIAL AND METHODS: Medical records from 103 children with diagnosis of interstitial bacterial nephritis were retrospectively reviewed. Diagnosis was supported by the dramatic involvement of renal parenquima, which is not addressed as "urinary tract infection". RESULTS: From all 103 patients, 49 were 2-years-old or younger, 33 were between 2 and 5-years-old, and 21 were between 6 to 10. Clinical picture inc...

  14. Technique of after-loading interstitial implants.

    Science.gov (United States)

    Syed, A M; Feder, B H

    1977-01-01

    Interstitial implants are either removable or permanent (and occasionally a combination of both). Permanent implants are generally utilized where tumors are not accessible enough to permit easy removal of sources or where accurate source distribution is less critical. They are useful for cancers of the lung, pancreas, prostate, bladder, lymph nodes, etc. Radon and gold-198 have been largely replaced by iodine-125. Our major interests are in the removable after-loading iridium-192 implant techniques. Template (steel guide) and non-template (plastic tube) techniques are utilized. Templates are preferred where the tumor volume can only be approached from one side and where accurate positioning of sources would otherwise be difficult. They are useful for cancers of the cervix, vagina, urethra, and rectum. Non-template (plastic tube) techniques are preferred where the tumor volume can be approached from at least two sides and where templates are either not feasible or not essential for accurate positioning of sources. The single needle non-template approach is useful for cancers of lip, nodes, and breast (plastic button) and for cancers of the oral cavity and oropharynx (gold button). The paired needle non-template approach is useful for cancers of the gum, retromolar trigone, and base of tongue (loop technique) and for cancers of the palate (arch technique). Procedures for each technique are described in detail.

  15. Interstitial guidance of cancer invasion.

    Science.gov (United States)

    Gritsenko, Pavlo G; Ilina, Olga; Friedl, Peter

    2012-01-01

    Cancer cell invasion into healthy tissues develops preferentially along pre-existing tracks of least resistance, followed by secondary tissue remodelling and destruction. The tissue scaffolds supporting or preventing guidance of invasion vary in structure and molecular composition between organs. In the brain, the guidance is provided by myelinated axons, astrocyte processes, and blood vessels which are used as invasion routes by glioma cells. In the human breast, containing interstitial collagen-rich connective tissue, disseminating breast cancer cells preferentially invade along bundled collagen fibrils and the surface of adipocytes. In both invasion types, physical guidance prompted by interfaces and space is complemented by molecular guidance. Generic mechanisms shared by most, if not all, tissues include (i) guidance by integrins towards fibrillar interstitial collagen and/or laminins and type IV collagen in basement membranes decorating vessels and adipocytes, and, likely, CD44 engaging with hyaluronan; (ii) haptotactic guidance by chemokines and growth factors; and likely (iii) physical pushing mechanisms. Tissue-specific, resticted guidance cues include ECM proteins with restricted expression (tenascins, lecticans), cell-cell interfaces, and newly secreted matrix molecules decorating ECM fibres (laminin-332, thrombospondin-1, osteopontin, periostin). We here review physical and molecular guidance mechanisms in interstitial tissue and brain parenchyma and explore shared principles and organ-specific differences, and their implications for experimental model design and therapeutic targeting of tumour cell invasion.

  16. Desquamative interstitial pneumonia: an analytic review with an emphasis on aetiology

    Directory of Open Access Journals (Sweden)

    Benoît Godbert

    2013-06-01

    Full Text Available Desquamative interstitial pneumonia (DIP is characterised by the accumulation of numerous pigmented macrophages within most of the distal airspace of the lung and, sometimes, the presence of giant cells. Diagnosis of DIP is not easy and requires surgical lung biopsy. DIP is usually associated with tobacco smoke. However, the association between smoking and DIP is less robust than that with respiratory bronchiolitis with interstitial lung disease or pulmonary Langerhans’ cell histiocytosis; approximately 10–42% of patients with DIP are nonsmokers. DIP can also occur in patients following exposure to certain inhaled toxins (occupational exposure and drugs, and may occur in the context of certain viral illnesses and autoimmune diseases. In the context of DIP, occupational exposure should be systematically investigated.

  17. 类风湿性关节炎患者间质性肺疾病的临床概况%Interstitial lung disease in patients with rheumatoid arthritis

    Institute of Scientific and Technical Information of China (English)

    杨福堂; 赵萌; 王淑云; 李燕; 吕培瑾; 侯庆民

    2009-01-01

    目的 了解类风湿性关节炎肺间质病变的发生情况、特征及相关因素分析,以便早期发现类风湿性关节炎的肺部病变.方法 类风湿性关节炎患者45例,免疫比浊法检测类风湿因子、C反应蛋白、免疫球蛋白及补体;间接免疫荧光法和免疫印迹法检测抗核抗体及亚类.血气分析测定氧分压、动脉血二氧化碳分压和肺泡-动脉血氧分压差.肺功能仪测定潮气容积、最大肺活量、用力肺活量、第1秒用力呼气容积、最大呼气中段流量、最大通气量和一氧化碳弥散功能.放射学检查包括胸部正侧位X线片、双手像和肺高分辨率CT扫描.结果 45例类风湿关节患者中,14例存在肺间质病变,其中10例有呼吸道症状.肺功能检测异常主要为弥散功能降低和限制性通气障碍.8例胸部X线片存在异常,14例高分辨率CT发现异常.肺高分辨率CT在发现类风湿性关节炎肺间质病变病变时优于普通胸部X线片.结论 类风湿性关节炎肺间质病变的发生与疾病活动性和严重性相关,肺弥散功能、高分辨率CT在早期发现病变时有诊断意义.%Objective To investigate the prevalence and clinical manifestation of rheumatoid arthritis-inter-stitial lung disease(RA-ILD). Methods Forty-five inpatients with rheumatoid arthritis(RA) were enrolled. Serum rheumatoid factor (RF), C reactive protein (CRP), immunoglobulin (IgG, IgA, IgM), and compliment (C3, C4) were measured by immunoncphelometry. Antinuclear antibodies (ANAs) and anti-extractable nuclear antigen(anti-ENA) antibodies were measured by indirect immunofluorescence technique and Western blot. Arterial gas analysis included PaO2, PaCO2, and PA-zO2. The following pulmonary function tests were performed: tidal volume(VT), maximal vital-capacity (Vcmax), forced vital capacity (FVC), one second forced expiratory volume (FEV1), FEV1/FVC, maximal mid-expiratory flow(MMFF), maximal voluntary ventilation(MVV) and

  18. Idiopathic interstitial pneumonias with connective tissue diseases features: A review.

    Science.gov (United States)

    Cottin, Vincent

    2016-02-01

    A systematic approach is recommended to search for clinical and biological features of connective tissue disease (CTD) in any patient with interstitial lung disease (ILD). In the diagnostic approach to ILD, a diagnosis of CTD should be considered particularly in women and subjects younger than 50 years, and in those with an imaging and/or pathological pattern of non-specific interstitial pneumonia. However, the diagnosis of CTD may be difficult when ILD is the presenting or the dominant manifestation of CTD. A proportion of patients with ILD present symptoms that belong to the spectrum of CTD and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given CTD. Some imaging and histopathological patterns may also suggest the presence of an underlying CTD. Although studies published to date used heterogeneous definitions and terminology for this condition, evidence is accumulating that even limited CTD features are relevant regarding symptoms, imaging features, pathological pattern and possibly evolution to overt CTD, whereas the impact on prognosis needs confirmation. Conversely, autoantibodies alone do not seem to impact the prognosis or management in patients with otherwise typical idiopathic pulmonary fibrosis and no extra-pulmonary manifestation. A collective international multidisciplinary effort has proposed a uniform definition and criteria for 'interstitial pneumonia with autoimmune features', a condition characterized by limited CTD features occurring in the setting of ILD, with the aim of fostering future clinical studies. Referral of ILD patients suspect to have CTD to a rheumatologist and possibly multidisciplinary discussion may contribute to a better management.

  19. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP.

    Science.gov (United States)

    Kokosi, Maria A; Nicholson, Andrew G; Hansell, David M; Wells, Athol U

    2016-05-01

    In the 2013 reclassification of the idiopathic interstitial pneumonias (IIPs), two rare IIPs (idiopathic lymphoid interstitial pneumonia (LIP), idiopathic pleuroparenchymal fibroelastosis (IPPFE)) and two rare histologic patterns (acute fibrinous and organizing pneumonia (AFOP), bronchiolocentric pattern of interstitial pneumonia (BPIP)) are described. All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. LIP is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin-walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. IPPFE is defined histologically by coexisting upper lobe pleural and intra-alveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. AFOP is a rare histologic pattern lying within the spectrum of acute/subacute lung injury, characterized by organizing pneumonia and intra-alveolar fibrin deposition without hyaline membranes. BPIP is characterized histologically by fibrosis and/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, AFOP and BPIP are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features.

  20. Diagnostic Value of Low Dose Multi-slice Spiral VHRCT (MSCT) CT in Children with Chronic Interstitial Lung Disease%小剂量多层螺旋CT(MSCT)VHRCT在小儿慢性肺间质病变中的诊断价值

    Institute of Scientific and Technical Information of China (English)

    李燕北; 李云秀; 王宝军

    2016-01-01

    目的:分析研讨小剂量多层螺旋CT(MSCT)VHRCT在小儿慢性肺间质病变中的诊断价值。方法:此研究中所研讨的32例患儿均随机从2013年5月至2014年11月期间我院收治的小儿慢性肺间质病变患儿中选取而出,回顾分析患儿临床病历资料,32例患儿均接受小剂量多层螺旋CT(MSCT)容积数据重组高分辨和HRCT扫描,对比此两者检查扫描方式在囊状透亮部位、网格影、结节影、肺大泡影、磨玻璃密度影等差异性,对比诊断准确性和图像质量评分状况。结果:对比同层面囊状透亮部位、网格影、结节影、肺大泡影、磨玻璃密度影、蜂窝影等显影状况,HRCT方式比MSCT方式要稍微高一点,但组间数据无统计学意义(P>0.05)。小剂量MSCT检查方式在图像质量评分上比VHRCT检查方式要稍高,但组间数据无统计学意义(P>0.05);在诊断准确率上,小剂量MSCT(93.75%)和VHRCT方式(96.87%)比较,虽后者稍高,但组间数据无统计学意义(P>0.05)。结论:HRCT检测结果比小剂量多层螺旋CTMSCT检查结果更加具有优势性,特别是针对小剂量多层螺旋CTMSCT和大龄患儿检查判定不佳时,可依据实际状况考虑是否采用HRCT,因此,建议临床在诊断疾病时,按照患儿实际状况确定检查方式,确保诊断准确性。%ABSTRACT:Objective:To analyze and discuss the value of low dose multi-slice spiral VHRCT (MSCT) CT in the diagnosis of chronic interstitial lung disease in children.Methods:32 children with this research studied were randomly from 2013 May to 2014 November period in our hospital pediatric chronic lung interstitial lesion in children with selected out, children with clin-ical data were retrospectively analyzed, 32 cases underwent low dose multislice spiral CT (MSCT) volume number according to the restructuring of high resolution scanning and HRCT, compared with the two scanning way

  1. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?

    Science.gov (United States)

    Tomassetti, Sara; Ryu, Jay H; Piciucchi, Sara; Chilosi, Marco; Poletti, Venerino

    2016-06-01

    We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. This hypothesis warrants further studies. Currently, two major radiologic-pathologic different profiles have been well established. First, the "inflammatory type" characterized by prominent lymphocytic inflammation both on biopsy and bronchoalveolar lavage (BAL), and high-resolution computed tomography (HRCT) with mixed NSIP/organizing pneumonia pattern that tends to have a better response to corticosteroid and immunosuppressive treatment. Second, the "highly fibrotic" subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into "full-blown idiopathic pulmonary fibrosis." The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients.

  2. [Lung is also involved in juvenile dermatomyositis].

    Science.gov (United States)

    Pouessel, G; Thumerelle, C; Nève, V; Santangelo, T; Flammarion, S; Pruvot, I; Tillie-Leblond, I; Deschildre, A

    2014-07-01

    Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children.

  3. Fibrotic idiopathic interstitial pneumonias: HRCT findings that predict mortality

    Energy Technology Data Exchange (ETDEWEB)

    Edey, Anthony J.; Hansell, David M. [The Royal Brompton Hospital, Department of Radiology, London (United Kingdom); Devaraj, Anand A. [St. George' s NHS Foundation Trust, Department of Radiology, Tooting (United Kingdom); Barker, Robert P. [Frimley Park Hosptal, Department of Radiology, Frimley, Surrey (United Kingdom); Nicholson, Andrew G. [The Royal Brompton Hospital, Department of Histopathology, London (United Kingdom); Wells, Athol U. [The Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom)

    2011-08-15

    The study aims were to identify CT features that predict outcome of fibrotic idiopathic interstitial pneumonia (IIP) when information from lung biopsy data is unavailable. HRCTs of 146 consecutive patients presenting with fibrotic IIP were studied. Visual estimates were made of the extent of abnormal lung and proportional contribution of fine and coarse reticulation, microcystic (cysts {<=}4 mm) and macrocystic honeycombing. A score for severity of traction bronchiectasis was also assigned. Using death as our primary outcome measure, variables were analysed using the Cox proportional hazards model. CT features predictive of a worse outcome were coarse reticulation, microcystic and macrocystic honeycombing, as well as overall extent of lung abnormality (p < 0.001). Importantly, increased severity of traction bronchiectasis, corrected for extent of parenchymal abnormality, was predictive of poor prognosis regardless of the background pattern of abnormal lung (HR = 1.04, CI = 1.03-1.06, p < 0.001). On bivariate Cox analysis microcystic honeycombing was a more powerful determinant of a poor prognosis than macrocystic honeycombing. In fibrotic IIPs we have shown that increasingly severe traction bronchiectasis is indicative of higher mortality irrespective of the HRCT pattern and extent of disease. Extent of microcystic honeycombing is a more powerful determinant of outcome than macrocystic honeycombing. (orig.)

  4. The classification, natural history and radiological/histological appearance of idiopathic pulmonary fibrosis and the other idiopathic interstitial pneumonias

    Directory of Open Access Journals (Sweden)

    G. Raghu

    2008-12-01

    Full Text Available The idiopathic interstitial pneumonias (IIPs are a heterogeneous group of rare interstitial lung diseases (ILDs or diffuse parenchymal lung diseases, which, as their name implies, are of unknown aetiology. The past 10 yrs have seen important advances in the classification of the IIPs into idiopathic pulmonary fibrosis (IPF and its corresponding histopathological pattern of usual interstitial pneumonia (UIP, plus six non-IPF IIP subtypes. The present article will look at the current classification of IIPs, arising from the Consensus Statement of the American Thoracic Society and European Respiratory Society, and discusses the importance of differential diagnosis of IPF from the non-IPF IIP subtypes, especially nonspecific interstitial pneumonia. Diagnosis of IIPs is a dynamic process involving close collaboration between pulmonologists, radiologists and pathologists. Increasingly accurate diagnosis of IPF has been made possible by the use of high-resolution computed tomography (HRCT and refinements in surgical lung biopsy. In IPF, a lung HRCT will typically reveal irregular reticular opacities, traction bronchiestasis and, most importantly, peripheral honeycombing. In contrast, histological examination shows evidence of UIP manifesting as typically subpleural and paraseptal established fibrosis, often with honeycomb changes, associated with mild chronic inflammation and varying numbers of fibroblastic foci in continuity with the edges of areas of established fibrosis. Despite these advances, obtaining a consistent and uniform diagnosis of idiopathic interstitial pneumonias is difficult, with studies showing significant disagreement in the diagnosis of interstitial lung diseases between academic centres of expertise and community-based clinicians. Greater interaction between academic and community clinicians, together with improved education, is needed to bridge this gap.

  5. Interstitial pneumonia associated with linear immunoglobulin A/immunoglobulin G bullous dermatosis.

    Science.gov (United States)

    Kakugawa, Tomoyuki; Tomimura, Saori; Hayashi, Tomayoshi; Sakamoto, Noriho; Ishimatsu, Yuji; Mukae, Hiroshi; Kohno, Shigeru

    2013-01-01

    A 76-year-old man with interstitial lung disease was admitted to our institution after developing persistent dyspnea upon effort. He also had a relapse of bullous eruptions on the skin of the trunk and extremities, previously diagnosed as vesicular pemphigoid. Direct immunofluorescence of a skin biopsy specimen using fluorescence microscopy showed the linear deposition of immunglobulin A (IgA), IgG and C3 along the basement membrane. These findings indicated a definitive diagnosis of linear IgA/IgG bullous dermatosis. Chest computed tomography, bronchoalveolar lavage and transbronchial lung biopsy findings suggested nonspecific interstitial pneumonia. Direct immunofluorescence of the lung biopsy specimens using fluorescence microscopy also showed a deposition of IgA, IgG an