Sample records for diffuse lung disease

  1. Diffuse parenchymal lung disease

    Sara Tomassetti


    Full Text Available Between September 2015 and August 2016 there were >1500 publications in the field of diffuse parenchymal lung diseases (DPLDs. For the Clinical Year in Review session at the European Respiratory Society Congress that was held in London, UK, in September 2016, we selected only five articles. This selection, made from the enormous number of published papers, does not include all the relevant studies that will significantly impact our knowledge in the field of DPLDs in the near future. This review article provides our personal view on the following topics: early diagnosis of idiopathic pulmonary fibrosis, current knowledge on the multidisciplinary team diagnosis of DPLDs and the diagnostic role of transbronchial cryobiopsy in this diagnostic setting, insights on the new entity of interstitial pneumonia with autoimmune features, and new therapeutic approaches for scleroderma-related interstitial lung disease.

  2. Spectrum of fibrosing diffuse parenchymal lung disease.

    Morgenthau, Adam S; Padilla, Maria L


    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.

  3. Diffuse interstitial lung disease: overlaps and uncertainties

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


    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.)

  4. Bronchoscopic cryobiopsy for the diagnosis of diffuse parenchymal lung disease.

    Jonathan A Kropski

    Full Text Available BACKGROUND: Although in some cases clinical and radiographic features may be sufficient to establish a diagnosis of diffuse parenchymal lung disease (DPLD, surgical lung biopsy is frequently required. Recently a new technique for bronchoscopic lung biopsy has been developed using flexible cryo-probes. In this study we describe our clinical experience using bronchoscopic cryobiopsy for diagnosis of diffuse lung disease. METHODS: A retrospective study of subjects who had undergone bronchoscopic cryobiopsy for evaluation of DPLD at an academic tertiary care center from January 1, 2012 through January 15, 2013 was performed. The procedure was performed using a flexible bronchoscope to acquire biopsies of lung parenchyma. H&E stained biopsies were reviewed by an expert lung pathologist. RESULTS: Twenty-five eligible subjects were identified. With a mean area of 64.2 mm(2, cryobiopsies were larger than that typically encountered with traditional transbronchial forceps biopsy. In 19 of the 25 subjects, a specific diagnosis was obtained. In one additional subject, biopsies demonstrating normal parenchyma were felt sufficient to exclude diffuse lung disease as a cause of dyspnea. The overall diagnostic yield of bronchoscopic cryobiopsy was 80% (20/25. The most frequent diagnosis was usual interstitial pneumonia (UIP (n = 7. Three of the 25 subjects ultimately required surgical lung biopsy. There were no significant complications. CONCLUSION: In patients with suspected diffuse parenchymal lung disease, bronchoscopic cryobiopsy is a promising and minimally invasive approach to obtain lung tissue with high diagnostic yield.

  5. Expiratory high-resolution CT in diffuse lung disease

    Arakawa, Hiroaki [St. Marianna Univ. School of Medicine, Kawasaki, Kanagawa (Japan)


    Expiratory high-resolution computed tomography (HRCT) is a powerful adjunct to inspiratory HRCT in the diagnosis of diffuse lung disease (DLD), revealing air-trapping even when the inspiratory scan is normal. Expiratory scans are also useful in the differentiation of inhomogeneous lung opacity, which is not uncommon in various types of DLD. The history and technique of expiratory HRCT are described as well as the basic understanding of lung attenuation and the diagnostic value of expiratory scans DLD. The clinical significance of the presence of expiratory air-trapping in the absence of inspiratory scan abnormality is also presented. (author)

  6. Scintigraphic studies of inflammation in diffuse lung disease

    Line, B.R. (Albany Medical College, New York (USA))


    67Ga lung scintigraphy is an established means to assess alveolar inflammation in a wide variety of diffuse lung diseases. It can be used to monitor the extent and activity of the alveolitis during the course of the disease and as a follow-up evaluation to therapy. Although the mechanism of 67Ga localization is not established firmly, the isotope appears to act as a tracer for disturbed protein and cellular fluxes within the interstitium and alveolar spaces. The radiolabeled aerosol study may also be applied to the study of these fluxes as a reflection of inflammation and injury. Although Tc-DTPA clearance studies are highly sensitive to lung injury, they may be too nonspecific to separate lung injury from other physiologic processes effectively. 117 references.




    Full Text Available Diffuse parenchyma lung disease (DPLD encompasses a hetero - geneous group of disorders, characterized by a spectrum of inflammatory and fibrotic changes affecting alveolar walls and air spaces. They comprise over 200 entities and include a wide spectrum of diseases, many uncommon and many of unknown etiology. The incidence and prevalence rates of DPLD have not been precisely estimated due to difficulties in ascertaining a specific diagnosis on a specific disease. MATERIAL & METHODS : Prospective observational study done on 20 adult patients with radiologically diffuse parenchymal lung disease admitted between January 2010 and May 2015 in Govt. General & Chest Hospital, Hyderabad were subjected for Transbronchial Lung Biopsy via flexible fibreoptic bronchoscopy, without fluoroscopic guidance. RESULTS : Out of 20 patients studied adequate lung tissue was obtained in 15 patients, yield of the procedure was 75%. Out of 15 patient’s histopathological diagnosis of chronic interstitial pneumonia is seen in 5 members, interstitial fibrosis is seen in 4 members, non caseating granulomas seen in 4 members, pulmonary alveolar protenosis was seen in 1 member and normal lung histopathology was seen in 1 members. Diagnostic yield of the procedure was 93.3% and overall diagnostic yield was 70%. Two patients developed post procedure pneumothorax. Both of them underwent closed - tube thoracostomy, lung expanded well and ICD was removed in 4 days. No significant bleeding was observed in any patient. No mortality was observed after the procedure . CONCLUSIONS : Transbronchial lung biopsy through flexible bronchoscopy is a simple, safe and effective procedure for the diagnosis of diffuse parenchymal lung diseases. Complications were observed in only few patients out of twenty, which were successfully managed with ICD.

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

    Wang, Jiahui; Li, Feng; Li, Qiang


    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.

  9. Processing of CT images for analysis of diffuse lung disease in the lung tissue research consortium

    Karwoski, Ronald A.; Bartholmai, Brian; Zavaletta, Vanessa A.; Holmes, David; Robb, Richard A.


    The goal of Lung Tissue Resource Consortium (LTRC) is to improve the management of diffuse lung diseases through a better understanding of the biology of Chronic Obstructive Pulmonary Disease (COPD) and fibrotic interstitial lung disease (ILD) including Idiopathic Pulmonary Fibrosis (IPF). Participants are subjected to a battery of tests including tissue biopsies, physiologic testing, clinical history reporting, and CT scanning of the chest. The LTRC is a repository from which investigators can request tissue specimens and test results as well as semi-quantitative radiology reports, pathology reports, and automated quantitative image analysis results from the CT scan data performed by the LTRC core laboratories. The LTRC Radiology Core Laboratory (RCL), in conjunction with the Biomedical Imaging Resource (BIR), has developed novel processing methods for comprehensive characterization of pulmonary processes on volumetric high-resolution CT scans to quantify how these diseases manifest in radiographic images. Specifically, the RCL has implemented a semi-automated method for segmenting the anatomical regions of the lungs and airways. In these anatomic regions, automated quantification of pathologic features of disease including emphysema volumes and tissue classification are performed using both threshold techniques and advanced texture measures to determine the extent and location of emphysema, ground glass opacities, "honeycombing" (HC) and "irregular linear" or "reticular" pulmonary infiltrates and normal lung. Wall thickness measurements of the trachea, and its branches to the 3 rd and limited 4 th order are also computed. The methods for processing, segmentation and quantification are described. The results are reviewed and verified by an expert radiologist following processing and stored in the public LTRC database for use by pulmonary researchers. To date, over 1200 CT scans have been processed by the RCL and the LTRC project is on target for recruitment of the

  10. Interstitial lung disease - adults - discharge

    Diffuse parenchymal lung disease - discharge; Alveolitis - discharge; Idiopathic pulmonary pneumonitis - discharge; IPP - discharge; Chronic interstitial lung - discharge; Chronic respiratory interstitial lung - discharge; Hypoxia - interstitial lung - discharge

  11. Diffuse cystic lung disease of unexplained cause with coexistent small airway disease: a possible causal relationship?

    Rowan, Camilla; Hansell, David M; Renzoni, Elisabetta; Maher, Toby M; Wells, Athol U; Polkey, Michael I; Rehal, Pauline K; Ibrahim, Wanis H; Kwong, Georges Ng Man; Colby, Thomas V; Pistolesi, Massimo; Bigazzi, Francesca; Comin, Camilla E; Nicholson, Andrew G


    Diffuse "true" cystic lung disease is rare, and the specificity of high-resolution computed tomography (HRCT) has reduced the need for biopsy. We present 5 patients with similar clinical and HRCT features of cystic lung disease that were sufficiently atypical to warrant surgical lung biopsies that showed coexistent small airway diseases (SAD). There were 4 female patients and 1 male patient with a mean age of 43 years. All were never smokers. Four had symptoms such as dyspnea (1), cough (2), or both (1). HRCTs showed variably sized thin-walled cystic airspaces without zonal distribution, some with prominent vessels in their walls. One case was unilateral. Surgical lung biopsy showed cystic change comprising localized loss of alveolar density with coexistent SADs [chronic bronchiolitis (n=2), eosinophilic bronchiolitis, probable asthma (n=1), and diffuse idiopathic neuroendocrine cell hyperplasia (n=2)]. Two patients who were tested for Birt-Hogg-Dube-related gene mutations proved negative, and all lacked other features of Birt-Hogg-Dube. We hypothesize that chronic damage to small airways may lead to cystic degeneration in a minority of patients. Precedents in relation to Sjogren syndrome and hypersensitivity pneumonitis raise the possibility of a causal association between pathologies in these 2 anatomic compartments, although HRCT data in relation to common SADs indicate that this would be a rare phenomenon. The driving factor remains unknown.

  12. Interstitial lung disease

    Diffuse parenchymal lung disease; Alveolitis; Idiopathic pulmonary pneumonitis (IPP) ... The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs expand with each ...

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

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


    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.

  14. Mixed Herbal Medicine Induced Diffuse Infiltrative Lung Disease: The HRCT and Histopathologic Findings

    Kim, Tae Gyu; Shin, Eun A [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Kim, Joung Sook [Mokdong Hospital, Ewha Womans University College of Medicine, Seoul (Korea, Republic of)


    The purpose of this study was to evaluate the high-resolution CT (HRCT) and pathologic findings of mixed herbal medicine-induced diffuse interstitial lung disease. Eight patients (6 women and 2 men, age range: 31 to 81 years, mean age: 51.4 years) who presented with cough or dyspnea after taking mixed herbal medicine were included in this study. All the patients underwent plain chest radiography and HRCT. We obtained pathologic specimens from 7 patients via fluoroscopy guided large bore cutting needle biopsy and transbronchial lung biopsy. All the patients were treated with steroid therapy. The most common HRCT finding was bilateral diffuse ground glass opacity (n=7), followed by peribronchial consolidation (n=5) and inter- or intralobular septal thickening (n=2). For the disease distribution, the lower lung zone was dominantly involved. The pathologic results of 7 patients were nonspecific interstitial pneumonia (n=3), bronchiolitis obliterans organizing pneumonia (n=2), hypersensitivity pneumonitis (n=1) and eosinophilic pneumonia (n=1). Irrespective of the pathologic results, all 8 patients improved clinically and radiologically after steroid treatment. The HRCT findings of mixed herbal medicine-induced diffuse infiltrative lung disease were mainly bilateral diffuse ground glass opacity, peribronchial consolidation and dominant involvement of the lower lung zone. Those pathologic findings were nonspecific and the differential diagnosis could include interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, hypersensitivity pneumonitis and eosinophilic pneumonia

  15. Lung disease

    ... this page: // Lung disease To use the sharing features on this page, ... fibrosis and sarcoidosis are examples of lung tissue disease. Lung circulation diseases -- These diseases affect the blood vessels ...

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

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


    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.

  17. Evaluation of three-dimensional distribution of foci of diffuse lung diseases with computed tomography

    Niwa, Masamitsu [Nagoya City Univ. (Japan). Medical School


    Three-dimensional distribution of foci of diffuse lung disease was evaluated by CT image, and it was examined whether each lung disease could be diagnosed by different distribution. Subjects were 120 cases (idiopathic interstitial pneumonia 15 cases, sarcoidosis 23 cases, miliary tuberculosis 10 cases, diffuse panbronchiolitis 7 cases, chronic pulmonary emphysema 6 cases, pulmonary metastasis 9 cases, SLE 6 cases, PSS 8 cases, dermatomyositis (DM) 6 cases, RA 7 cases, SjS 9 cases and others 14 cases). CT image was obtained from apex to base of lung with 10 mm gapless. The image was divided into three parts in both head-tail direction and back-front direction, and two parts in cortex medulla direction, and the ratio of foci to whole in each part was evaluated in five phases, and the part with the highest ratio was determined as the predominant side. In idiopathic interstitial pneumonia, sarcoidosis, miliary tuberculosis, diffuse panbronchiolitis, chronic pulmonary emphysema, pulmonary metastasis, PSS and DM, the predominant side was confirmed, respectively, and usefulness of differential diagnosis was recognized. Constant distribution wasn't recognized in SLE, RA and SjS, because the state of disease was not always same in the foci of these diseases. (K.H.)

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

    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.


    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 ...

  19. Diffuse pulmonary meningotheliomatosis: A literature review of a rare diffuse parenchymal lung disease with unclear clinical significance

    James B Gleason


    Full Text Available Introduction: Diffuse pulmonary meningotheliomatosis is a rare disease, with unclear clinical significance and very few reported cases in the literature. In this study, we review the demographics, presentation, imaging, diagnostic workup, and histologic findings of the 25 patients previously published in the literature with an outline of the disease history. Materials and Methods: We conducted a review of the literature through July 2016 for studies reporting cases of diffuse pulmonary meningotheliomatosis by searching multiple scholarly databases. Results: Of the 25 cases identified 2 were male (8%, and 23 were female (92%. Ages ranged from 37 to 73 with a median age of 59.5 years at diagnosis. 15 (60% were asymptomatic and imaging abnormalities were discovered incidentally. 8 (32% had unexplained respiratory complaints. 11 (44% had history of or active malignancy. 3 (12% were diagnosed by transbronchial biopsy while the remainder had surgical lung biopsies. Conclusion: Diffuse pulmonary meningotheliomatosis should be considered in all patients with diffuse bilateral pulmonary nodules on HRCT. The condition is more prevalent in females and its clinical significance is unclear, although nearly half of those diagnosed had a history of malignancy. CT imaging and surgical lung biopsy are the modalities of choice for diagnosis but transbronchial biopsies have recently been used obtain the diagnosis. Additional research needs to be done to further characterize the nature of this condition and the clinical scenarios in which is presents.

  20. [Diffuse infiltrative lung disease in scleroderma. Analysis of radio-clinical and functional semiology].

    El Khattabi, W; Afif, H; Moussali, N; Aichane, A; Abdelouafi, A; Bouayad, Z


    Scleroderma (SD) is a systemic disease that predominantly affects the skin. Diffuse infiltrative lung disease (DILD) is rare and occurs most often in the course of the disease. We analyzed seven cases of DILO of SD recorded between 2003 and 2010 among 196 PID (3.6%). Functional signs were limited to respiratory dyspnea, it was associated to dysphagia in six cases, dry syndrome in five cases and Raynaud's phenomenon in four cases. Clinical examination found crackles in the bases of the thorax in all cases and specific cutaneous signs in six cases. The chest radiograph showed that interstitial disease predominates at the lung bases in all cases with a large aspect of the pulmonary arteries in two cases. The chest CT scan confirmed the predominance of basal and peripheral damage with signs of fibrosis in six cases. The pulmonary function objectified a severe restrictive ventilatory defect in all cases. Bronchoscopy showed a normal macroscopic appearance in all cases, the broncho-alveolar lavage was predominated by neutrophilic formula in four cases. SCL 70 antibodies were positive in four cases. All patients were treated by steroids with improvement of dyspnea and stabilization of radiographs. A patient had died in an array of acute respiratory failure and one patient was lost to follow-up. DILD in scleroderma is rare and seldom reveals the disease, it affects the patient's prognosis especially when associated with arterial pulmonary hypertension. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  1. Lung Diseases

    When you breathe, your lungs take in oxygen from the air and deliver it to the bloodstream. The cells in your body need oxygen to ... you breathe nearly 25,000 times. People with lung disease have difficulty breathing. Millions of people in ...

  2. Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease

    Guttentag Susan


    Full Text Available Abstract Background Abnormalities of the intracellular metabolism of the hydrophobic surfactant proteins SP-B and SP-C and their precursors may be causally linked to chronic childhood diffuse lung diseases. The profile of these proteins in the alveolar space is unknown in such subjects. Methods We analyzed bronchoalveolar lavage fluid by Western blotting for SP-B, SP-C and their proforms in children with pulmonary alveolar proteinosis (PAP, n = 15, children with no SP-B (n = 6, children with chronic respiratory distress of unknown cause (cRD, n = 7, in comparison to children without lung disease (n = 15 or chronic obstructive bronchitis (n = 19. Results Pro-SP-B of 25–26 kD was commonly abundant in all groups of subjects, suggesting that their presence is not of diagnostic value for processing defects. In contrast, pro-SP-B peptides cleaved off during intracellular processing of SP-B and smaller than 19–21 kD, were exclusively found in PAP and cRD. In 4 of 6 children with no SP-B, mutations of SFTPB or SPTPC genes were found. Pro-SP-C forms were identified at very low frequency. Their presence was clearly, but not exclusively associated with mutations of the SFTPB and SPTPC genes, impeding their usage as candidates for diagnostic screening. Conclusion Immuno-analysis of the hydrophobic surfactant proteins and their precursor forms in bronchoalveolar lavage is minimally invasive and can give valuable clues for the involvement of processing abnormalities in pediatric pulmonary disorders.

  3. Evaluation of diffusing capacity in patients with a restrictive lung disease

    H. Stam (Hendrik); T.A.W. Splinter (Ted); A. Versprille (Adrian)


    textabstractBACKGROUND: In healthy volunteers, the single-breath diffusing capacity of the lung for carbon monoxide (DLCO) decreases and DLCO normalized per liter alveolar volume (VA; DLCO/VA) increases if VA is decreased. We hypothesized that comparison of DLCO/VA with

  4. A sparse representation based method to classify pulmonary patterns of diffuse lung diseases.

    Zhao, Wei; Xu, Rui; Hirano, Yasushi; Tachibana, Rie; Kido, Shoji


    We applied and optimized the sparse representation (SR) approaches in the computer-aided diagnosis (CAD) to classify normal tissues and five kinds of diffuse lung disease (DLD) patterns: consolidation, ground-glass opacity, honeycombing, emphysema, and nodule. By using the K-SVD which is based on the singular value decomposition (SVD) and orthogonal matching pursuit (OMP), it can achieve a satisfied recognition rate, but too much time was spent in the experiment. To reduce the runtime of the method, the K-Means algorithm was substituted for the K-SVD, and the OMP was simplified by searching the desired atoms at one time (OMP1). We proposed three SR based methods for evaluation: SR1 (K-SVD+OMP), SR2 (K-Means+OMP), and SR3 (K-Means+OMP1). 1161 volumes of interest (VOIs) were used to optimize the parameters and train each method, and 1049 VOIs were adopted to evaluate the performances of the methods. The SR based methods were powerful to recognize the DLD patterns (SR1: 96.1%, SR2: 95.6%, SR3: 96.4%) and significantly better than the baseline methods. Furthermore, when the K-Means and OMP1 were applied, the runtime of the SR based methods can be reduced by 98.2% and 55.2%, respectively. Therefore, we thought that the method using the K-Means and OMP1 (SR3) was efficient for the CAD of the DLDs.

  5. An adaptive knowledge-driven medical image search engine for interactive diffuse parenchymal lung disease quantification

    Tao, Yimo; Zhou, Xiang Sean; Bi, Jinbo; Jerebkoa, Anna; Wolf, Matthias; Salganicoff, Marcos; Krishnana, Arun


    Characterization and quantification of the severity of diffuse parenchymal lung diseases (DPLDs) using Computed Tomography (CT) is an important issue in clinical research. Recently, several classification-based computer-aided diagnosis (CAD) systems [1-3] for DPLD have been proposed. For some of those systems, a degradation of performance [2] was reported on unseen data because of considerable inter-patient variances of parenchymal tissue patterns. We believe that a CAD system of real clinical value should be robust to inter-patient variances and be able to classify unseen cases online more effectively. In this work, we have developed a novel adaptive knowledge-driven CT image search engine that combines offline learning aspects of classification-based CAD systems with online learning aspects of content-based image retrieval (CBIR) systems. Our system can seamlessly and adaptively fuse offline accumulated knowledge with online feedback, leading to an improved online performance in detecting DPLD in both accuracy and speed aspects. Our contribution lies in: (1) newly developed 3D texture-based and morphology-based features; (2) a multi-class offline feature selection method; and, (3) a novel image search engine framework for detecting DPLD. Very promising results have been obtained on a small test set.

  6. Novel algorithm to identify and differentiate specific digital signature of breath sound in patients with diffuse parenchymal lung disease.

    Bhattacharyya, Parthasarathi; Mondal, Ashok; Dey, Rana; Saha, Dipanjan; Saha, Goutam


    Auscultation is an important part of the clinical examination of different lung diseases. Objective analysis of lung sounds based on underlying characteristics and its subsequent automatic interpretations may help a clinical practice. We collected the breath sounds from 8 normal subjects and 20 diffuse parenchymal lung disease (DPLD) patients using a newly developed instrument and then filtered off the heart sounds using a novel technology. The collected sounds were thereafter analysed digitally on several characteristics as dynamical complexity, texture information and regularity index to find and define their unique digital signatures for differentiating normality and abnormality. For convenience of testing, these characteristic signatures of normal and DPLD lung sounds were transformed into coloured visual representations. The predictive power of these images has been validated by six independent observers that include three physicians. The proposed method gives a classification accuracy of 100% for composite features for both the normal as well as lung sound signals from DPLD patients. When tested by independent observers on the visually transformed images, the positive predictive value to diagnose the normality and DPLD remained 100%. The lung sounds from the normal and DPLD subjects could be differentiated and expressed according to their digital signatures. On visual transformation to coloured images, they retain 100% predictive power. This technique may assist physicians to diagnose DPLD from visual images bearing the digital signature of the condition. © 2015 Asian Pacific Society of Respirology.

  7. Rheumatoid lung disease

    Lung disease - rheumatoid arthritis; Rheumatoid nodules; Rheumatoid lung ... They often cause no symptoms. The cause of lung disease associated with rheumatoid arthritis is unknown. Sometimes, the ...

  8. Effect of yoga regimen on lung functions including diffusion capacity in coronary artery disease patients: A randomized controlled study.

    Yadav, Asha; Singh, Savita; Singh, Kp; Pai, Preeti


    Lung functions are found to be impaired in coronary artery disease (CAD), congestive heart failure, left ventricular dysfunction, and after cardiac surgery. Diffusion capacity progressively worsens as the severity of CAD increases due to reduction in lung tissue participating in gas exchange. Pranayama breathing exercises and yogic postures may play an impressive role in improving cardio-respiratory efficiency and facilitating gas diffusion at the alveolo-capillary membrane. This study was done to see the effect of yoga regimen on lung functions particularly diffusion capacity in CAD patients. A total of 80 stable CAD patients below 65 years of age of both sexes were selected and randomized into two groups of 40 each. Group I CAD patients were given yoga regimen for 3 months which consisted of yogic postures, pranayama breathing exercises, dietary modification, and holistic teaching along with their conventional medicine while Group II CAD patients were put only on conventional medicine. Lung functions including diffusion capacity were recorded thrice in both the groups: 0 day as baseline, 22(nd) day and on 90(th) day by using computerized MS medisoft Cardio-respiratory Instrument, HYP'AIR Compact model of cardio-respiratory testing machine was manufactured by P K Morgan, India. The recorded parameters were statistically analyzed by repeated measures ANOVA followed by Tukey's test in both the groups. Cardiovascular parameters were also compared before and after intervention in both the groups. Statistically significant improvements were seen in slow vital capacity, forced vital capacity, peak expiratory flow rate, maximum voluntary ventilation, and diffusion factor/ transfer factor of lung for carbon monoxide after 3 months of yoga regimen in Group I. Forced expiratory volume in 1(st) sec (FEV1), and FEV1 % also showed a trend toward improvement although not statistically significant. HR, SBP and DBP also showed significant improvement in Group-I patients who

  9. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study

    Walsh, Simon L F; Wells, Athol U; Desai, Sujal R;


    . This difference is of particular importance, because accurate and consistent diagnoses of IPF are needed if clinical outcomes are to be optimised. Inter-multidisciplinary team agreement for a diagnosis of hypersensitivity pneumonitis is low, highlighting an urgent need for standardised diagnostic guidelines......-multidisciplinary team agreement for the diagnosis of diffuse parenchymal lung disease. METHODS: We did a multicentre evaluation of clinical data of patients who presented to the interstitial lung disease unit of the Royal Brompton and Harefield NHS Foundation Trust (London, UK; host institution) and required...... multidisciplinary team meeting (MDTM) characterisation between March 1, 2010, and Aug 31, 2010. Only patients whose baseline clinical, radiological, and, if biopsy was taken, pathological data were undertaken at the host institution were included. Seven MDTMs, consisting of at least one clinician, radiologist...

  10. Cryobiopsy in the diagnosis of diffuse interstitial lung disease: yield and cost-effectiveness analysis.

    Hernández-González, Fernanda; Lucena, Carmen M; Ramírez, José; Sánchez, Marcelo; Jimenez, María José; Xaubet, Antoni; Sellares, Jacobo; Agustí, Carlos


    Assessment of patients with suspected interstitial lung disease (ILD) includes surgical lung biopsy (SLB) when clinical and radiological data are inconclusive. However, cryobiopsy is acquiring an important role in the ILD diagnostic process. The objective of this study was to evaluate the diagnostic yield, safety and economic costs of the systematic use of cryobiopsy in the assessment of patients with suspected ILD. This was a retrospective observational study of patients who had undergone transbronchial cryobiopsy for evaluation of ILD from January 2011 to January 2014. The procedures were performed with a video bronchoscope using a cryoprobe for the collection of lung parenchyma specimens, which were analyzed by pathologists. Diagnostic yield, complications and economic costs of this technique were analyzed. Criobiopsy specimens from a total of 33 patients were included. A specific diagnosis was obtained in 26, producing a diagnostic yield of 79%. In 5 patients, SLB was required for a histopathological confirmation of disease, but the procedure could not be performed in 4, due to severe comorbidities. The most frequent complications were pneumothorax (12%) and gradei (9%) or gradeii (21%) bleeding. There were no life-threatening complications. The systematic use of cryobiopsy saved up to €59,846. Cryobiopsy is a safe and potentially useful technique in the diagnostic assessment of patients with ILD. Furthermore, the systematic use of cryobiopsy has an important economic impact. Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.

  11. Anxiety and depression among patients with Bronchial asthma, chronic obstructive pulmonary disease and diffuse parenchymatous lung diseases

    Taghreed S. Farag*, Manal R. Hafez*, Taghreed Elshafie**and Omaima I. Abo-Elkheir


    Full Text Available Background: Psychosocial stressors, such as death of a spouse or divorce are closely related to relapses and aggravations of respiratory disease pointing to a link between psychological factors and chronic pulmonary disease. Patients with chronic obstructive pulmonary disease (COPD cannot cope adequately with everyday needs. This inadequacy may lead to heightened anxiety and depression, which in turn may worsen the everyday inadequacy. It has been reported that this is probably a factor that leads bronchial asthma (BA and COPD patients to frequent hospital admissions, lower treatment adherence and even intensive care unit hospitalizations.Study objectives: to assess anxiety and depressive symptoms among a sample of Egyptian patients with chronic obstructive pulmonary disease (COPD, bronchial asthma (BA, and diffuse parenchymatous lung diseases (DPLD.Subjects & methods: A cross-sectional study conducted over a one year period on a sample of 258 Egyptian patients with chronic breathing disorders who attending the outpatient chest clinic at Al-Zahraa University Hospital. All patients diagnosed as having COPD, BA or DPLD were enrolled into the study. Anxiety and depression were assessed by using the Hospital anxiety and depression scale (HADS and the Beck Depression Inventory (BDI.Results: Psychiatric disorders were most prevalent among DPLD cases (80.0%, followed by COPD patients (74.0%, while BA group showed the least reported cases (38.8% with psychiatric disorders, with a statistically significant difference among the studied groups (P-value < 0.05.Depression was prevalent among 60.1% (155/258 of the studied cases, of them 23.2% had severe depression, followed by 38.7 % had moderate depression and 38.1% had mild depression. DPLD cases had the highest proportion of severe depression (31.8, COPD cases had the highest proportion of moderate depression (52.4 and BA group had the highest proportion of mild depression (68.4%, with a statistically

  12. Reflux and Lung Disease

    ... Healthy Eating Reflux and Lung Disease Reflux and Lung Disease Make an Appointment Ask a Question Find a Doctor Many people with chronic lung disease also suffer from gastroesophageal reflux (GERD). In this ...

  13. Effect of yoga regimen on lung functions including diffusion capacity in coronary artery disease patients: A randomized controlled study

    Asha Yadav


    Conclusions: Yoga regimen was found to improve lung functions and diffusion capacity in CAD patients besides improving cardiovascular functions. Thus, it can be used as a complimentary or adjunct therapy along with the conventional medicine for their treatment and rehabilitation.

  14. Effect and mechanism of acute graft versus host disease on early diffuse murine lung injury following allogeneic stem cell transplantation


    To explore the effect and pathogenssis of acute graft-versus-host disease (aGVHD) on early diffuse lung injury in allogeneic hematopoietic stem cell transplantation (allo-HSCT), we established an aGVHD model of C57BL/6→BALB/c mice. Chest computed tomography (CT) scans, histopathology and the levels of cytokines including tumor necrosis factor α (TNFα) and Interferon (IFNγ) in lungs were dynamically detected in recipient mice after transplantation. The incidence of aGVHD was respectively 0%, 0% and 100% in simple irradiation group (A), syngeneic transplant group(B) and allogeneic transplant group (C). Chest CT scans of recipient mice were normal in 3 groups on days +3 and +7 after transplantation. CT showed that two of ten mice had bilateral lung diffuse infiltrate on day +12 (on the brink of death) in group A and 6 of 10 mice had bilateral lung diffuse infiltrate on day +14 (3 d after aGVHD occurring) in group C, and were normal on days +12 and +14 in group B after transplantation. Histopathology of lungs in the 3 groups was similar, consisting of minor interstitial pneumonitis on day +3. Group A showed edema, hyperplasia of epithelial cells and widened alveolar interval on day +7, and epithelial cell necrosis, lymphocyte infiltration, hemorrhage, protein leakage, and local consolidation on day +12. The histopathology of group B showed slight edema of epithelial cells on +7 day, which were slighter than that on day +3, and virtually normal on day +14. The histopathology in group C was characterized by the significant expansion and congestion of capillaries, and lymphocyte infiltration on day +7, the acute pneumonitis was present involving tissue edema, lymphocyte and macrophage infiltration, protein leakage and perivascular inflammation on day +14. In group A, the levels of TNFα were lower on day +7 than on day +3. In group B, the levels of TNFα attained a peak on day +3, which decreased on days +7 and +14. In group C, the levels of TNFα were highest on day

  15. 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



    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.

  16. Assessment of pathologically diagnosed patients with Castleman's disease associated with diffuse parenchymal lung involvement using the diagnostic criteria for IgG4-related disease.

    Ogoshi, Takaaki; Kido, Takashi; Yatera, Kazuhiro; Oda, Keishi; Kawanami, Toshinori; Ishimoto, Hiroshi; Sakamoto, Noriho; Sano, Arisa; Yoshii, Chiharu; Shimajiri, Shohei; Mukae, Hiroshi


    IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman's disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement. We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD). We also compared the clinical and laboratory findings observed in these patients. Six of the eight patients exhibited abundant IgG4-positive plasmacytes in the lung and lymph node tissues and elevated serum IgG4 levels, thereby fulfilling the diagnostic criteria of IgG4RD with DL (DL-IgG4RD) in addition to having obstructive phlebitis and massive lymphoplasmacytic infiltration with fibrosis. However, three of these six patients exhibited higher levels of serum interleukin-6 and were still diagnosed with DL-PCD. Accordingly, three of these eight patients were considered as IgG4RD with DL (DL-IgG4RD), and the other five patients were ultimately given a diagnosis of DL-PCD. These two diseases have different characteristics in terms of age, symptoms, serum levels of C-reactive protein, and IgA, complicating allergic disorders, response to corticosteroids, and prognosis. This is the first report to show a high prevalence of DL-IgG4RD in DL-PCD patients, although additional large investigations are necessary. Clinical and laboratory findings are important for distinguishing between these two diseases in other organs, as previously described.

  17. Feasibility of Automated Quantification of Regional Disease Patterns Depicted on High-Resolution Computed Tomography in Patients with Various Diffuse Lung Diseases

    Park, Sang Ok; Seo, Joon Beom; Kim, Nam Kug; Park, Bum Woo; Sung, Yu Sub; Lee, Young Joo [Asan Medical Center, Seoul (Korea, Republic of); Park, Seong Hoon [Wonkwang University Hospital, Iksan (Korea, Republic of); Lee, Young Kyung [East-West Neo Medical Center of Kyunghee University, Seoul (Korea, Republic of); Lee, Jeong Jin [The Catholic University of Korea, Seoul (Korea, Republic of); Kang, Suk Ho [Seoul National University, Seoul (Korea, Republic of)


    This study was designed to develop an automated system for quantification of various regional disease patterns of diffuse lung diseases as depicted on high-resolution computed tomography (HRCT) and to compare the performance of the automated system with human readers. A total of 600 circular regions-of-interest (ROIs), 10 pixels in diameter, were utilized. The 600 ROIs comprised 100 ROIs that represented six typical regional patterns (normal, ground-glass opacity, reticular opacity, honeycombing, emphysema, and consolidation). The ROIs were used to train the automated classification system based on the use of a Support Vector Machine classifier and 37 features of texture and shape. The performance of the classification system was tested with a 5-fold cross-validation method. An automated quantification system was developed with a moving ROI in the lung area, which helped classify each pixel into six categories. A total of 92 HRCT images obtained from patients with different diseases were used to validate the quantification system. Two radiologists independently classified lung areas of the same CT images into six patterns using the manual drawing function of dedicated software. Agreement between the automated system and the readers and between the two individual readers was assessed. The overall accuracy of the system to classify each disease pattern based on the typical ROIs was 89%. When the quantification results were examined, the average agreement between the system and each radiologist was 52% and 49%, respectively. The agreement between the two radiologists was 67%. An automated quantification system for various regional patterns of diffuse interstitial lung diseases can be used for objective and reproducible assessment of disease severity.

  18. Interstitial Lung Diseases

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

  19. TGF-β1 in bronchoalveolar lavage fluid in diffuse parenchymal lung diseases and high-resolution computed tomography score

    Artur Szlubowski


    Full Text Available INTRODUCTION: In the pathogenesis of diffuse parenchymal lung diseases (DPLDs, growth factors, including transforming growth factor β1 (TGF-β1, are responsible for cell proliferation, apoptosis, chemotaxis, and angiogenesis, and also for the production and secretion of some components of the extracellular matrix. OBJECTIVES: The aim of the study was to evaluate correlations in DPLDs between TGF-β1 levels in bronchoalveolar lavage (BAL fluid and high-resolution computed tomography (HRCT score. PATIENTS AND METHODS: The study was performed in 31 DPLD patients in whom a selection of lung segments with high and low intensity of abnormalities was estimated by HRCT score. All patients underwent BAL with TGF-β1 measured by an enzyme immunoassay in BAL fluid and video-assisted thoracic surgery lung biopsy from both selected segments. RESULTS: All 31 patients were diagnosed, and based on histopathology, they were classified into 2 groups: idiopathic interstitial pneumonia (usual interstitial pneumonia – 12, nonspecific interstitialpneumonia – 2, cryptogenic organizing pneumonia – 2, and desquamative interstitial pneumonia – 1 and granulomatous disease (sarcoidosis – 7, extrinsic allergic alveolitis – 5, and histiocytosis X – 2. The final analysis was performed in 28 patients who showed nonhomogenous distribution on HRCT. TGF-β1 levels in BAL fluid were significantly higher in the areas with high intensity of abnormalities assessed by HRCT score (P = 0.018, analysis of variance. These levels were not different between the groups, but a trend towards higher levels in idiopathic interstitial pneumonia was observed. CONCLUSIONS: The results confirm that TGF-β1 may be a good but not specific marker of fibrosis in DPLDs. A significant positive correlation between TGF-β1 levels in BAL fluid and the HRCT score was observed.

  20. Diffusion capacity of the lung for carbon monoxide - A potential marker of impaired gas exchange or of systemic deconditioning in chronic obstructive lung disease?

    Weinreich, Ulla Møller; Thomsen, Lars Pilegaard; Brock, Christina; Karbing, Dan Stieper; Rees, Stephen Edward


    Gas exchange impairment is primarily caused by ventilation-perfusion mismatch in chronic obstructive pulmonary disease (COPD), where diffusing capacity of the lungs for carbon monoxide (DLCO) remains the clinical measure. This study investigates whether DLCO: (1) can predict respiratory impairment in COPD, that is, changes in oxygen and carbon dioxide (CO2); (2) is associated with combined risk assessment score for COPD (Global Initiative for Chronic Obstructive Lung Disease (GOLD) score); and (3) is associated with blood glucose and body mass index (BMI). Fifty patients were included retrospectively. DLCO; arterial blood gas at inspired oxygen (FiO2) = 0.21; oxygen saturation (SpO2) at FiO2 = 0.21 (SpO2 (21)) and FiO2 = 0.15 (SpO2 (15)) were registered. Difference between arterial and end-tidal CO2 (ΔCO2) was calculated. COPD severity was stratified according to GOLD score. The association between DLCO, SpO2, ΔCO2, GOLD score, blood glucose, and BMI was investigated. Multiple regression showed association between DLCO and GOLD score, BMI, and glucose level (R (2) = 0.6, p < 0.0001). Linear and multiple regression showed an association between DLCO and SpO2 (21) (R (2) = 0.3, p = 0.001 and p = 0.03, respectively) without contribution from SpO2 (15) or ΔCO2. A stronger association between DLCO and GOLD score than between DLCO and SpO2 could indicate that DLCO is more descriptive of systemic deconditioning than gas exchange in COPD patients. However, further larger studies are needed. A weaker association is seen between DLCO and SpO2 (21) without contribution from SpO2 (15) and ΔCO2. This could indicate that DLCO is more descriptive of systemic deconditioning than gas exchange in COPD patients. However, further larger studies are needed.

  1. Reader Accuracy and Confidence in Diagnosing Diffuse Lung Disease on High-Resolution Computed Tomography of the Lungs: Impact of Sampling Frequency

    Sundaram, B.; Gross, B.H.; Oh, E.; Mueller, N.; Myles, J.D.; Kazerooni, E.A. (Dept. of Radiology, Michigan Institute for Clinical Health Research, Univ. of Michigan Health System, Ann Arbor, Michigan (United States))


    Background: The accuracy of the number of high-resolution computed tomography (HRCT) images necessary to diagnose diffuse lung disease (DLD) is not well established. Purpose: To evaluate the impact of HRCT sampling frequency on reader confidence and accuracy for diagnosing DLD. Material and Methods: HRCT images of 100 consecutive patients with proven DLD were reviewed. They were: 48 usual interstitial pneumonia, 22 sarcoidosis, six hypersensitivity pneumonitis, five each of desquamative interstitial pneumonitis, eosinophilic granulomatosis, and lymphangioleiomyomatosis, and nine others. Inspiratory images at 1-cm increments throughout the lungs and three specified levels formed complete and limited examinations. In random order, three experts (readers 1, 2, and 3) ranked their top three diagnoses and rated confidence for their top diagnosis, independently and blinded to clinical information. Results: Using the complete versus limited examinations for correct first-choice diagnosis, accuracy for reader 1 (R1) was 81% versus 80%, respectively, for reader 2 (R2) 70% versus 70%, and for reader 3 (R3) 64% versus 59%. Reader accuracy within their top three choices for complete versus limited examinations was: R1 91% versus 91% of cases, respectively, R2 84% versus 83%, and R3 79% versus 72% of cases. No statistically significant differences were found between the diagnosis methods (P=0.28 for first diagnosis and P=0.17 for top three choices). The confidence intervals for individual raters showed considerable overlap, and the point estimates are almost identical. The mean interreader agreement for complete versus limited HRCT for both top and top three diagnoses were the same (moderate and fair, respectively). The mean intrareader agreement between complete and limited HRCT for top and top three diagnoses were substantial and moderate, respectively. Conclusion: Overall reader accuracy and confidence in diagnosis did not significantly differ when fewer or more HRCT images

  2. A 22-Year-Old Nonsmoker With Diffuse Cystic Lung Disease.

    Foust, Kimber; Albores, Jeffrey; Fishbein, Gregory A; Genshaft, Scott; Wang, Tisha


    A 22-year-old previously healthy woman was evaluated in pulmonary clinic for shortness of breath and cough that had been slowly progressive over 3 months. She otherwise reported being fully functional and attended her college graduation a week prior to evaluation. She had no history of smoking, illicit drug use, connective tissue disease, or noxious exposures.

  3. The impact of iterative reconstruction in low-dose computed tomography on the evaluation of diffuse interstitial lung disease

    Lim, Hyun Ju; Chung, Myung Jin; Shin, Kyung Eun; Hwang, Hye Sun; Lee, Kyung Soo [Dept. of Radiology nd Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)


    To evaluate the impact of iterative reconstruction (IR) on the assessment of diffuse interstitial lung disease (DILD) using CT. An American College of Radiology (ACR) phantom (module 4 to assess spatial resolution) was scanned with 10-100 effective mAs at 120 kVp. The images were reconstructed using filtered back projection (FBP), adaptive statistical iterative reconstruction (ASIR), with blending ratios of 0%, 30%, 70% and 100%, and model-based iterative reconstruction (MBIR), and their spatial resolution was objectively assessed by the line pair structure method. The patient study was based on retrospective interpretation of prospectively acquired data, and it was approved by the institutional review board. Chest CT scans of 23 patients (mean age 64 years) were performed at 120 kVp using 1) standard dose protocol applying 142-275 mA with dose modulation (high-resolution computed tomography [HRCT]) and 2) low-dose protocol applying 20 mA (low dose CT, LDCT). HRCT images were reconstructed with FBP, and LDCT images were reconstructed using FBP, ASIR, and MBIR. Matching images were randomized and independently reviewed by chest radiologists. Subjective assessment of disease presence and radiological diagnosis was made on a 10-point scale. In addition, semi-quantitative results were compared for the extent of abnormalities estimated to the nearest 5% of parenchymal involvement. In the phantom study, ASIR was comparable to FBP in terms of spatial resolution. However, for MBIR, the spatial resolution was greatly decreased under 10 mA. In the patient study, the detection of the presence of disease was not significantly different. The values for area under the curve for detection of DILD by HRCT, FBP, ASIR, and MBIR were as follows: 0.978, 0.979, 0.972, and 0.963. LDCT images reconstructed with FBP, ASIR, and MBIR tended to underestimate reticular or honeycombing opacities (-2.8%, -4.1%, and -5.3%, respectively) and overestimate ground glass opacities (+4.6%, +8.9%, and

  4. Lung Diseases and Conditions

    ... Share this page from the NHLBI on Twitter. Lung Diseases and Conditions Breathing is a complex process. If ... to a disease called COPD (chronic obstructive pulmonary disease). COPD prevents proper airflow in and out of your lungs and can hinder gas exchange in the air ...

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

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


    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.

  6. Diagnostic value of diffuse and peripheral lung lesions by transbronchial lung biopsy

    Shi Zhihong; Wei Xia; Wei Xiaohong; Zhu Bo


    Objective To evaluate the diagnostic value of transbronchial lung biopsy (TBLB) via bronchoscope for lung diffuse lesions and peripheral pulmonary lesions. Methods The results of TBLB were retrospectively analyzed from 256 diagnoses of unknown patients with lung diffuse lesions or peripheral lung lesions. Results Eighty-nine final diagnosis cases via TBLB only once, the diagnostic rate of double lung diffuse lesions was the highest, [51.06% (24/47)], local non-nodule lesions, nodule lesions, cavity lesions were 33.33%(23/69), 29.91%(35/117), and 28.57%(4/14), respectively. Among 24 diagnostic cases of double lung diffuse lesions, there were 6 cases of pneumonia of pulmonary tuberculosis, 9 cases of lung carcinoma, 7 cases of adenecarcinoma; 6 cases of pneumonia, 2 cases of fungous infection, and 1 case of lung hemosiderusis. The diagnostic yield about 58 cases of local lesions was low, among which non-nodule lesions accounted for 33.33% and nodule lesions accounted for 29.91%. In 89 cases of peripheral lung lesions with histological diagnosis, lung carcinoma and pulmonary tuberculosis were frequent diseases, which accounted for 86.52%. Conclusion TBLB is a reliable, safe, effective and repeatable operation method in diagnosis of lung diffuse lesions and peripheral pulmonary lesions.

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

    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)


    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.)

  8. Cyclophosphamide in diffuse lung damage.

    Musiatowicz, B; Sulkowska, M; Sulik, M; Famulski, W; Dziecioł, J; Sobaniec-Lotowska, M; Baltaziak, M; Arciuch, L; Rółkowski, R; Jabłońska, E


    Some cyclophosphamide toxic effects on lung tissue are presented. Cyclophosphamide metabolism, pathogenesis of lung damage and morphological lung tissue changes caused by that agent were characterized. Attention was focused on BAL evaluation as a useful method in the monitoring of lung tissue damage degree.

  9. Diffusion and perfusion MRI of the lung and mediastinum

    Henzler, Thomas, E-mail: [Institute of Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Medical Faculty Mannheim - Heidelberg University (Germany); Schmid-Bindert, Gerald [Interdisciplinary Thoracic Oncology, University Medical Center Mannheim, Medical Faculty Mannheim - Heidelberg University (Germany); Schoenberg, Stefan O.; Fink, Christian [Institute of Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Medical Faculty Mannheim - Heidelberg University (Germany)


    With ongoing technical improvements such as multichannel MRI, systems with powerful gradients as well as the development of innovative pulse sequence techniques implementing parallel imaging, MRI has now entered the stage of a radiation-free alternative to computed tomography (CT) for chest imaging in clinical practice. Whereas in the past MRI of the lung was focused on morphological aspects, current MRI techniques also enable functional imaging of the lung allowing for a comprehensive assessment of lung disease in a single MRI exam. Perfusion imaging can be used for the visualization of regional pulmonary perfusion in patients with different lung diseases such as lung cancer, chronic obstructive lung disease, pulmonary embolism or for the prediction of postoperative lung function in lung cancer patients. Over the past years diffusion-weighted MR imaging (DW-MRI) of the thorax has become feasible with a significant reduction of the acquisition time, thus minimizing artifacts from respiratory and cardiac motion. In chest imaging, DW-MRI has been mainly suggested for the characterization of lung cancer, lymph nodes and pulmonary metastases. In this review article recent MR perfusion and diffusion techniques of the lung and mediastinum as well as their clinical applications are reviewed.

  10. Multiple cystic lung disease

    Flavia Angélica Ferreira Francisco


    Full Text Available Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt–Hogg–Dubé; other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.

  11. 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

    袁新宇; 叶滨宾; 陈慧中


    @@ 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.

  12. 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

    龙雄; 郭树彬; 邓春涛; 杜铁宽


    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

  13. CT引导下经皮肺穿刺在肺弥漫性病变中诊断价值%Diagnostic value of CT guided percutaneous lung biopsy in diffuse lung diseases

    宋新宇; 陈世雄; 曾凡军; 高宝安


    目的 评价CT引导下经皮肺穿刺在肺弥漫性病变中的诊断价值.方法 应用16 GBARD活检针穿刺32例肺部弥漫性病变患者,主要影像学改变包括弥漫性网格样结节或结节影,网状线形阴影和弥漫性磨玻璃影.结果 32例患者均取材成功,并获得明确诊断,10例恶性肿瘤,(其中5例肺泡癌,5例肺癌肺内转移),22例良性病变(7例粟粒性肺结核,7例间质性肺炎,2例肺泡蛋白沉积征,2例肺真菌病,1例闭塞性支气管炎伴机化性肺炎,1例肺结节病,1例过敏性肺炎,1例弥漫性泛细支气管炎).主要并发症是气胸(22.5%)和出血(21.9%).结论 CT引导下经皮肺穿刺是一种实用、安全、并发症低的技术,对弥漫性肺疾病有很高的确诊率.%Objective To assess the diagnostic value of CT-guided percutaneous lung biopsy in diffuse lung diseases. Methods CT-guided percutaneous lung biopsy was performed in 32 patients with diffuse lung diseases by 16G BARD biopsy needle. The main imaging changes of these patients included network of diffuse nodular or nodular, diffuse reticular lines shadow and diffuse ground-glass density in the lungs. Results Punctures were successful in all 32 patients, and the diseases were clearly diagnosed, which included 10 patients with malignant tumor ( 5 cases of bronchioloalveolar carcinoma and 5 cases of metastatic carcimoma ) and 22 patients with benign tumor ( 7 cases of disseminated pulmonary tuberculosis, 7 cases of interstitial pneumonia, 2 cases of pulmonary alveolar proteinosis, 2 cases of pulmonary mycoses, 1 case of hronchiolitis obliterans with organizing pneumonia, 1 case of sarcoidosis of lung, 1 case of allergic pneumonia and 1 diffuse panbronchiolitis ). The major complications of puncture were pneumothorax ( 22. 5% ) and bleeding ( 21. 9% ). Conclusion CT-guided percutaneous lung biopsy is a useful and safe technique with low complication and high accuracy rate in the diagnosis of patients with diffuse lung

  14. Interstitial lung disease


    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

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

    Jones, Kirk D; Urisman, Anatoly


    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.

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

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


    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.

  17. Inhalational Lung Disease

    S Kowsarian


    Full Text Available Inhalational lung diseases are among the most important occupational diseases. Pneumoconiosis refers to a group of lung diseases result from inhalation of usually inorganic dusts such as silicon dioxide, asbestos, coal, etc., and their deposition in the lungs. The resultant pulmonary disorders depend on the susceptibility of lungs; size, concentration, solubility and fibrogenic properties of the inhaled particles; and duration of exposure. Radiographic manifestations of pneumoconiosis become apparent several years after exposure to the particles. However, for certain types of dusts, e.g., silicone dioxide crystal and beryllium, heavy exposure within a short period can cause an acute disease. Pulmonary involvement in asbestosis is usually in the lower lobes. On the contrary, in silicosis and coal worker pneumoconiosis, the upper lobes are involved predominantly. For imaging evaluation of pneumoconiosis, high-resolution computed tomography (CT is superior to conventional chest x-ray. Magnetic resonance imaging (MRI and positron emission tomography (PET scan are helpful in those with suspected tumoral lesions. In this essay, we reviewed the imaging aspects of inhalational lung disease.

  18. Cough in interstitial lung disease.

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


    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.

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

    Schaefer-Prokop, C.M.


    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

  20. Preclinical lung disease in early rheumatoid arthritis.

    Robles-Perez, Alejandro; Luburich, Patricio; Rodriguez-Sanchon, Benigno; Dorca, Jordi; Nolla, Joan Miquel; Molina-Molina, Maria; Narvaez-Garcia, Javier


    Early detection and treatment of lung disease in patients with rheumatoid arthritis (RA) may ameliorate disease progression. The objectives of this study were to investigate the frequency of asymptomatic lung abnormalities in early RA patients and the potential association of positive RA blood reactive biomolecules with lung involvement. A prospective observational study was performed in a cohort of patients with early RA (joint symptoms disease with a baseline chest radiograph (CR) and complete pulmonary function tests (PFTs). In those patients with lung abnormalities on the CR or PFTs, a high-resolution chest computed tomography scan (HRCT) was performed. We included 40 patients (30 women). Altered PFTs were detected in 18 (45%) of these patients. These cases had a diffusion lung transfer capacity of carbon monoxide (DLCO) of disease is present in up to 45% of early RA patients and can be determined by PFTs and ACPA levels.

  1. Flavorings-Related Lung Disease

    ... and Products Programs Contact NIOSH NIOSH FLAVORINGS-RELATED LUNG DISEASE Recommend on Facebook Tweet Share Compartir On this ... practices that place workers at risk. Flavorings-Related Lung Disease Microwave popcorn plant and flavoring plant workers have ...

  2. Immunologic lung disease

    Harman, E.M.


    The term immunologic lung disease comprises a broad spectrum of disease. The authors have covered a few entities in which recent studies have been particularly helpful in elucidating pathophysiology though not in uncovering the inciting cause. Common to all of these entities is the problem of finding appropriate methods of defining disease activity and response to treatment. As exemplified by the improved outlook for Goodpasture's syndrome with elucidation of its underlying immunopathology, it is likely that better understanding of the immunologic basis of sarcoid and interstitial disease may be helpful in planning more effective treatment strategies. 44 references.

  3. [Preoperative assessment of lung disease patients.].

    Ramos, Gilson; Ramos Filho, José; Pereira, Edísio; Junqueira, Marcos; Assis, Carlos Henrique C


    Lung complications are the most frequent causes of postoperative morbidity-mortality, especially in lung disease patients. So, those patients should be preoperatively carefully evaluated and prepared, both clinically and laboratorially. This review aimed at determining surgical risk and at establishing preoperative procedures to minimize peri and postoperative morbidity-mortality in lung disease patients. Major anesthetic-surgical repercussions in lung function have already been described. Similarly, we tried to select higher-risk patients, submitted or not to lung resection. To that end, clinical and laboratorial propedeutics were used. Finally, a proposal of a preoperative algorithm was presented for procedures with lung resection. Lung disease patients, especially those with chronic evolution, need to be preoperatively thoroughly evaluated. ASA physical status and Goldmans cardiac index are important risk forecasting factors for lung disease patients not candidates for lung resection. Adding to these criteria, estimated postoperative max VO2, FEV1 and diffusion capacity are mandatory for some patients submitted to lung resection. beta2-agonists and steroids should be considered in the preoperative period of these patients.

  4. Cystic Lung Diseases: Algorithmic Approach.

    Raoof, Suhail; Bondalapati, Praveen; Vydyula, Ravikanth; Ryu, Jay H; Gupta, Nishant; Raoof, Sabiha; Galvin, Jeff; Rosen, Mark J; Lynch, David; Travis, William; Mehta, Sanjeev; Lazzaro, Richard; Naidich, David


    Cysts are commonly seen on CT scans of the lungs, and diagnosis can be challenging. Clinical and radiographic features combined with a multidisciplinary approach may help differentiate among various disease entities, allowing correct diagnosis. It is important to distinguish cysts from cavities because they each have distinct etiologies and associated clinical disorders. Conditions such as emphysema, and cystic bronchiectasis may also mimic cystic disease. A simplified classification of cysts is proposed. Cysts can occur in greater profusion in the subpleural areas, when they typically represent paraseptal emphysema, bullae, or honeycombing. Cysts that are present in the lung parenchyma but away from subpleural areas may be present without any other abnormalities on high-resolution CT scans. These are further categorized into solitary or multifocal/diffuse cysts. Solitary cysts may be incidentally discovered and may be an age related phenomenon or may be a remnant of prior trauma or infection. Multifocal/diffuse cysts can occur with lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, tracheobronchial papillomatosis, or primary and metastatic cancers. Multifocal/diffuse cysts may be associated with nodules (lymphoid interstitial pneumonia, light-chain deposition disease, amyloidosis, and Langerhans cell histiocytosis) or with ground-glass opacities (Pneumocystis jirovecii pneumonia and desquamative interstitial pneumonia). Using the results of the high-resolution CT scans as a starting point, and incorporating the patient's clinical history, physical examination, and laboratory findings, is likely to narrow the differential diagnosis of cystic lesions considerably. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  5. Interstitial lung disease


    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

  6. Interstitial lung disease

    Vincent Cottin


    Full Text Available This article reviews the most important articles published in interstitial lung disease, as reviewed during the Clinical Year in Review session at the 2012 annual European Respiratory Society Congress in Vienna, Austria. Since the recent international guidelines for the management of idiopathic pulmonary fibrosis (IPF, important new evidence is available. The anti-fibrotic drug pirfenidone has been recently approved in Europe. Other pharmacological agents, especially nintedanib, are still being tested. The so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended as treatment. Although the clinical course of IPF is highly variable, novel tools have been developed for individual prediction of prognosis. Acute exacerbations of IPF are associated with increased mortality and may occur with higher frequency in IPF patients with associated pulmonary hypertension. Interstitial lung disease associated with connective tissue disease has been definitely established to have a better long-term survival than IPF. A subset of patients present with symptoms and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given autoimmune disease; this condition is associated with a higher prevalence of nonspecific interstitial pneumonia pattern, female sex and younger age, although survival relevance is unclear.

  7. Respiratory Viral Infections in Chronic Lung Diseases.

    Britto, Clemente J; Brady, Virginia; Lee, Seiwon; Dela Cruz, Charles S


    Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), asthma, cystic fibrosis (CF) and interstitial lung diseases (ILD), affect many individuals worldwide. Patients with these chronic lung diseases are susceptible to respiratory lung infections and some of these viral infections can contribute to disease pathogenesis. This review highlights the associations of lung infections and the respective chronic lung diseases and how infection in the different lung diseases affects disease exacerbation and progression. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Recruitment of lung diffusing capacity: update of concept and application.

    Hsia, Connie C W


    Lung diffusing capacity (DL) for carbon monoxide (DLCO), nitric oxide (DLNO) or oxygen (DLO2) increases from rest to peak exercise without reaching an upper limit; this recruitment results from interactions among alveolar volume (VA), and cardiac output (q), as well as changing physical properties and spatial distribution of capillary erythrocytes, and is critical for maintaining a normal arterial oxygen saturation. DLCO and DLNO can be used to interpret the effectiveness of diffusive oxygen transport and track structural alterations of the alveolar-capillary barrier, providing sensitive noninvasive indicators of microvascular integrity in health and disease. Clinical interpretation of DL should take into account Q in addition to VA and hemoglobin concentration.

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

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


    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.

  10. Marijuana and lung diseases.

    Joshi, Manish; Joshi, Anita; Bartter, Thaddeus


    Cannabis sativa (marijuana) is used throughout the world, and its use is increasing. In much of the world, marijuana is illicit. While inhalation of smoke generated by igniting dried components of the plant is the most common way marijuana is used, there is concern over potential adverse lung effects. The purpose of this review is to highlight recent studies that explore the impact upon the respiratory system of inhaling marijuana smoke. Smoking marijuana is associated with chronic bronchitis symptoms and large airway inflammation. Occasional use of marijuana with low cumulative use is not a risk factor for the development of chronic obstructive pulmonary disease. The heavy use of marijuana alone may lead to airflow obstruction. The immuno-histopathologic and epidemiologic evidence in marijuana users suggests biological plausibility of marijuana smoking as a risk for the development of lung cancer; at present, it has been difficult to conclusively link marijuana smoking and cancer development. There is unequivocal evidence that habitual or regular marijuana smoking is not harmless. A caution against regular heavy marijuana usage is prudent. The medicinal use of marijuana is likely not harmful to lungs in low cumulative doses, but the dose limit needs to be defined. Recreational use is not the same as medicinal use and should be discouraged.

  11. Mitochondria in lung disease.

    Cloonan, Suzanne M; Choi, Augustine M K


    Mitochondria are a distinguishing feature of eukaryotic cells. Best known for their critical function in energy production via oxidative phosphorylation (OXPHOS), mitochondria are essential for nutrient and oxygen sensing and for the regulation of critical cellular processes, including cell death and inflammation. Such diverse functional roles for organelles that were once thought to be simple may be attributed to their distinct heteroplasmic genome, exclusive maternal lineage of inheritance, and ability to generate signals to communicate with other cellular organelles. Mitochondria are now thought of as one of the cell's most sophisticated and dynamic responsive sensing systems. Specific signatures of mitochondrial dysfunction that are associated with disease pathogenesis and/or progression are becoming increasingly important. In particular, the centrality of mitochondria in the pathological processes and clinical phenotypes associated with a range of lung diseases is emerging. Understanding the molecular mechanisms regulating the mitochondrial processes of lung cells will help to better define phenotypes and clinical manifestations associated with respiratory disease and to identify potential diagnostic and therapeutic targets.

  12. Lung alveolar epithelium and interstitial lung disease.

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


    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. Evaluation of diffuse lung uptake in radiogallium scans

    Kamei, Tetsuya (Toyama Medical and Pharmaceutical Univ. (Japan). Faculty of Medicine); Tatsuno, Ikuo


    Diffuse lung uptake of radiogallium was observed in 12 of 239 scans (5.0%). All of 12 patients received radiation therapy (average dose: 34Gy) and 9 of 12 patients were also placed on chemotherapy (average duration: 7 weeks). The most common cause of diffuse lung uptake was infection which included interstitial pneumonitis and the second was pleuritis. Of the 12 patients, 7 patients died an average of 3.6 months later since diffuse lung uptake was observed. Therefore, when we recognize diffuse lung uptake, we must perform closer examination and appropriate therapy as early as possible.

  14. Diffuse Alveolar Damage: A Common Phenomenon in Progressive Interstitial Lung Disorders

    Riitta Kaarteenaho


    Full Text Available It has become obvious that several interstitial lung diseases, and even viral lung infections, can progress rapidly, and exhibit similar features in their lung morphology. The final histopathological feature, common in these lung disorders, is diffuse alveolar damage (DAD. The histopathology of DAD is considered to represent end stage phenomenon in acutely behaving interstitial pneumonias, such as acute interstitial pneumonia (AIP and acute exacerbations of idiopathic pulmonary fibrosis (IPF. Acute worsening and DAD may occur also in patients with nonspecific interstitial pneumonias (NSIPs, and even in severe viral lung infections where there is DAD histopathology in the lung. A better understanding of the mechanisms underlying the DAD reaction is needed to clarify the treatment for these serious lung diseases. There is an urgent need for international efforts for studying DAD-associated lung diseases, since the prognosis of these patients has been and is still dismal.

  15. Is there a uniform approach to the management of diffuse parenchymal lung disease (DPLD in the UK? A national benchmarking exercise

    Partridge Martyn R


    Full Text Available Abstract Background Benchmarking is the comparison of a process to the work or results of others. We conducted a national benchmarking exercise to determine how UK pulmonologists manage common clinical scenarios in diffuse parenchymal lung disease (DPLD, and to determine current use and availability of investigative resources. We compared management decisions to existing international guidelines. Methods Consultant members of the British Thoracic Society were mailed a questionnaire seeking their views on the management of three common scenarios in DPLD. They were asked to choose from various management options for each case. Information was also obtained from the respondents on time served as a consultant, type of institution in which they worked and the availability of a local radiologist and histopathologist with an interest/expertise in thoracic medicine. Results 370 out of 689 consultants replied (54% response rate. There were many differences in the approach to the management of all three cases. Given a scenario of relapsing pulmonary sarcoidosis in a lady with multiple co-morbidities, half of respondents would institute treatment with a variety of immunosuppressants while a half would simply observe. 42% would refer a 57-year old lady with new onset DPLD for a surgical lung biopsy, while a similar number would not. 80% would have referred her for transplantation, but a fifth would not. 50% of consultants from district general hospitals would have opted for a surgical biopsy compared to 24% from cardiothoracic centres: this may reflect greater availability of a radiologist with special interest in thoracic imaging in cardiothoracic centres, obviating the need for tissue diagnosis. Faced with an elderly male with high resolution CT thorax (HRCT evidence of usual interstitial pneumonia (UIP, three quarters would observe, while a quarter would start immunosuppressants. 11% would refer for a surgical biopsy. 14% of UK pulmonologists responding

  16. What Are Asbestos-Related Lung Diseases?

    ... page from the NHLBI on Twitter. What Are Asbestos-Related Lung Diseases? Asbestos-related lung diseases are ... as the peritoneum (PER-ih-to-NE-um). Asbestos-Related Lung Diseases Figure A shows the location ...

  17. Rheumatoid arthritis-associated interstitial lung disease

    Brown KK


    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

  18. 经支气管镜肺活检对弥漫性肺实质疾病的诊断价值%The diagnostic value of transbronchial lung biopsy in diffuse parenchymal lung diseases

    施举红; 许文兵; 刘鸿瑞; 冯瑞娥; 朱元珏; 肖毅; 蔡柏蔷


    Objective This study was to evaluate the efficacy and limitations of transbronchial lung biopsy(TBLB)in the diagnosis of diffuse parenchymal lung diseases(DPLD). Methods TBLB was performed in 416 patients with diffuse lung diseases from January 2001 to October 2006 in Peking Union Medical College Hospital. The results of clinical data and pathologic diagnosis were retrospectively analyzed.Results Confirmed diagnosis by TBLB was obtained in 124 patients,the total positive diagnostic rate was 29.8%. The diseases included pulmonary sarcoidosis(52/124,41.9%),bronchiolitis obliterans organizing pneumonia/organizing pneumonia(BOOP/OP)(28/124,22.6%),pulmonary alveolar proteinosis(19/124,15.3%),lung cancer(12/124,9.7%),pulmonary vasculitis(5/124,4.0%),pulmonary tuberculosis(3/124, 2.4%), and pneumocystis carinii pneumonia,lung fungal infection,lymphangiomyomatosis,and pulmonary amyloidosis(each 1/124,0.8%). Open lung or thoracoscopic biopsy was performed in 104 cases in whom the diagnosis was undetermined by TBLB. Confirmed diagnosis Was obtained in 109 patients,including nonspecific interstitial pneumonia(37/104,37.7%),usual interstitial pneumonia(18/104,18.4%),pulmonary sarcoidosis(11/104,11%),BOOP/OP(6/104,6%),and lung cancer(5/104,5.1%). Conclusions Pathologic diagnosis can be obtained by TBLB in about 30% of the cases with DPLD,and therefore it should be considered to be a routine diagnostic procedure before open lung or thoracoscopic biopsy.%目的 评价经支气管镜肺活检(TBLB)在弥漫性肺实质病变诊断中的作用.方法 回顾性分析2001年1月至2006年10月在北京协和医院住院、经TBLB检查且具有完整临床资料的肺部弥漫性疾病患者416例,男157例,女259例,平均年龄(42.6±18.9)岁.结果 416例中124例(29.8%)经支气管镜肺活检确诊,其中结节病52例(41.9%),闭塞型细支气管炎伴机化性肺炎(BOOP/OP)28例(22.6%),肺泡蛋白沉积症(PAP)19例(15.3%),肺部肿瘤12例(9.7%),肺血管炎5例(4.0%),

  19. Aspergillus-Related Lung Disease

    Alia Al-Alawi


    Full Text Available Aspergillus is a ubiquitous dimorphic fungus that causes a variety of human diseases ranging in severity from trivial to life-threatening, depending on the host response. An intact host defence is important to prevent disease, but individuals with pre-existing structural lung disease, atopy, occupational exposure or impaired immunity are susceptible. Three distinctive patterns of aspergillus-related lung disease are recognized: saprophytic infestation of airways, cavities and necrotic tissue; allergic disease including extrinsic allergic alveolitis, asthma, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis and chronic eosinophilic pneumonia; and airway and tissue invasive disease -- pseudomembranous tracheobronchitis, acute bronchopneumonia, angioinvasive aspergillosis, chronic necrotizing aspergillosis and invasive pleural disease. A broad knowledge of these clinical presentations and a high index of suspicion are required to ensure timely diagnosis and treatment of the potentially lethal manifestations of aspergillus-related pulmonary disease. In the present report, the clinical, radiographic and pathological aspects of the various aspergillus-related lung diseases are briefly reviewed.

  20. Regional diffusing capacity in normal lungs during a slow exhalation.

    MacIntyre, N R; Nadel, J A


    From an analysis of carbon monoxide uptake and xenon-133 distribution after two bolus inhalations of these gases, we calculated regional diffusing capacity in the upper and lower volume halves of the lungs during the middle 60% of an exhaled vital capacity in five seated normal subjects. We found that the regional diffusing capacity of the upper half of the lungs was 11.6 +/- 4.2 (mean +/- SD) ml.min-1.Torr-1 and that the regional diffusing capacity of the lower half of the lungs was 24.4 +/- 2.4 ml.min-1.Torr-1 after 25% of the vital capacity had been exhaled. These values remained relatively constant as lung volume decreased from 25 to 75% of the exhaled vital capacity. Diffusing capacity in the upper half of the lungs ranged from 9.4 to 12.4 ml.min-1.Torr-1 during exhalation, and in the lower half of the lungs from 21.0 to 28.6 ml.min-1.Torr-1 during exhalation. These results suggest that total lung diffusing capacity remains relatively constant over this midrange of lung volumes and that this occurs because the regional diffusing capacities in both the upper and lower halves of the lungs remain relatively constant.

  1. Cystic lung disease: Achieving a radiologic diagnosis

    Trotman-Dickenson, Beatrice, E-mail:


    Diffuse cystic lung disease represents a diverse group of uncommon disorders with characteristic appearance on high resolution CT imaging. The combination of imaging appearance with clinical features and genetic testing where appropriate permits a confident and accurate diagnosis in the majority of the diseases without recourse for open lung biopsy. The mechanism of cyst development disease is unclear but in some disorders appears to be related to small airways obstruction. These diseases are incurable, with the exception of Langerhans cell histiocytosis which may spontaneously remit or resolve on smoking cessation. Disease progression is unpredictable; in general older patients have a more benign disease, while young patients may progress rapidly to respiratory failure. An understanding of the complications of cystic lung disease and the appearance of disease progression is essential for the management of these patients. A number of these disorders are associated with malignancy, recognition of the potential tumors permits appropriate imaging surveillance. Due to the widespread use of CT, pulmonary cysts are increasingly discovered incidentally in an asymptomatic individual. The diagnostic challenge is to determine whether these cysts represent an early feature of a progressive disease or have no clinical significance. In the elderly population the cysts are unlikely to represent a progressive disease. In individuals <50 years further evaluation is recommended.

  2. CT引导下经皮肺活检在弥漫性实质性肺疾病的临床应用%The diagnostic value of CT-guided percutaneous needle lung biopsy in diffuse parenchymal lung diseases

    彭敏; 冯瑞娥; 刘鸿瑞; 朱元珏; 许文兵; 施举红; 蔡柏蔷; 田欣伦; 柳涛; 张弘; 肖教; 刘巍


    目的 探讨CT引导下经皮切割针肺活检在影像学表现为弥漫性实质性肺疾病诊断中的作用和应用范围.方法 选择2000年1月至2008年12月北京协和医院行CT引导下经皮肺活检、胸部CT表现为双肺弥漫病变且临床资料完整的弥漫性实质性肺疾病患者248例.排除胸部CT为单发病变及单肺病变的病例.回顾分析所有病例的病史、血免疫学、支气管镜、胸部高分辨CT及肺活检病理等检查结果.结果 男114例,女134例,年龄13~78岁,平均年龄(50±16)岁.248例中经皮肺活检获得病理形态学诊断130例(52.4%),确诊疾病分别为肺部感染性疾病46例(35.4%),肺部肿瘤33例(25.4%),闭塞型细支气管炎伴机化性肺炎/机化性肺炎(BOOP/OP) 29例(22.3%),血管炎8例(6.2%),肉芽肿病变6例(4.6%),结节病3例(2.3%),弥漫性肺泡损伤和肺淀粉样变各2例(1.5%),以及肺泡蛋白沉积症(PAP)1例(0.7%).经皮肺活检未确定诊断的118例患者中有37例进行了开胸或胸腔镜肺活检,36例确定诊断,分别为非特异性间质性肺炎(NSIP) 12例,寻常型间质性肺炎(UIP)3例,感染6例,肿瘤3例,淋巴细胞间质性肺炎(LIp)、肺血管炎和过敏性肺炎(HP)各2例,结节病、ABPA、肺透明样肉芽肿病、肺尘埃沉积症、Castleman病及淋巴增殖性疾病各1例.经皮肺活检未确定诊断的118例患者中有69例结合临床及其他检查获得诊断.结论 约半数弥漫性实质性肺疾病患者通过CT引导下经皮肺活检能够获得特定的病理形态学诊断,尤其是影像学表现为双肺弥漫病变的感染及肿瘤病例确诊阳性率较高,但对于特发性间质性肺炎诊断价值有限,应结合患者的临床实际情况选择应用.%Objective This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD).Methods A total of 481 patients admitted

  3. Diagnostic value of diffuse and peripheral lung lesions by transbronchial lung biopsy

    石志红; 魏霞; 卫小红; 朱柏


    Objective To evaluate the diagnostic value of transbronchial lung biopsy(TBLB)via bronchoscope for lung diffuse lesions and peripheral pulmonary lesions.Methods The results of TBLB were retrospectively analyzed from 256 diagnoses of unknown patients with lung diffuse lesions or peripheral lung lesions.Results Eighty-nine final diagnosis cases via TBLB only once,the diagnostic rate of double lung diffuse lesions was the highest,[51.06%(24/47)],local non-nodule lesions,nodule lesions,cavity lesions were 33.33...

  4. Smoking and interstitial lung diseases.

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


    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.

  5. Smoking and interstitial lung diseases

    George A. Margaritopoulos


    Full Text Available 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.

  6. Smoking-related interstitial lung disease.

    Hagmeyer, Lars; Randerath, Winfried


    Smoking-related interstitial lung diseases (SR-ILDs) are a heterogeneous group of diseases with major clinical significance. Reliable epidemiological data are not yet available. Review of pertinent literature retrieved by a selective search in PubMed. The available data on many aspects of SR-ILDs are sparse, but recent studies on the pathophysiology and targeted treatment of these conditions have revealed ways in which clinical outcomes can be improved. Highresolution computerized tomography should be used for differential diagnosis; lung biopsy is often unnecessary. Oncogenic mutations play a role in the pathogenesis of pulmonary Langerhans-cell histiocytosis (PLCH). In the future, cladribine and vemurafenib may be treatment options for PLCH. Desquamative interstitial pneumonia (DIP) may be difficult to distinguish from respiratorybronchiolitis-associated interstitial lung disease (RB-ILD); DIP is treated with steroids and sometimes with immune suppressants. In idiopathic pulmonary fibrosis (IPF), the antifibrotic drugs pirfenidone and nintedanib can delay disease progression. Smoking is also a risk factor for combined pulmonary fibrosis and emphysema (CPFE), rheumatoid-arthritis-associated interstitial lung disease (RA-ILD), pulmonary alveolar proteinosis (PAP), acute eosinophilic pneumonia (AEP), and diffuse alveolar hemorrhage (DAH) in Goodpasture syndrome. In smokers with exertional dyspnea and/or a nonproductive cough, SR-ILDs must be considered in the differential diagnosis. If an SR-ILD is suspected, the patient should be referred to a pulmonary specialist. Early treatment and smoking cessation can improve clinical outcomes, particularly in the acute and chronically progressive types of SR-ILD.

  7. 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

    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)


    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.)

  8. Comorbidities in interstitial lung diseases

    George A. Margaritopoulos


    Full Text Available Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death. Therefore, early identification and accurate treatment of comorbidities is essential.

  9. Interstitial Lung disease in Systemic Sclerosis

    Ooi, G.C.; Mok, M.Y.; Tsang, K.W.T.; Khong, P.L.; Fung, P.C.W.; Chan, S.; Tse, H.F.; Wong, R.W.S.; Lam, W.K.; Lau, C.S. [Queen Mary Hospital, Hong Kong (China). Dept. of Diagnostic Radiology and Medicine; Wong, Y. [Queen Mary Hospital, Hong Kong (China). Dept. of Radiology


    Purpose: To evaluate high-resolution CT (HRCT) parameters of inflammation and fibrosis in systemic sclerosis (SSc), for correlation with lung function, skin scores and exercise tolerance. Material and Methods: : 45 SSc patients (40 women, 48.5{+-}13.4 years), underwent thoracic HRCT, lung function assessment, and modified Rodnan skin scores. Exercise tolerance was also graded. HRCT were scored for extent of 4 HRCT patterns of interstitial lung disease (ILD): ground glass opacification (GGO), reticular, mixed and honeycomb pattern in each lobe. Total HRCT score, inflammation index (GGO and mixed score) and fibrosis index (reticular and honeycomb scores) were correlated with lung function and clinical parameters. Results: ILD was present in 39/45 (86.7%) patients. Abnormal (<80% predicted) forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusion factor (DLco) were detected in 30%, 22% and 46% of patients. Total HRCT score correlated with FVC (r=0.43, p=0.008), FEV1 (forced expiratory volume) (r=-0.37, p=0.03), TLC (r=-0.47, p=0.003), and DLCO (r=-0.43, p=0.008); inflammatory index with DLCO (r=-0.43, p=0.008) and exercise tolerance (r=-0.39, p < 0.05); and fibrosis index with FVC (r=-0.31, p=0.05) and TLC (r=-0.38, p=0.02). Higher total HRCT score, and inflammation and fibrosis indices were found in patients with abnormal lung function. Conclusion: Qualitative HRCT is able to evaluate inflammation and fibrosis, showing important relationships with diffusion capacity and lung volume, respectively.

  10. 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

    赵栋梁; 白云苹; 王明航; 李建生


    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

  11. Interstitial lung disease in scleroderma.

    Schoenfeld, Sara R; Castelino, Flavia V


    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.

  12. Complement system in lung disease.

    Pandya, Pankita H; Wilkes, David S


    In addition to its established contribution to innate immunity, recent studies have suggested novel roles for the complement system in the development of various lung diseases. Several studies have demonstrated that complement may serve as a key link between innate and adaptive immunity in a variety of pulmonary conditions. However, the specific contributions of complement to lung diseases based on innate and adaptive immunity are just beginning to emerge. Elucidating the role of complement-mediated immune regulation in these diseases will help to identify new targets for therapeutic interventions.

  13. Interstitial lung disease


    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-

  14. Nitric oxide and carbon monoxide diffusing capacity of the lung

    Lee, I. van der


    The single breath diffusion capacity of the lung for carbon monoxide (DLCO) is measure for gas uptake by the lung, and consists of a membrane and a vascular component. Nitric oxide (NO) binds 400 times faster to hemoglobin than carbon monoxide, thus the uptake of NO by the blood is very large.

  15. Nitric oxide and carbon monoxide diffusing capacity of the lung

    Lee, I. van der


    The single breath diffusion capacity of the lung for carbon monoxide (DLCO) is measure for gas uptake by the lung, and consists of a membrane and a vascular component. Nitric oxide (NO) binds 400 times faster to hemoglobin than carbon monoxide, thus the uptake of NO by the blood is very large. There

  16. Interstitial lung disease


    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.

  17. Diffusion-weighted MR imaging of the normal fetal lung

    Balassy, Csilla; Kasprian, Gregor; Weber, Michael; Hoermann, Marcus; Bankier, Alexander; Herold, Christian J.; Prayer, Daniela [Medical University of Vienna, Department of Radiology, Vienna (Austria); Brugger, Peter C. [Medical University of Vienna, Center of Anatomy and Cell Biology, Vienna (Austria); Csapo, Bence [Medical University of Vienna, Department of Obstetrics and Gyneocology, Vienna (Austria); Bammer, Roland [University of Stanford, Department of Radiology, Stanford, CA (United States)


    To quantify apparent diffusion coefficient (ADC) changes in fetuses with normal lungs and to determine whether ADC can be used in the assessment of fetal lung development. In 53 pregnancies (20-37th weeks of gestation), we measured ADC on diffusion-weighted imaging (DWI) in the apical, middle, and basal thirds of the right lung. ADCs were correlated with gestational age. Differences between the ADCs were assessed. Fetal lung volumes were measured on T2-weighted sequences and correlated with ADCs and with age. ADCs were 2.13 {+-} 0.44 {mu}m{sup 2}/ms (mean {+-} SD) in the apex, 1.99 {+-} 0.42 {mu}m{sup 2}/ms (mean {+-} SD) in the middle third, and 1.91 {+-} 0.41 {mu}m{sup 2}/ms (mean {+-} SD) in the lung base. Neither the individual ADC values nor average ADC values showed a significant correlation with gestational age or with lung volumes. Average ADCs decreased significantly from the lung apex toward the base. Individual ADCs showed little absolute change and heterogeneity. Lung volumes increased significantly during gestation. We have not been able to identify a pattern of changes in the ADC values that correlate with lung maturation. Furthermore, the individual, gravity-related ADC changes are subject to substantial variability and show nonuniform behavior. ADC can therefore not be used as an indicator of lung maturity. (orig.)

  18. Diffusion-weighted MR imaging of the normal fetal lung.

    Balassy, Csilla; Kasprian, Gregor; Brugger, Peter C; Csapo, Bence; Weber, Michael; Hörmann, Marcus; Bankier, Alexander; Bammer, Roland; Herold, Christian J; Prayer, Daniela


    To quantify apparent diffusion coefficient (ADC) changes in fetuses with normal lungs and to determine whether ADC can be used in the assessment of fetal lung development. In 53 pregnancies (20-37th weeks of gestation), we measured ADC on diffusion-weighted imaging (DWI) in the apical, middle, and basal thirds of the right lung. ADCs were correlated with gestational age. Differences between the ADCs were assessed. Fetal lung volumes were measured on T2-weighted sequences and correlated with ADCs and with age. ADCs were 2.13 +/- 0.44 microm(2)/ms (mean +/- SD) in the apex, 1.99 +/- 0.42 microm(2)/ms (mean +/- SD) in the middle third, and 1.91 +/- 0.41 microm(2)/ms (mean +/- SD) in the lung base. Neither the individual ADC values nor average ADC values showed a significant correlation with gestational age or with lung volumes. Average ADCs decreased significantly from the lung apex toward the base. Individual ADCs showed little absolute change and heterogeneity. Lung volumes increased significantly during gestation. We have not been able to identify a pattern of changes in the ADC values that correlate with lung maturation. Furthermore, the individual, gravity-related ADC changes are subject to substantial variability and show nonuniform behavior. ADC can therefore not be used as an indicator of lung maturity.

  19. Cilia Dysfunction in Lung Disease

    Tilley, Ann E.; Walters, Matthew S.; Shaykhiev, Renat; Crystal, Ronald G.


    A characteristic feature of the human airway epithelium is the presence of ciliated cells bearing motile cilia, specialized cell surface projections containing axonemes comprised of microtubules and dynein arms, which provide ATP-driven motility. In the airways, cilia function in concert with airway mucus to mediate the critical function of mucociliary clearance, cleansing the airways of inhaled particles and pathogens. The prototypical disorder of respiratory cilia is primary ciliary dyskinesia, an inherited disorder that leads to impaired mucociliary clearance, repeated chest infections, and progressive destruction of lung architecture. Numerous acquired lung diseases are also marked by abnormalities in both cilia structure and function. In this review we summarize current knowledge regarding airway ciliated cells and cilia, how they function to maintain a healthy epithelium, and how disorders of cilia structure and function contribute to inherited and acquired lung disease. PMID:25386990

  20. Interstitial lung disease: Diagnostic approach

    Kaushik Saha


    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.

  1. Acute exacerbations of fibrotic interstitial lung disease.

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


    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.

  2. Aeroparticles, composition and lung diseases

    Carlos Ivan Falcon-Rodriguez


    Full Text Available Urban air pollution is a serious worldwide problem due to its impact on human health. In the past sixty years, growing evidence established a correlation between exposure to air pollutants and the developing of severe respiratory diseases. Recently Particulate matter (PM is drawing more public attention to various aspects including historical backgrounds, physicochemical characteristics and its pathological role. Therefore, this review is focused on these aspects. The most famous air pollution disaster happened in London on December 1952; it has been calculated that more than 4000 deaths occurred during this event. Air pollution is a complex mix of gases and particles. Gaseous pollutants disseminate deeply into the alveoli, allowing its diffusion through the blood-air barrier to several organs. Meanwhile, PM is a mix of solid or liquid particles suspended in the air. PM is deposited at different levels of the respiratory tract, depending on its size: Coarse particles (PM10 in upper airways and fine particles (PM2.5 can be accumulated in the lung parenchyma, inducing several respiratory diseases. Additionally to size, the composition of particulate matter has been associated with different toxicological outcomes on clinical, epidemiological, as well as in vivo and in vitro animal and human studies. PM can be constituted by organic, inorganic and biological compounds. All these compounds are capable of modifying several biological activities including alterations in cytokine production, coagulation factors balance, pulmonary function, respiratory symptoms, and cardiac function. It can also generate different modifications during its passage through the airways, like inflammatory cells recruitment, with the release of cytokines and reactive oxygen species (ROS. These inflammatory mediators can activate different pathways such as MAP-kinases, NF-B, and stat-1, or induce DNA adducts. All these alterations can mediate obstructive or restrictive

  3. Pneumoproteins in interstitial lung diseases

    Janssen, Rob


    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

  4. Lung Cancer Mortality among Uranium Gaseous Diffusion Plant Workers: A Cohort Study 1952–2004

    LW Figgs


    Full Text Available Background: 9%–15% of all lung cancers are attributable to occupational exposures. Reports are disparate regarding elevated lung cancer mortality risk among workers employed at uranium gaseous diffusion plants. Objective: To investigate whether external radiation exposure is associated with lung cancer mortality risk among uranium gaseous diffusion workers. Methods: A cohort of 6820 nuclear industry workers employed from 1952 to 2003 at the Paducah uranium gaseous diffusion plant (PGDP was assembled. A job-specific exposure matrix (JEM was used to determine likely toxic metal exposure categories. In addition, radiation film badge dosimeters were used to monitor cumulative external ionizing radiation exposure. International Classification for Disease (ICD codes 9 and 10 were used to identify 147 lung cancer deaths. Logistic and proportional hazards regression were used to estimate lung cancer mortality risk. Results: Lung cancer mortality risk was elevated among workers who experienced external radiation >3.5 mrem and employment duration >12 years. Conclusion: Employees of uranium gaseous diffusion plants carry a higher risk of lung cancer mortality; the mortality is associated with increased radiation exposure and duration of employment.

  5. Quick-Relief Medications for Lung Diseases

    ... relief medications are used to treat asthma, other lung disease symptoms or an acute episode (such as an ... about the following quick-relief asthma and other lung disease medications: Anticholinergics Anticholinergics are quick-relief asthma and ...

  6. Unusual progression and subsequent improvement in cystic lung disease in a child with radiation-induced lung injury

    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)


    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.)

  7. 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

    张晶莹; 刘秀云; 彭芸; 周春菊


    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

  8. Interstitial lung disease


    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-

  9. Interstitial lung disease


    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

  10. Diffusion Reaction of Carbon Monoxide in the Human Lung

    Kang, M.-Y.; Guénard, H.; Sapoval, B.


    The capture of CO, a standard lung function test, results from diffusion-reaction processes of CO with hemoglobin inside red blood cells (RBCs). In its current understanding, suggested by Roughton and Forster in 1957, the capture is represented by two independent resistances in series, one for diffusion from the gas to the RBC periphery, the second for internal diffusion reaction. Numerical studies in 3D model structures described here contradict the independence hypothesis. This results from two different theoretical reasons: (i) The RBC peripheries are not equi-concentrations; (ii) diffusion times in series are not additive.

  11. Pulmonary hypertension in chronic interstitial lung diseases

    Antonella Caminati


    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.

  12. Imaging of nonmalignant occupational lung disease

    Kim, J.S.; Lynch, D.A. [University of Colorado, Denver, CO (United States). Health Science Center, Dept. of Radiology


    The radiologist plays an important partnership role in detecting presymptomatic disease in those at risk for occupational lung disease, contributing to the specificity of the diagnosis and recognizing sentinel events. Medicolegal roles for imaging include confirming the presence of a morphologic abnormality compatible with occupational lung disease, identifying other potential causes for disability, and determining the morphologic extent of disease. This article describes and illustrates the imaging appearance of a wide range of occupational lung diseases.

  13. Agricultural lung diseases.

    Kirkhorn, S R; Garry, V F


    Agriculture is considered one of the most hazardous occupations. Organic dusts and toxic gases constitute some of the most common and potentially disabling occupational and environmental hazards. The changing patterns of agriculture have paradoxically contributed to both improved working conditions and increased exposure to respiratory hazards. Animal confinement operations with increasing animal density, particularly swine confinement, have contributed significantly to increased intensity and duration of exposure to indoor air toxins. Ongoing research has implicated bacterial endotoxins, fungal spores, and the inherent toxicity of grain dusts as causes of upper and lower airway inflammation and as immunologic agents in both grain and animal production. Animal confinement gases, particularly ammonia and hydrogen sulfide, have been implicated as additional sources of respiratory irritants. It has become evident that a significant percentage of agricultural workers have clinical symptoms associated with long-term exposure to organic dusts and animal confinement gases. Respiratory diseases and syndromes, including hypersensitivity pneumonitis, organic dust toxic syndrome, chronic bronchitis, mucous membrane inflammation syndrome, and asthmalike syndrome, result from ongoing acute and chronic exposures. In this review we focus upon the emerging respiratory health issues in a changing agricultural economic and technologic environment. Environmental and occupational hazards and exposures will be emphasized rather than clinical diagnosis and treatment. Methods of prevention, from both engineering controls and personal respiratory perspectives, are also addressed.

  14. A videotoracoscopia no diagnóstico das doenças difusas do parênquima pulmonar Video-assisted thoracoscopy for the diagnosis of diffuse parenchymal lung disease

    Renato Tadao Ishie


    Full Text Available OBJETIVO: Analisar o papel da videotoracoscopia no diagnóstico das doenças difusas do parênquima pulmonar. MÉTODOS: Os prontuários de pacientes com suspeita de doenças difusas do parênquima pulmonar e os resultados do exame anatomopatológico das amostras de biópsia pulmonar por videotoracoscopia foram analisados retrospectivamente. RESULTADOS: Dos 48 pacientes incluídos no estudo, 25 (52,08% eram do sexo feminino, e 23 (47,92% eram do sexo masculino. A idade média foi de 58,77 anos, variando entre 20 e 76 anos. Foi realizado o exame anatomopatológico de 54 fragmentos de biópsia pulmonar: 24 (44,44% da língula; 10 (18,52% do lobo inferior esquerdo; 7 (12,96% do lobo médio; 6 (11,11% do lobo inferior direito; 5 (9,26% do lobo superior esquerdo; e 2 (3,71% do lobo superior direito. O tempo médio de drenagem torácica foi de 2,2 dias. Como eventos adversos, houve conversão para toracotomia em 2 pacientes (4,17% e pneumotórax residual em 1 (2,08%. O diagnóstico definitivo foi obtido em 46 (95,83% casos, com predomínio das pneumonias intersticiais idiopáticas (54,18%. Os diagnósticos mais frequentes foram pneumonia intersticial usual (29,27%, pneumonia intersticial não-específica (16,67% e pneumonia por hipersensibilidade (12,50%. CONCLUSÕES: A videotoracoscopia com biópsia pulmonar é um procedimento eficaz, seguro e viável para o diagnóstico das doenças difusas do parênquima pulmonar.OBJECTIVE: To evaluate the role of video-assisted thoracoscopy in the diagnosis of diffuse parenchymal lung diseases. METHODS: The medical charts of patients suspected of having diffuse parenchymal lung disease were retrospectively reviewed, as were the results of the anatomopathological examination of lung biopsy specimens collected through video-assisted thoracoscopy. RESULTS: Of the 48 patients included in the study, 25 (52.08% were female and 23 (47.92% were male. The mean age was 58.77 years (range, 20-76 years. A total of 54 biopsy

  15. Diffusion of gases into the lung: How physics can help to understand physiology

    M Filoche; B Sapoval


    In the human lung, the gas transfer between air and blood is achieved in terminal units that are called `acini'. Whereas convection is still the predominant transport phenomenon at the acinus entrance, most of the acinar surface is in fact accessed by diffusion. The transition between convection and diffusion, and thus the size of the diffusion unit, depends on the air velocity at the acinus entrance. In this paper, we present a gas transport model which takes into account both the diffusion into the acinus and the diffusion across the alveolar membrane. It is shown that the physiological sizes of the diffusion unit in the lung, at rest or at exercise, can be explained by physical arguments. In that sense, diffusion is the `dimensioning criterion' of the lung at the acinar level. This approach shows that, due to diffusional screening at inspiration and at rest, there exists a permanent spatial inhomogeneity of oxygen and carbon dioxide partial pressure which reduces the effective surface efficiency of the human acinus to a value of only 30 to 40%. This model casts a new light on the properties of this physiological transport system. It permits in particular to understand how several diseases among which pulmonary edema may remain asymptomatic in their early stages.

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

    Kostas Archontogeorgis


    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.

  17. IgG4 Related Lung Disease

    Mihir Patel


    Full Text Available IgG4 related disease is a poorly understood immune mediated condition. Lung involvement is rare and difficult to diagnose and can mimic primary lung malignancy on imaging. A patient who was found to have an incidental lung lesion with risk factors for primary pulmonary malignancy is reported.

  18. Diffusion on Networks and Diffusion Weighted NMR of the Human Lung

    Buhl, Niels


    been studied by many authors within the mathematical and physical communities. Here we use ideas from both of those fields to develop three simple and easy to use expressions for the diffusion propagator, i.e., the fundamental solution of the diffusion equation, on general metric graphs with equal...... with a finite interval, naturally arise as simplified models of network structures in many areas of science ranging from free-electron models of conjugated molecules to models of fluid diffusion in porous materials. The description of diffusion on metric graphs, together with a variety of related problems, have...... application of the above mentioned theory, given that the human lung consists of a large network of bifurcating tube like airways. 90-95% of the gas in a human lung resides in the ~30000 pulmonary acini, each of these consists of ~500 airways, which are connected as the edges in a binary tree. We model...

  19. CT of chronic infiltrative lung disease: Prevalence of mediastinal lymphadenopathy

    Niimi, Hiroshi; Kang, Eun-Young; Kwong, S. [Univ. of British Columbia and Vancouver Hospital and Health Sciences Centre (Canada)] [and others


    Our goal was to determine the prevalence of mediastinal lymph node enlargement at CT in patients with diffuse infiltrative lung disease. The study was retrospective and included 175 consecutive patients with diffuse infiltrative lung diseases. Diagnoses included idiopathic pulmonary fibrosis (IPF) (n = 61), usual interstitial pneumonia associated with collagen vascular disease (CVD) (n = 20), idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) (n = 22), extrinsic allergic alveolitis (EAA) (n = 17), and sarcoidosis (n = 55). Fifty-eight age-matched patients with CT of the chest performed for unrelated conditions served as controls. The presence, number, and sites of enlarged nodes (short axis {ge}10 mm in diameter) were recorded. Enlarged mediastinal nodes were present in 118 of 175 patients (67%) with infiltrative lung disease and 3 of 58 controls (5%) (p < 0.001). The prevalence of enlarged nodes was 84% (46 of 55) in sarcoidosis, 67% (41 of 61) in IPF, 70% (14 of 20) in CVD, 53% (9 of 17) in EAA, and 36% (8 of 22) in BOOP. The mean number of enlarged nodes was higher in sarcoidosis (mean 3.2) than in the other infiltrative diseases (mean 1.2) (p < 0.001). Enlarged nodes were most commonly present in station 10R, followed by 7, 4R, and 5. Patients with infiltrative lung disease frequently have enlarged mediastinal lymph nodes. However, in diseases other than sarcoid, usually only one or two nodes are enlarged and their maximal short axis diameter is <15 mm. 11 refs., 2 figs., 1 tab.

  20. Mortality and Respiratory Failure After Thoracoscopic Lung Biopsy for Interstitial Lung Disease.

    Durheim, Michael T; Kim, Sunghee; Gulack, Brian C; Burfeind, William R; Gaissert, Henning A; Kosinski, Andrzej S; Hartwig, Matthew G


    Surgical lung biopsy contributes to establishing a specific diagnosis among many patients with interstitial lung disease (ILD). The risks of death and respiratory failure associated with elective thoracoscopic surgical lung biopsy, and patient characteristics associated with these outcomes, are not well understood. This is a retrospective cohort study of patients who underwent elective thoracoscopic lung biopsy for ILD between 2008 and 2014, according to The Society of Thoracic Surgeons database. The study determined the incidence of operative mortality and of postoperative respiratory failure. Multivariable models were used to identify risk factors for these adverse outcomes. Among 3,085 patients, 46 (1.5%) died before hospital discharge or within 30 days of thoracoscopic lung biopsy. Postoperative respiratory failure occurred in 90 (2.9%) patients. Significant risk factors for operative mortality among patients with ILD included a diagnosis of pulmonary hypertension, preoperative corticosteroid treatment, and low diffusion capacity. Elective thoracoscopic lung biopsy among patients with ILD is associated with a low risk of operative mortality and postoperative respiratory failure. Attention to the presence of pulmonary hypertension, preoperative corticosteroid treatment, and diffusion capacity may help inform risk stratification for thoracoscopic lung biopsy among patients with ILD. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

    Campo, Ilaria; Zorzetto, Michele; Bonella, Francesco


    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.

  2. 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

    王洪武; 李庆棣; 朱元珏


    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。

  3. Diffusion on Networks and Diffusion Weighted NMR of the Human Lung

    Buhl, Niels


    This dissertation deals with the analytical description of diffusion on metric graphs and the application of this theory to diffusion weighted Nuclear Magnetic Resonance (NMR) of the human lung and other branched structures. Metric graphs, i.e., graphs where each edge has been associated with a f......This dissertation deals with the analytical description of diffusion on metric graphs and the application of this theory to diffusion weighted Nuclear Magnetic Resonance (NMR) of the human lung and other branched structures. Metric graphs, i.e., graphs where each edge has been associated...... most useful expression, is an eigenfunction expansion. The theory used to construct the latter is directly related to certain eigenvalue spectra studied in the field of spectral graph theory. This link opens the door to a wealth of results which, e.g., can be used to relate the long time behavior...... application of the above mentioned theory, given that the human lung consists of a large network of bifurcating tube like airways. 90-95% of the gas in a human lung resides in the ~30000 pulmonary acini, each of these consists of ~500 airways, which are connected as the edges in a binary tree. We model...

  4. Endoplasmic reticulum stress in lung disease

    Stefan J. Marciniak


    Full Text Available Exposure to inhaled pollutants, including fine particulates and cigarette smoke is a major cause of lung disease in Europe. While it is established that inhaled pollutants have devastating effects on the genome, it is now recognised that additional effects on protein folding also drive the development of lung disease. Protein misfolding in the endoplasmic reticulum affects the pathogenesis of many diseases, ranging from pulmonary fibrosis to cancer. It is therefore important to understand how cells respond to endoplasmic reticulum stress and how this affects pulmonary tissues in disease. These insights may offer opportunities to manipulate such endoplasmic reticulum stress pathways and thereby cure lung disease.

  5. Lung Disease Including Asthma and Adult Vaccination

    ... Healthcare Professionals Lung Disease including Asthma and Adult Vaccination Language: English Español (Spanish) Recommend on Facebook Tweet ... more about health insurance options. Learn about adult vaccination and other health conditions Asplenia Diabetes Heart Disease, ...

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

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


    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.

  7. Lung diffusion capacity in children with respiratory symptoms and untreated GERD

    MIRIĆ, MIRJANA; Turkalj, Mirjana; Nogalo, Boro; Erceg, Damir; Perica, Marija; Plavec, Davor


    Background Gastroesophageal reflux disease (GERD) is associated with many respiratory disorders, among which, chronic cough, laryngitis, and asthma are among the most common. We investigated lung function, including gas diffusion capacity, in children with poor asthma control or chronic laryngitis with untreated GERD. Material/Methods A total of 71 children, aged 6–17 years, with chronic respiratory and other symptoms suggestive for GERD, were enrolled and divided into 2 groups: chronic laryn...

  8. Usefulness of CT in diffuse pleural disease

    Ashizawa, Kazuto; Uetani, Masataka; Mori, Masaichi; Matsunaga, Naofumi; Hayashi, Kuniaki; Kawahara, Katsunobu; Ayabe, Kimiji; Tsuda, Nobuo (Nagasaki Univ. (Japan). School of Medicine)


    Alterations of the pleura and extrapleural fat were assessed by CT in 114 patients with diffuse pleural disease. The diseases included malignant pleural mesothelioma, pleuritis carcinomatosa, acute and chronic empyema, asbestosis and transudatory effusion. The pleural changes were classified into five types according to the degree, extent and contour of pleural thickening. Each type was relatively specific for the diagnosis of diffuse pleural diseases. Increased thickness of the extrapleural fat was seen in malignant pleural disease and empyema, and homogenous increased attenuation of extrapleural was a relatively characteristic finding in acute empyema. CT was useful in evaluating diffuse pleural disease. (author).

  9. Recurrence and metastasis of lung cancer demonstrate decreased diffusion on diffusion-weighted magnetic resonance imaging.

    Usuda, Katsuo; Sagawa, Motoyasu; Motomo, Nozomu; Ueno, Masakatsu; Tanaka, Makoto; Machida, Yuichiro; Maeda, Sumiko; Matoba, Munetaka; Tonami, Hisao; Ueda, Yoshimichi; Sakuma, Tsutomu


    Diffusion-weighted magnetic resonance imaging (DWI) is reported to be useful for detecting malignant lesions. The purpose of this study is to clarify characteristics of imaging, detection rate and sensitivity of DWI for recurrence or metastasis of lung cancer. A total of 36 lung cancer patients with recurrence or metastasis were enrolled in this study. While 16 patients underwent magnetic resonance imaging (MRI), computed tomography (CT) and positron emission tomography-computed tomography (PET-CT), 17 underwent MRI and CT, and 3 underwent MRI and PET-CT. Each recurrence or metastasis showed decreased diffusion, which was easily recognized in DWI. The detection rate for recurrence or metastasis was 100% (36/36) in DWI, 89% (17/19) in PET-CT and 82% (27/33) in CT. Detection rate of DWI was significantly higher than that of CT (p=0.0244) but not significantly higher than that of PET-CT (p=0.22). When the optimal cutoff value of the apparent diffusion coefficient value was set as 1.70?10-3 mm2/sec, the sensitivity of DWI for diagnosing recurrence or metastasis of lung cancer was 95.6%. DWI is useful for detection of recurrence and metastasis of lung cancer.

  10. Ground-glass opacity in diffuse lung diseases: high-resolution computed tomography-pathology correlation; Opacidades em vidro fosco nas doencas pulmonares difusas: correlacao da tomografia computadorizada de alta resolucao com a anatomopatologia

    Santos, Maria Lucia de Oliveira; Vianna, Alberto Domingues; Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Disciplina de Radiologia; Moraes, Heleno Pinto de [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Patologia]. E-mail:


    Ground-glass opacity is a finding frequently seen in high-resolution computed tomography examinations of the chest and is characterized by hazy increased attenuation of lung, however without blurring of bronchial and vascular margins. Due to its un specificity, association with other radiological, clinical and pathological findings must be considered for an accurate diagnostic interpretation. In this paper were reviewed 62 computed tomography examinations of patients with diffuse pulmonary diseases of 14 different etiologies in which ground-glass opacity was the only or the most remarkable finding, and correlated this findings with pathology abnormalities seen on specimens obtained from biopsies or necropsies. In pneumocystosis, ground-glass opacities correlated histologically with alveolar occupation by a foaming material containing parasites, in bronchiole alveolar cell carcinoma with thickening of the alveolar septa and occupation of the lumen by mucus and tumoral cells, in paracoccidioidomycosis with thickening of the alveolar septa, areas of fibrosis and alveolar bronchopneumonia exudate, in sarcoidosis with fibrosis or clustering of granulomas and in idiopathic pulmonary fibrosis with alveolar septa thickening due to fibrosis. Alveolar occupation by blood was found in cases of leptospirosis, idiopathic hemo siderosis, metastatic kidney tumor and invasive aspergillosis whereas oily vacuole were seen in lipoid pneumonia, proteinaceous and lipo proteinaceous material in silico proteinosis and pulmonary alveolar proteinosis, and edematous fluid in cardiac failure. (author)

  11. Lung ultrasound has limited diagnostic value in rare cystic lung diseases

    Davidsen, Jesper Rømhild; Bendstrup, Elisabeth; Henriksen, Daniel P


    Background: Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). However, the applicability of LUS...... in patients with DPLD subtypes as rare cystic lung diseases has not previously been investigated. This study aimed to observe if distinctive LUS findings could be found in patients with lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHDS). Methods......: This single centre case-based cross-sectional study of patients diagnosed with LAM, PCLH and BHDS was conducted at a Danish DPLD specialist centre. Patients underwent clinical examination including LUS. LUS findings were compared to findings scored according to a modified Belmaati score on HRCT and reviewed...

  12. Diffuse and vascular hepatic diseases; Diffuse und vaskulaere Lebererkrankungen

    Kreimeyer, S.; Grenacher, L. [Universitaetsklinikum Heidelberg, Abteilung Diagnostische und Interventionelle Radiologie, Heidelberg (Germany)


    In addition to focal liver lesions, diffuse and vascular disorders of the liver represent a wide spectrum of liver diseases which are from the radiological point of view often difficult or nearly impossible to diagnose. Classical diagnostic methods are computed tomography and magnetic resonance imaging in addition to ultrasound. Diffuse parenchymal damage caused by diseases of various etiologies is therefore difficult to evaluate because it often lacks characteristic morphological features. For hepatic steatosis, hemochromatosis/siderosis as an example of a diffuse storage disease and sarcoidosis and candidiasis as infectious/inflammatory diseases, an image-based diagnosis is appropriate in some cases. For most diffuse liver diseases, however only nonspecific changes are visualized. Vascular pathologies of the liver, such as the Budd-Chiari syndrome and portal vein thrombosis, however, can usually be diagnosed very clearly using radiology and there is also a very effective interventional radiological treatment. Chronic diseases very often culminate in liver cirrhosis which is highly associated with an increased risk of liver cancer. (orig.) [German] Neben den fokalen Leberlaesionen stellen diffuse und vaskulaere Lebererkrankungen ein weites Spektrum an Erkrankungen der Leber dar, die radiologisch oft schwer oder gar nicht diagnostizierbar sind. Klassische diagnostische Verfahren sind dabei neben dem Ultraschall die Computertomographie und die Magnetresonanztomographie. Diffuse Parenchymschaeden, bedingt durch Erkrankungen unterschiedlichster Aetiologie, sind deshalb schwierig evaluierbar, weil haeufig charakteristische bildmorphologische Merkmale fehlen. Die Steatosis hepatis, die Haemochromatose/Siderose als Beispiel der Speicherkrankheiten sowie die Sarkoidose und die Candidose als infektioes-entzuendliche Erkrankungen sind einer bildbasierten Diagnosestellung z. T. zugaenglich, bei den meisten diffusen Lebererkrankungen jedoch zeigen sich lediglich unspezifische

  13. Imaging of macrophage-related lung diseases

    Marten, Katharina; Hansell, David M. [Royal Brompton Hospital, Department of Radiology, London (United Kingdom)


    Macrophage-related pulmonary diseases are a heterogeneous group of disorders characterized by macrophage accumulation, activation or dysfunction. These conditions include smoking-related interstitial lung diseases, metabolic disorders such as Niemann-Pick or Gaucher disease, and rare primary lung tumors. High-resolution computed tomography abnormalities include pulmonary ground-glass opacification secondary to infiltration by macrophages, centrilobular nodules or interlobular septal thickening reflecting peribronchiolar or septal macrophage accumulation, respectively, emphysema caused by macrophage dysfunction, and honeycombing following macrophage-related lung matrix remodeling. (orig.)

  14. Detecting lung cancer symptoms with analogic CNN algorithms based on a constrained diffusion template

    Hirakawa, Satoshi; Nishio, Yoshifumi; Ushida, Akio; Ueno, Junji; Kasem, I.; Nishitani, Hiromu [Tokushima Univ. (Japan); Rekeczky, C.; Roska, T.


    In this article, a new type of diffusion template and an analogic CNN algorithm using this diffusion template for detecting some lung cancer symptoms in X-ray films are proposed. The performance of the diffusion template is investigated and our CNN algorithm is verified to detect some key lung cancer symptoms, successfully. (author)

  15. Diagnosis and treatment of cystic lung disease

    Park, Sanghoon; Lee, Eun Joo


    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

  16. Rheumatic manifestations associated with lung diseases

    Marco Aurelio Scarpinella Bueno


    Full Text Available Rheumatic manifestations in the more prevalent lung diseasessuch as asthma, chronic pulmonary disease or pneumonia are notfrequent. Exceptions to this rule are represented by lung cancerand sarcoidosis, where the appearance of the digital clubbing,hypertrophic osteoarthropathy, and arthtritis can correspond to thefi rst symptoms of the illness.

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

    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)


    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.)

  18. Video-assisted medical thoracoscopic (VAMT lung biopsy in the diagnosis of diffuse pulmonary infiltrates

    Tamer Elhadidy


    Conclusion: we concluded that lung sampling can be performed safely by VAMT (video assisted medical thoracoscopy with a good diagnostic yield in cases of diffuse lung infiltrates of unknown origin and represent an excellent alternative for VATS and open biopsy.

  19. Long-Term Control Medications for Lung Diseases

    ... medications are taken daily to control and prevent lung disease symptoms. These medicines should be taken every day ... long-acting beta-agonist. They improve symptoms of lung disease and increase lung function. Inhaled Steroids Inhaled steroids ...

  20. Autophagy and Obesity-Related Lung Disease.

    Pabon, Maria A; Ma, Kevin C; Choi, Augustine M K


    Obesity-related disease is a significant source of premature death and economic burden globally. It is also a common comorbidity in patients suffering from lung disease, affecting both severity and treatment success. However, this complex association between obesity and the lung is poorly understood. Autophagy is a self-recycling homeostatic process that has been linked to beneficial or deleterious effects, depending on the specific lung disease. Obesity affects autophagy in a tissue-specific manner, activating autophagy in adipocytes and impairing autophagy in hepatocytes, immune cells, and pancreatic β-cells, among others. Obesity is also characterized by chronic low-grade inflammation that can be modulated by the pro- and antiinflammatory effects of the autophagic machinery. Scant evidence exists regarding the impact of autophagy in obesity-related lung diseases, but there are communal pathways that could be related to disease pathogenesis. Important signaling molecules in obesity, including IL-17, leptin, adiponectin, NLRP3 inflammasome, and TLR-4, have been implicated in the pathogenesis of lung disease. These mediators are known to be modulated by autophagy activity. In this perspective, we highlight the recent advances in the understanding of autophagy in obesity-related conditions, as well as the potential mechanisms that can link autophagy and obesity in the pathogenesis of lung disease.

  1. Cystic fibrosis lung disease in adult patients.

    Vender, Robert L


    As the longevity of all patients with cystic fibrosis (CF) continues to increase (median 2005 survival=36.8 years), more adult patients will be receiving their medical care from nonpediatric adult-care providers. Cystic fibrosis remains a fatal disease, with more than 80% of patients dying after the age of 18 years, and most deaths resulting from pulmonary disease. The changing epidemiology requires adult-care providers to become knowledgeable and competent in the clinical management of adults with CF. Physicians must understand the influence of specific genotype on phenotypic disease presentation and severity, the pathogenic factors determining lung disease onset and progression, the impact of comorbid disease factors such as CF-related diabetes and malnutrition upon lung disease severity, and the currently approved or standard accepted therapies used for chronic management of CF lung disease. This knowledge is critical to help alleviate morbidity and improve mortality for the rapidly expanding population of adults with CF.

  2. NOD-like receptors in lung diseases

    Catherine eChaput


    Full Text Available The lung is a particularly vulnerable organ at the interface of the body and the exterior environment. It is constantly exposed to microbes and particles by inhalation. The innate immune system needs to react promptly and adequately to potential dangers posed by these microbes and particles, while at the same time avoiding extensive tissue damage. NOD-like receptors (NLRs represent a group of key sensors for microbes and damage in the lung. As such they are important players in various infectious as well as acute and chronic sterile inflammatory diseases, such as pneumonia, chronic obstructive lung disease (COPD, acute lung injury/ARDS, pneumoconiosis and asthma. Activation of most known NLRs leads to the production and release of pro-inflammatory cytokines, and/or to the induction of cell death. We will review NLR functions in the lung during infection and sterile inflammation.

  3. Lung Cancer Mortality among Uranium Gaseous Diffusion Plant Workers: A Cohort Study 1952–2004


    Background: 9%–15% of all lung cancers are attributable to occupational exposures. Reports are disparate regarding elevated lung cancer mortality risk among workers employed at uranium gaseous diffusion plants.Objective: To investigate whether external radiation exposure is associated with lung cancer mortality risk among uranium gaseous diffusion workers.Methods: A cohort of 6820 nuclear industry workers employed from 1952 to 2003 at the Paducah uranium gaseous diffusion plant (PGDP) was ass...

  4. Surfactant gene polymorphisms and interstitial lung diseases

    Pantelidis Panagiotis


    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.

  5. Drug induced lung disease; Medikamenteninduzierte Lungenveraenderungen

    Schaefer-Prokop, Cornelia [Medizinisches Zentrum Meander (MMC) Amersfoort (Netherlands). Abt. fuer Radiologie; Eisenhuber, Edith [Krankenhaus Goettlicher Heiland, Wien (Austria). Abt. fuer Radiologie


    There is an ever increasing number of drugs that can cause lung disease. Imaging plays an important role in the diagnosis, since the clinical symptoms are mostly nonspecific. Various HRCT patterns can be correlated - though with overlaps - to lung changes caused by certain groups of drugs. Alternative diagnosis such as infection, edema or underlying lung disease has to be excluded by clinical-radiological means. Herefore is profound knowledge of the correlations of drug effects and imaging findings essential. History of drug exposure, suitable radiological findings and response to treatment (corticosteroids and stop of medication) mostly provide the base for the diagnosis. (orig.)

  6. Warning Signs of Lung Disease

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

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


    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.)

  8. Exercise-induced haemoptysis as a rare presentation of a rare lung disease.

    Mihalek, Andrew D; Haney, Carissa; Merino, Maria; Roy-Chowdhuri, Sinchita; Moss, Joel; Olivier, Kenneth N


    Amyloid primarily affecting the lungs is a seldom seen clinical entity. This case discusses the work-up of a patient presenting with exercise-induced haemoptysis and diffuse cystic lung disease on radiographic imaging. The common clinical and radiographic findings of diffuse cystic lung diseases as well as a brief overview of pulmonary amyloid are presented. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to

  9. Interstitial lung disease in systemic sclerosis.

    Wells, Athol U


    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.

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

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


    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.

  11. The bacterial microbiota in inflammatory lung diseases.

    Huffnagle, Gary B; Dickson, Robert P


    Numerous lines of evidence, ranging from recent studies back to those in the 1920s, have demonstrated that the lungs are NOT bacteria-free during health. We have recently proposed that the entire respiratory tract should be considered a single ecosystem extending from the nasal and oral cavities to the alveoli, which includes gradients and niches that modulate microbiome dispersion, retention, survival and proliferation. Bacterial exposure and colonization of the lungs during health is most likely constant and transient, respectively. Host microanatomy, cell biology and innate defenses are altered during chronic lung disease, which in turn, alters the dynamics of bacterial turnover in the lungs and can lead to longer term bacterial colonization, as well as blooms of well-recognized respiratory bacterial pathogens. A few new respiratory colonizers have been identified by culture-independent methods, such as Pseudomonas fluorescens; however, the role of these bacteria in respiratory disease remains to be determined.

  12. The Bacterial Microbiota in Inflammatory Lung Diseases

    Huffnagle, Gary B.; Dickson, Robert P.


    Numerous lines of evidence, ranging from recent studies back to those in the 1920's, have demonstrated that the lungs are NOT bacteria-free during health. We have recently proposed that the entire respiratory tract should be considered a single ecosystem extending from the nasal and oral cavities to the alveoli, which includes gradients and niches that modulate microbiome dispersion, retention, survival and proliferation. Bacterial exposure and colonization of the lungs during health is most likely constant and transient, respectively. Host microanatomy, cell biology and innate defenses are altered during chronic lung disease, which in turn, alters the dynamics of bacterial turnover in the lungs and can lead to longer term bacterial colonization, as well as blooms of well-recognized respiratory bacterial pathogens. A few new respiratory colonizers have been identified by culture-independent methods, such as Pseudomonas fluorescens; however, the role of these bacteria in respiratory disease remains to be determined. PMID:26122174

  13. Uncommon causes of occupational interstitial lung diseases.

    Gong, H


    Uncommon causes of occupational interstitial lung disease, or pneumoconiosis, are being increasingly recognized and diagnosed. The fibrogenic potential of numerous types of respirable inorganic particles remains poorly understood but is significantly determined by lung deposition and clearance, the agent's size and solubility, host susceptibility, and other factors. Microanalytic techniques have improved the identification of uncommon or unusual biopersistent particles or elements in fibrotic lung tissue. Recent findings in workers exposed to manmade vitreous fibers, silicon carbide, talc, titanium, cerium, and polyvinyl chloride provide new clinical insights into not only their specific fibrogenic capabilities but also in the broader appreciation that many cases of unexplained interstitial lung disease may be caused by occupational exposures to one or more uncommon airborne substances.

  14. Telomere length in interstitial lung diseases

    Snetselaar, Reinier; Van Moorsel, Coline H M; Kazemier, Karin M.; Van Der Vis, Joanne J.; Zanen, Pieter; Van Oosterhout, Matthijs F M; Grutters, Jan C.


    Background: Interstitial lung disease (ILD) is a heterogeneous group of rare diseases that primarily affect the pulmonary interstitium. Studies have implicated a role for telomere length (TL) maintenance in ILD, particularly in idiopathic interstitial pneumonia (IIP). Here, we measure TL in a wide

  15. Inflammatory Lung Disease in Rett Syndrome

    Claudio De Felice


    Full Text Available Rett syndrome (RTT is a pervasive neurodevelopmental disorder mainly linked to mutations in the gene encoding the methyl-CpG-binding protein 2 (MeCP2. Respiratory dysfunction, historically credited to brainstem immaturity, represents a major challenge in RTT. Our aim was to characterize the relationships between pulmonary gas exchange abnormality (GEA, upper airway obstruction, and redox status in patients with typical RTT (n = 228 and to examine lung histology in a Mecp2-null mouse model of the disease. GEA was detectable in ~80% (184/228 of patients versus ~18% of healthy controls, with “high” (39.8% and “low” (34.8% patterns dominating over “mixed” (19.6% and “simple mismatch” (5.9% types. Increased plasma levels of non-protein-bound iron (NPBI, F2-isoprostanes (F2-IsoPs, intraerythrocyte NPBI (IE-NPBI, and reduced and oxidized glutathione (i.e., GSH and GSSG were evidenced in RTT with consequently decreased GSH/GSSG ratios. Apnea frequency/severity was positively correlated with IE-NPBI, F2-IsoPs, and GSSG and negatively with GSH/GSSG ratio. A diffuse inflammatory infiltrate of the terminal bronchioles and alveoli was evidenced in half of the examined Mecp2-mutant mice, well fitting with the radiological findings previously observed in RTT patients. Our findings indicate that GEA is a key feature of RTT and that terminal bronchioles are a likely major target of the disease.

  16. Lung transplantation for chronic obstructive pulmonary disease

    Liou TG


    Full Text Available Theodore G Liou, Sanjeev M Raman, Barbara C CahillDivision of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Medicine, School of Medicine, University of Utah, Salt Lake City, Utah, USAAbstract: Patients with end-stage chronic obstructive pulmonary disease (COPD comprise the largest single lung disease group undergoing transplantation. Selection of appropriate candidates requires consideration of specific clinical characteristics, prognosis in the absence of transplantation, and likely outcome of transplantation. Increased availability of alternatives to transplantation for end-stage patients and the many efforts to increase the supply of donor organs have complicated decision making for selecting transplant candidates. Many years of technical and clinical refinements in lung transplantation methods have improved survival and quality of life outcomes. Further advances will probably come from improved selection methods for the procedure. Because no prospective trial has been performed, and because of confounding and informative censoring bias inherent in the transplant selection process in studies of the existing experience, the survival effect of lung transplant in COPD patients remains undefined. There is a lack of conclusive data on the impact of lung transplantation on quality of life. For some patients with end-stage COPD, lung transplantation remains the only option for further treatment with a hope of improved survival and quality of life. A prospective trial of lung transplantation is needed to provide better guidance concerning survival benefit, resource utilization, and quality of life effects for patients with COPD.Keywords: outcomes, emphysema, COPD, alpha-1-antitrypsin deficiency, survival, single lung transplant, bilateral sequential single lung transplant, lung volume reduction, referral, guidelines, health related quality of life

  17. Nitrofurantoin-induced immune-mediated lung and liver disease

    Milić Rade


    Full Text Available Introduction. Nitrofurantoin, a furan derivative, introduced in the fifties has widely been used as an effective agent for the treatment and prevention of urinary tract infections (UTI. Spectrum of adverse reactions to nitrofurantoin is wide, ranging from eosinophilic interstitial lung disease, acute hepatitis and granulomatous reaction, to the chronic active hepatitis, a very rare adverse effect, that can lead to cirrhosis and death. Case report. We presented a 55-year-old female patient with eosinophilic interstitial lung disease, severe chronic active hepatitis and several other immune- mediated multisystemic manifestations of prolonged exposure to nitrofurantoin because of the recurrent UTI caused by Escherichia coli. We estimated typical radiographic and laboratory disturbances, also restrictive ventilatory changes, severe reduction of carbon monoxide diffusion capacity and abnormal liver function tests. Lymphocytic-eosinophylic alveolitis was consistent with druginduced reaction. Hepatitis was confirmed by liver biopsy. After withdrawal of nitrofurantoin and application of high dose of glicocorticosteroids, prompt clinical and laboratory recovery was achieved. Conclusion. Adverse drug reactions should be considered in patients with concomitant lung and liver disease. The mainstay of treatment is drug withdrawal and the use of immunosuppressive drugs in severe cases. Consideration should be given to monitor lung and liver function tests during long term nitrofurantoin therapy.

  18. The Therapeutic Potential of Differentiated Lung Cells from Embryonic Stem Cells in Lung Diseases.

    Mokhber Dezfouli, Mohammad Reza; Chaleshtori, Sirous Sadeghian; Dehghan, Mohammad Mehdi; Tavanaeimanesh, Hamid; Baharvand, Hossein; Tahamtani, Yaser


    Lung diseases cause great morbidity and mortality. The choice of effective medical treatment is limited and the number of lung diseases are difficult to treat with current treatments. The embryonic stem cells (ESCs) have the potential to differentiate into cell types of all three germinal layers, including lung epithelial cells. So they can be a potential source for new cell therapies for hereditary or acquired diseases of the airways and lungs. One method for treatment of lung diseases is cell therapy and the use of ESCs that can replace the damaged epithelial and endothelial cells. Progress using ESCs has developed slowly for lung regeneration because differentiation of lung cells from ESCs is more difficult as compared to differentiation of other cells. The review studies the therapeutic effects of differentiated lung cells from embryonic stem cells in lung diseases. There are few studies of differentiation of ESCs into a lineage of respiratory and then investigation of this cell in experimental model of lung diseases.

  19. [Lung transplantation in pulmonary fibrosis and other interstitial lung diseases].

    Berastegui, Cristina; Monforte, Victor; Bravo, Carlos; Sole, Joan; Gavalda, Joan; Tenório, Luis; Villar, Ana; Rochera, M Isabel; Canela, Mercè; Morell, Ferran; Roman, Antonio


    Interstitial lung disease (ILD) is the second indication for lung transplantation (LT) after emphysema. The aim of this study is to review the results of LT for ILD in Hospital Vall d'Hebron (Barcelona, Spain). We retrospectively studied 150 patients, 87 (58%) men, mean age 48 (r: 20-67) years between August 1990 and January 2010. One hundred and four (69%) were single lung transplants (SLT) and 46 (31%) bilateral-lung transplants (BLT). The postoperative diagnoses were: 94 (63%) usual interstitial pneumonia, 23 (15%) nonspecific interstitial pneumonia, 11 (7%) unclassifiable interstitial pneumonia and 15% miscellaneous. We describe the functional results, complications and survival. The actuarial survival was 87, 70 and 53% at one, 3 and 5 years respectively. The most frequent causes of death included early graft dysfunction and development of chronic rejection in the form of bronchiolitis obliterans (BOS). The mean postoperative increase in forced vital capacity and forced expiratory volume in the first second (FEV1) was similar in SLT and BLT. The best FEV1 was reached after 10 (r: 1-36) months. Sixteen percent of patients returned to work. At some point during the evolution, proven acute rejection was diagnosed histologically in 53 (35%) patients. The prevalence of BOS among survivors was 20% per year, 45% at 3 years and 63% at 5 years. LT is the best treatment option currently available for ILD, in which medical treatment has failed. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

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

    Aygül Güzel


    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.

  1. Relationship of lung function to radiographic reading (ILO) in patients with asbestos related lung disease.

    Cotes, J E; King, B


    The 1980 International Labour Office (ILO) classification of posteroanterior chest radiographs was used to obtain the scores for profusion of small opacities and pleural abnormalities of 172 men with confirmed or suspected disease of the lungs due to asbestos. After allowance had been made for age, stature, and smoking habit the quantitative score for area of diffuse pleural thickening seen in profile on both lateral chest walls contributed to reductions in inspiratory capacity, expiratory reserve volume, and forced expiratory flow rates. Occlusion of one or both costophrenic angles in the presence of diffuse thickening was associated with further reduction in inspiratory capacity. Profusion of small opacities was associated with a reduction in transfer factor. Diffuse pleural thickening and occlusion of costophrenic angles were associated with relatively low values for the forced expiratory flow rates (MEF50FVC) and FEV1/FVC, whereas small opacities were associated with relatively high values. Thus overall increased, normal, or reduced values of MEF50FVC and FEV1/FVC might occur, depending on the distribution of the radiographic abnormalities. The findings contribute to the validation of the ILO pleural scores; those for diffuse pleural thickening and occlusion of costophrenic angles should be used jointly with the scores for profusion of parenchymal small opacities in interpreting the lung function of persons exposed to asbestos.

  2. Lung Surfactant and Its Use in Lung Diseases

    O. A. Rosenberg


    Full Text Available The review considers the present views of lung surfactant (LS functions with emphasis on its protective and barrier properties and ability to maintain local and adaptive immunity. The composition of commercial LS formulations is analyzed. Data on qualitative and quantitative LS abnormalities are presented in various diseases in neonates and adults. The results of clinical trials of different LS formulations in the treatment of acute respiratory distress syndrome in adults are analyzed in detail. Recent data on the results of and prospects for surfactant therapy for bronchial asthma, chronic obstructive pulmonary disease and pulmonary tuberculosis are given. 

  3. Lung involvement in systemic connective tissue diseases

    Plavec Goran


    Full Text Available Background/Aim. Systemic connective tissue diseases (SCTD are chronic inflammatory autoimmune disorders of unknown cause that can involve different organs and systems. Their course and prognosis are different. All of them can, more or less, involve the respiratory system. The aim of this study was to find out the frequency of respiratory symptoms, lung function disorders, radiography and high-resolution computerized tomography (HRCT abnormalities, and their correlation with the duration of the disease and the applied treatment. Methods. In 47 non-randomized consecutive patients standard chest radiography, HRCT, and lung function tests were done. Results. Hypoxemia was present in nine of the patients with respiratory symptoms (20%. In all of them chest radiography was normal. In five of these patients lung fibrosis was established using HRCT. Half of all the patients with SCTD had symptoms of lung involvement. Lung function tests disorders of various degrees were found in 40% of the patients. The outcome and the degree of lung function disorders were neither in correlation with the duration of SCTD nor with therapy used (p > 0.05 Spearmans Ro. Conclusion. Pulmonary fibrosis occurs in about 10% of the patients with SCTD, and possibly not due to the applied treatment regimens. Hypoxemia could be a sing of existing pulmonary fibrosis in the absence of disorders on standard chest radiography.

  4. Lung Compliance and Chronic Obstructive Pulmonary Disease

    D. Papandrinopoulou


    Full Text Available Chronic obstructive pulmonary disease, namely, pulmonary emphysema and chronic bronchitis, is a chronic inflammatory response of the airways to noxious particles or gases, with resulting pathological and pathophysiological changes in the lung. The main pathophysiological aspects of the disease are airflow obstruction and hyperinflation. The mechanical properties of the respiratory system and its component parts are studied by determining the corresponding volume-pressure (P-V relationships. The consequences of the inflammatory response on the lung structure and function are depicted on the volume-pressure relationships.

  5. [Parasternal mediastino-pleuroscopy in the diagnosis of diffuse lesions of the lungs].

    Motus, I Ia


    A procedure for parasternal mediastinopleuroscopy (PMS) has been proposed for biopsy of pulmonary tissue and intrasternal lymph nodes. A surgical technique of intervention is described in the paper. PMS was performed in 81 patients with diffuse lesions of the lungs and the disease was morphologically verified. The clinical and X-ray diagnosis was found to be incoincident with their final morphological diagnosis after PMS had been performed in 48 out of 81 patients. To obtain an informative biopsy specimen needed for a morphological diagnosis, biopsies of the lung and lymph nodes should be taken in all cases. Complications occurred in 2 patients. These included bleedings from the intercostal arteries, which required vascular ligation.

  6. Clinical evaluation of bilateral diffuse lung uptake of sup 67 Ga-citrate

    Kobayashi, Hidetoshi; Tanaka, Kouji (Gifu Prefectural Tajimi Hospital (Japan)); Sakuma, Sadayuki


    This is a retrospective study of 23 patients in whom bilateral diffuse lung uptake (BDLU) of radioactivity was seen on Ga-67 scans. Of the 23 patients, 10 patients were histologically or clinically diagnosed as having respiratory diffuse or active diseases, consisting of lung cancer (5), pulmonary tuberculosis (3), silicosis (one), lung fibrosis (one), 4 as having malignant lymphoma, 3 as having multiple myeloma, and single patients as having chronic myelocytic leukemia, hepatoma, bladder cancer, skin cancer, cholecystitis, and fever of unknown origin. In all of the patients, except for one, the estimated causes of BDLU were clarified as follows: silicosis in 9 patients, pleuritis in 4, chemotherapy in 6, and radiation pneumonitis, and tuberculosis in each one. The extent of BDLU on Ga-67 scans was larger than lesions seen on chest X-ray films. This implies the involvement of blood flow changes in Ga-67 uptake. BDLU on Ga-67 scans, except for cases of pleuritis associated with malignant lymphoma, did not imply poor prognosis. Silicosis accounted for 40% of all patients, which may reflect the endemic prevalence in the present population living in a pottery district. (N.K.).

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

    Fink, L


    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.

  8. Drug induced lung disease - amiodarone in focus

    Vasić Nada R.


    Full Text Available More than 380 medications are known to cause pulmonary toxicity. Selected drugs that are important causes of pulmonary toxicity fall into the following classes: cytotoxic, cardiovascular, anti-inflammatory, antimicrobial, illicit drugs, miscellaneous. The adverse reactions can involve the pulmonary parenchyma, pleura, the airways, pulmonary vascular system, and mediastinum. Drug-induced lung diseases have no pathognomonic clinical, laboratory, physical, radiographic or histological findings. A drug-induced lung disease is usually considered a diagnosis of exclusion of other diseases. The diagnosis of drug-mediated pulmonary toxicity is usually made based on clinical findings. In general, laboratory analyses do not help in establishing the diagnosis. High-resolution computed tomography scanning is more sensitive than chest radiography for defining radiographic abnormalities. The treatment of drug-induced lung disease consists of immediate discontinuation of the offending drug and appropriate management of the pulmonary symptoms. Glucocorticoids have been associated with rapid improvement in gas exchange and reversal of radiographic abnormalities. Before starting any medication, patients should be educated about the potential adverse effects of the drug. Amiodarone is an antiarrhythmic agent used in the treatment of many types of tachyarrhythmia. Amiodarone-caused pulmonary toxicity is a well-known side effect (complication of this medication. The incidence of amiodarone-induced lung disease is approximately 5-7%.

  9. Autophagy in lung disease pathogenesis and therapeutics

    Stefan W. Ryter


    Full Text Available Autophagy, a cellular pathway for the degradation of damaged organelles and proteins, has gained increasing importance in human pulmonary diseases, both as a modulator of pathogenesis and as a potential therapeutic target. In this pathway, cytosolic cargos are sequestered into autophagosomes, which are delivered to the lysosomes where they are enzymatically degraded and then recycled as metabolic precursors. Autophagy exerts an important effector function in the regulation of inflammation, and immune system functions. Selective pathways for autophagic degradation of cargoes may have variable significance in disease pathogenesis. Among these, the autophagic clearance of bacteria (xenophagy may represent a crucial host defense mechanism in the pathogenesis of sepsis and inflammatory diseases. Our recent studies indicate that the autophagic clearance of mitochondria, a potentially protective program, may aggravate the pathogenesis of chronic obstructive pulmonary disease by activating cell death programs. We report similar findings with respect to the autophagic clearance of cilia components, which can contribute to airways dysfunction in chronic lung disease. In certain diseases such as pulmonary hypertension, autophagy may confer protection by modulating proliferation and cell death. In other disorders, such as idiopathic pulmonary fibrosis and cystic fibrosis, impaired autophagy may contribute to pathogenesis. In lung cancer, autophagy has multiple consequences by limiting carcinogenesis, modulating therapeutic effectiveness, and promoting tumor cell survival. In this review we highlight the multiple functions of autophagy and its selective autophagy subtypes that may be of significance to the pathogenesis of human disease, with an emphasis on lung disease and therapeutics.

  10. Asbestos-induced lung disease in small-scale clutch manufacturing workers

    Gothi, Dipti; Gahlot, Tanushree; Sah, Ram B.; Saxena, Mayank; Ojha, U. C.; Verma, Anand K.; Spalgais, Sonam


    Background: The crocidolite variety of asbestos is banned. However, chrysotile, which is not prohibited, is still used in developing countries in making products such as clutch plate. Fourteen workers from a small-scale clutch plate-manufacturing factory were analyzed for asbestos-induced lung disease as one of their colleagues had expired due to asbestosis. Aims: This study was conducted to evaluate the awareness of workers, the prevalence and type of asbestos-induced lung disease, and the sensitivity and specificity of diffusion test. Materials and Methods: History, examination, chest radiograph, spirometry with diffusion, and high resolution computed tomography (HRCT) thorax was performed in all the workers. The diagnosis of asbestos-induced lung disease was suspected on the basis of HRCT. This was subsequently confirmed on transbronchial lung biopsy (TBLB). Results: None of the workers had detailed information about asbestos and its ill effects. Eleven out of 14 (71.42%) workers had asbestos-induced lung disease. All 11 had small airway disease (SAD). Three had SAD alone, 6 had additional interstitial lung disease (ILD), and 2 patients had additional ILD and chronic obstructive pulmonary disease. Sensitivity and specificity of residual volume (RV) or total lung capacity (TLC) for detecting SAD was 90% and 100%, respectively, and that of diffusion capacity of lung for carbon monoxide (DLCO) for detecting ILD was 100%. Conclusion: The awareness about asbestos in small-scale clutch-plate manufacturing industry is poor. The usage of chrysotile should be strictly regulated as morbidity and mortality is high. DLCO and RV/TLC are sensitive and specific in detecting nonmalignant asbestos induced lung disease.

  11. Microstructural alterations of sputum in cystic fibrosis lung disease

    Duncan, Gregg A.; Jung, James; Joseph, Andrea; Thaxton, Abigail L.; West, Natalie E.; Boyle, Michael P.; Hanes, Justin


    The stasis of mucus secretions in the lungs of cystic fibrosis (CF) patients leads to recurrent infections and pulmonary exacerbations, resulting in decreased survival. Prior studies have assessed the biochemical and biophysical features of airway mucus in individuals with CF. However, these measurements are unable to probe mucus structure on microscopic length scales relevant to key players in the progression of CF-related lung disease, namely, viruses, bacteria, and neutrophils. In this study, we quantitatively determined sputum microstructure based on the diffusion of muco-inert nanoparticle probes in CF sputum and found that a reduction in sputum mesh pore size is characteristic of CF patients with reduced lung function, as indicated by measured FEV1. We also discovered that the effect of ex vivo treatment of CF sputum with rhDNase I (Pulmozyme) on microstructure is dependent upon the time interval between the most recent inhaled rhDNase I treatment and the sample collection. Microstructure of mucus may serve as a marker for the extent of CF lung disease and as a parameter for assessing the effectiveness of mucus-altering agents. PMID:27812540

  12. Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease.

    De Santis, Maria; Bosello, Silvia Laura; Peluso, Giusy; Pinnelli, Michela; Alivernini, Stefano; Zizzo, Gaetano; Bocci, Mario; Capacci, Annunziata; La Torre, Giuseppe; Mannocci, Alice; Pagliari, Gabriella; Varone, Francesco; Pistelli, Roberto; Danza, Francesco Maria; Ferraccioli, Gianfranco


    So far no clinical or experimental evidences clearly explain how and which systemic sclerosis (SSc) patients will experience a functional and radiological progression of interstitial lung disease (ILD). The aim of the study was to investigate whether any bronchoalveolar lavage fluid (BALF) characteristic, compared with clinical, functional and radiological parameters, is associated with the risk of progression of ILD and worse survival in SSc patients. Lung involvement was evaluated in 110 consecutively examined SSc patients with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT); 73 patients with evidence of ILD on HRCT underwent BAL. The progression of ILD was evaluated with PFTs and HRCT after 1-year follow-up. A 36-month survival analysis was assessed. ILD patients with alveolitis had a higher risk to have restrictive lung disease and honeycombing, to experience a worsening in honeycombing score or to develop honeycombing. ILD progression was associated with the evidence of honeycombing on HRCT, with the presence of eosinophils, with an inverted CD4/CD8 ratio and with a higher CD19 percentage count in the BALF or with a positive BALF microbiological culture. The patients with ILD had a worse overall survival. The diffuse disease was the only independent risk factor of overall mortality, and the extent of honeycombing on HRCT was the only independent risk factor of lung disease-related mortality. Our study suggests the importance of evaluating ILD with HRCT and BAL in order to characterize the risk factors of SSc lung involvement progression. © 2010 Blackwell Publishing Ltd.

  13. Computational modeling of the obstructive lung diseases asthma and COPD.

    Burrowes, Kelly Suzanne; Doel, Tom; Brightling, Chris


    Asthma and chronic obstructive pulmonary disease (COPD) are characterized by airway obstruction and airflow imitation and pose a huge burden to society. These obstructive lung diseases impact the lung physiology across multiple biological scales. Environmental stimuli are introduced via inhalation at the organ scale, and consequently impact upon the tissue, cellular and sub-cellular scale by triggering signaling pathways. These changes are propagated upwards to the organ level again and vice versa. In order to understand the pathophysiology behind these diseases we need to integrate and understand changes occurring across these scales and this is the driving force for multiscale computational modeling. There is an urgent need for improved diagnosis and assessment of obstructive lung diseases. Standard clinical measures are based on global function tests which ignore the highly heterogeneous regional changes that are characteristic of obstructive lung disease pathophysiology. Advances in scanning technology such as hyperpolarized gas MRI has led to new regional measurements of ventilation, perfusion and gas diffusion in the lungs, while new image processing techniques allow these measures to be combined with information from structural imaging such as Computed Tomography (CT). However, it is not yet known how to derive clinical measures for obstructive diseases from this wealth of new data. Computational modeling offers a powerful approach for investigating this relationship between imaging measurements and disease severity, and understanding the effects of different disease subtypes, which is key to developing improved diagnostic methods. Gaining an understanding of a system as complex as the respiratory system is difficult if not impossible via experimental methods alone. Computational models offer a complementary method to unravel the structure-function relationships occurring within a multiscale, multiphysics system such as this. Here we review the currentstate

  14. Prevalence and prognosis of unclassifiable interstitial lung disease.

    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


    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.



    Apr 2, 2011 ... The mechanisms behind accelerated emphysema in adults with HIV infection and the HIV-infected smoking population are both ... HI virus itself is a predisposing factor in the pathogenesis of bullous lung disease.1-3. In 1989 ...

  16. Lymphomatoid granulomatosis mimicking interstitial lung disease.

    Braham, Emna; Ayadi-Kaddour, Aïda; Smati, Belhassen; Ben Mrad, Sonia; Besbes, Mohammed; El Mezni, Faouzi


    Lymphoid granulomatosis is a rare form of pulmonary angiitis. This case report presents a patient with lymphoid granulomatosis in whom the clinical presentation, radiological features and the partial response to corticosteroid therapy mimicked interstitial lung disease. Lymphoid granulomatosis was only diagnosed at post-mortem examination. The range of reported clinical presentations, diagnostic approaches and outcomes are described.

  17. Interstitial lung disease probably caused by imipramine.

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


    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.

  18. Pathophysiology of Pulmonary Hypertension in Chronic Parenchymal Lung Disease.

    Singh, Inderjit; Ma, Kevin Cong; Berlin, David Adam


    Pulmonary hypertension commonly complicates chronic obstructive pulmonary disease and interstitial lung disease. The association of chronic lung disease and pulmonary hypertension portends a worse prognosis. The pathophysiology of pulmonary hypertension differs in the presence or absence of lung disease. We describe the physiological determinants of the normal pulmonary circulation to better understand the pathophysiological factors implicated in chronic parenchymal lung disease-associated pulmonary hypertension. This review will focus on the pathophysiology of 3 forms of chronic lung disease-associated pulmonary hypertension: idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease, and sarcoidosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. How Are Asbestos-Related Lung Diseases Treated?

    ... the NHLBI on Twitter. How Are Asbestos-Related Lung Diseases Treated? No treatments can reverse the effects of ... ease pain, or relieve other complications of your disease. If you have lung cancer or mesothelioma, talk with your doctor about ...

  20. Protein misfolding and obstructive lung disease.

    Greene, Catherine M


    The endoplasmic reticulum has evolved a number of mechanisms to manage the accumulation of incorrectly folded proteins. This results in loss of function of these proteins, but occasionally, in conditions such as α-1 antitrpysin (A1AT) deficiency, the misfolded protein can acquire a toxic gain of function promoting exaggerated ER stress responses and inflammation. Mutations leading to deficiency in a second serine proteinase inhibitor, α-1 antichymotrpysin (ACT), can induce potentially similar consequences. A1AT and ACT deficiencies are associated with chronic obstructive lung disease. Until recently, it was thought that the lung diseases associated with these conditions were entirely due to loss of antiprotease protection in the lung (i.e., loss of function), whereas gain of function was the major cause of the liver disease associated with A1AT deficiency. This paradigm is being increasingly challenged because ER stress is being recognized in bronchial epithelial cells and inflammatory cells normally resident in the lung, giving rise to an inflammatory phenotype that adds to the proteolytic burden associated with these conditions. In this article, we describe the cellular mechanisms that are activated to cope with an increasing burden of misfolded proteins within the ER in A1AT and ACT deficiency, show how these events are linked to inflammation, and outline the therapeutic strategies that can potentially interfere with production of misfolded proteins.

  1. Interstitial lung disease in the connective tissue diseases.

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


    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.

  2. Translational models of lung disease.

    Mercer, Paul F; Abbott-Banner, Katharine; Adcock, Ian M; Knowles, Richard G


    The 2nd Cross Company Respiratory Symposium (CCRS), held in Horsham, U.K. in 2012, brought together representatives from across the pharmaceutical industry with expert academics, in the common interest of improving the design and translational predictiveness of in vivo models of respiratory disease. Organized by the respiratory representatives of the European Federation of Pharmaceutical Industries and Federations (EFPIA) group of companies involved in the EU-funded project (U-BIOPRED), the aim of the symposium was to identify state-of-the-art improvements in the utility and design of models of respiratory disease, with a view to improving their translational potential and reducing wasteful animal usage. The respiratory research and development community is responding to the challenge of improving translation in several ways: greater collaboration and open sharing of data, careful selection of the species, complexity and chronicity of the models, improved practices in preclinical research, continued refinement in models of respiratory diseases and their sub-types, greater understanding of the biology underlying human respiratory diseases and their sub-types, and finally greater use of human (and especially disease-relevant) cells, tissues and explants. The present review highlights these initiatives, combining lessons from the symposium and papers published in Clinical Science arising from the symposium, with critiques of the models currently used in the settings of asthma, idiopathic pulmonary fibrosis and COPD. The ultimate hope is that this will contribute to a more rational, efficient and sustainable development of a range of new treatments for respiratory diseases that continue to cause substantial morbidity and mortality across the world.

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

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


    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.

  4. SLPI and inflammatory lung disease in females.

    McKiernan, Paul J


    During the course of certain inflammatory lung diseases, SLPI (secretory leucoprotease inhibitor) plays a number of important roles. As a serine antiprotease it functions to protect the airways from proteolytic damage due to neutrophil and other immune cell-derived serine proteases. With respect to infection it has known antimicrobial and anti-viral properties that are likely to contribute to host defence. Another of its properties is the ability to control inflammation within the lung where it can interfere with the transcriptional induction of pro-inflammatory gene expression induced by NF-kappaB (nuclear factor kappaB). Thus, factors that regulate the expression of SLPI in the airways can impact on disease severity and outcome. Gender represents once such idiosyncratic factor. In females with CF (cystic fibrosis), it is now thought that circulating oestrogen contributes, in part, to the observed gender gap whereby females have worse disease and poorer prognosis than males. Conversely, in asthma, sufferers who are females have more frequent exacerbations at times of low-circulating oestrogen. In the present paper, we discuss how SLPI participates in these events and speculate on whether regulatory mechanisms such as post-transcriptional modulation by miRNAs (microRNAs) are important in the control of SLPI expression in inflammatory lung disease.

  5. Two novel mutations in surfactant protein-C, lung function and obstructive lung disease

    Baekvad-Hansen, Marie; Nordestgaard, Børge G; Tybjaerg-Hansen, Anne


    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....

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

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


    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.

  7. [Swimming pool lung -- extrinsic allergic alveolitis or mycobacterial disease?].

    Koschel, D; Pietrzyk, C; Sennekamp, J; Müller-Wening, D


    There have been several recent reports of pulmonary disease resulting from exposure to Mycobacterium avium complex in indoor hot tubs. The disease is thought to be due either to infection or extrinsic allergic alveolitis (EAA). In this report we describe the case of a patient who developed episodes of fever, dyspnea and cough 4-6 hours after cleaning his indoor swimming pool. A diagnosis of EAA was made on finding a restrictive lung function pattern with gas exchange abnormalities, a predominant lymphocytosis in the bronchoalveolar lavage, diffuse ground-glass opacities in the lower lobes on high-resolution computer tomography, and specific IgG antibody activity to the swimming pool water. There was no precipitin reaction or specific IgG antibody activity to microbes extracted from the water. Interestingly, the water contained Mycobacterium avium complex (MAC) in huge amounts and in this case the histopathological features of the lung biopsy specimens differed from those seen in typical EAA, but were similar to those described in "hot tub lung" caused by mycobacteria. Solely by avoidance of cleaning the swimming pool, without any pharmacological treatment, the patient recovered completely within three months. To the best of our knowledge, this is the first report of EAA possibly associated with MAC exposure in a swimming pool environment.

  8. Long-term efficacy of B cell depletion therapy on lung and skin involvement in diffuse systemic sclerosis.

    Bosello, Silvia L; De Luca, Giacomo; Rucco, Manuela; Berardi, Giorgia; Falcione, Matteo; Danza, Francesco Maria; Pirronti, Tommaso; Ferraccioli, Gianfranco


    To assess the long-term efficacy and safety of single and multiple courses of rituximab therapy in systemic sclerosis (SSc) patients with and without lung disease. A total of 20 SSc patients with a diffuse disease were treated with rituximab. At baseline and during follow-up the lung involvement was evaluated with pulmonary function tests (FVC and DLCO) and with lung high-resolution computed tomography (HRCT). The skin score, activity, and severity indices improved significantly after 12 months and at final follow-up compared to baseline. After 12 months, there was a significant increase of FVC and TLC compared to baseline (p = 0.024 and p = 0.005, respectively), while the mean DLCO value remained stable. Considering the last available follow-up in six patients with restrictive lung disease at baseline, two patients (33.3%) experienced an increase of more than 10% of FVC, one patient had a decrease of FVC >10%, while in three patients FVC remained stable (50%). After the mean follow-up of 48.5 ± 20.4 months, among the patients with normal lung parameters at baseline, FVC remained stable in 12 (85.7%) and in one patient (14.3%) it increased by more than 10%. At the final follow-up, the alveolar and interstitial HRCT scores remained stable in more than 80% of patients, both in patients with and without restrictive lung disease at baseline. Anti-CD20 B cell depletion therapy is effective on skin involvement but seems also to preserve the pulmonary function, as supported by a stable or improved FVC and stable interstitial score, suggesting a possible role of rituximab as a modifying therapy overall in early diffuse SSc. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Obstructive lung disease models: what is valid?

    Ferdinands, Jill M; Mannino, David M


    Use of disease simulation models has led to scrutiny of model methods and demand for evidence that models credibly simulate health outcomes. We sought to describe recent obstructive lung disease simulation models and their validation. Medline and EMBASE were used to identify obstructive lung disease simulation models published from January 2000 to June 2006. Publications were reviewed to assess model attributes and four types of validation: first-order (verification/debugging), second-order (comparison with studies used in model development), third-order (comparison with studies not used in model development), and predictive validity. Six asthma and seven chronic obstructive pulmonary disease models were identified. Seven (54%) models included second-order validation, typically by comparing observed outcomes to simulations of source study cohorts. Seven (54%) models included third-order validation, in which modeled outcomes were usually compared qualitatively for agreement with studies independent of the model. Validation endpoints included disease prevalence, exacerbation, and all-cause mortality. Validation was typically described as acceptable, despite near-universal absence of criteria for judging adequacy of validation. Although over half of recent obstructive lung disease simulation models report validation, inconsistencies in validation methods and lack of detailed reporting make assessing adequacy of validation difficult. For simulation modeling to be accepted as a tool for evaluating clinical and public health programs, models must be validated to credibly simulate health outcomes of interest. Defining the required level of validation and providing guidance for quantitative assessment and reporting of validation are important future steps in promoting simulation models as practical decision tools.

  10. In vivo lung morphometry with hyperpolarized 3He diffusion MRI: Theoretical background

    Sukstanskii, A. L.; Yablonskiy, D. A.


    MRI-based study of 3He gas diffusion in lungs may provide important information on lung microstructure. Lung acinar airways can be described in terms of cylinders covered with alveolar sleeve [Haefeli-Bleuer, Weibel, Anat. Rec. 220 (1988) 401]. For relatively short diffusion times (on the order of a few ms) this geometry allows description of the 3He diffusion attenuated MR signal in lungs in terms of two diffusion coefficients—longitudinal (D) and transverse (D) with respect to the individual acinar airway axis [Yablonskiy et al., PNAS 99 (2002) 3111]. In this paper, empirical relationships between D and D and the geometrical parameters of airways and alveoli are found by means of computer Monte Carlo simulations. The effects of non-Gaussian signal behavior (dependence of D and D on b-value) are also taken into account. The results obtained are quantitatively valid in the physiologically important range of airway parameters characteristic of healthy lungs and lungs with mild emphysema. In lungs with advanced emphysema, the results provide only "apparent" characteristics but still could potentially be used to evaluate emphysema progression. This creates a basis for in vivo lung morphometry—evaluation of the geometrical parameters of acinar airways from hyperpolarized 3He diffusion MRI, despite the airways being too small to be resolved by direct imaging. These results also predict a rather substantial dependence of 3He ADC on the experimentally-controllable diffusion time, Δ. If Δ is decreased from 3 ms to 1 ms, the ADC in normal human lungs may increase by almost 50%. This effect should be taken into account when comparing experimental data obtained with different pulse sequences.

  11. In vivo lung morphometry with hyperpolarized 3He diffusion MRI: theoretical background.

    Sukstanskii, A L; Yablonskiy, D A


    MRI-based study of (3)He gas diffusion in lungs may provide important information on lung microstructure. Lung acinar airways can be described in terms of cylinders covered with alveolar sleeve [Haefeli-Bleuer, Weibel, Anat. Rec. 220 (1988) 401]. For relatively short diffusion times (on the order of a few ms) this geometry allows description of the (3)He diffusion attenuated MR signal in lungs in terms of two diffusion coefficients-longitudinal (D(L)) and transverse (D(T)) with respect to the individual acinar airway axis [Yablonskiy et al., PNAS 99 (2002) 3111]. In this paper, empirical relationships between D(L) and D(T) and the geometrical parameters of airways and alveoli are found by means of computer Monte Carlo simulations. The effects of non-Gaussian signal behavior (dependence of D(L) and D(T) on b-value) are also taken into account. The results obtained are quantitatively valid in the physiologically important range of airway parameters characteristic of healthy lungs and lungs with mild emphysema. In lungs with advanced emphysema, the results provide only "apparent" characteristics but still could potentially be used to evaluate emphysema progression. This creates a basis for in vivo lung morphometry-evaluation of the geometrical parameters of acinar airways from hyperpolarized (3)He diffusion MRI, despite the airways being too small to be resolved by direct imaging. These results also predict a rather substantial dependence of (3)He ADC on the experimentally-controllable diffusion time, Delta. If Delta is decreased from 3 ms to 1 ms, the ADC in normal human lungs may increase by almost 50%. This effect should be taken into account when comparing experimental data obtained with different pulse sequences.

  12. Social media use for occupational lung disease.

    Harber, Philip; Leroy, Gondy


    Social media have great impact on all aspects of life throughout the world. The utilization of social media for occupational lung disease, however, has been much more limited. This article summarizes recent literature concerning social media for occupational lung disease and identifies areas for additional use. Social media are used in six relevant areas: information dissemination, peer-to-peer communication, survey research data collection, participatory research and exposome data acquisition, assessing public concerns, and knowledge generation. There are very clear advantages for information dissemination from experts to workers and on a peer-to-peer basis, although variable credibility and accuracy concerns persist. For research, social media have been used for acquiring data posted for nonresearch purposes and for efficiently collecting information specifically for research. The benefits of efficiency, democracy, and very large data sources may counterbalance concerns about inadequate specification of recruitment strategies and limited control over data quality. The potential benefits of using social media for lung health-workplace interactions are much greater than the very limited current utilization.

  13. Fetal lung apparent diffusion coefficient measurement using diffusion-weighted MRI at 3 Tesla: Correlation with gestational age.

    Afacan, Onur; Gholipour, Ali; Mulkern, Robert V; Barnewolt, Carol E; Estroff, Judy A; Connolly, Susan A; Parad, Richard B; Bairdain, Sigrid; Warfield, Simon K


    To evaluate the feasibility of using diffusion-weighted magnetic resonance imaging (DW-MRI) to assess the fetal lung apparent diffusion coefficient (ADC) at 3 Tesla (T). Seventy-one pregnant women (32 second trimester, 39 third trimester) were scanned with a twice-refocused Echo-planar diffusion-weighted imaging sequence with 6 different b-values in 3 orthogonal diffusion orientations at 3T. After each scan, a region-of-interest (ROI) mask was drawn to select a region in the fetal lung and an automated robust maximum likelihood estimation algorithm was used to compute the ADC parameter. The amount of motion in each scan was visually rated. When scans with unacceptable levels of motion were eliminated, the lung ADC values showed a strong association with gestational age (P < 0.01), increasing dramatically between 16 and 27 weeks and then achieving a plateau around 27 weeks. We show that to get reliable estimates of ADC values of fetal lungs, a multiple b-value acquisition, where motion is either corrected or considered, can be performed. J. Magn. Reson. Imaging 2016;44:1650-1655. © 2016 International Society for Magnetic Resonance in Medicine.

  14. Cystic lung disease in tuberculosis: An unusual presentation

    Animesh Ray


    Full Text Available Cysts in the lung can arise due to large number of causes out of which tuberculosis is very rare, We report a case of tuberculosis in a young female presenting as a febrile illness and respiratory failure with radiological features of cystic lung disease. With treatment,fever and respiratory distress subsided and cysts in the lungs showed partial regression. We highlight the need to consider tuberculosis in the differential diagnoses of cystic lung disease under appropriate circumstances.

  15. Unclassifiable interstitial lung disease: A review.

    Skolnik, Kate; Ryerson, Christopher J


    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.

  16. Clinical practice. The impact of lung disease on the heart and cardiac disease on the lungs.

    Healy, Fiona; Hanna, Brian D; Zinman, Raezelle


    Pathologies in both the respiratory and cardiovascular systems frequently coexist and impact on each other. This manuscript introduces an approach to the interpretation of this complex relationship. Pulmonary hypertension can be a significant consequence of many respiratory diseases. This in turn can lead to right ventricular dysfunction and cor pulmonale. Many childhood illnesses can result in cor pulmonale and can be conveniently grouped into three categories: idiopathic pulmonary hypertension, neonatal lung diseases, and lung disease beyond the neonatal period. When considering the impact of cardiac disease on the lung, one must consider two main pathologies: compression of the pediatric airway and increased lung water. In conclusion, thorough attention must be given to the interpretation of the complex relationship between cardiac and respiratory diseases. Pulmonary hypertension is a complication to consider in respiratory illness at all ages. In addition, when dealing with the complexities of congenital heart disease, one must always be aware of the risks of pulmonary complications whether parenchymal or airway. Ongoing improvements in ventilation strategies, vasodilator therapy, and surgical interventions continue to improve the outlook for these complex cases.

  17. Pemphigus vulgaris-associated interstitial lung disease.

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


    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.


    Gagiya Ashok K


    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

  19. [Interstitial lung disease in rheumatoid arthritis].

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


    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.

  20. Lung-resident γδ T cells and their roles in lung diseases.

    Cheng, Min; Hu, Shilian


    γδ T cells are greatly enriched in mucosal and epithelial sites, such as the skin, respiratory, digestive and reproductive tracts, and they are defined as tissue-resident immune cells. In these tissues, the characteristics and biological roles of γδ T cells are distinguished from each other. The lungs represent the most challenging immunological dilemma for the host, and they have their own effective immune system. The abundance of γδ T cells, an estimated 8-20% of resident pulmonary lymphocytes in the lung, maintains lung tissue homeostasis. In this review, we summarize the recent research progress regarding lung-resident γδ T cells, including their development, residency and immune characteristics, and discuss the involvement of γδ T cells in infectious diseases of the lung, including bacterial, viral and fungal infections; lung allergic disease; lung inflammation and fibrosis; and lung cancer. © 2017 John Wiley & Sons Ltd.

  1. Bullous lung diseases as a risk factor for lung cancer: A case report

    Nagorni-Obradović Ljudmila


    Full Text Available Introduction. A possible association between lung cancer and bullous lung disease has been suggested and recently supported by the results of genetic studies. Case report. A previously healthy 43-year-old man, smoker, was diagnosed with bullous lung disease at the age of 31 years. He was followed up for 12 years when lung cancer (adenocarcinoma was found at the site. In the meantime, he was treated for recurrent respiratory infections. Conclusion. There is the need for active approach in following up the patients with pulmonary bulla for potential development of lung cancer.

  2. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases.

    Gian Luca Casoni

    Full Text Available BACKGROUND: Histology is a key element for the multidisciplinary diagnosis of fibrotic diffuse parenchymal lung diseases (f-DPLD when the clinical-radiological picture is nondiagnostic. Transbronchial lung cryobiopsy (TBLC have been shown to be useful for obtaining large and well-preserved biopsies of lung parenchyma, but experience with TBLC in f-DPLD is limited. OBJECTIVES: To evaluate safety, feasibility and diagnostic yield of TBLC in f-DPLD. METHOD: Prospective study of 69 cases of TBLC using flexible cryoprobe in the clinical-radiological setting of f-DPLD with nondiagnostic high resolution computed tomography (HRCT features. RESULTS: SAFETY: pneumothorax occurred in 19 patients (28%. One patient (1.4% died of acute exacerbation. Feasibility: adequate cryobiopsies were obtained in 68 cases (99%. The median size of cryobiopsies was 43.11 mm(2 (range, 11.94-76.25. Diagnostic yield: among adequate TBLC the pathologists were confident ("high confidence" that histopathologic criteria sufficient to define a specific pattern in 52 patients (76%, including 36 of 47 with UIP (77% and 9 nonspecific interstitial pneumonia (6 fibrosing and 3 cellular, 2 desquamative interstitial pneumonia/respiratory bronchiolitis-interstitial lung disease, 1 organizing pneumonia, 1 eosinophilic pneumonia, 1 diffuse alveolar damage, 1 hypersensitivity pneumonitis and 1 follicular bronchiolitis. In 11 diagnoses of UIP the pathologists were less confident ("low confidence". Agreement between pathologists in the detection of UIP was very good with a Kappa coefficient of 0.83 (95% CI, 0.69-0.97. Using the current consensus guidelines for clinical-radiologic-pathologic correlation 32% (20/63 of cases were classified as Idiopathic Pulmonary Fibrosis (IPF, 30% (19/63 as possible IPF, 25% (16/63 as other f-DPLDs and 13% (8/63 were unclassifiable. CONCLUSIONS: TBLC in the diagnosis of f-DPLD appears safe and feasible. TBLC has a good diagnostic yield in the clinical

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

    Elham Vali Khojeini


    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.

  4. Invasive Aspergillus infections in hospitalized patients with chronic lung disease

    Wessolossky M


    Full Text Available Mireya Wessolossky,1 Verna L Welch,2 Ajanta Sen,1 Tara M Babu,1 David R Luke21Division of Infectious Diseases, University of Massachusetts Medical School, Worcester, MA, USA; 2Medical Affairs, Pfizer Inc, Collegeville, PA, USABackground: Although invasive pulmonary aspergillosis (IPA is more prevalent in immunocompromised patients, critical care clinicians need to be aware of the occurrence of IPA in the nontraditional host, such as a patient with chronic lung disease. The purpose of this study was to describe the IPA patient with chronic lung disease and compare the data with that of immunocompromised patients.Methods: The records of 351 patients with Aspergillus were evaluated in this single-center, retrospective study for evidence and outcomes of IPA. The outcomes of 57 patients with chronic lung disease and 56 immunocompromised patients were compared. Patients with chronic lung disease were defined by one of the following descriptive terms: emphysema, asthma, idiopathic lung disease, bronchitis, bronchiectasis, sarcoid, or pulmonary leukostasis.Results: Baseline demographics were similar between the two groups. Patients with chronic lung disease were primarily defined by emphysema (61% and asthma (18%, and immunocompromised patients primarily had malignancies (27% and bone marrow transplants (14%. A higher proportion of patients with chronic lung disease had a diagnosis of IPA by bronchoalveolar lavage versus the immunocompromised group (P < 0.03. The major risk factors for IPA were found to be steroid use in the chronic lung disease group and neutropenia and prior surgical procedures in the immunocompromised group. Overall, 53% and 69% of chronic lung disease and immunocompromised patients were cured (P = 0.14; 55% of chronic lung patients and 47% of immunocompromised patients survived one month (P = 0.75.Conclusion: Nontraditional patients with IPA, such as those with chronic lung disease, have outcomes and mortality similar to that in the

  5. Diffusion of Molecular Diagnostic Lung Cancer Tests: A Survey of German Oncologists

    Julius Alexander Steffen


    Full Text Available This study was aimed at examining the diffusion of diagnostic lung cancer tests in Germany. It was motivated by the high potential of detecting and targeting oncogenic drivers. Recognizing that the diffusion of diagnostic tests is a conditio sine qua non for the success of personalized lung cancer therapies, this study analyzed the diffusion of epidermal growth factor receptor (EGFR and anaplastic lymphoma kinase (ALK tests in Germany. Qualitative and quantitative research strategies were combined in a mixed-method design. A literature review and subsequent Key Opinion Leader interviews identified a set of qualitative factors driving the diffusion process, which were then translated into an online survey. The survey was conducted among a sample of 961 oncologists (11.34% response rate. The responses were analyzed in a multiple linear regression which identified six statistically significant factors driving the diffusion of molecular diagnostic lung cancer tests: reimbursement, attitude towards R&D, information self-assessment, perceived attitudes of colleagues, age and test-pathway strategies. Besides the important role of adequate reimbursement and relevant guidelines, the results of this study suggest that an increasing usage of test-pathway strategies, especially in an office-based setting, can increase the diffusion of molecular diagnostic lung cancer tests in the future.

  6. Diffusion of molecular diagnostic lung cancer tests: a survey of german oncologists.

    Steffen, Julius Alexander


    This study was aimed at examining the diffusion of diagnostic lung cancer tests in Germany. It was motivated by the high potential of detecting and targeting oncogenic drivers. Recognizing that the diffusion of diagnostic tests is a conditio sine qua non for the success of personalized lung cancer therapies, this study analyzed the diffusion of epidermal growth factor receptor (EGFR) and anaplastic lymphoma kinase (ALK) tests in Germany. Qualitative and quantitative research strategies were combined in a mixed-method design. A literature review and subsequent Key Opinion Leader interviews identified a set of qualitative factors driving the diffusion process, which were then translated into an online survey. The survey was conducted among a sample of 961 oncologists (11.34% response rate). The responses were analyzed in a multiple linear regression which identified six statistically significant factors driving the diffusion of molecular diagnostic lung cancer tests: reimbursement, attitude towards R&D, information self-assessment, perceived attitudes of colleagues, age and test-pathway strategies. Besides the important role of adequate reimbursement and relevant guidelines, the results of this study suggest that an increasing usage of test-pathway strategies, especially in an office-based setting, can increase the diffusion of molecular diagnostic lung cancer tests in the future.

  7. Rituximab-induced interstitial lung disease

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


    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....

  8. Lung Ultrasonography in the Evaluation of Interstitial Lung Disease in Systemic Connective Tissue Diseases: Criteria and Severity of Pulmonary Fibrosis - Analysis of 52 Patients.

    Buda, N; Piskunowicz, M; Porzezińska, M; Kosiak, W; Zdrojewski, Z


    Patients with a diagnosed systemic connective tissue disease require regular monitoring from the point of view of interstitial lung disease. The main aim of this work is a description of the criteria for pulmonary fibrosis and the degree of the severity of the fibrosis during the course of interstitial lung disease through the TLU (transthoracic lung ultrasound). 52 patients with diagnosed diffuse interstitial lung disease were qualified for this research, together with 50 volunteers in the control group. The patients in both groups were over 18 years of age and were of both sexes. The results of the TLU of the patients underwent statistical analysis and were compared to High-Resolution Computed Tomography (HRCT) results. As a consequence of the statistical analysis, we defined our own criteria for pulmonary fibrosis in TLU: irregularity of the pleura line, tightening of the pleura line, the fragmentary nature of the pleura line, blurring of the pleura line, thickening of the pleura line, artifacts of line B ≤ 3 and ≥ 4, artifacts of Am line and subpleural consolidations Lung Ultrasonography (TLU) gives a new outlook on the diagnostic possibilities, non-invasive and devoid of ionising radiation, of pulmonary fibrosis. This research work has allowed to discover two new ultrasound symptoms of pulmonary fibrosis (blurred pleural line and Am lines). © Georg Thieme Verlag KG Stuttgart · New York.

  9. Lung involvement and drug-induced lung disease in patients with rheumatoid arthritis.

    Atzeni, Fabiola; Boiardi, Luigi; Sallì, Salvatore; Benucci, Maurizio; Sarzi-Puttini, Piercarlo


    Interstitial lung disease (ILD) is a common extra-articular manifestation of rheumatoid arthritis (RA) and a significant cause of morbidity and mortality. Usual interstitial pneumonia and nonspecific interstitial pneumonia seem to be the most frequent patterns in RA patients with ILD, although the proportion of patients with usual interstitial pneumonia is higher than among patients with other systemic rheumatic autoimmune diseases. RA patients with ILD most frequently present with chronic symptoms of cough and dyspnea when climbing stairs or walking uphill. A physical examination may reveal inhalatory crackles and a pulmonary function test demonstrates restrictive physiology, often with reduced diffusing capacity. High-resolution computed tomography is generally sufficient to confirm a diagnosis of ILD, although a minority of cases may require a surgical lung biopsy. Conventional disease-modifying antirheumatic drugs such as methotrexate (MTX) or leflunomide (LEF) and biological agents such as TNF-blocking agents or rituximab may trigger or aggravate ILD in RA patients, and infections may contribute to increased mortality in such patients. LEF should not be used in patients with a history of MTX pneumonitis. The prevalence of interstitial pneumonia among RA patients treated with anti-TNF agents ranges from 0.5 to 3%; however, as the evidence that anti-TNF increases or decreases the risk of ILD is controversial, it is not clear whether this indicates more severe RA requiring biological therapy or the effect of exposure to potentially toxic drugs such as MTX or LEF. The development of treatment-related ILD is a paradoxical adverse event, and patients should be warned about this rare but serious complication of biological or disease-modifying antirheumatic drug therapy.

  10. Diffuse lung neoplasms. Correlation between high resolution computerized tomography and anatomopathology; Neoplasias pulmonares difusas: correlacao da tomografia computadorizada de alta resolucao com a anatomopatologia

    Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia; Irion, Klaus L. [Complexo Hospitalar Santa Casa de Porto Alegre, RS (Brazil). Servico de Radiologia do Pavilhao Pereira Filho; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto, SP (Brazil)]. E-mail:


    A short comparative evaluation study between high resolution computerized tomography and anatomopathologic findings is presented. The association of these two diagnostic techniques is discussed as a factor to enhance the accuracy of diffuse lung diseases, such as carcinomatous lymphangitis, Kaposi's Sarcoma, lymphomas, hematogenic metastases, bronchiole-alveolar carcinoma and leukemia. (MAC)

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

    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)


    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

  12. Common lung conditions: chronic obstructive pulmonary disease.

    Delzell, John E


    The etiology of chronic obstructive pulmonary disease (COPD) is chronic lung inflammation. In the United States, this inflammation most commonly is caused by smoking. COPD is diagnosed when an at-risk patient presents with respiratory symptoms and has irreversible airway obstruction indicated by a forced expiratory volume in 1 second/forced vital capacity ratio of less than 0.7. Management goals for COPD include smoking cessation, symptom reduction, exacerbation reduction, hospitalization avoidance, and improvement of quality of life. Stable patients with COPD who remain symptomatic despite using short-acting bronchodilators should start inhaled maintenance drugs to reduce symptoms and exacerbations, avoid hospitalizations, and improve quality of life. A long-acting anticholinergic or a long-acting beta2-agonist (LABA) can be used for initial therapy; these drugs have fewer adverse effects than inhaled corticosteroids (ICS). If patients remain symptomatic despite monotherapy, dual therapy with a long-acting anticholinergic and a LABA, or a LABA and an ICS, may be beneficial. Triple therapy (ie, a long-acting anticholinergic, a LABA, and an ICS) also is used, but it is unclear if triple therapy is superior to dual therapy. Roflumilast, an oral selective inhibitor of phosphodiesterase 4, is used to manage moderate to severe COPD. Continuous oxygen therapy is indicated for patients with COPD who have severe hypoxemia (ie, PaO2 less than 55 mm Hg or an oxygen saturation less than 88% on room air). Nonpharmacologic strategies also are useful to improve patient outcomes. Pulmonary rehabilitation improves dyspnea and quality of life. Pulmonary rehabilitation after an acute exacerbation reduces hospitalizations and mortality, and improves quality of life and exercise capacity. Smoking cessation is the most effective management strategy for reducing morbidity and mortality in patients with COPD. Lung volume reduction surgery, bullectomy, and lung transplantation are

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

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


    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.

  14. 3D MRI of non-Gaussian 3He gas diffusion in the rat lung

    Jacob, Richard E.; Laicher, Gernot; Minard, Kevin R.


    In 3He magnetic resonance images of pulmonary air spaces, the confining architecture of the parenchymal tissue results in a non-Gaussian distribution of signal phase that non-exponentially attenuates image intensity as diffusion weighting is increased. Here, two approaches previously used for the analysis of non-Gaussian effects in the lung are compared and related using diffusion-weighted 3He MR images of mechanically ventilated rats. One approach is model-based and was presented by Yablonskiy et al., while the other approach utilizes the second order decay contribution that is predicted from the cumulant expansion theorem. Total lung coverage is achieved using a hybrid 3D pulse sequence that combines conventional phase encoding with sparse radial sampling for efficient gas usage. This enables the acquisition of nine 3D images using a total of only ˜1 L of hyperpolarized 3He gas. Diffusion weighting ranges from 0 s/cm 2 to 40 s/cm 2. Results show that the non-Gaussian effects of 3He gas diffusion in healthy rat lungs are directly attributed to the anisotropic geometry of lung microstructure as predicted by the Yablonskiy model, and that quantitative analysis over the entire lung can be reliably repeated in time-course studies of the same animal.

  15. Heterogeneity of mononuclear phagocytes in interstitial lung diseases

    H.C. Hoogsteden (Henk)


    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

  16. Hard metal lung disease in an oil industry worker.

    Bezerra, Patrícia Nunes; Vasconcelos, Ana Giselle Alves; Cavalcante, Lílian Loureiro Albuquerque; Marques, Vanessa Beatriz de Vasconcelos; Nogueira, Teresa Neuma Albuquerque Gomes; Holanda, Marcelo Alcantara


    Hard metal lung disease, which manifests as giant cell interstitial pneumonia, is caused by exposure to hard metal dust. We report the case of an oil industry worker diagnosed with hard metal lung disease. The diagnosis was based on the clinical, radiological and anatomopathological analysis, as well as on pulmonary function testing.

  17. Heterogeneity of mononuclear phagocytes in interstitial lung diseases

    H.C. Hoogsteden (Henk)


    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

  18. Structural and functional lung disease in primary ciliary dyskinesia

    F. Santamaria (Francesca); S. Montella (Silvia); H.A.W.M. Tiddens (Harm); G. Guidi (Guido); V. Casotti (Valeria); M. Maglione (Marco); P.A. de Jong (Pim)


    textabstractBackground: High-resolution CT (HRCT) scan data on primary ciliary dyskinesia (PCD) related lung disease are scarce. Study objectives: We evaluated the lung disease in children and adults with PCD by a modified Brody composite HRCT scan score to assess the prevalence of the structural ab

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

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


    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.

  20. 11.5.Interstitial lung disease


    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-

  1. Smart Technology in Lung Disease Clinical Trials.

    Geller, Nancy L; Kim, Dong-Yun; Tian, Xin


    This article describes the use of smart technology by investigators and patients to facilitate lung disease clinical trials and make them less costly and more efficient. By "smart technology" we include various electronic media, such as computer databases, the Internet, and mobile devices. We first describe the use of electronic health records for identifying potential subjects and then discuss electronic informed consent. We give several examples of using the Internet and mobile technology in clinical trials. Interventions have been delivered via the World Wide Web or via mobile devices, and both have been used to collect outcome data. We discuss examples of new electronic devices that recently have been introduced to collect health data. While use of smart technology in clinical trials is an exciting development, comparison with similar interventions applied in a conventional manner is still in its infancy. We discuss advantages and disadvantages of using this omnipresent, powerful tool in clinical trials, as well as directions for future research.

  2. An Unusual Radiologic Manifestation of Pulmonary Tuberculosis with Bilateral Multiple Lung Nodules and Diffuse Alveolar Hemorrhage: A Case Report

    Jeong, Seo In; Seon, Hyun Ju; Kim, Yun Hyeon [Dept. of Radiology, Chunnam National University Hospital, Gwangju (Korea, Republic of); Choi, Sung [Dept. of Radiology, Chunnam National University Hwasun Hospital, Hwasun(Korea, Republic of)


    Pulmonary tuberculosis presenting as bilateral multiple lung nodules or diffuse alveolar hemorrhage is very rare. Here, we report a case of pulmonary tuberculosis presenting as bilateral multiple lung nodules and diffuse alveolar hemorrhage mimicking granulomatous vasculitis, such as Wegener's granulomatosis.

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

    Daniel Antunes Silva Pereira


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

    EY Chan


    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.

  5. Clinical application of exhaled nitric oxide measurement in pediatric lung diseases

    Manna Angelo


    Full Text Available Summary Fractional exhaled nitric oxide (FeNO is a non invasive method for assessing the inflammatory status of children with airway disease. Different ways to measure FeNO levels are currently available. The possibility of measuring FeNO levels in an office setting even in young children, and the commercial availability of portable devices, support the routine use of FeNO determination in the daily pediatric practice. Although many confounding factors may affect its measurement, FeNO is now widely used in the management of children with asthma, and seems to provide significantly higher diagnostic accuracy than lung function or bronchial challenge tests. The role of FeNO in airway infection (e.g. viral bronchiolitis and common acquired pneumonia, in bronchiectasis, or in cases with diffuse lung disease is less clear. This review focuses on the most recent advances and the current clinical applications of FeNO measurement in pediatric lung disease.

  6. Complications in Transbronchial Lung Biopsy

    Hue, Sung Ho


    Transbronchial lung biopsy was performed in 68 patients with various lung diseases. As an inital diagnostic procedure in diffuse and localized lung diseases, trasbronchial lung biopsy offers an attractive alternative to open lung biopsy. Recently imporvement in the development of medical technology has greatly facilitated this technique, but it must be kept in mind that a transbronchial lung biopsy is not without complication and sometimes potentially serious complications may occur. This stu...

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

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


    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...

  8. Promotion of Lung Health: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases

    Camargo, Carlos A.; Budinger, G. R. Scott; Escobar, Gabriel J.; Hansel, Nadia N.; Corrine K Hanson; Gary B Huffnagle; Buist, A. Sonia


    Lung-related research primarily focuses on the etiology and management of diseases. In recent years, interest in primary prevention has grown. However, primary prevention also includes “health promotion” (actions in a population that keep an individual healthy). We encourage more research on population-based (public health) strategies that could not only maximize lung health but also mitigate “normal” age-related declines—not only for spirometry but across multiple measures of lung health. In...

  9. Pleuropneumonectomy for diffuse pleural metastasis in primary lung cancer

    Wen-Bo Jin


    Full Text Available Objective: The purpose of this study is to analyze a single institution experience with pleuropneumonectomy for pleural metastasis and malignant pleural effusion in primary lung cancer. Materials and Methods: From August 1978 to August 2011, 66 consecutive patients with lung cancer underwent pleuropneumonectomy. Patients were followed-up after the operation. The quality-of-life and the survival time were recorded. Results: All the 66 patients were successfully operated on, including 38 patients in early years (1978-1993 and 28 patients in recent years (1994-2011. Two patients in early years died after the operation. Post-operative complications occurred including heart arrhythmia, respiratory insufficiency and bacterial infection of residual lung, chylothoraxin and mental disorder. A total of 61 patients have been successfully followed-up and three patients in early years were lost in 1 year after the operation. Local recurrence was found in seven cases (4 in early years, 3 in recent years and distant metastasis was found in 48 cases (29 in early years, 19 in recent years. A total of 54 patients died from tumors, seven patients survived. The actuarial 1, 2 and 3-year survival rates are 72.7%, 27.2% and 6.1% of 36 in patients of early years and 85.7%, 46.4% and 21.4% in 28 patients of recent years. The mean survival and the median survival of the total 64 patients were 20.0 ± 10.9 months and 17 months respectively. Further analysis showed that the mean survival and the median survival of the 36 patients in early years were 17.2 ± 9.7 months and 15 months, in contrast to 23.4 ± 11.3 months and 18 months of the 28 patients in recent years. Conclusion: Pleuropneumonectomy is an option of patients with advanced-stage lung cancer associated with uncontrolled malignant pleural fluid by conservative therapies. Strict selection of patient to be operated, careful procedures to eradicate obvious tumors and metastasis and enhanced post-operative combined

  10. Prevention and treatment of chronic lung disease

    Fabio Mosca


    Full Text Available The increased survival among very low birth weight (VLBW contributes to the overall increase in the incidence of chronic lung disease (CLD, also known as bronchopulmonary dysplasia (BPD, that remains a major complication of prematurity. The long-term health consequences of BPD include early and long term respiratory disease, susceptibility to respiratory infections, pulmonary hypertension, repeated hospitalizations, neurodevelopmental impairment and increased mortality. BPD pathogenesis is multifactorial and includes exposure to mechanical ventilation, oxygen toxicity, infection, and inflammation, but the real causes in single individuals have not been well clarified. In this review the current and potential future postnatal pharmacological (caffeine, diuretics, postnatal corticosteroids, bronchodilators, pulmonary vasodilators, anti-oxidants and non-pharmacological  strategies (ventilatory support, stem cells in the prevention and management of BPD will be presented. Proceedings of the 9th International Workshop on Neonatology · Cagliari (Italy · October 23rd-26th, 2013 · Learned lessons, changing practice and cutting-edge research

  11. The role of fibrocytes in sickle cell lung disease.

    Joshua J Field

    Full Text Available BACKGROUND: Interstitial lung disease is a frequent complication in sickle cell disease and is characterized by vascular remodeling and interstitial fibrosis. Bone marrow-derived fibrocytes have been shown to contribute to the pathogenesis of other interstitial lung diseases. The goal of this study was to define the contribution of fibrocytes to the pathogenesis of sickle cell lung disease. METHODOLOGY/PRINCIPAL FINDINGS: Fibrocytes were quantified and characterized in subjects with sickle cell disease or healthy controls, and in a model of sickle cell disease, the NY1DD mouse. The role of the chemokine ligand CXCL12 in trafficking of fibrocytes and phenotype of lung disease was examined in the animal model. We found elevated concentration of activated fibrocytes in the peripheral blood of subjects with sickle cell disease, which increased further during vaso-occlusive crises. There was a similar elevations in the numbers and activation phenotype of fibrocytes in the bone marrow, blood, and lungs of the NY1DD mouse, both at baseline and under conditions of hypoxia/re-oxygenation. In both subjects with sickle cell disease and the mouse model, fibrocytes expressed a hierarchy of chemokine receptors, with CXCR4 expressed on most fibrocytes, and CCR2 and CCR7 expressed on a smaller subset of cells. Depletion of the CXCR4 ligand, CXCL12, in the mouse model resulted in a marked reduction of fibrocyte trafficking into the lungs, reduced lung collagen content and improved lung compliance and histology. CONCLUSIONS: These data support the notion that activated fibrocytes play a significant role in the pathogenesis of sickle cell lung disease.

  12. Diagnosis and therapy of primary lung diffuse large B cell lymphoma: a case report%原发性肺大B细胞淋巴瘤诊断治疗一例报道

    Lei Zhou; Li Duan; Min Hu


    Objective: We studied the diagnosis and therapy of primary lung diffuse large B cell lymphoma (DLBCL). Meth-ods: Analysis the clinical manifestations, pathologic character and immunohistochemical character of one tung diffuse B cell lymphoma patent. Results: In visual observation, it's a gray irregular Iobulated mass, section was gray, fish-like, and number of necrotic foci. Observed under the microscope, subepithelial respiratory center oocyte-like cells diffuse proliferative, infiltra-tion in lung tissue. Immunohistochemistry: CDJ0 (+), CD790 (+), CD3 (-), CD45RO (-), PCK (-). Conclusion: Diffuse large B cell lymphoma is the most common subtype in non-Hodgkin lymphoma, but the primary lung diffuse large B cell lymphoma is rare. This disease is lack of typical clinical manifestations, so easily misdiagnosed. The diagnosis of diffuse large B cell lymphoma should be based on pathology and immunohistochemistry.

  13. Combined prednisolone and pirfenidone in bleomycin-induced lung disease

    Preyas J Vaidya


    Full Text Available Bleomycin is a cytostatic drug commonly employed in the treatment of Hodgkin's disease, seminomas, and choriocarcinoma. Bleomycin may induce a chronic pulmonary inflammation that may progress to fibrosis. So far, only corticosteroids have been used in the treatment of bleomycin-induced lung disease with variable results. Pirfenidone is an antifibrotic drug that has been approved for the treatment of idiopathic pulmonary fibrosis. We report two cases of bleomycin-induced lung disease treated successfully with pirfenidone and oral corticosteroids.

  14. Rheumatoid arthritis associated interstitial lung disease: a review

    Deborah Assayag


    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.

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

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


    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.

  16. Diffusion weighted imaging in cystic fibrosis disease: beyond morphological imaging

    Ciet, Pierluigi [Erasmus Medical Center, Department of Radiology, Rotterdam (Netherlands); Erasmus Medical Center - Sophia Children' s Hospital, Department of Paediatrics, Respiratory Medicine and Allergology, P.O. Box 2060, Rotterdam, Zuid-Holland (Netherlands); Ca' Foncello - General Hospital, Department of Radiology, Treviso (Italy); Serra, Goffredo; Catalano, Carlo [University of Rome ' ' Sapienza' ' , Department of Radiology, Rome (Italy); Andrinopoulou, Eleni Rosalina [Erasmus Medical Center, Department of Biostatistics, Rotterdam (Netherlands); Bertolo, Silvia; Morana, Giovanni [Ca' Foncello - General Hospital, Department of Radiology, Treviso (Italy); Ros, Mirco [Ca' Foncello Hospital, Department of Pediatrics, Treviso (Italy); Colagrande, Stefano [University of Florence - Azienda Ospedaliero-Universitaria Careggi, Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit n. 2, Florence (Italy); Tiddens, Harm A.W.M. [Erasmus Medical Center, Department of Radiology, Rotterdam (Netherlands); Erasmus Medical Center - Sophia Children' s Hospital, Department of Paediatrics, Respiratory Medicine and Allergology, P.O. Box 2060, Rotterdam, Zuid-Holland (Netherlands)


    To explore the feasibility of diffusion-weighted imaging (DWI) to assess inflammatory lung changes in patients with Cystic Fibrosis (CF) CF patients referred for their annual check-up had spirometry, chest-CT and MRI on the same day. MRI was performed in a 1.5 T scanner with BLADE and EPI-DWI sequences (b = 0-600 s/mm{sup 2}). End-inspiratory and end-expiratory scans were acquired in multi-row scanners. DWI was scored with an established semi-quantitative scoring system. DWI score was correlated to CT sub-scores for bronchiectasis (CF-CT{sub BE}), mucus (CF-CT{sub mucus}), total score (CF-CT{sub total-score}), FEV{sub 1}, and BMI. T-test was used to assess differences between patients with and without DWI-hotspots. Thirty-three CF patients were enrolled (mean 21 years, range 6-51, 19 female). 4 % (SD 2.6, range 1.5-12.9) of total CF-CT alterations presented DWI-hotspots. DWI-hotspots coincided with mucus plugging (60 %), consolidation (30 %) and bronchiectasis (10 %). DWI{sub total-score} correlated (all p < 0.0001) positively to CF-CT{sub BE} (r = 0.757), CF-CT{sub mucus} (r = 0.759) and CF-CT{sub total-score} (r = 0.79); and negatively to FEV{sub 1} (r = 0.688). FEV{sub 1} was significantly higher (p < 0.0001) in patients without DWI-hotspots. DWI-hotspots strongly correlated with radiological and clinical parameters of lung disease severity. Future validation studies are needed to establish the exact nature of DWI-hotspots in CF patients. (orig.)

  17. Sex Differences and Sex Steroids in Lung Health and Disease

    Townsend, Elizabeth A.; Miller, Virginia M.


    Sex differences in the biology of different organ systems and the influence of sex hormones in modulating health and disease are increasingly relevant in clinical and research areas. Although work has focused on sex differences and sex hormones in cardiovascular, musculoskeletal, and neuronal systems, there is now increasing clinical evidence for sex differences in incidence, morbidity, and mortality of lung diseases including allergic diseases (such as asthma), chronic obstructive pulmonary disease, pulmonary fibrosis, lung cancer, as well as pulmonary hypertension. Whether such differences are inherent and/or whether sex steroids play a role in modulating these differences is currently under investigation. The purpose of this review is to define sex differences in lung structure/function under normal and specific disease states, with exploration of whether and how sex hormone signaling mechanisms may explain these clinical observations. Focusing on adult age groups, the review addresses the following: 1) inherent sex differences in lung anatomy and physiology; 2) the importance of certain time points in life such as puberty, pregnancy, menopause, and aging; 3) expression and signaling of sex steroid receptors under normal vs. disease states; 4) potential interplay between different sex steroids; 5) the question of whether sex steroids are beneficial or detrimental to the lung; and 6) the potential use of sex steroid signaling as biomarkers and therapeutic avenues in lung diseases. The importance of focusing on sex differences and sex steroids in the lung lies in the increasing incidence of lung diseases in women and the need to address lung diseases across the life span. PMID:22240244

  18. Estimation of pulmonary hypertension in lung and valvular heart diseases by perfusion lung scintigraphy

    Fujii, Tadashige [Shinshu Univ., Matsumoto, Nagano (Japan). School of Allied Medical Sciences; Tanaka, Masao; Yazaki, Yoshikazu; Kitabayashi, Hirosi; Koizumi, Tomonori; Kubo, Keisi; Sekiguchi, Morie; Yano, Kesato


    To estimate pulmonary hypertension, we measured postural differences in pulmonary blood flow for the lateral decubitus positions on perfusion lung scintigrams with Tc-99 m macro-aggregated albumin, applying the method devised by Tanaka et al (Eur J Nucl Med 17: 320-326, 1990). Utilizing a scintillation camera coupled to a minicomputer system, changes in the distribution of pulmonary blood flow caused by gravitational effects, namely, changes in the total count ratios for the right lung versus the left lung in the right and left lateral decubitus positions (R/L), were obtained for 44 patients with lung disease, 95 patients with valvular heart disease, and 23 normal subjects. Mean standard deviation in the R/L ratios was 3.09{+-}1.28 for the normal subjects, 1.97{+-}0.89 for the patients with lung disease, and 1.59{+-}0.59 for the patients with valvular heart disease. The R/L ratios correlated with mean pulmonary arterial pressure and cardio-thoracic ratios in the lung disease and valvular heart disease groups, with pulmonary arteriolar resistance in the former, and with pulmonary capillary wedge pressure in the latter. Defining pulmonary hypertension (>20 mmHg) as an R/L ratio of less than 1.81, which is the mean-1 standard deviation for normal subjects, the sensitivity and the specificity of the R/L ratio for the diagnosis of pulmonary hypertension were 62.9% and 76.2%, respectively, for the lung disease patients, and 80.3% and 61.8%, respectively, for the valvular heart disease patients. This method seems to be useful for the pathophysiologic evaluation of pulmonary perfusion in cases of lung disease and valvular heart disease. (author)

  19. Modulatory potential of resveratrol during lung inflammatory disease.

    Vargas, José Eduardo; Souto, André Arigony; Pitrez, Paulo Márcio Condessa; Stein, Renato Tetelbom; Porto, Bárbara Nery


    Neutrophils are the first cells to achieve the sites of infection or inflammation in the lungs. The massive accumulation of these cells is associated with acute and chronic lung injury. Therefore, they have been implicated in the pathogenesis of many lung diseases through the release of reactive oxygen intermediates, proteolytic enzymes and Neutrophil Extracellular Traps (NETs). The excessive and continuous release of NETs, fibers composed by decondensed chromatin coated with neutrophil proteins, are associated to the impairment of lung function in different pathological settings. Flavonoids inhibit the respiratory burst of neutrophils in mammals. However, one of these flavonoids, resveratrol has a particular chemical property. It reduce Cu(II) to Cu(I) form with concomitant formation of reactive oxygen species, which can produce DNA breakage as reported in several in vitro models. We hypothesize that direct resveratrol administration in lungs can cleave DNA in NETs, improving lung function during acute airway infections or chronic inflammatory lung diseases. If the hypothesis is correct, the control of NET formation can be used to reduce the inflammatory environment in lung after neutrophil stimuli. Additionally, the production of proinflammatory cytokines by neutrophils could be also diminished by resveratrol administration. In this sense, this flavonoid provides a multifaceted opportunity for treatment of lung diseases with strong or chronic neutrophil activation. Copyright © 2016 Elsevier Ltd. All rights reserved.

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

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


    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.

  1. Biometric monitoring in silicosis to detect early disease and monitor lung injury: not quite there

    Daniel Weiler Ravell; Hashem Bishara


    Any attempt to prevent the development of silicosis in exposed workers by identifying occult disease undetectable by conventional methodology, is a welcome and worthy cause. The recently published paper by Ophir et al. [1] addresses this issue by using biometric monitoring of functional (spirometry and diffusion capacity of the lung for carbon monoxide) and inflammatory parameters (percentage of neutrophils in a sample of induced sputum) in exposed workers. Using novel technology, they also q...

  2. Serial perfusion in native lungs in patients with idiopathic pulmonary fibrosis and other interstitial lung diseases after single lung transplantation.

    Sokai, Akihiko; Handa, Tomohiro; Chen, Fengshi; Tanizawa, Kiminobu; Aoyama, Akihiro; Kubo, Takeshi; Ikezoe, Kohei; Nakatsuka, Yoshinari; Oguma, Tsuyoshi; Hirai, Toyohiro; Nagai, Sonoko; Chin, Kazuo; Date, Hiroshi; Mishima, Michiaki


    Lung perfusions after single lung transplantation (SLT) have not been fully clarified in patients with interstitial lung disease (ILD). The present study aimed to investigate temporal changes in native lung perfusion and their associated clinical factors in patients with ILD who have undergone SLT. Eleven patients were enrolled. Perfusion scintigraphy was serially performed up to 12 months after SLT. Correlations between the post-operative perfusion ratio in the native lung and clinical parameters, including pre-operative perfusion ratio and computed tomography (CT) volumetric parameters, were evaluated. On average, the perfusion ratio of the native lung was maintained at approximately 30% until 12 months after SLT. However, the ratio declined more significantly in idiopathic pulmonary fibrosis (IPF) than in other ILDs (p = 0.014). The perfusion ratio before SLT was significantly correlated with that at three months after SLT (ρ = 0.64, p = 0.048). The temporal change of the perfusion ratio in the native lung did not correlate with those of the CT parameters. The pre-operative perfusion ratio may predict the post-operative perfusion ratio of the native lung shortly after SLT in ILD. Perfusion of the native lung may decline faster in IPF compared with other ILDs. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. The validity of static lung compliance in asbestos-induced diseases.

    Schneider, Joachim; Arhelger, Rolf; Raab, Wolfgang; Hering, Kurt Georg


    Pulmonary compliance can be viewed as an indicator of distensibility of the lungs, not only in asbestos-induced pulmonary disorders but also in visceral pleural fibrosis extending into the lung parenchyma. In this study we evaluated static compliance measurements in asbestos-derived diseases, especially in patients with parietal pleura plaques. Lung function analyses, especially static lung compliance, were correlated with high-resolution computer tomography examinations. Sixty-three patients with parietal pleural plaques, 10 with visceral pleural fibrosis, 39 with parenchymal pulmonary asbestosis together with parietal pleural plaques, and 42 with parenchymal pulmonary asbestosis together with visceral pleural fibrosis were enrolled in the study. In comparison with patients having only parietal pleural plaques, those having asbestosis and visceral pleural fibrosis showed significant decreases in static lung compliance, diffusing capacity, and vital capacity. Visceral pleural thickening was also associated with significantly reduced FEV(1), MEF(50), and FEV(1)/FVC ratios. Multiple regression analyses indicated that the existence of visceral pleural fibrosis (p = 0.017) is the most important factor accounting for a decrease in static compliance. Reference values of static lung compliance differ notably. In comparison with mean reference values, the sensitivity of detecting reduced lung compliance was calculated to be between 9.7 and 45.5 %. Other respiratory function variables failed to show any significant differences. Our data indicate that the measurement of static compliance is not sufficient for early detection of pulmonary function impairment in patients with parietal pleural plaques.

  4. Sonic hedgehog signaling in the lung. From development to disease.

    Kugler, Matthias C; Joyner, Alexandra L; Loomis, Cynthia A; Munger, John S


    Over the past two decades, the secreted protein sonic hedgehog (SHH) has emerged as a critical morphogen during embryonic lung development, regulating the interaction between epithelial and mesenchymal cell populations in the airway and alveolar compartments. There is increasing evidence that the SHH pathway is active in adult lung diseases such as pulmonary fibrosis, asthma, chronic obstructive pulmonary disease, and lung cancer, which raises two questions: (1) What role does SHH signaling play in these diseases? and (2) Is it a primary driver of the disease or a response (perhaps beneficial) to the primary disturbance? In this review we aim to fill the gap between the well-studied period of embryonic lung development and the adult diseased lung by reviewing the hedgehog (HH) pathway during the postnatal period and in adult uninjured and injured lungs. We elucidate the similarities and differences in the epithelial-mesenchymal interplay during the fibrosis response to injury in lung compared with other organs and present a critical appraisal of tools and agents available to evaluate HH signaling.

  5. CT in the diagnosis of interstitial lung disease

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


    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.

  6. Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation.

    Izbicki, Gabriel; Shitrit, David; Schechtman, Itzhak; Bendayan, Danielle; Fink, Gershon; Sahar, Gideon; Saute, Milton; Ben-Gal, Tuvia; Kramer, Mordechai R


    Pulmonary veno-occlusive disease, a rare cause of pulmonary hypertension, is characterized by extensive and diffuse occlusion of pulmonary veins by fibrous tissue. Although the diagnosis can be suspected by the presence of the classic clinical triad of severe pulmonary arterial hypertension, radiographic evidence of pulmonary hypertension and edema, and normal pulmonary artery occlusion pressure, the definitive diagnosis is histopathologic. The prognosis of pulmonary veno-occlusive disease is poor with most described patients dying within 2 years of diagnosis. Although anti-coagulation, oxygen, and vasodilator therapies are effective temporarily, the definitive treatment is lung transplantation. We describe the recurrence of pulmonary veno-occlusive disease at 3 months after heart-lung transplantation in a 26-year-old man. Recurrence after transplantation for this disease has not been reported previously, and lung transplantation was thought to be definitive treatment. With this 1st report of early recurrence of pulmonary veno-occlusive disease after heart-lung transplantation, we believe that extrapulmonary factors may play a role in the pathogenesis of this rare disease.

  7. Medical imaging in occupational and environmental lung disease.

    Cox, Christian W; Lynch, David A


    The purpose of this review is to provide an up-to-date summary of developments in medical imaging in the diagnosis, surveillance, treatment, and screening of occupational and environmental lung diseases, focusing on articles published within the past 2 years. Many new exposures resulting in lung disease have been described worldwide; medical imaging, particularly computed tomography (CT), is often pivotal in recognition and characterization of these new patterns of lung injury. Chest radiography remains important to surveillance studies tracking the long-term evolution of disease and effectiveness of air quality regulation. Finally, studies are proving the utility of screening with low-dose CT, and technical advances offer the prospect of further CT dose reduction with ultra-low-dose CT. In understanding the best practices and new developments in medical imaging, the occupational and environmental medicine clinician can optimize diagnosis and management of related lung diseases.

  8. Collagenolytic Matrix Metalloproteinases in Chronic Obstructive Lung Disease and Cancer

    Woode, Denzel; Shiomi, Takayuki; D’Armiento, Jeanine, E-mail: [Department of Anesthesiology, Columbia University, College of Physicians and Surgeons, New York, NY 10033 (United States)


    Chronic obstructive pulmonary disease (COPD) and lung cancer result in significant morbidity and mortality worldwide. In addition to the role of environmental smoke exposure in the development of both diseases, recent epidemiological studies suggests a connection between the development of COPD and lung cancer. Furthermore, individuals with concomitant COPD and cancer have a poor prognosis when compared with individuals with lung cancer alone. The modulation of molecular pathways activated during emphysema likely lead to an increased susceptibility to lung tumor growth and metastasis. This review summarizes what is known in the literature examining the molecular pathways affecting matrix metalloproteinases (MMPs) in this process as well as external factors such as smoke exposure that have an impact on tumor growth and metastasis. Increased expression of MMPs provides a unifying link between lung cancer and COPD.

  9. Perioperative Management of Interscalene Block in Patients with Lung Disease

    Eric S. Schwenk


    Full Text Available Interscalene nerve block impairs ipsilateral lung function and is relatively contraindicated for patients with lung impairment. We present a case of an 89-year-old female smoker with prior left lung lower lobectomy and mild to moderate lung disease who presented for right shoulder arthroplasty and insisted on regional anesthesia. The patient received a multimodal perioperative regimen that consisted of a continuous interscalene block, acetaminophen, ketorolac, and opioids. Surgery proceeded uneventfully and postoperative analgesia was excellent. Pulmonary physiology and management of these patients will be discussed. A risk/benefit discussion should occur with patients having impaired lung function before performance of interscalene blocks. In this particular patient with mild to moderate disease, analgesia was well managed through a multimodal approach including a continuous interscalene block, and close monitoring of respiratory status took place throughout the perioperative period, leading to a successful outcome.

  10. Collagenolytic Matrix Metalloproteinases in Chronic Obstructive Lung Disease and Cancer

    Denzel Woode


    Full Text Available Chronic obstructive pulmonary disease (COPD and lung cancer result in significant morbidity and mortality worldwide. In addition to the role of environmental smoke exposure in the development of both diseases, recent epidemiological studies suggests a connection between the development of COPD and lung cancer. Furthermore, individuals with concomitant COPD and cancer have a poor prognosis when compared with individuals with lung cancer alone. The modulation of molecular pathways activated during emphysema likely lead to an increased susceptibility to lung tumor growth and metastasis. This review summarizes what is known in the literature examining the molecular pathways affecting matrix metalloproteinases (MMPs in this process as well as external factors such as smoke exposure that have an impact on tumor growth and metastasis. Increased expression of MMPs provides a unifying link between lung cancer and COPD.

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

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


    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.

  12. Severity and outcome of cystic lung disease in women with tuberous sclerosis complex.

    Taveira-DaSilva, Angelo M; Jones, Amanda M; Julien-Williams, Patricia; Yao, Jianhua; Stylianou, Mario; Moss, Joel


    What are the clinical features, severity, and rate of progression of lung disease in women with tuberous sclerosis and lymphangioleiomyomatosis (LAM) and how do they differ from patients with sporadic LAM? Data from 94 tuberous sclerosis/LAM and 460 sporadic LAM women were compared. 40 tuberous sclerosis/LAM and 40 sporadic LAM patients were age- and lung function-matched, and changes in volume occupied by cysts (cyst score) and pulmonary function occurring over 6.5 years were evaluated. Tuberous sclerosis/LAM patients had better lung function than sporadic LAM patients, but no difference was observed from sporadic LAM patients in yearly rates of change in forced expiratory volume in 1 s (-1.9±2.7 versus -1.9±1.9% predicted; p=0.302), diffusing capacity of the lung for CO (-2.1±2.8 versus -1.9±2.7% predicted; p=0.282) or cyst scores (+1.0±1.3 versus +1.4±1.7%, p=0.213). However, the proportion of patients with abnormal lung function and higher rates of FEV1 decline was greater in sporadic LAM. Some young tuberous sclerosis/LAM patients (mean age 25.7±3 years) progressed rapidly from minimal to severe lung disease. Tuberous sclerosis/LAM patients may experience abrupt declines in lung function. Consequently, women with tuberous sclerosis found to have lung cysts should undergo periodic functional and radiological testing to follow disease progression and determine need for therapy.

  13. Computed Tomography Measure of Lung at Risk and Lung Function Decline in Chronic Obstructive Pulmonary Disease.

    Bhatt, Surya P; Bodduluri, Sandeep; Hoffman, Eric A; Newell, John D; Sieren, Jessica C; Dransfield, Mark T; Reinhardt, Joseph M


    The rate of decline of lung function is greater than age-related change in a substantial proportion of patients with chronic obstructive pulmonary disease, even after smoking cessation. Regions of the lung adjacent to emphysematous areas are subject to abnormal stretch during respiration, and this biomechanical stress likely influences emphysema initiation and progression. To assess whether quantifying this penumbra of lung at risk would predict FEV1 decline. We analyzed paired inspiratory-expiratory computed tomography images at baseline of 680 subjects participating in a large multicenter study (COPDGene) over approximately 5 years. By matching inspiratory and expiratory images voxel by voxel using image registration, we calculated the Jacobian determinant, a measure of local lung expansion and contraction with respiration. We measured the distance between each normal voxel to the nearest emphysematous voxel, and quantified the percentage of normal voxels within each millimeter distance from emphysematous voxels as mechanically affected lung (MAL). Multivariable regression analyses were performed to assess the relationship between the Jacobian determinant, MAL, and FEV1 decline. The mean (SD) rate of decline in FEV1 was 39.0 (58.6) ml/yr. There was a progressive decrease in the mean Jacobian determinant of both emphysematous and normal voxels with increasing disease stage (P lung are mechanically influenced by emphysematous areas and this lung at risk is associated with lung function decline. Clinical trial registered with (NCT00608764).

  14. Dynamic ventilation 3He MRI for the quantification of disease in the rat lung.

    Kyriazis, Angelos; Rodriguez, I; Nin, N; Izquierdo-Garcia, J L; Lorente, J A; Perez-Sanchez, J M; Pesic, J; Olsson, L E; Ruiz-Cabello, J


    Pulmonary diseases are known to be largely inhomogeneous. To evaluate such inhomogeneities, we are testing an image-based method to measure gas flow in the lung regionally. Dynamic, spin-density-weighted hyperpolarized (3)He MR images performed during slow inhalation of this gas were analyzed to quantify regional inflation rate. This parameter was measured in regions of interest (ROIs) that were defined by a rectangular grid that covered the entire rat lung and grew dynamically with it during its inflation. We used regional inflation rate to quantify elastase-induced emphysema and to differentiate healthy (n = 8) from elastase-treated (n = 9) rat lungs as well as healthy from elastase-treated areas of one rat unilaterally treated with elastase in the left lung. Emphysema was also assessed by gold standard morphological and well-established hyperpolarized (3)He MRI diffusion measurements. Mean values of regional inflation rates were significantly different for healthy and elastase-treated animals and correlated well with the apparent diffusion coefficient of (3)He and morphological measurements. The image-based biomarker inflation rate may be useful for the assessment of regional lung ventilation.

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

    Lutterbey, G. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany)]. E-mail:; 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)


    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

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

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


    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.

  17. Unevenness of lung perfusion images and pulmonary diseases

    Teshima, Takeo; Isawa, Toyoharu; Hirano, Tomio; Anazawa, Yoshiki; Miki, Makoto; Konno, Kiyoshi; Motomiya, Masakichi (Tohoku Univ., Sendai (Japan). Research Inst. for Tuberculosis and Cancer)


    The purpose of the study was to quantify the unevenness of perfusion distribution in the lungs in conjunction with underlying lung pathology. Twenty-one parameters as described previously were defined out of horizontal radioactive count profiles on perfusion lung image data taken in 64x64 matrixes. Principal component analysis has revealed that the 1st component or Z1 is represented by AREA, the area of the lung, and ANG, the slope of the mean count profile, Z2, by N, the number of peaks, Z3 and Z4, by YG and XG, the barycentric coordinates of count distribution, Z5, by MAC, the maximal count and Z6, by CSD, the degree of scatter in count from the peak count. How those parameters differ in each lung pathology has been determined from 657 lung perfusion image data. In pulmonary emphysema, the lung volumes are larger than those in normal subjects. The AREA and ANG were consequently larger in value and N was also significantly larger, indicating the increased regional alveolar pressure and the compressed or destroyed vascular beds. In diffuse panbronchiolitis (DPB), N was increased probably because the distal airways were either narrowed or obstructed by inflammatory processes inducing regional alveolar hypoxia and/or alveolar hyperinflation. In fibrosis, both AREA and N were significantly smaller. In congestive heart failure with postcapillary pulmonary hypertension, YG was smaller probably because of 'reversal of perfusion'. In pulmonary sarcoidosis, an increase in YG was the only abnormality. (author).

  18. New insights into lung diseases using hyperpolarized gas MRI.

    Flors, L; Altes, T A; Mugler, J P; de Lange, E E; Miller, G W; Mata, J F; Ruset, I C; Hersman, F W


    Hyperpolarized (HP) gases are a new class of contrast agents that permit to obtain high temporal and spatial resolution magnetic resonance images (MRI) of the lung airspaces. HP gas MRI has become important research tool not only for morphological and functional evaluation of normal pulmonary physiology but also for regional quantification of pathologic changes occurring in several lung diseases. The purpose of this work is to provide an introduction to MRI using HP noble gases, describing both the basic principles of the technique and the new information about lung disease provided by clinical studies with this method. The applications of the technique in normal subjects, smoking related lung disease, asthma, and cystic fibrosis are reviewed. Copyright © 2014 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

  19. Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome.

    Kim, Jung Hoon; Song, Won Jun; Jun, Ji Eun; Ryu, Duck Hyun; Lee, Ji Eun; Jeong, Ho Jung; Jeong, Suk Hyeon; Kang, Hyung Koo; Kim, Jung Soo; Lee, Hyun; Chon, Hae Ri; Jeon, Kyeongman; Kim, Dohun; Kim, Jhingook; Koh, Won-Jung


    Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.

  20. Successful Lung Transplantation for Pulmonary Disease Associated With Erdheim-Chester Disease.

    Hashimoto, Kohei; Miyoshi, Kentaroh; Mizutani, Hisao; Otani, Shinji; Sugimoto, Seiichiro; Yamane, Masaomi; Oto, Takahiro


    A 53-year-old man with pulmonary fibrosis associated with Erdheim-Chester disease achieved long-term survival after lung transplantation. Major clinical manifestations included lung and bone injuries, and other vital organs were functionally unaffected by the disease. After a careful observation for the disease progression, he underwent bilateral deceased-donor lung transplantation. He has returned to his normal social life and is doing well without recurrence of Erdheim-Chester disease in the lung allograft or progression in other organs 5 years after transplant. Lung transplantation is a potentially reasonable treatment option for Erdheim-Chester disease involving the lungs if the functions of other vital organs remain stable. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

    Ahrens, P; Weimer, B; Hofmann, D


    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.

  2. The airway microbiota in early cystic fibrosis lung disease.

    Frayman, Katherine B; Armstrong, David S; Grimwood, Keith; Ranganathan, Sarath C


    Infection plays a critical role in the pathogenesis of cystic fibrosis (CF) lung disease. Over the past two decades, the application of molecular and extended culture-based techniques to microbial analysis has changed our understanding of the lungs in both health and disease. CF lung disease is a polymicrobial disorder, with obligate and facultative anaerobes recovered alongside traditional pathogens in varying proportions, with some differences observed to correlate with disease stage. While healthy lungs are not sterile, differences between the lower airway microbiota of individuals with CF and disease-controls are already apparent in childhood. Understanding the evolution of the CF airway microbiota, and its relationship with clinical treatments and outcome at each disease stage, will improve our understanding of the pathogenesis of CF lung disease and potentially inform clinical management. This review summarizes current knowledge of the early development of the respiratory microbiota in healthy children and then discusses what is known about the airway microbiota in individuals with CF, including how it evolves over time and where future research priorities lie. © 2017 Wiley Periodicals, Inc.

  3. Genetic Variation of αENaC Influences Lung Diffusion During Exercise in Humans

    Baker, Sarah E.; Wheatley, Courtney M.; Cassuto, Nicholas A.; Foxx-Lupo, William T.; Sprissler, Ryan; Snyder, Eric M.


    Exercise, decompensated heart failure, and exposure to high altitude have been shown to cause symptoms of pulmonary edema in some, but not all, subjects, suggesting a genetic component to this response. Epithelial Na+ Channels (ENaC) regulate Na+ and fluid reabsorption in the alveolar airspace in the lung. An increase in number and/or activity of ENaC has been shown to increase lung fluid clearance. Previous work has demonstrated common functional genetic variants of the α-subunit of ENaC, including an A→T substitution at amino acid 663 (αA663T). We sought to determine the influence of the T663 variant of αENaC on lung diffusion at rest and at peak exercise in healthy humans. Thirty healthy subjects were recruited for study and grouped according to their SCNN1A genotype [n= 17vs.13, age=25±7vs.30±10yrs., BMI= 23±4vs.25±4kg/m2, V̇O2peak= 95±30vs.100±31%pred., mean±SD, for AA (homozygous for αA663) vs. AT/TT groups (at least one αT663), respectively]. Measures of the diffusing capacity of the lungs for carbon monoxide (DLCO), the diffusing capacity of the lungs for nitric oxide (DLNO), alveolar volume (VA), and alveolar-capillary membrane conductance (DM) were taken at rest and at peak exercise. Subjects expressing the AA polymorphism of ENaC showed a significantly greater percent increase in DLCO and DLNO, and a significantly greater decrease in systemic vascular resistance from rest to peak exercise than those with the AT/TT variant (DLCO=51±12vs.36±17%, DLNO=51±24vs.32±25%, SVR=−67±3vs.−50±8%, p<0.05). The AA ENaC group also tended to have a greater percent increase in DLCO/VA from rest to peak exercise, although this did not reach statistical significance (49±26vs.33±26%, p=0.08). These results demonstrate that genetic variation of the α-subunit of ENaC at amino acid 663 influences lung diffusion at peak exercise in healthy humans, suggesting differences in alveolar Na+ and, therefore, fluid handling. These findings could be important

  4. Automated segmentation of lungs with severe interstitial lung disease in CT.

    Wang, Jiahui; Li, Feng; Li, Qiang


    Accurate segmentation of lungs with severe interstitial lung disease (ILD) in thoracic computed tomography (CT) is an important and difficult task in the development of computer-aided diagnosis (CAD) systems. Therefore, we developed in this study a texture analysis-based method for accurate segmentation of lungs with severe ILD in multidetector CT scans. Our database consisted of 76 CT scans, including 31 normal cases and 45 abnormal cases with moderate or severe ILD. The lungs in three selected slices for each CT scan were first manually delineated by a medical physicist, and then confirmed or revised by an expert chest radiologist, and they were used as the reference standard for lung segmentation. To segment the lungs, we first employed a CT value thresholding technique to obtain an initial lung estimate, including normal and mild ILD lung parenchyma. We then used texture-feature images derived from the co-occurrence matrix to further identify abnormal lung regions with severe ILD. Finally, we combined the identified abnormal lung regions with the initial lungs to generate the final lung segmentation result. The overlap rate, volume agreement, mean absolute distance (MAD), and maximum absolute distance (dmax) between the automatically segmented lungs and the reference lungs were employed to evaluate the performance of the segmentation method. Our segmentation method achieved a mean overlap rate of 96.7%, a mean volume agreement of 98.5%, a mean MAD of 0.84 mm, and a mean dmax of 10.84 mm for all the cases in our database; a mean overlap rate of 97.7%, a mean volume agreement of 99.0%, a mean MAD of 0.66 mm, and a mean dmax of 9.59 mm for the 31 normal cases; and a mean overlap rate of 96.1%, a mean volume agreement of 98.1%, a mean MAD of 0.96 mm, and a mean dmax of 11.71 mm for the 45 abnormal cases with ILD. Our lung segmentation method provided accurate segmentation results for abnormal CT scans with severe ILD and would be useful for developing CAD systems

  5. Human CD56+ cytotoxic lung lymphocytes kill autologous lung cells in chronic obstructive pulmonary disease.

    Christine M Freeman

    Full Text Available CD56+ natural killer (NK and CD56+ T cells, from sputum or bronchoalveolar lavage of subjects with chronic obstructive pulmonary disease (COPD are more cytotoxic to highly susceptible NK targets than those from control subjects. Whether the same is true in lung parenchyma, and if NK activity actually contributes to emphysema progression are unknown. To address these questions, we performed two types of experiments on lung tissue from clinically-indicated resections (n = 60. First, we used flow cytometry on fresh single-cell suspension to measure expression of cell-surface molecules (CD56, CD16, CD8, NKG2D and NKp44 on lung lymphocytes and of the 6D4 epitope common to MICA and MICB on lung epithelial (CD326+ cells. Second, we sequentially isolated CD56+, CD8+ and CD4+ lung lymphocytes, co-cultured each with autologous lung target cells, then determined apoptosis of individual target cells using Annexin-V and 7-AAD staining. Lung NK cells (CD56+ CD3- and CD56+ T cells (CD56+ CD3+ were present in a range of frequencies that did not differ significantly between smokers without COPD and subjects with COPD. Lung NK cells had a predominantly "cytotoxic" CD56+ CD16+ phenotype; their co-expression of CD8 was common, but the percentage expressing CD8 fell as FEV1 % predicted decreased. Greater expression by autologous lung epithelial cells of the NKG2D ligands, MICA/MICB, but not expression by lung CD56+ cells of the activating receptor NKG2D, correlated inversely with FEV1 % predicted. Lung CD56+ lymphocytes, but not CD4+ or CD8+ conventional lung T cells, rapidly killed autologous lung cells without additional stimulation. Such natural cytotoxicity was increased in subjects with severe COPD and was unexplained in multiple regression analysis by age or cancer as indication for surgery. These data show that as spirometry worsens in COPD, CD56+ lung lymphocytes exhibit spontaneous cytotoxicity of autologous structural lung cells, supporting their

  6. 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

    黄慧; 李珊; 张婷婷; 王艳勋; 黄诚; 李立; 李单青; 冯瑞娥; 刘鸿瑞


    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

  7. Stem cell biology and regenerative medicine for neonatal lung diseases.

    Kang, Martin; Thébaud, Bernard


    Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells has been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease. Endothelial progenitor cells are optimally suited to promote lung vascular growth and attenuate pulmonary hypertension in infants with congenital diaphragmatic hernia or a vascular bronchopulmonary dysplasia phenotype. Induced pluripotent stem cells (iPSCs) are one of the most exciting breakthroughs of the past decade. Patient-specific iPSCs can be derived from somatic cells and differentiated into any cell type. iPSCs can be capitalized upon to develop personalized regenerative cell products for surfactant protein deficiencies-lethal lung disorders without treatment-that affect a single gene in a single cell type and thus lend themselves to phenotype-specific cell replacement. While the clinical translation has begun, more needs to be learned about the biology of these repair cells to make this translation successful.Pediatric Research accepted article preview online, 18 September 2017. doi:10.1038/pr.2017.232.

  8. [Basic lung ultrasound. Part 2. Parenchymal diseases].

    de la Quintana Gordon, F B; Nacarino Alcorta, B; Fajardo Pérez, M


    In this second part, an analysis is made of the pathology of lung parenchyma. This text is structured into different sections, including the study of atelectasias, pneumonia and abscess, interstitial/alveolar or Blines patterns, and finally an analysis is made of pulmonary embolism. With this second part, the basic knowledge to develop lung ultrasound in the anesthesia department has been presented. Copyright © 2014 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Diffusion-weighted magnetic resonance imaging for assessment of lung lesions: repeatability of the apparent diffusion coefficient measurement

    Bernardin, L.; Douglas, N.H.M.; Collins, D.J.; Giles, S.L.; O' Flynn, E.A.M.; Orton, M.; DeSouza, N.M. [Institute of Cancer Research and Royal Marsden NHS Foundation Trust, CRUK and EPSRC Cancer Imaging Centre, Surrey (United Kingdom)


    To establish repeatability of apparent diffusion coefficients (ADCs) acquired from free-breathing diffusion-weighted magnetic resonance imaging (DW-MRI) in malignant lung lesions and investigate effects of lesion size, location and respiratory motion. Thirty-six malignant lung lesions (eight patients) were examined twice (1- to 5-h interval) using T1-weighted, T2-weighted and axial single-shot echo-planar DW-MRI (b = 100, 500, 800 s/mm{sup 2}) during free-breathing. Regions of interest around target lesions on computed b = 800 s/mm{sup 2} images by two independent observers yielded ADC values from maps (pixel-by-pixel fitting using all b values and a mono-exponential decay model). Intra- and inter-observer repeatability was assessed per lesion, per patient and by lesion size (> or <2 cm) or location. ADCs were similar between observers (mean ± SD, 1.15 ± 0.28 x 10{sup -3} mm{sup 2}/s, observer 1; 1.15 ± 0.29 x 10{sup -3} mm{sup 2}/s, observer 2). Intra-observer coefficients of variation of the mean [median] ADC per lesion and per patient were 11 % [11.4 %], 5.7 % [5.7 %] for observer 1 and 9.2 % [9.5 %], 3.9 % [4.7 %] for observer 2 respectively; inter-observer values were 8.9 % [9.3 %] (per lesion) and 3.0 % [3.7 %] (per patient). Inter-observer coefficient of variation (CoV) was greater for lesions <2 cm (n = 20) compared with >2 cm (n = 16) (10.8 % vs 6.5 % ADC{sub mean}, 11.3 % vs 6.7 % ADC{sub median}) and for mid (n = 14) vs apical (n = 9) or lower zone (n = 13) lesions (13.9 %, 2.7 %, 3.8 % respectively ADC{sub mean}; 14.2 %, 2.8 %, 4.7 % respectively ADC{sub median}). Free-breathing DW-MRI of whole lung achieves good intra- and inter-observer repeatability of ADC measurements in malignant lung tumours. (orig.)

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

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


    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.

  11. Prevalence of gastroesophageal reflux in end-stage lung disease candidates for lung transplant.

    D'Ovidio, Frank; Singer, Lianne G; Hadjiliadis, Denis; Pierre, Andrew; Waddell, Thomas K; de Perrot, Marc; Hutcheon, Micheal; Miller, Linda; Darling, Gail; Keshavjee, Shaf


    Aspiration secondary to gastroesophageal reflux has been postulated to be a contributing factor in bronchiolitis obliterans after lung transplantation. It is not clear whether gastroesophageal reflux is a preexisting condition or secondary to intraoperative vagal injury or drug-induced prolonged gastric emptying. The prevalence of gastroesophageal reflux was examined in 78 consecutive end-stage lung disease patients assessed for lung transplantation: emphysema, 21; cystic fibrosis, 5; idiopathic pulmonary fibrosis, 26; scleroderma, 10; and miscellaneous diseases, 16. All underwent esophageal manometry. Two-channel esophageal 24-hour pH testing was completed in 76 patients. Gastric emptying studies were conducted in 36 patients. Typical gastroesophageal reflux symptoms were documented in 63% of patients. The lower esophageal sphincter was hypotensive in 72% of patients, and 33% had esophageal body dysmotility. Prolonged gastric emptying was documented in 44%, and 38% had abnormal pH testing. The overall DeMeester score was above normal in 32% of patients, and 20% had abnormal proximal pH probe readings. Gastroesophageal reflux is highly prevalent in end-stage lung disease patients who are candidates for lung transplantation. Further investigation is needed to study the prevalence of gastroesophageal reflux after lung transplantation and its contribution to chronic allograft dysfunction.

  12. Lung function tests in neonates and infants with chronic lung disease: lung and chest-wall mechanics.

    Gappa, Monika; Pillow, J Jane; Allen, Julian; Mayer, Oscar; Stocks, Janet


    This is the fifth paper in a review series that summarizes available data and critically discusses the potential role of lung function testing in infants and young children with acute neonatal respiratory disorders and chronic lung disease of infancy (CLDI). This review focuses on respiratory mechanics, including chest-wall and tissue mechanics, obtained in the intensive care setting and in infants during unassisted breathing. Following orientation of the reader to the subject area, we focused comments on areas of enquiry proposed in the introductory paper to this series. The quality of the published literature is reviewed critically with respect to relevant methods, equipment and study design, limitations and strengths of different techniques, and availability and appropriateness of reference data. Recommendations to guide future investigations in this field are provided. Numerous different methods have been used to assess respiratory mechanics with the aims of describing pulmonary status in preterm infants and assessing the effect of therapeutic interventions such as surfactant treatment, antenatal or postnatal steroids, or bronchodilator treatment. Interpretation of many of these studies is limited because lung volume was not measured simultaneously. In addition, populations are not comparable, and the number of infants studied has generally been small. Nevertheless, results appear to support the pathophysiological concept that immaturity of the lung leads to impaired lung function, which may improve with growth and development, irrespective of the diagnosis of chronic lung disease. To fully understand the impact of immaturity on the developing lung, it is unlikely that a single parameter such as respiratory compliance or resistance will accurately describe underlying changes. Assessment of respiratory mechanics will have to be supplemented by assessment of lung volume and airway function. New methods such as the low-frequency forced oscillation technique, which

  13. NET balancing: A problem in inflammatory lung diseases

    Olivia Z Cheng


    Full Text Available Neutrophil extracellular traps (NETs are beneficial antimicrobial defense structures that can help fight against invading pathogens in the host. However, recent studies reveal that NETs exert adverse effects in a number of diseases including many lung diseases. Most of the inflammatory lung diseases are characterized with a massive influx of neutrophils into the airways. Neutrophils contribute to the pathology of these diseases. To date, NETs have been identified in the lungs of cystic fibrosis, acute lung injury, allergic asthma and lungs infected with bacteria, virus, or fungi. These microbes and several host factors can stimulate NET formation, or NETosis. Different forms of NETosis have been identified; these NETotic pathways are dependent on the types of stimuli. All of these pathways however appear to result in the formation of NETs with DNA, modified extracellular histones, proteases and cytotoxic enzymes. Some of the NET components are immunogenic and damaging to the host tissue. Innate immune collectins such as pulmonary surfactant protein D (SP-D binds NETs, and enhances the clearance of dying cells and DNA by alveolar macrophages. In many inflammatory lung diseases, bronchoalveolar SP-D levels are altered and its deficiency results in the accumulation of DNA in the lungs. Some of the other therapeutic molecules under consideration for treating NET-related diseases include DNases, antiproteases, myeloperoxidase inhibitors, peptidylarginine deiminase-4 inhibitors and anti-histone antibodies. Too much of the good thing can be a bad thing. Maintaining the right balance of NET formation and reducing the amounts of NETs that accumulate in the tissues are essential for harnessing the power of NETs with minimal damage to the hosts.

  14. Lung Regeneration Therapy for Chronic Obstructive Pulmonary Disease

    Oh, Dong Kyu; Kim, You-sun; Oh, Yeon-Mok


    Chronic obstructive pulmonary disease (COPD) is a critical condition with high morbidity and mortality. Although several medications are available, there are no definite treatments. However, recent advances in the understanding of stem and progenitor cells in the lung, and molecular changes during re-alveolization after pneumonectomy, have made it possible to envisage the regeneration of damaged lungs. With this background, numerous studies of stem cells and various stimulatory molecules have...

  15. Diffusion-weighted and diffusion-tensor imaging of normal and diseased uterus

    Duygu; Kara; Bozkurt; Murat; Bozkurt; Mehmet; Ali; Nazli; Ilhan; Nahit; Mutlu; Ozgur; Kilickesmez


    Owing to technical advances and improvement of the software, diffusion weighted imaging and diffusion tensor imaging(DWI and DTI) greatly improved the diagnostic value of magnetic resonance imaging(MRI) of the pelvic region. These imaging sequences can exhibit important tissue contrast on the basis of random diffusion(Brownian motion) of water molecules in tissues. Quantitative measurements can be done with DWI and DTI by apparent diffusion coefficient(ADC) and fractional anisotropy(FA) values respectively. ADC and FA values may be changed by various physiological and pathological conditions providing additional information to conventional MRI. The quantitative DWI assists significantly in the differentiation of benign and malignant lesions. It can demonstrate the microstructural architecture and celluler density of the normal and diseased uterine zones. On the other hand, DWI and DTI are useful for monitoring the treatment outcome of the uterine lesions. In this review, we discussed advantages of DWI and DTI of the normal and diseased uterus.

  16. [Occupational lung diseases other than asbestos- and indium-related disease].

    Kimura, Kiyonobu; Nakano, Ikuo; Ohtsuka, Yosinori; Igarashi, Takeshi; Okamoto, Kenzo


    In our country, pneumoconiosis used to hold an overwhelmingly majority in respiratory occupational lung diseases. Although the number of pneumoconiosis cases has been decreasing certainly, new cases have been arising even today. In addition, in place of pneumoconiosis or asbestos-related diseases, occupational asthma has become the most common forms of occupational lung disease in many industrialized countries. Occupational asthma has been implicated in 9 to 15% of adult asthma in the United States. Although the environmental causes of occupational lung disease are clear, the mechanisms of the diseases are not fully understood and need to be further elucidated.

  17. Estimation of {sup 123}I-metaiodobenzylguanidine lung uptake in heart and lung diseases. With reference to lung uptake ratio and decrease of lung uptake

    Fujii, Tadashige [Shinshu Univ., Matsumoto, Nagano (Japan). School of Allied Medical Sciences; Tanaka, Masao; Yazaki, Yoshikazu; Kitabayashi, Hiroshi; Koizumi, Tomonori; Sekiguchi, Morie; Gomi, Tsutomu; Yano, Kesato; Itoh, Atsuko


    {sup 123}I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy was performed in 64 patients with heart and lung diseases. Distribution of MIBG in the chest was evaluated by planar images, using counts ratios of the heart to the mediastinum (H/M) and the unilateral lung to the mediastinum (Lu/M). Most of patients with heart diseases showed obvious lung uptake of MIBG. The ratios of H/M were 1.75{+-}0.20 in the group without heart failure and 1.55{+-}0.19 in the group with heart failure. The ratios of Lu/M in the right and left lung were 1.56{+-}0.16 and 1.28{+-}0.16 in the group without heart failure. And those were 1.45{+-}0.16 and 1.19{+-}0.15 in the group with heart failure. But 3 patients complicated with chronic pulmonary emphysema and one patient with interstitial pneumonia due to dermatomyositis showed markedly decreased lung uptake. The ratios of Lu/M in the right and left lung of these patients were 1.20, 1.17; 1.17, 1.13; 1.01, 0.97 and 1.27, 0.94, respectively. These results suggest that the lung uptake of MIBG may reflect the state of pulmonary endothelial cell function in clinical situations, considering that it has been demonstrated that MIBG may be useful as a marker of pulmonary endothelial cell function in the isolated rat lung. (author)

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

    Karen L Heathcote


    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.

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

    Shahrzad M Lari


    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

  20. Case-based lung image categorization and retrieval for interstitial lung diseases: clinical workflows.

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


    Clinical workflows and user interfaces of image-based computer-aided diagnosis (CAD) for interstitial lung diseases in high-resolution computed tomography are introduced and discussed. Three use cases are implemented to assist students, radiologists, and physicians in the diagnosis workup of interstitial lung diseases. In a first step, the proposed system shows a three-dimensional map of categorized lung tissue patterns with quantification of the diseases based on texture analysis of the lung parenchyma. Then, based on the proportions of abnormal and normal lung tissue as well as clinical data of the patients, retrieval of similar cases is enabled using a multimodal distance aggregating content-based image retrieval (CBIR) and text-based information search. The global system leads to a hybrid detection-CBIR-based CAD, where detection-based and CBIR-based CAD show to be complementary both on the user's side and on the algorithmic side. The proposed approach is in accordance with the classical workflow of clinicians searching for similar cases in textbooks and personal collections. The developed system enables objective and customizable inter-case similarity assessment, and the performance measures obtained with a leave-one-patient-out cross-validation (LOPO CV) are representative of a clinical usage of the system.

  1. Antioxidant supplementation for lung disease in cystic fibrosis

    Ciofu, Oana; Lykkesfeldt, Jens


    BACKGROUND: Airway infection leads to progressive damage of the lungs in cystic fibrosis and oxidative stress has been implicated in the etiology. Supplementation of antioxidant micronutrients (vitamin E, vitamin C, ß-carotene and selenium) or glutathione may therefore potentially help maintain...... an oxidant-antioxidant balance. Current literature suggests a relationship between oxidative status and lung function. OBJECTIVES: To synthesize existing knowledge of the effect of antioxidants such as vitamin C, vitamin E, ß-carotene, selenium and glutathione in cystic fibrosis lung disease. SEARCH METHODS...... COLLECTION AND ANALYSIS: Two authors independently selected studies, extracted data and assessed the risk of bias in the included studies. We contacted trial investigators to obtain missing information. Primary outcomes are lung function and quality of life; secondary outcomes are oxidative stress...

  2. Lung Regeneration Therapy for Chronic Obstructive Pulmonary Disease.

    Oh, Dong Kyu; Kim, You-Sun; Oh, Yeon-Mok


    Chronic obstructive pulmonary disease (COPD) is a critical condition with high morbidity and mortality. Although several medications are available, there are no definite treatments. However, recent advances in the understanding of stem and progenitor cells in the lung, and molecular changes during re-alveolization after pneumonectomy, have made it possible to envisage the regeneration of damaged lungs. With this background, numerous studies of stem cells and various stimulatory molecules have been undertaken, to try and regenerate destroyed lungs in animal models of COPD. Both the cell and drug therapies show promising results. However, in contrast to the successes in laboratories, no clinical trials have exhibited satisfactory efficacy, although they were generally safe and tolerable. In this article, we review the previous experimental and clinical trials, and summarize the recent advances in lung regeneration therapy for COPD. Furthermore, we discuss the current limitations and future perspectives of this emerging field.

  3. Utility of surgical lung biopsy in critically ill patients with diffuse pulmonary infiltrates: a retrospective review.

    Donaldson, L H; Gill, A J; Hibbert, M


    There are conflicting reports regarding the role of surgical lung biopsies in patients who present to the intensive care unit (ICU) with unexplained respiratory failure and diffuse pulmonary infiltrates on imaging. To describe the utility of surgical lung biopsies in patients presenting to the ICU with unexplained respiratory failure and diffuse pulmonary infiltrates. A retrospective cohort study was performed. All patients admitted to the ICU who underwent a surgical lung biopsy for the investigation of respiratory failure and unexplained pulmonary infiltrates between 1998 and 2012 were included. The primary outcome measures for this descriptive study were the biopsy histopathology, changes in patient management following biopsy and in-hospital mortality. A total of 30 patients was included in the review. Biopsies in 22 patients (73%) demonstrated diffuse alveolar damage (DAD), with 15 of these biopsies (50%) suggesting a specific underlying aetiology. In 73% of cases (n = 22), the biopsy finding was associated with a change in management, although this generally involved the escalation of an existing therapy rather than initiation of a new treatment. Biopsies were performed at a median 10 days after admission (interquartile range 5-17 days), with the majority of patients being treated empirically prior to the biopsy with systemic steroids and broad-spectrum antimicrobials. Mortality was 53%. In this series, DAD was the most frequent pathology. The biopsy result was associated with a change in management in a majority of the subjects, most frequently an escalation of prior empiric therapy. Mortality was high. © 2016 Royal Australasian College of Physicians.

  4. Morphological respiratory diffusion capacity of the lungs of ball pythons (Python regius).

    Starck, J Matthias; Aupperle, Heike; Kiefer, Ingmar; Weimer, Isabel; Krautwald-Junghanns, Maria-Elisabeth; Pees, Michael


    This study aims at a functional and morphological characterization of the lung of a boid snake. In particular, we were interested to see if the python's lungs are designed with excess capacity as compared to resting and working oxygen demands. Therefore, the morphological respiratory diffusion capacity of ball pythons (Python regius) was examined following a stereological, hierarchically nested approach. The volume of the respiratory exchange tissue was determined using computed tomography. Tissue compartments were quantified using stereological methods on light microscopic images. The tissue diffusion barrier for oxygen transport was characterized and measured using transmission electron micrographs. We found a significant negative correlation between body mass and the volume of respiratory tissue; the lungs of larger snakes had relatively less respiratory tissue. Therefore, mass-specific respiratory tissue was calculated to exclude effects of body mass. The volume of the lung that contains parenchyma was 11.9±5.0mm(3)g(-1). The volume fraction, i.e., the actual pulmonary exchange tissue per lung parenchyma, was 63.22±7.3%; the total respiratory surface was, on average, 0.214±0.129m(2); it was significantly negatively correlated to body mass, with larger snakes having proportionally smaller respiratory surfaces. For the air-blood barrier, a harmonic mean of 0.78±0.05μm was found, with the epithelial layer representing the thickest part of the barrier. Based on these findings, a median diffusion capacity of the tissue barrier ( [Formula: see text] ) of 0.69±0.38ml O(2)min(-1)mmHg(-1) was calculated. Based on published values for blood oxygen concentration, a total oxygen uptake capacity of 61.16mlO(2)min(-1)kg(-1) can be assumed. This value exceeds the maximum demand for oxygen in ball pythons by a factor of 12. We conclude that healthy individuals of P. regius possess a considerable spare capacity for tissue oxygen exchange.

  5. Imaging of cystic fibrosis lung disease and clinical interpretation

    Wielpuetz, M.O.; Eichinger, M.; Kauczor, H.U. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Biederer, J. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Gross-Gerau Community Hospital (Germany). Radiologie Darmstadt; Wege, S. [Heidelberg University Hospital (Germany). Dept. of Pulmonology and Respiratory Medicine; Stahl, M.; Sommerburg, O. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Mall, M.A. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Heidelberg University Hospital (Germany). Dept. of Translational Pulmonology; Puderbach, M. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Hufeland Hospital, Bad Langensalza (Germany). Dept. of Diagnostic and Interventional Radiology


    Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine. Chest X-ray (CXR), computed tomography (CT) and magnetic resonance imaging (MRI) are now available as routine modalities, each with individual strengths and drawbacks, which need to be considered when choosing the optimal modality adapted to the clinical situation of the patient. CT stands out with the highest morphological detail and has often been a substitute for CXR for regular severity monitoring at specialized centers. Multidetector CT data can be post-processed with dedicated software for a detailed measurement of airway dimensions and bronchiectasis and potentially a more objective and precise grading of disease severity. However, changing to CT was inseparably accompanied by an increase in radiation exposure of CF patients, a young population with high sensitivity to ionizing radiation and lifetime accumulation of dose. MRI as a cross-sectional imaging modality free of ionizing radiation can depict morphological hallmarks of CF lung disease at lower spatial resolution but excels with comprehensive functional lung imaging, with time-resolved perfusion imaging currently being most valuable.

  6. Bleb Point: Mimicker of Pneumothorax in Bullous Lung Disease

    Gelabert, Christopher


    Full Text Available In patients presenting with severe dyspnea, several diagnostic challenges arise in distinguishing the diagnosis of pneumothorax versus several other pulmonary etiologies like bullous lung disease, pneumonia, interstitial lung disease, and acute respiratory distress syndrome. Distinguishing between large pulmonary bullae and pneumothorax is of the utmost importance, as the acute management is very different. While multiple imaging modalities are available, plain radiographs may be inadequate to make the diagnosis and other advanced imaging may be difficult to obtain. Ultrasound has a very high specificity for pneumothorax. We present a case where a large pulmonary bleb mimics the lung point and therefore inaccurately suggests pneumothorax. [West J Emerg Med. 2015;16(3:447–449.

  7. Lung fibrosis quantified by HRCT in scleroderma patients with different disease forms and ANA specificities

    S. Mancin


    Full Text Available Objective: to define the prevalence of interstitial lung fibrosis in systemic sclerosis (SSc and its relationship with the different clinical forms of disease and ANA specificities. Methods: fifty patients with SSc were submitted to pulmonary high resolution computed tomography (HRCT. Lung abnormalities were evaluated according to Warrick’s score that considers both the severity and the extent of fibrotic lesions. Results: pulmonary HRCT abnormalities were observed in 84% of SSc patients. Ground glass aspects (60%, irregular pleural margins (56% and septal/subpleural lines (68% were the most common lesions. The distribution of these abnormalities favoured the posterior basilar segments of both lungs. HRCT findings were significantly more frequent in males and in patients with the cutaneous diffuse form of SSc and with the specific antibody anti-Scl70. Conclusions: HRCT is a very useful method for the diagnosis of interstitial lung fibrosis in SSc. Warrick’s score permits to quantify the HRCT findings and to evaluate their relationship with the disease clinical forms and ANA specificities.

  8. Lung Disease Associated With Marijuana Use.

    Chatkin, José Miguel; Zabert, Gustavo; Zabert, Ignacio; Chatkin, Gustavo; Jiménez-Ruiz, Carlos Andrés; de Granda-Orive, Jose Ignacio; Buljubasich, Daniel; Solano Reina, Segismundo; Figueiredo, Ana; Ravara, Sofia; Riesco Miranda, Juan Antonio; Gratziou, Christina


    Marijuana is the most widely usedillegal drug in the world, with a prevalence of 2.5%-5%, and the second most commonly smoked substance after tobacco. The components of smoke from combustion of marijuana are similar to those produced by the combustion of tobacco, but they differ in terms of psychoactive components and use. Inhalation of cannabis smoke affects the respiratory tract, so the available evidence must be updated in order to provide pulmonologists with the latest scientific information. In this article, we review the impact of cannabis consumption on the lungs, taking into account that the respiratory route is the most popular route of cannabis consumption. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Diffusion formation and psychiatric diseases; Diffusionsbildung und psychiatrische Erkrankungen

    Reith, W.; Kulikovski, J. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)


    The basic principle behind diffusion is Brownian motion. The diffusion parameters obtained in a clinical association provide information on the spatial distribution of water molecule mobility and, therefore, evidence of the morphological integrity of the white and grey matters of the brain. In recent years functional magnetic resonance imaging (fMRI) could contribute to obtaining a detailed understanding of the cortical and subcortical cerebral networks. Diffusion tensor imaging (DTI) investigations can demonstrate the extent of anisotropy and the fiber pathways in so-called parametric images. For example, in Alzheimer's disease DTI reveals a reduced structural connectivity between the posterior cingulum and the hippocampus. This article shows examples of the application of diffusion-weighted imaging (DWI) in psychiatric disorders. (orig.) [German] Die Grundlagen der Diffusion bildet die brownsche Molekularbewegung. Die im klinischen Zusammenhang gewonnenen Diffusionsparameter geben Auskunft ueber die raeumliche Verteilung der Wassermolekuelmobilitaet und damit Hinweise auf die morphologische Integritaet der weissen und grauen Hirnsubstanz. Die funktionelle MRT (fMRT) konnte in den letzten Jahren dazu beitragen, ein detailliertes Verstaendnis der kortikalen und subkortikalen zerebralen Netzwerke zu erlangen. Diffusion-tensor-imaging(DTI)-Untersuchungen koennen in sogenannten Parameterbildern das Ausmass der Anisotropie und den Faserverlauf darstellen. So zeigte z. B. die DTI bei der Alzheimer-Demenz eine verminderte strukturelle Konnektivitaet zwischen dem posterioren Zingulum und dem Hippokampus. Dieser Beitrag zeigt Beispiele der Anwendung der Diffusionsbildgebung (''diffusion-weighted imaging'', DWI) bei psychiatrischen Erkrankungen. (orig.)

  10. Rheumatoid interstitial lung disease presenting as cor pulmonale

    Acharya Sourya


    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.

  11. Rheumatoid interstitial lung disease presenting as cor pulmonale.

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


    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.

  12. Lung Dendritic cells: Targets for therapy in allergic disease

    B.N.M. Lambrecht (Bart)


    textabstractDendritic cells are crucial in determining the functional outcome of allergen encounter in the lung. Antigen presentation by myeloid DCs leads to Th2 sensitization typical of allergic disease, whereas antigen presentation by plasmacytoid DCs serves to dampen inflammation. It is increasin

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

    G. De Marchi


    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.

  14. Diffusion-weighted MRI of maple syrup urine disease encephalopathy

    Cavalleri, F.; Mavilla, L. [Servizio di Neuroradiologia, Azienda Ospedaliera Policlinico, Modena (Italy); Berardi, A.; Ferrari, F. [Servizio di Neonatologia, Azienda Ospedaliera Policlinico, Modena (Italy); Burlina, A.B. [Dipartimento di Pediatria, Azienda Ospedaliera, Universita di Padova, Padua (Italy)


    We report the case of a newborn child with maple syrup urine disease (MSUD), diagnosed at 10 days of life. Diffusion-weighted echoplanar MRI showed marked hyperintensity of the cerebellar white matter, the brainstem, the cerebral peduncles, the thalami, the dorsal limb of the internal capsule and the centrum semiovale, while conventional dual-echo sequence evidenced only a weak diffuse T2 hyperintensity in the cerebellar white matter and in the dorsal brainstem. The apparent diffusion coefficient (ADC) of these regions was markedly (>80%) decreased. Therefore, in agreement with current hypotheses on MSUD pathogenesis, MSUD oedema proves to be a cytotoxic oedema. Diffusion-weighted MRI may be a valuable tool, more sensitive than conventional spin-echo techniques, to assess the extent and progression of cytotoxicity in MSUD, as well as the effectiveness of the therapeutic interventions. (orig.)

  15. 18F-FDG Uptake in Less Affected Lung Field Provides Prognostic Stratification in Patients with Interstitial Lung Disease.

    Nobashi, Tomomi; Kubo, Takeshi; Nakamoto, Yuji; Handa, Tomohiro; Koyasu, Sho; Ishimori, Takayoshi; Mishima, Michiaki; Togashi, Kaori


    This study evaluated the clinical significance of (18)F-FDG PET/CT in patients with interstitial lung disease (ILD), by investigating the relationships between (18)F-FDG PET/CT parameters and clinical indicators and by evaluating the prognostic implications of (18)F-FDG PET/CT results. Ninety patients (51 men, 39 women; mean age, 55.4 y; age range, 26-78 y) with ILD who underwent (18)F-FDG PET/CT were retrospectively analyzed. SUVmean was defined as the mean SUV of the less-affected lung field, SUVTF as adjusted SUVmean using tissue fraction (TF), and CTmean as the mean attenuation of the corresponding region of interest on high-resolution CT. SUVmean, SUVTF, and CTmean were compared in the 90 ILD patients and in 15 age- and sex-matched controls. Correlations of SUVmax, SUVmean, SUVTF, and CTmean with clinical indicators, including estimated percentage of forced vital capacity (%FVC), estimated percentage of diffusion capacity of the lungs for carbon monoxide (%DLco), sialylated carbohydrate antigen Krebs von den Lungen-6 (KL-6), surfactant protein D (SP-D), C-reactive protein (CRP), lactate dehydrogenase (LDH), and ILD-sex-age-physiology (GAP) index, were evaluated using the Spearman rank correlation test and the Tukey-Kramer test. A Cox proportional hazards model was used for univariate and multivariate analyses of factors associated with lung transplantation-free survival. SUVmean, SUVTF, and CTmean were significantly higher in ILD patients than in healthy controls, except for CTmean in patients with a nonusual interstitial pneumonia pattern. SUVmean and CTmean were significantly correlated with %FVC, %DLco, KL-6, and SP-D; SUVTF was significantly correlated with %DLco, KL-6, SP-D, and LDH; and SUVmax was weakly correlated with KL-6 and CRP. Univariate analysis showed that SUVmean, SUVTF, sex, %FVC, %DLco, KL-6, and ILD-GAP index were significantly prognostic of lung transplantation-free survival; and multivariate analysis showed that SUVmean and ILD-GAP index

  16. Lung clearance index in the assessment of airways disease.

    Horsley, Alex


    In the last few years there has been a growing interest in lung clearance index (LCI), a measure of lung physiology derived from multiple breath washout tests. This resurgence of interest was initially driven by the recognition that such assessments were capable of detecting early airways disease in children, and are more sensitive and easier to perform in this population than conventional lung function tests [Aurora P, Kozlowska W, Stocks J. Gas mixing efficiency from birth to adulthood measured by multiple-breath washout. Respir Physiol Neurobiol, 2005;148(1-2):125-39]. With an appreciation of the importance of earlier identification of airways dysfunction, and prevention of irreversible structural airway changes, methods of following airways disease in these "silent years" are especially important. LCI has now been reported in studies involving all age groups, from infants to adults [Lum S, Gustafsson P, Ljungberg H, Hulskamp G, Bush A, Carr SB, et al. Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax, 2007;62(4):341-7; Horsley AR, Gustafsson PM, Macleod K, Saunders CJ, Greening AP, Porteous D, et al. Lung clearance index is a sensitive, repeatable and practical measure of airways disease in adults with cystic fibrosis. Thorax, 2008;63:135-40], and has a narrow range of normal over this wide age range, making it especially suitable for long-term follow-up studies. In cystic fibrosis (CF) particularly, there is a pressing need for sensitive and repeatable clinical endpoints for therapeutic interventions [Rosenfeld M. An overview of endpoints for cystic fibrosis clinical trials: one size does not fit all. Proc Am Thorac Soc, 2007;4(4):299-301], and LCI has been proposed as an outcome measure in future CF gene therapy studies [Davies JC, Cunningham S, Alton EW, Innes JA. Lung clearance index in CF: a sensitive marker of lung disease severity. Thorax, 2008;63(2):96-7]. This review will consider how LCI is

  17. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

    Stephen C Juvet


    Full Text Available Pulmonary Langerhans’ cell histiocytosis (PLCH is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.

  18. Occupational lung diseases and the mining industry in Mongolia.

    Lkhasuren, Oyuntogos; Takahashi, Ken; Dash-Onolt, Lkhamsuren


    Mining production has accounted for around 50% of the gross industrial product in Mongolia since 1998. Dust-induced chronic bronchitis and pneumoconiosis currently account for the largest relative share (67.8%) of occupational diseases in Mongolia, and cases are increasing annually. In 1967-2004, medically diagnosed cases of occupational diseases in Mongolia numbered 7,600. Of these, 5,154 were confirmed cases of dust-induced chronic bronchitis and pneumoconiosis. Lung diseases and other mining-sector health risks pose major challenges for Mongolia. Gold and coal mines, both formal and informal, contribute significantly to economic growth, but the prevalence of occupational lung diseases is high and access to health care is limited. Rapid implementation of an effective national program of silicosis elimination and pneumoconiosis reduction is critical to ensure the health and safety of workers in this important sector of the Mongolian economy.

  19. Occupational lung diseases and the mining industry in Mongolia

    Lkhasuren, O.; Takahashi, K.; Dash-Onolt, L. [Health Science University of Mongolia, Ulaanbaatar (Mongolia)


    Mining production has accounted for around 50% of the gross industrial product in Mongolia since 1998. Dust-induced chronic bronchitis and pneumoconiosis currently account for the largest relative share (67.8%) of occupational diseases in Mongolia, and cases are increasing annually. In 1967-2004, medically diagnosed cases of occupational diseases in Mongolia numbered 7,600. Of these, 5,154 were confirmed cases of dust-induced chronic bronchitis and pneumoconiosis. Lung diseases and other mining-sector health risks pose major challenges for Mongolia. Gold and coal mines, both formal and informal, contribute significantly to economic growth, but the prevalence of occupational lung diseases is high and access to health care is limited. Rapid implementation of an effective national program of silicosis elimination and pneumoconiosis reduction is critical to ensure the health and safety of workers in this important sector of the Mongolian economy.

  20. [Pulmonary diffusion test to NO and CO time course during thoracic radiotherapy for lung cancer: the CONORT prospective study protocol].

    Zarza, V; Couraud, S; Hassouni, A; Prévost, C; Souquet, P-J; Letanche, G; Hammou, Y; Girard, N; Viart-Ferber, C; Mornex, F


    Thoracic radiotherapy is a usual treatment for lung cancer. Early-stages may be treated in stereotactic mode while locally advanced stages are usually treated with conventional radiotherapy mode. Pulmonary function tests show that thoracic irradiation has no impact on lung volume such as forced expiratory volume in one second (FEV1) or forced vital capacity (FCV). However, some studies found that CO (carbon monoxide) diffusing capacity (TLCO) may be altered under thoracic radiotherapy. DLCO alteration is usually symptomatic of either a lesion in the alveolar membrane or a pulmonary capillary alteration. Pulmonary diffusion may be also appreciated by the NO (azote monoxide) diffusion capacity. Moreover, using a double measurement of NO and CO diffusing capacities permit to assess which lung compartment (capillary or membrane) is affected. CONORT is an observational prospective monocentric study, aiming to assess the CO and NO diffusing capacity (as well as other pulmonary function tests) during thoracic radiotherapy. Inclusion criteria are patients with lung cancer, treated by thoracic radiotherapy (conformational or stereotactic), who signed consent. Pulmonary function tests are performed before, during, at the end and six weeks and six months after thoracic irradiation. To estimate a difference of 15% in diffusing capacity test, we have to include 112 patients with a 90% power and a 5% alpha risk. Four months after beginning, 36 patients were included. Preliminary data will be presented at the SFRO meeting.

  1. Lung clearance index for monitoring early lung disease in alpha-1-antitrypsin deficiency.

    Fuchs, Susanne I; Schwerk, Nicolaus; Pittschieler, Klaus; Ahrens, Frank; Baden, Winfried; Bals, Robert; Fähndrich, Sebastian; Gleiber, Wolfgang; Griese, Matthias; Hülskamp, Georg; Köhnlein, Thomas; Reckling, Ludmilla; Rietschel, Ernst; Staab, Doris; Gappa, Monika


    Patients with alpha-1-antitrypsin deficiency (AATD) and a PI-ZZ genotype are at high risk to develop severe emphysema during adulthood. However, little is known about early stages of emphysema and disease manifestation in other PI-types. Spirometry is commonly used for monitoring although early manifestation of emphysema is suspected within the peripheral airways that are not accessible by forced expiratory manoeuvres. We hypothesized that the Lung Clearance Index (LCI) derived from multiple breath nitrogen-washout (N2-washout) is useful to bridge this diagnostic gap. Patients from age 4 years onward and different PI-types performed N2-washout and spirometry. Results were compared to controls. 193 patients (4-79 years, 75% PI-ZZ) and 33 controls (8-60 years) were included. Mean (SD) LCI in patients was 9.1 (3.1) and 6.3 (0.6) in controls (p ≤ 0.001). 47% of adult patients with other than PI-ZZ genotypes and 39% of all patients with normal spirometry had abnormal LCIs. The LCI measured by N2-washout discriminates between patients with AATD and controls, reflects AATD related lung disease in all stages and appears to identify early peripheral lung changes in younger age than spirometry. We conclude that a normal spirometry does not exclude presence of AATD related lung disease even in genotypes other than PI-ZZ.

  2. Lung diffusing capacity for nitric oxide at lowered and raised ambient pressures.

    Linnarsson, Dag; Hemmingsson, Tryggve E; Frostell, Claes; Van Muylem, Alain; Kerckx, Yannick; Gustafsson, Lars E


    Lung diffusing capacity for NO (DLNO) was determined in eight subjects at ambient pressures of 505, 1015, and 4053hPa (379, 761 and 3040mmHg) as they breathed normoxic gases. Mean values were 116.9±11.1 (SEM), 113.4±11.1 and 99.3±10.1mlmin(-1)hPa(-1)at 505, 1015, and 4053hPa, with a 13% difference between the two higher pressures (P=0.017). The data were applied to a model with two serially coupled conductances; the gas phase (DgNO, variable with pressure), and the alveolo-capillary membrane (DmNO, constant). The data fitted the model well and we conclude that diffusive transport of NO in the peripheral lung is inversely related to gas density. At normal pressure DmNO was approximately 5% larger than DLNO, suggesting that the Dg factor then is not negligible. We also conclude that the density of the breathing gas is likely to impact the backdiffusion of naturally formed NO from conducting airways to the alveoli.

  3. Symptom Burden of Chronic Lung Disease Compared with Lung Cancer at Time of Referral for Palliative Care Consultation.

    Wysham, Nicholas G; Cox, Christopher E; Wolf, Steven P; Kamal, Arif H


    A growing evidence base supports provision of palliative care services alongside life-prolonging care. Whereas palliative care processes have been implemented widely in the care of patients with lung cancer, the same is not true for patients with chronic, progressive lung disease. To compare the symptom burden of chronic lung disease with that of lung cancer at the time of initial palliative care consultation. Data were abstracted from the Carolinas Palliative Care Consortium's Quality Data Collection Tool, an electronic database used by seven academic and community palliative care practices in multiple states for quality improvement purposes. We analyzed data derived from first palliative care encounters collected during a 2-year period, including the primary diagnosis of chronic lung disease or lung cancer, unresolved symptoms, setting of initial palliative care encounter, Palliative Performance Scale status, and on that basis we estimated prognosis for survival. We compared key clinical variables between chronic lung disease and lung cancer using Kruskal-Wallis and χ(2) tests. We identified 152 patients with lung cancer and 86 patients with chronic lung disease. Of the total sample, 53% were women and 87% were white. Patients with chronic lung disease were more likely than those with lung cancer to have the initial palliative care encounter occur in the intensive care unit (17% vs. 6%; P = 0.005) and less likely as an outpatient (20% vs. 56%; P < 0.0001). Patients with chronic lung disease were also less likely to have a high Palliative Performance Scale status (14% vs. 30%; P = 0.009) but more likely to have an estimated prognosis for survival longer than 6 months (51% vs. 28%; P = 0.002). The most prevalent symptoms were dyspnea (55% vs. 42%) and pain (40% vs. 52%), neither of which differed between groups (P = 0.08). Patients with chronic lung disease have symptom burdens similar to those of patients with lung cancer at the time of first

  4. Lung transplantation in the elderly: Influence of age, comorbidities, underlying disease, and extended criteria donor lungs.

    Ehrsam, Jonas P; Benden, Christian; Seifert, Burkhardt; Opitz, Isabelle; Schneiter, Didier; Weder, Walter; Inci, Ilhan


    As large registries show an increased risk for lung transplant recipients aged 60 years or more, few single centers report favorable outcomes for carefully selected older recipients without providing essential details. The purpose of our study was to determine variables that influence survival in the elderly. All adult bilateral first lung transplants between January 2000 and December 2014 were divided in 2 groups: those aged less than 60 years (N = 223) and those aged 60 years or more (N = 83). The Charlson-Deyo Index determined recipient comorbidities. The Oto Donor Score assessed donor lung quality. Recipients aged 60 years or more had a significant lower median survival compared with their younger counterparts (48 vs 112 months, respectively, P disease, and donor lung quality appear to be more important than age in reducing long-term survival. Older age serves as a marker for a complex constellation of factors that might be considered the relative or absolute contraindication to lung transplantation rather than age, per se. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  5. 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

    丁艳芩; 朱红; 姚婉贞; 赵鸣武


    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

  6. [Postnatal corticosteroids in preterm infants with immature lung disease].

    Hinriksdottir, Erna; Brynjarsson, Hrolfur; Thorkelsson, Thordur


    Corticosteroids have been used in preterm infants with immature lungs to decrease their need for supplemental oxygen and mechanical ventilation. Whether the benefits of the treatment outweigh possible adverse effects remains controversial. The main objective of the study was to evaluate the effects of intravenous and inhalation corticosteroids on preterm infants' need for supplemental oxygen and mechanical ventilation and potential adverse effects. This was a retrospective cohort study on preterm infants at the Neonatal Intensive Care Unit of Children's Hospital Iceland, born between 2000-2014 and treated with intravenous (n=28) or inhalation (n=30) corticosteroids for immature lung disease. For each infant receiving steriods one infant who did not receive steriods was selected as control, matched on gestational age. There was a significant decrease in the need for supplemental oxygen following intravenous and inhalation corticosteroids administration, and a significant decrease in the need for mechanical ventilation following intravenous corticosteroids administration, but not in controls. Infants receiving intravenous corticosteroids gained significantly less weight than controls during treatment, but no significant difference in weight between groups was found at 35 weeks postmenstrual age, or in other possible adverse effects such as the prevalence of cerebral palsy. Intravenous and inhalation corticosteroids decrease the need for supplemental oxygen in preterm infants with immature lung disease and intravenous steriods facilitate earlier weaning from mechanical ventilation, without significant adverse effects. Therefore, it seems justifiable in selected cases to use corticosteroids in treatment of preterm infants with severe immature lung disease. Corticosteroids, preterm infants, chronic lung disease, mechanical ventilation. Correspondence: Thorður Thorkelsson,

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

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


    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...

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

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


    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. Copyright © 2011 Elsevier Ltd. All rights reserved.

  9. The Lung Microbiome and Airway Disease.

    Lynch, Susan V


    A growing body of literature has demonstrated relationships between the composition of the airway microbiota (mixed-species communities of microbes that exist in the respiratory tract) and critical features of immune response and pulmonary function. These studies provide evidence that airway inflammatory status and capacity for repair are coassociated with specific taxonomic features of the airway microbiome. Although directionality has yet to be established, the fact that microbes are known drivers of inflammation and tissue damage suggests that in the context of chronic inflammatory airway disease, the composition and, more importantly, the function, of the pulmonary microbiome represent critical factors in defining airway disease outcomes.

  10. Antimicrobial Peptides and Innate Lung Defenses: Role in Infectious and Noninfectious Lung Diseases and Therapeutic Applications.

    Hiemstra, Pieter S; Amatngalim, Gimano D; van der Does, Anne M; Taube, Christian


    Respiratory infections are a major clinical problem, and treatment is increasingly complicated by the emergence of microbial antibiotic resistance. Development of new antibiotics is notoriously costly and slow; therefore, alternative strategies are needed. Antimicrobial peptides, central effector molecules of the immune system, are being considered as alternatives to conventional antibiotics. These peptides display a range of activities, including not only direct antimicrobial activity, but also immunomodulation and wound repair. In the lung, airway epithelial cells and neutrophils in particular contribute to their synthesis. The relevance of antimicrobial peptides for host defense against infection has been demonstrated in animal models and is supported by observations in patient studies, showing altered expression and/or unfavorable circumstances for their action in a variety of lung diseases. Importantly, antimicrobial peptides are active against microorganisms that are resistant against conventional antibiotics, including multidrug-resistant bacteria. Several strategies have been proposed to use these peptides in the treatment of infections, including direct administration of antimicrobial peptides, enhancement of their local production, and creation of more favorable circumstances for their action. In this review, recent developments in antimicrobial peptides research in the lung and clinical applications for novel therapies of lung diseases are discussed.

  11. Detection of interstitial lung disease in PA chest radiographs

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


    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.

  12. Segmentation of interstitial lung disease patterns in HRCT images

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


    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.

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

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


    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.

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

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


    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.

  15. Risk of postoperative complications in chronic obstructive lung diseases patients considered fit for lung cancer surgery: beyond oxygen consumption.

    Shafiek, Hanaa; Valera, Jose Luis; Togores, Bernat; Torrecilla, Juan Antonio; Sauleda, Jaume; Cosío, Borja G


    Patients with poor lung function usually undergo cardiopulmonary exercise testing (CPET) and those with a predicted postoperative maximal oxygen consumption (VO2 max) of >10 ml/kg/min undergo lung resection surgery and still some complications are observed. We aimed to determine other parameters beyond VO2 able to predict postoperative complications in patients undergoing lung resection surgery. This is an observational study with longitudinal follow-up. Patients with forced expiratory volume in 1 second (FEV1) or diffusing capacity for carbon monoxide of VO2 max of >10 ml/kg/min were considered fit for surgery. Patients were followed up prospectively for 12 months and postoperative complications and survival were recorded. Physiological parameters obtained during CPET and pulmonary function tests were analysed. Eighty-three chronic obstructive pulmonary disease (COPD) patients were evaluated for surgery between 2010 and 2015. Twenty-four patients were considered unfit for surgery and received an alternative therapy. Fifty-five patients had a VO2 max of >10 ml/kg/min and underwent lung surgery. Among them, 4% died and 41% developed complications postoperatively. Baseline minute ventilation to carbon dioxide output (VE/VCO2) slope was significantly higher among those who developed postoperative complications or died (P = 0.047). Furthermore, VE/VCO2 slope of >35 (at maximal exercise) was the single parameter most strongly associated with the probability of mortality and postoperative complications (hazard ratio 5.14) with a survival probability of 40% after 1 year of follow-up. In a multivariable model, VO2, VE/VCO2 slope of >35 and work load were independently associated with the probability of having an event. VO2 is not the unique parameter to consider when CPET is performed to evaluate the postoperative risk of lung cancer surgery in COPD patients. The signs of ventilatory inefficiency such as VE/VCO2 slope predict complications better than VO2 does. © The

  16. Suppressing disease spreading by using information diffusion on multiplex networks

    Wang, Wei; Cai, Shi-Min; Tang, Ming; Braunstein, Lidia A; Stanley, H Eugene


    Although there is always an interplay between the dynamics of information diffusion and disease spreading, the empirical research on the systemic coevolution mechanisms connecting these two spreading dynamics is still lacking. Here we investigate the coevolution mechanisms and dynamics between information and disease spreading by utilizing real data and a proposed model on multiplex network. Our empirical analysis finds asymmetrical interactions between the information and disease spreading dynamics. Our results obtained from both the theoretical framework and intensive stochastic numerical simulations suggest that an information outbreak can be triggered on a communication network by its own spreading dynamics or the disease outbreak on a contact network, but that the disease threshold is not affected by information spreading. Our key finding is that there is an optimal information transmission rate that markedly suppresses the disease speading. We find that the time evolution of the dynamics in the proposed...

  17. Apparent diffusion coefficient values of diffusion-weighted imaging for distinguishing focal pulmonary lesions and characterizing the subtype of lung cancer: a meta-analysis

    Shen, Guohua; Jia, Zhiyun; Deng, Houfu [Sichuan University, Department of Nuclear Medicine, West China Hospital, Chengdu, Sichuan (China)


    The potential performance of apparent diffusion coefficient (ADC) values for distinguishing malignant and benign pulmonary lesions, further characterizing the subtype of lung cancer was assessed. PubMed, EMBASE, Cochrane Library, EBSCO, and three Chinese databases were searched to identify eligible studies on diffusion-weighted imaging (DWI) of focal pulmonary lesions. ADC values of malignant and benign lesions were extracted by lesion type and statistically pooled based on a linear mixed model. Further analysis for subtype of lung cancer was also performed. The methodological quality was assessed using the quality assessment of diagnostic accuracy studies tool. Thirty-four articles involving 2086 patients were included. Malignant pulmonary lesions have significantly lower ADC values than benign lesions [1.21 (95 % CI, 1.19-1.22) mm{sup 2}/s vs. 1.76 (95 % CI, 1.72-1.80) mm{sup 2}/s; P < 0.05]. There is a significant difference between ADC values of small cell lung cancer and non-small cell lung cancer (P < 0.05), while the differences were not significant among histological subtypes of lung cancer. The methodological quality was relatively high, and the data points from Begg's test indicated that there was probably no obvious publication bias. The ADC value is helpful for distinguishing malignant and benign pulmonary lesions and provides a promising method for differentiation of SCLC from NSCLC. (orig.)

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

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


    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_

  19. Prioritization of candidate disease genes by topological similarity between disease and protein diffusion profiles.

    Zhu, Jie; Qin, Yufang; Liu, Taigang; Wang, Jun; Zheng, Xiaoqi


    Identification of gene-phenotype relationships is a fundamental challenge in human health clinic. Based on the observation that genes causing the same or similar phenotypes tend to correlate with each other in the protein-protein interaction network, a lot of network-based approaches were proposed based on different underlying models. A recent comparative study showed that diffusion-based methods achieve the state-of-the-art predictive performance. In this paper, a new diffusion-based method was proposed to prioritize candidate disease genes. Diffusion profile of a disease was defined as the stationary distribution of candidate genes given a random walk with restart where similarities between phenotypes are incorporated. Then, candidate disease genes are prioritized by comparing their diffusion profiles with that of the disease. Finally, the effectiveness of our method was demonstrated through the leave-one-out cross-validation against control genes from artificial linkage intervals and randomly chosen genes. Comparative study showed that our method achieves improved performance compared to some classical diffusion-based methods. To further illustrate our method, we used our algorithm to predict new causing genes of 16 multifactorial diseases including Prostate cancer and Alzheimer's disease, and the top predictions were in good consistent with literature reports. Our study indicates that integration of multiple information sources, especially the phenotype similarity profile data, and introduction of global similarity measure between disease and gene diffusion profiles are helpful for prioritizing candidate disease genes. Programs and data are available upon request.

  20. Diffusion-weighted MR imaging of fetal lung maturation in sheep: effect of prenatal cortisone administration on ADC values.

    Much, Chressen Catharina; Schoennagel, Björn Phillip; Yamamura, Jin; Buchert, Ralph; Kooijman, Hendrik; Schätzle, Anne-Kathrin; Adam, Gerhard; Wedegaertner, Ulrike


    To assess changes in diffusion properties in the fetal lung after cortisone administration with diffusion-weighted imaging (DWI) in fetal sheep. DWI was performed on 11 pregnant sheep with singleton pregnancies on a 1.5-T MRI scanner. Four animals received cortisone injections before baseline imaging. Seven animals served as controls. Apparent diffusion coefficient (ADC) was measured on DWI in the fetal lungs by two independent readers. The Pearson test was used to correlate ADC and gestational age. A t-test was performed to compare differences in ADC values at the baseline and follow-up images within and between groups. Inter-rater reliability was calculated. In the cortisone group, ADC values increased about 10 % between the baseline and follow-up images (P = 0.039). Comparing the cortisone and control groups, ADC values of the baseline images did not differ; whereas in the follow-up imaging, ADC values were significantly higher in the cortisone group (P = 0.024). Lung ADC values did not correlate with gestational age (P = 0.970). Inter-rater reliability was high (0.970, P = 0.000). In this experimental model, MR-DWI can detect cortisone-induced changes in diffusion properties of the fetal lung. • Corticosteroids are frequently administered antenatally to prevent fetal lung immaturity at birth • DWI can detect changes in the fetal lung after corticosteroid administration • Changes can be detected as early as 5 days after treatment • Fetal MRI may offer a non-invasive method of monitoring lung maturation.

  1. Nanomedicine and therapy of lung diseases

    Garcia, Fabricio de Melo, E-mail: [Faculdade de Medicina Nova Esperanca, Joao Pessoa, PB (Brazil)


    The use of nanotechnology has significantly increased in different fields of science, including the development of drug delivery systems. Currently, the most modern pharmaceutical nanocarriers, such as liposomes, micelles, nanoemulsions and polymeric nanoparticles, demonstrate extremely useful properties from the point of view of drug therapy. In this context, the development of nanocarriers for pulmonary application has been much debated by the scientific community in recent decades. Although research on the use of nanoparticles for pulmonary application are still in the initial phase, the studies conducted to date suggest that the development of drug delivery systems for systemic or local treatment of diseases that affect the respiratory system may be promising. (author)

  2. Lung microbiome for clinicians. New discoveries about bugs in healthy and diseased lungs.

    Segal, Leopoldo N; Rom, William N; Weiden, Michael D


    Microbes are readily cultured from epithelial surfaces of the skin, mouth, and colon. In the last 10 years, culture-independent DNA-based techniques demonstrated that much more complex microbial communities reside on most epithelial surfaces; this includes the lower airways, where bacterial culture had failed to reliably demonstrate resident bacteria. Exposure to a diverse bacterial environment is important for adequate immunological development. The most common microbes found in the lower airways are also found in the upper airways. Increasing abundance of oral characteristic taxa is associated with increased inflammatory cells and exhaled nitric oxide, suggesting that the airway microbiome induces an immunological response in the lung. Furthermore, rhinovirus infection leads to outgrowth of Haemophilus in patients with chronic obstructive pulmonary disease, and human immunodeficiency virus-infected subjects have more Tropheryma whipplei in the lower airway, suggesting a bidirectional interaction in which the host immune defenses also influence the microbial niche. Quantitative and/or qualitative changes in the lung microbiome may be relevant for disease progression and exacerbations in a number of pulmonary diseases. Future investigations with longitudinal follow-up to understand the dynamics of the lung microbiome may lead to the development of new therapeutic targets.

  3. Immunoglobulin G4-related Lung Disease: A Disease with Many Different Faces

    Philip Hui


    Full Text Available Immunoglobulin (Ig G4-related lung disease is a fibroinflammatory entity that presents in protean ways. Diagnostically, IgG4-related lung disease requires a high clinical index of suspicion complemented by elevated serum IgG4 levels and/or biopsy that shows the characteristic pathological features. The disease is almost always responsive to systemic corticosteroids. However, relapse is common following their discontinuation. The authors present three cases to highlight the diverse clinical features, and to illustrate the diagnostic and therapeutic approaches to this disease.

  4. NHLBI viewpoint: Lung health and disease prevention research starting in childhood.

    Blaisdell, Carol J; Weinmann, Gail G


    Lung health begins in utero when the complex structure of the airway, alveolar, and vascular structures are formed. To really impact the United States and global burden of chronic lung diseases in both adults and children, we must understand normal and abnormal development, the outcomes of disrupted development, and the effects of in utero and postnatal exposures on lung health. With increasing recognition of early life origins of adult diseases,(1) it is important to know what early events and interventions can alter the trajectory of lung development, growth, and decline to help promote lung health and reduce chronic lung disease.

  5. Severe nitrofurantoin lung disease resolving without the use of steroids

    Bhullar S


    Full Text Available We report a case of an elderly woman who developed a severe, chronic pulmonary reaction to nitrofurantoin therapy that she had taken continuously for three years to prevent urinary tract infections. The patient was taking no other drug known to cause lung disease but the diagnosis was delayed by failure to recognize the association between nitrofurantoin and adverse drug reactions affecting the lung. When originally seen, the patient was unable to care for herself due to dyspnea. Bronchoscopy with biopsy ruled out other causes of her pulmonary disease. Immediate withdrawal of nitrofurantoin led to substantial, sustained improvement and disappearance of symptoms over several months without administration of corticosteroids. Nitrofurantoin toxicity should always be considered in any person taking that drug who develops bilateral infiltrates.

  6. [Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases].

    Jiang, Xiaojing; Sun, Xiuzhu; Du, Weihua; Hao, Haisheng; Zhao, Xueming; Wang, Dong; Zhu, Huabin; Liu, Yan


    Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases.

  7. Nanomedicine as an innovative therapeutic strategy for pediatric lung diseases.

    Tian, Ye; Chen, Jian; Zahtabi, Fatemeh; Keijzer, Richard; Xing, Malcolm


    Nanomedicine is a rapidly emerging technology and represents an innovative field for therapy. Nanomaterials have intrinsically defined features for biomedical applications due to the high specific surface area, the amazing diversity, versatility in structure and function and the possibility of surface charge. In particular, the functionalization of targeting or stimuli-responsive unit on the surface of these materials gives them specific targeted therapeutic properties. There are many pediatric lung diseases that could potentially benefit from nanomedicine. Herein, we aim to review various drug carrier systems and release systems specifically targeting pediatric lung diseases. The injection of nanomedicine into in vivo models and their elimination will also be discussed. Finally, the potential toxicity of nanomaterials will be addressed.

  8. Pulmonary Surfactants for Acute and Chronic Lung Diseases (Part II

    O. A. Rozenberg


    Full Text Available Part 2 of the review considers the problem of surfactant therapy for acute respiratory distress syndrome (ARDS in adults and young and old children. It gives information on the results of surfactant therapy and prevention of ARDS in patients with severe concurrent trauma, inhalation injuries, complications due to complex expanded chest surgery, or severe pneumonias, including bilateral pneumonia in the presence of A/H1N1 influenza. There are data on the use of a surfactant in obstetric care and prevention of primary graft dysfunction during lung transplantation. The results of longterm use of surfactant therapy in Russia, suggesting that death rates from ARDS may be substantially reduced (to 20% are discussed. Examples of surfactant therapy for other noncritical lung diseases, such as permanent athelectasis, chronic obstructive pulmonary diseases, and asthma, as well tuberculosis, are also considered.

  9. Drug-induced lung disease: High-resolution CT and histological findings

    Cleverley, Joanne R.; Screaton, Nicholas J.; Hiorns, Melanie P.; Flint, Julia D.A.; Mueller, Nestor L


    AIM: To compare the parenchymal high-resolution computed tomography (HRCT) appearances with histological findings in patients with drug-induced lung disease and to determine the prognostic value of HRCT. MATERIALS AND METHODS: Drug history, HRCT features, histological findings and outcome at 3 months in 20 patients with drug induced-lung disease were reviewed retrospectively. The HRCT images were assessed for the pattern and distribution of abnormalities and classified as most suggestive of interstitial pneumonitis/fibrosis, diffuse alveolar damage (DAD), organizing pneumonia (OP) reaction, or a hypersensitivity reaction. RESULTS: On histopathological examination there were eight cases of interstitial pneumonitis/fibrosis, five of DAD, five of OP reactions, one of hypersensitivity reaction and one of pulmonary eosinophilia. The most common abnormalities on HRCT were ground-glass opacities (n = 17), consolidation (n = 14), interlobular septal thickening (n = 15) and centrilobular nodules (n 8). HRCT interpretation and histological diagnosis were concordant in only nine (45%) of 20 patients. The pattern, distribution, and extent of HRCT abnormalities were of limited prognostic value: all eight patients with histological findings of OP, hypersensitivity reaction, or eosinophilic infiltrate improved on follow-up compared to only five of 13 patients with interstitial pneumonitis/fibrosis or DAD. CONCLUSION: In many cases of drug-induced lung injury HRCT is of limited value in determining the histological pattern and prognosis. Cleverly, J.R. et al.

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

    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


    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.

  11. [Intersticial lung disease as the sole manifestation of antisynthetase syndrome].

    Monteiro, Paulo; Coutinho, Margarida; Machado, Pedro; Garcia, Jorge; Salvador, Maria João; Inês, Luís; Silva, Jorge; Malcata, Armando


    The authors report a clinical case of a woman who had a 3 years diagnosis of hipersensitivity pneumonitis based on intersticial lung disease without other manifestations. The diagnosis of antisynthetase syndrome was made three years after the initial symptoms upon the onset of systemic manifestations with articular involvement, myositis and determination of anti-PL 7 antibodies. In this syndrome, the isolated pulmonary involvement is rare.

  12. Pleuroparenchymal Lung Disease Secondary to Nonoccupational Exposure to Vermiculite

    Fahad Al-Ghimlas


    Full Text Available An unusual case of pleuroparenchymal lung disease caused by the inhalation of vermiculite dust, presumably containing asbestos fibers is described. The uniqueness of the case lies in the very indirect nature of exposure – the wife of a factory owner, rather than a worker exposed to asbestos, whose factory manufactured vermiculite. The present case illustrates the importance of taking careful occupational histories of all household members when presented with a patient whose chest radiograph exhibits features consistent with asbestos exposure.

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

    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


    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

  14. Pulmonary hypertension in chronic obstructive and interstitial lung diseases

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


    The purpose of the present review is to summarize the current knowledge on PH in relation to COPD and ILD from a clinical perspective with emphasis on diagnosis, biomarkers, prevalence, impact, treatment, and practical implications. PH in COPD and ILD is associated with a poor prognosis, and is c......The purpose of the present review is to summarize the current knowledge on PH in relation to COPD and ILD from a clinical perspective with emphasis on diagnosis, biomarkers, prevalence, impact, treatment, and practical implications. PH in COPD and ILD is associated with a poor prognosis......, 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...... 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...

  15. Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

    Amirmasoud Zangiabadi


    Full Text Available Group 3 pulmonary hypertension (PH is a common complication of chronic lung disease (CLD, including chronic obstructive pulmonary disease (COPD, interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor prognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and only a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right ventricle (RV range between early RV remodeling, hypertrophy, dilatation, and eventual failure with associated increased mortality. The golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical examination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management of the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as phosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients with CLD and moderate-to-severe PH.

  16. Mechanisms of Physical Activity Limitation in Chronic Lung Diseases

    Ioannis Vogiatzis


    Full Text Available In chronic lung diseases physical activity limitation is multifactorial involving respiratory, hemodynamic, and peripheral muscle abnormalities. The mechanisms of limitation discussed in this paper relate to (i the imbalance between ventilatory capacity and demand, (ii the imbalance between energy demand and supply to working respiratory and peripheral muscles, and (iii the factors that induce peripheral muscle dysfunction. In practice, intolerable exertional symptoms (i.e., dyspnea and/or leg discomfort are the main symptoms that limit physical performance in patients with chronic lung diseases. Furthermore, the reduced capacity for physical work and the adoption of a sedentary lifestyle, in an attempt to avoid breathlessness upon physical exertion, cause profound muscle deconditioning which in turn leads to disability and loss of functional independence. Accordingly, physical inactivity is an important component of worsening the patients’ quality of life and contributes importantly to poor prognosis. Identifying the factors which prevent a patient with lung disease to easily carry out activities of daily living provides a unique as well as important perspective for the choice of the appropriate therapeutic strategy.

  17. Real-time in vivo tissue characterization with diffuse reflectance spectroscopy during transthoracic lung biopsy: a clinical feasibility study

    Spliethoff, Jarich; Prevoo, Warner; Meier, Mark A.J.; de Jong, Jeroen; Evers, Daniel; Evers, Daniel J.; Sterenborg, Hendricus J.C.M.; Lucassen, Gerald; Lucassen, Gerald W.; Hendriks, Benno H.W.; Ruers, Theo J.M.


    Purpose: This study presents the first in vivo real-time tissue characterization during image-guided percutaneous lung biopsies using diffuse reflectance spectroscopy (DRS) sensing at the tip of a biopsy needle with integrated optical fibers. Experimental Design: Tissues from 21 consented patients

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

    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)


    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 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

  19. The Gut-Lung Axis in Respiratory Disease.

    Marsland, Benjamin J; Trompette, Aurélien; Gollwitzer, Eva S


    Host-microorganism interactions shape local cell functionality, immune responses, and can influence disease development. Evidence indicates that the impact of host-microbe interactions reaches far beyond the local environment, thus influencing responses in peripheral tissues. There is a vital cross-talk between the mucosal tissues of our body, as exemplified by intestinal complications during respiratory disease and vice versa. Although, mechanistically, this phenomenon remains poorly defined, the existence of the gut-lung axis and its implications in both health and disease could be profoundly important for both disease etiology and treatment. In this review, we highlight how changes in the intestinal microenvironment, with a particular focus on the intestinal microbiota, impact upon respiratory disease.

  20. Cartography of Pathway Signal Perturbations Identifies Distinct Molecular Pathomechanisms in Malignant and Chronic Lung Diseases.

    Arakelyan, Arsen; Nersisyan, Lilit; Petrek, Martin; Löffler-Wirth, Henry; Binder, Hans


    Lung diseases are described by a wide variety of developmental mechanisms and clinical manifestations. Accurate classification and diagnosis of lung diseases are the bases for development of effective treatments. While extensive studies are conducted toward characterization of various lung diseases at molecular level, no systematic approach has been developed so far. Here we have applied a methodology for pathway-centered mining of high throughput gene expression data to describe a wide range of lung diseases in the light of shared and specific pathway activity profiles. We have applied an algorithm combining a Pathway Signal Flow (PSF) algorithm for estimation of pathway activity deregulation states in lung diseases and malignancies, and a Self Organizing Maps algorithm for classification and clustering of the pathway activity profiles. The analysis results allowed clearly distinguish between cancer and non-cancer lung diseases. Lung cancers were characterized by pathways implicated in cell proliferation, metabolism, while non-malignant lung diseases were characterized by deregulations in pathways involved in immune/inflammatory response and fibrotic tissue remodeling. In contrast to lung malignancies, chronic lung diseases had relatively heterogeneous pathway deregulation profiles. We identified three groups of interstitial lung diseases and showed that the development of characteristic pathological processes, such as fibrosis, can be initiated by deregulations in different signaling pathways. In conclusion, this paper describes the pathobiology of lung diseases from systems viewpoint using pathway centered high-dimensional data mining approach. Our results contribute largely to current understanding of pathological events in lung cancers and non-malignant lung diseases. Moreover, this paper provides new insight into molecular mechanisms of a number of interstitial lung diseases that have been studied to a lesser extent.

  1. Mechanisms of protein misfolding in conformational lung diseases.

    McElvaney, N G


    Genetic or environmentally-induced alterations in protein structure interfere with the correct folding, assembly and trafficking of proteins. In the lung the expression of misfolded proteins can induce a variety of pathogenetic effects. Cystic fibrosis (CF) and alpha-1 antitrypsin (AAT) deficiency are two major clinically relevant pulmonary disorders associated with protein misfolding. Both are genetic diseases the primary causes of which are expression of mutant alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) and SERPINA1, respectively. The most common and best studied mutant forms of CFTR and AAT are ΔF508 CFTR and the Glu342Lys mutant of AAT called ZAAT, respectively. Non-genetic mechanisms can also damage protein structure and induce protein misfolding in the lung. Cigarette-smoke contains oxidants and other factors that can modify a protein\\'s structure, and is one of the most significant environmental causes of protein damage within the lung. Herein we describe the mechanisms controlling the folding of wild type and mutant versions of CFTR and AAT proteins, and explore the consequences of cigarette-smoke-induced effects on the protein folding machinery in the lung.

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

    Bestaev, D V


    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.

  3. Providing nutritional information to people with lung disease.

    Nicholls, Carol

    Studies have shown that about 30 per cent of people who have chronic obstructive pulmonary disease (COPD) lose weight. Weight loss has been shown to be associated with a reduction in lung function (Poole, 1993). Conversely, patients who are overweight have an increased respiratory workload due to their extra weight. Excess weight also increases the risk of hypertension, diabetes, heart disease and osteoarthritis (Collins, 2003). Many patients are unaware of changes in their nutritional status. The case study in Box 1 provides an illustration of this.

  4. Diffusion MR imaging in sporadic Creutzfeldt-Jakob disease

    Burcak Cakir Pekoz


    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. Diffusion-weighted imaging became an important tool for early diagnosis of CJD because of the high sensitivity. We present 59-year-old female patient diagnosed as sporadic CJD with typical MR imagings. [Cukurova Med J 2014; 39(4.000: 880-883

  5. An approach to interstitial lung disease in India

    J N Pande


    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 connective tissue disease--mechanisms and management.

    Wells, Athol U; Denton, Christopher P


    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.

  7. Occupational Lung Diseases among Soldiers Deployed to Iraq and Afghanistan.

    Szema, Anthony M


    Military personnel deployed to Iraq and Afghanistan, from 2004 to the present, has served in a setting of unique environmental conditions. Among these are exposures to burning trash in open air "burn pits" lit on fire with jet fuel JP-8. Depending on trash burned--water bottles, styrofoam trays, medical waste, unexploded munitions, and computers--toxins may be released such as dioxins and n-hexane and benzene. Particulate matter air pollution culminates from these fires and fumes. Additional environmental exposures entail sandstorms (Haboob, Shamal, and Sharqi) which differ in direction and relationship to rain. These wars saw the first use of improvised explosive devices (roadside phosphate bombs),as well as vehicle improvised explosive devices (car bombs), which not only potentially aerosolize metals, but also create shock waves to induce lung injury via blast overpressure. Conventional mortar rounds are also used by Al Qaeda in both Iraq and Afghanistan. Outdoor aeroallergens from date palm trees are prevalent in southern Iraq by the Tigris and Euphrates rivers, while indoor aeroallergen aspergillus predominates during the rainy season. High altitude lung disease may also compound the problem, particularly in Kandahar, Afghanistan. Clinically, soldiers may present with new-onset asthma or fixed airway obstruction. Some have constrictive bronchiolitis and vascular remodeling on open lung biopsy - despite having normal spirometry and chest xrays and CT scans of the chest. Others have been found to have titanium and other metals in the lung (rare in nature). Still others have fulminant biopsy-proven sarcoidiosis. We found DNA probe-positive Mycobacterium Avium Complex in lung from a soldier who had pneumonia, while serving near stagnant water and camels and goats outside Abu Gharib. This review highlights potential exposures, clinical syndromes, and the Denver Working Group recommendations on post-deployment health.

  8. NETs and CF Lung Disease: Current Status and Future Prospects

    Robert D. Gray


    Full Text Available Cystic Fibrosis (CF is the most common fatal monogenic disease among Caucasians. While CF affects multiple organ systems, the principle morbidity arises from progressive destruction of lung architecture due to chronic bacterial infection and inflammation. It is characterized by an innate immune defect that results in colonization of the airways with bacteria such as Staphylococcus aureus and Pseudomonas aeruginosa from an early age. Within the airway microenvironment the innate immune cells including epithelial cells, neutrophils, and macrophages have all been implicated in the host defense defect. The neutrophil, however, is the principal effector cell facilitating bacterial killing, but also participates in lung damage. This is evidenced by a disproportionately elevated neutrophil burden in the airways and increased neutrophil products capable of tissue degradation, such as neutrophil elastase. The CF airways also contain an abundance of nuclear material that may be originating from neutrophils. Neutrophil extracellular traps (NETs are the product of a novel neutrophil death process that involves the expulsion of nuclear material embedded with histones, proteases, and antimicrobial proteins and peptides. NETs have been postulated to contribute to the bacterial killing capacity of neutrophils, however they also function as a source of proteases and other neutrophil products that may contribute to lung injury. Targeting nuclear material with inhaled DNase therapy improves lung function and reduces exacerbations in CF and some of these effects may be due to the degradation of NETs. We critically discuss the evidence for an antimicrobial function of NETs and their potential to cause lung damage and inflammation. We propose that CF animal models that recapitulate the human CF phenotype such as the CFTR−/− pig may be useful in further elucidating a role for NETs.

  9. Diffusion abnormality maps in demyelinating disease: correlations with clinical scores.

    Onu, Mihaela; Roceanu, Adina; Sboto-Frankenstein, Uta; Bendic, Robert; Tarta, Eugen; Preoteasa, Florentin; Bajenaru, Ovidiu


    Magnetic resonance imaging (MRI) has been explored as a noninvasive tool to assess pathology in multiple sclerosis (MS) patients. However, the correlation between classical MRI measures and physical disability is modest in MS. The diffusion tensor imaging (DTI) MRI technique holds particular promise in this regard. The present study shows brain regions where FA and individual diffusivities abnormalities are present and check their correlations with physical disability clinical scores. Eight patients and 12 matched healthy controls were recruited. The Multiple Sclerosis Functional Composite was administered. For MR-DTI acquisitions, a Genesis Signa 1.5 T MR system, an EP/SE scanning sequence, 25 gradient directions were used. Tract Based Spatial Statistics (TBSS) group comparisons showed reduced FA and increased individual diffusivities in several brain regions in patients. Significant correlations were found between FA and: EDSS, 9-HPT(NON)DOM and 25 FW score; between λ2 and: P100 (r&l), 9-HPT(NON)DOM and 25 FW; between λ3 and: 9-HPT(NON)DOM and 25 FW score. Fractional anisotropy and individual radial diffusivities proved to be important markers of motor disabilities in MS patients when the disease duration mean and the disability scores values range are relatively high. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  10. A rare case of occupational lung disease – Talcosis

    Sathish Kumar M, Dhipu Mathew, Thilagavathy, Aruna Shanmuganathan, Srinivasan R


    Full Text Available Talcosis/ Talcpneumoconiosis is one of the rarer forms of magnesium silicate induced lung disease, It usually occurs in the fourth decade and affects people working in talc related industries like roof, shingle, pharmaceutical companies, talcum powder industries, electric ceramics, rubber industry etc. We report a case of talc pneumoconiosis/talcosis in a 51yr old male who presented with breathlessness and dry cough for the past 5 yrs and progressively worsening for the past 5 days. Who was working in a talcum powder manufacturing company for >28yrs in the packaging section. The diagnosis was possible by history, clinical examination, Chest X-ray, PFT/DLCO, HRCT chest, Bronchoscopy & Trans bronchial lung biopsy showing interstitial fibrosis.

  11. Quantitative Epstein-Barr virus (EBV) serology in lung transplant recipients with primary EBV infection and/or post-transplant lymphoproliferative disease

    Verschuuren, E; van der Bij, W; de Boer, W; Timens, W; Middeldorp, J; The, TH


    The Epstein-Barr virus (EBV)-specific antibody response was studied in lung transplant patients to assess their value in the diagnosis and prognosis of post-transplant lymphoproliferative disease. Recently developed synthetic peptides representing Epstein-Barr nuclear antigen-1 (EBNA-1), diffuse ear

  12. Acute and subacute non-infectious lung diseases. Usefulness of HRCT for evaluation of activity especially in follow-up

    Johkoh, Takeshi; Tomiyama, Noriyuki; Honda, Osamu [Osaka Univ., Suita (Japan). Medical School] (and others)


    The purpose of this study was to survey the usefulness of high-resolution CT (HRCT) for the evaluation of activity in acute and subacute non-infectious diffuse infiltrative lung diseases before and after corticosteroid treatment. Sequential HRCT images and chest radiographs obtained before and after treatment were retrospectively evaluated in 33 patients with acute or subacute non-infectious diffuse infiltrative lung diseases. All these patients were histologically confirmed to have pulmonary Inflammation and to have responded to treatment with corticosteroid. Radiographic and CT scores were correlated with the degree of dyspnea and the results of arterial blood gas analysis using Spearman's rank-correlation coefficient. On follow-up HRCT, the profusion score of areas with increased attenuation was significantly correlated with arterial oxygen tension (PaO{sub 2}) (p=.003, r=-.53) and the alveolar-arterial oxygen tension difference (AaDO{sub 2}) (p=.001, r=.57). No other correlation was found after treatment. Nodular and linear opacities were more commonly seen on follow-up chest radiographs and HRCT images than on initial ones. HRCT is useful for the evaluation of disease activity in acute and subacute non-infectious infiltrative lung diseases before and after treatment if paying special attention to the profusion of ground-glass attenuation. Even if pretreatment HRCT has not been performed, posttreatment HRCT should be examined. (author)

  13. Spirometry utilisation among Danish adults initiating medication targeting obstructive lung disease

    Koefoed, Mette


    with pharmacotherapy targeting obstructive lung disease and only few have additional tests conducted, although the predictive value of respiratory symptoms for diagnosing obstructive lung disease has proven to be low. Spirometry is recommended as the gold standard for confirming obstructive lung disease, and testing...... and being exposed to unnecessary economic costs and medication risks. The literature has indicated that many users of medication targeting obstructive lung medication have not had spirometry performed and do not actually have obstructive lung disease. This potential quality gap needs to be assessed. Also......, in order to target interventions enhancing earlier spirometry utilisation among patients initiating medication targeting obstructive lung disease, improved knowledge on patient and practice factors associated with spirometry testing is needed. AIMS: Among first time users of obstructive lung medication we...

  14. Outcomes in Systemic Sclerosis-related Lung Disease following Lung Transplantation

    Sottile, Peter D; Iturbe, David; Katsumoto, Tamiko R; Connolly, M Kari; Collard, Harold R; Leard, Lorriana A; Hays, Steven; Golden, Jeffrey A; Hoopes, Charles; Kukreja, Jasleen; Singer, Jonathan P


    Background Lung disease (LD) is the leading cause of death in systemic sclerosis (SSc). The diagnosis of SSc-related LD (SSc-LD) is often a contraindication to lung transplantation (LT) due to concerns that extra-pulmonary involvement will yield worse outcomes. We sought to evaluate post-transplant outcomes in persons with SSc-LD with esophageal involvement compared to persons with non-connective tissue disease related interstitial lung disease (nCTD-ILD). Methods From 1998-2012, persons undergoing LT for SSc-LD were age and gender matched in a 2:1 fashion to controls undergoing LT for nCTD-ILD. Esophageal function was assessed by pH testing and manometry. We defined esophageal dysfunction as the presence of a DeMeester score >14 or dysmotility more severe than “mild non-specific disorder”. The primary outcome was post-transplant survival. Secondary outcomes included freedom from bronchiolitis obliterans syndrome (fBOS) and rates of acute rejection. Survival and fBOS were estimated with Kaplan-Meier methods. Acute rejection was compared with Students t-test. Results Survival was similar in 23 persons with SSc-LD and 46 controls who underwent LT (p=0.47). For the SSc-LD group, 1- and 5-year survival was 83% and 76% compared to 91% and 64% in the nCTD-ILD group. There were no differences in fBOS (p=0.83). Rates of acute rejection were less in SSc-ILD (p=0.05). Esophageal dysfunction was not associated with worse outcomes (p>0.55). Conclusions Persons with SSc-LD appear to have similar survival and fBOS as persons transplanted for nCTD-ILD. The risk of acute rejection after transplant may be reduced in persons with SSc-LD. Esophageal involvement does not appear to impact outcomes. PMID:23545509

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

    刘萍; 秦兴国


    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.

  16. Open lung biopsy

    ... CT scan Disseminated tuberculosis Granulomatosis with polyangiitis Lung cancer - small cell Lung disease Lung needle biopsy Malignant mesothelioma Pulmonary tuberculosis Rheumatoid lung disease Sarcoidosis Simple pulmonary eosinophilia ...

  17. CT perfusion at early stage of hepatic diffuse disease

    Sheng Guan; Wei-Dong Zhao; Kang-Rong Zhou; Wei-Jun Peng; Jian Mao; Feng Tang


    AIM: To determine the validity of the non-invasive method of CT perfusion (CTP) in rat model of hepatic diffuse disease. METHODS: Twenty-eight Wistar rats were divided into two groups. Liver diffuse lesions were induced by dietthylnitrosamine in 14 rats of test group. Rats in control groupwere bred with pure water. From the 1st to 12th wk after the test group was intervened, both groups were studied every week with CTP. CTP parameters of liver parenchymain different periods and pathologic changes in two groups were compared and analyzed.RESULTS: The process of hepatic diffuse lesions in test groups was dassified into three stages or periods according to the pathologic alterations, namely hepattitis, hepatic fibrosis, and cirrhosis. During this period, hepatic artery flow (HAF)of control group declined slightly, mean transit time (MTT),blood flow (BF) and volume (BV) increased, but there wereno significant differences between different periods. Tntest group, HAF tended to increase gradually, MTT prolonged obviously, BV and BF decreased at the same time. The results of statistical analysis revealed that the difference in the HAF ratio of test group to control group was significant.The ratio of BV and BF in test group to control group in stageof hepatitis and hepatic cirrhosis, hepatic fibrosis and early stage of hepatic cirrhosis was significantly different, but there was no significant difference between hepatitis and hepatic fibrosis. The main pathological changes in stage of hepatitis were swelling of hepatic cells, while sinusoid capillarization and deposition of collagen aggravated gradually in the extravascular Disse's spaces in stage of fibrosis and early stage of cirrhosis.CONCLUSION: The technique could reflect some early changes of hepatic blood perfusion in rat with liver diffuse disease and is valuable for their early diagnosis.

  18. Lung hyperinflation in chronic obstructive pulmonary disease: mechanisms, clinical implications and treatment.

    Langer, Daniel; Ciavaglia, Casey E; Neder, J Alberto; Webb, Katherine A; O'Donnell, Denis E


    Lung hyperinflation is highly prevalent in patients with chronic obstructive pulmonary disease and occurs across the continuum of the disease. A growing body of evidence suggests that lung hyperinflation contributes to dyspnea and activity limitation in chronic obstructive pulmonary disease and is an important independent risk factor for mortality. In this review, we will summarize the recent literature on pathogenesis and clinical implications of lung hyperinflation. We will outline the contribution of lung hyperinflation to exercise limitation and discuss its impact on symptoms and physical activity. Finally, we will examine the physiological rationale and efficacy of selected pharmacological and non-pharmacological 'lung deflating' interventions aimed at improving symptoms and physical functioning.

  19. Cardiopulmonary exercise factors predict survival in patients with advanced interstitial lung disease referred for lung transplantation.

    Layton, Aimee M; Armstrong, Hilary F; Kim, Hanyoung P; Meza, Kimbery S; D'Ovidio, Frank; Arcasoy, Selim M


    The purpose of this work was to determine if parameters assessed during Cardiopulmonary Exercise Testing (CPET) while using supplemental oxygen can independently predict one-year transplant-free survival in patients with Interstitial Lung Disease (ILD) referred for lung transplant evaluation. We performed a chart review of patients with ILD who completed CPET with 30% FiO2 and gathered spirometry, pulmonary hemodynamic, six-minute walk, and CPET data. The primary end-point was death or lung transplantation within one-year of CPET. The final data set included 192 patients. 79 patients died/underwent transplant, 113 survived transplant-free. Multivariable Cox regression revealed peak workload % predicted, nadir CPET SpO2, and FVC% predicted as independent predictors of one-year transplant-free survival. Of the independent predictors of survival, receiver operating characteristics analysis revealed peak workload %predicted cutoff of 35% to be highly discriminatory, more so than nadir CPET SpO2 or FVC % predicted in identifying patients at risk for one-year mortality or transplant (peak workload % predicted < 35% HR = 4.71, 95% CI = 2.64-8.38 and area under the curve (AUC) = 0.740, nadir CPET SpO2 < 86% HR = 2.27, 95%CI = 1.41-3.68, AUC = 0.645, FVC %predicted <45% HR = 1.82, 95% CI = 1.15-2.87, AUC = 0.624). Peak workload % predicted, nadir CPET SpO2, and FVC% predicted in ILD patients referred for lung transplant evaluation are independently predictive of one-year mortality or need for transplant. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. Proteasomes in lungs from organ donors and patients with end-stage pulmonary diseases.

    Baker, T A; Bach, H H; Gamelli, R L; Love, R B; Majetschak, M


    Proteasomes appear to be involved in the pathophysiology of various acute and chronic lung diseases. Information on the human lung proteasome in health and disease, however, is sparse. Therefore, we studied whether end-stage pulmonary diseases are associated with alterations in lung 20S/26S proteasome content, activity and 20S subunit composition. Biopsies were obtained from donor lungs (n=7) and explanted lungs from patients undergoing lung transplantation because of end stage chronic obstructive pulmonary disease (COPD; n=7), idiopathic pulmonary fibrosis (IPF, n=7) and pulmonary sarcoidosis (n=5). 20S/26S proteasomes in lung extracts were quantified by ELISA, chymotrypsin-like proteasome peptidase activities measured and 20S proteasome beta subunits analyzed by Western blot. As compared with donor lungs, proteasome content was increased in IPF and sarcoidosis, but not in COPD. The relative distribution of free 20S and 26S proteasomes was similar; 20S proteasome was predominant in all extracts. Proteasome peptidase activities in donor and diseased lungs were indistinguishable. All extracts contained a mixed composition of inducible 20S beta immuno-subunits and their constitutive counterparts; a disease associated distribution could not be identified. A higher content of lung proteasomes in IPF and pulmonary sarcoidosis may contribute to the pathophysiology of human fibrotic lung diseases.

  1. Lung carcinogenesis from chronic obstructive pulmonary disease: characteristics of lung cancer from COPD and contribution of signal transducers and lung stem cells in the inflammatory microenvironment.

    Sekine, Yasuo; Hata, Atsushi; Koh, Eitetsu; Hiroshima, Kenzo


    Chronic obstructive pulmonary disease (COPD) and lung cancer are closely related. The annual incidence of lung cancer arising from COPD has been reported to be 0.8-1.7 %. Treatment of lung cancer from COPD is very difficult due to low cardiopulmonary function, rapid tumor growth, and resistance to molecularly targeted therapies. Chronic inflammation caused by toxic gases can induce COPD and lung cancer. Carcinogenesis in the inflammatory microenvironment occurs during cycles of tissue injury and repair. Cellular damage can induce induction of necrotic cell death and loss of tissue integrity. Quiescent normal stem cells or differentiated progenitor cells are introduced to repair injured tissues. However, inflammatory mediators may promote the growth of bronchioalveolar stem cells, and activation of NF-κB and signal transducer and activator of transcription 3 (STAT3) play crucial roles in the development of lung cancer from COPD. Many of the protumorgenic effects of NF-κB and STAT3 activation in immune cells are mediated through paracrine signaling. NF-κB and STAT3 also contribute to epithelial-mesenchymal transition. To improve lung cancer treatment outcomes, lung cancer from COPD must be overcome. In this article, we review the characteristics of lung cancer from COPD and the mechanisms of carcinogenesis in the inflammatory microenvironment. We also propose the necessity of identifying the mechanisms underlying progression of COPD to lung cancer, and comment on the clinical implications with respect to lung cancer prevention, screening, and therapy.

  2. Liver and spleen elastography in patients with diffuse liver diseases

    Alexey Borsukov


    Full Text Available The purpose of the research was to estimate the clinical-diagnostic and predictive value of non-invasive ultrasonic elastography in dynamic monitoring in patients with diffuse liver disease. A number of 114 patients with diffuse liver disease were examined, specifically 68 (59.6% men and 46 (40.4% women. The patients were divided into three groups: 40 patients with steatosis; 38 with hepatitis; 36 with cirrhosis. The research included clinical and bio-chemical analysis, ultrasound examination of liver and spleen with doppler v. portae and v. lienalis, elastography of liver and spleen. The study found a high correlation of elastography data as regards the liver and spleen in patients with alcoholic cirrhosis (r=0.96, average correlation (r=0.69 in patients with steatosis and hepatitis of alcoholic etiology. On the basis of the statistical program receiver operating characteristic (ROC-analysis it was ascertained that the spleen is in perfect condition (AUC 0.9-1.0, and the liver is in a very good condition (0.8-0.9. The research revealed therapeutically significant factor ΔF/ΔL for dynamic monitoring: the ΔF/ΔL 1 can predict a more favorable course of the disease. Noninvasive ultrasound elastography helps to forecast the process of the disease and correct the therapeutic approach. The research contributes to the search for additional and reliable techniques of identifying the stage of disease of patients with hepatic fibrosis, the dynamics of the disease as well as forecasting further complications.

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

    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


    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.

  4. [Pulsed hypoxia in the treatment of obstructive lung diseases].

    Sil'vestrov, V P; Kovalenko, E A; Krysin, Iu S


    A new approach to the treatment of chronic nonspecific pulmonary diseases is proposed: helium-oxygen therapy combined with repeated interrupted hypoxic stimuli. Helium inclusion into hypoxic gas mixture leads to reduced air density. Gas mixture containing 10-15% of oxygen is more effective at the level of alveolocapillary membrane. When conducted in normal atmospheric pressure, the method involved no complications and produced positive responses in coronary heart disease, hypertension, alimentary diseases. The mixture of helium with oxygen (85-90% of helium, 10-15% oxygen) in combination with impulse normobaric hypoxia has been tried in 25 chronics with obstructive bronchitis and bronchial asthma. The results were indicative of the treatment efficacy: bronchial permeability improved in 67% of the cases, forced vital capacity of the lungs increased, inspiratory reserve volume grew, dyspnea and cough diminished, sputum discharge improved, general tonicity and performance status changed positively. Six-month follow-up evidenced positive shifts too.

  5. Pulmonary aspergillosis and aflatoxins in chronic lung diseases.

    Ali, Sana; Malik, Abida; Shahid, Mohd; Bhargava, Rakesh


    Fungal infections of lung have become increasingly common during the last few decades. Aspergillosis and the role of aflatoxins in various chronic lung diseases have not been extensively studied. Bronchoalveolar lavage (BAL) samples and sera from 40 patients of chronic lung diseases were analyzed for galactomannan antigen (GM) and aflatoxin by enzyme-linked immunosorbent assay. Direct microscopy and culture of BAL samples were also done to detect the Aspergillus species. Results revealed that 15 (37.5 %) of the 40 patients had growth of Aspergillus on BAL culture. Out of these culture-positive cases, 13 (86.7 %) patients were positive for galactomannan antigen also. About 62.5 % cases did not show growth of Aspergillus in BAL culture. However, galactomannan antigen could be detected in 20 % of these patients. Overall, 20 % patients were diagnosed as proven invasive fungal disease (IFD), 32.5 % were of probable IFD, 17.5 % of possible IFD. Aspergillus growth was observed in 100 % of proven and 53.8 % of probable IFD cases. Galactomannan antigen was found in 100 % cases of proven and 76.9 % of probable IFD. Ten (25 %) patients were found to be positive for aflatoxins. It was detected in 6 (40 %) of culture-positive cases. About 62.5 % of the cases with proven IFD and 46.1 % of probable IFD had aflatoxin in their samples. Aflatoxin positivity was found to be more in patients with proven IFD than in probable IFD, and higher level of aflatoxins was detected in cases with proven IFD. Significant difference was observed in aflatoxin positivity among food grain workers when compared to other occupations.

  6. Altered Pulmonary Lymphatic Development in Infants with Chronic Lung Disease

    Emily M. McNellis


    Full Text Available Pulmonary lymphatic development in chronic lung disease (CLD has not been investigated, and anatomy of lymphatics in human infant lungs is not well defined. Hypothesis. Pulmonary lymphatic hypoplasia is present in CLD. Method. Autopsy lung tissues of eighteen subjects gestational ages 22 to 40 weeks with and without history of respiratory morbidity were stained with monoclonal antipodoplanin and reviewed under light microscopy. Percentage of parenchyma podoplanin stained at the acinar level was determined using computerized image analysis; 9 CLD and 4 control subjects gestational ages 27 to 36 weeks were suitable for the analysis. Results. Distinct, lymphatic-specific staining with respect to other vascular structures was appreciated in all gestations. Infants with and without respiratory morbidity had comparable lymphatic distribution which extended to the alveolar ductal level. Podoplanin staining per parenchyma was increased and statistically significant in the CLD group versus controls at the alveolar ductal level (0.06% ± 0.02% versus 0.04% ± 0.01%, 95% CI −0.04% to −0.002%, P<0.03. Conclusion. Contrary to our hypothesis, the findings show that there is an increase in alveolar lymphatics in CLD. It is suggested that the findings, by expanding current knowledge of CLD pathology, may offer insight into the development of more effective therapies to tackle CLD.

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

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


    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.

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

    Susanna Cappelli


    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.

  9. Importance of lysosomal cysteine proteases in lung disease

    Chapman Harold A


    Full Text Available Abstract The human lysosomal cysteine proteases are a family of 11 proteases whose members include cathepsins B, C, H, L, and S. The biology of these proteases was largely ignored for decades because of their lysosomal location and the belief that their function was limited to the terminal degradation of proteins. In the past 10 years, this view has changed as these proteases have been found to have specific functions within cells. This review highlights some of these functions, specifically their roles in matrix remodeling and in regulating the immune response, and their relationship to lung diseases.

  10. Concise review: current status of stem cells and regenerative medicine in lung biology and diseases.

    Weiss, Daniel J


    Lung diseases remain a significant and devastating cause of morbidity and mortality worldwide. In contrast to many other major diseases, lung diseases notably chronic obstructive pulmonary diseases (COPDs), including both asthma and emphysema, are increasing in prevalence and COPD is expected to become the third leading cause of disease mortality worldwide by 2020. New therapeutic options are desperately needed. A rapidly growing number of investigations of stem cells and cell therapies in lung biology and diseases as well as in ex vivo lung bioengineering have offered exciting new avenues for advancing knowledge of lung biology as well as providing novel potential therapeutic approaches for lung diseases. These initial observations have led to a growing exploration of endothelial progenitor cells and mesenchymal stem (stromal) cells in clinical trials of pulmonary hypertension and COPD with other clinical investigations planned. Ex vivo bioengineering of the trachea, larynx, diaphragm, and the lung itself with both biosynthetic constructs as well as decellularized tissues have been used to explore engineering both airway and vascular systems of the lung. Lung is thus a ripe organ for a variety of cell therapy and regenerative medicine approaches. Current state-of-the-art progress for each of the above areas will be presented as will discussion of current considerations for cell therapy-based clinical trials in lung diseases.

  11. Suppressing disease spreading by using information diffusion on multiplex networks

    Wang, Wei; Liu, Quan-Hui; Cai, Shi-Min; Tang, Ming; Braunstein, Lidia A.; Stanley, H. Eugene


    Although there is always an interplay between the dynamics of information diffusion and disease spreading, the empirical research on the systemic coevolution mechanisms connecting these two spreading dynamics is still lacking. Here we investigate the coevolution mechanisms and dynamics between information and disease spreading by utilizing real data and a proposed spreading model on multiplex network. Our empirical analysis finds asymmetrical interactions between the information and disease spreading dynamics. Our results obtained from both the theoretical framework and extensive stochastic numerical simulations suggest that an information outbreak can be triggered in a communication network by its own spreading dynamics or by a disease outbreak on a contact network, but that the disease threshold is not affected by information spreading. Our key finding is that there is an optimal information transmission rate that markedly suppresses the disease spreading. We find that the time evolution of the dynamics in the proposed model qualitatively agrees with the real-world spreading processes at the optimal information transmission rate.

  12. Inflammatory/granulomatous diseases of the lung; Entzuendliche/granulomatoese Erkrankungen der Lunge

    Ivancevic, V.; Munz, D.L. [Humboldt Univ., Berlin (Germany). Kinik und Poliklinik fuer Nuklearmedizin


    The term `inflammatory` and `granulomatous` lung disease represents a pool of many etiologically different diseases, the pathologic mechanisms of which are characterized by inflammatory reactions of varying intensity and cell composition. In sarcoidosis and other granulomatous diseases as well as in lung fibroses, gallium scintigraphy allows reliable non-invasive estimation of alveolitis activity and is suitable for therapy monitoring. Granulomatous diseases seem to be detectable sensitively by means of somatostatin receptor scintigraphy as well. It is yet uncertain, whether positron emission tomography with F-18 fluordeoxyglucose will play a role in quantitative assessment of disease activity in sarcoidosis. Gallium scintigraphy is very useful in the early detection of pulmonary complications in AIDS patients. Pneumocystis carinii pneumonia, which is important in this patient population, can also be detected by both Tc-99m and In-111 labelled polyclonal human immunoglobulin, and in future possibly with a monoclonal antibody fragment against Pneumocystis carinii as well. The significance of primary bacterial pneumonias has decreased and nuclear medicine procedures for diagnosing inflammation are needed only exceptionally in this indication. (orig.) [Deutsch] Der Begriff der `entzuendlichen` und `granulomatoesen` Erkrankungen der Lunge stellt ein Sammelbecken fuer eine Vielzahl aetiologisch verschiedener Erkrankungen dar, deren Pathomechanismen durch eine entzuendliche Reaktion unterschiedlicher Intensitaet und Zellzusammensetzung gepraegt werden. Bei der Sarkoidose und anderen granulomatoesen Erkrankungen mit pulmonaler Manifestation sowie den Lungenfibrosen erlaubt die Galliumszintigraphie eine zuverlaessige nicht invasive Beurteilung des Aktivitaetsgrades der Alveolitis und eignet sich zum Therapiemonitoring. Granulomatoese Erkrankungen scheinen auch mit der Somatostatinrezeptorszintigraphie sensitiv nachweisbar zu sein. Ob die Positronen

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

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


    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.

  14. Sex-specific differences in hyperoxic lung injury in mice: Implications for acute and chronic lung disease in humans

    Lingappan, Krithika, E-mail: [Department of Pediatrics, Section of Neonatology, Texas Children' s Hospital, Baylor College of Medicine, 1102 Bates Avenue, MC: FC530.01, Houston, TX 77030 (United States); Jiang, Weiwu; Wang, Lihua; Couroucli, Xanthi I. [Department of Pediatrics, Section of Neonatology, Texas Children' s Hospital, Baylor College of Medicine, 1102 Bates Avenue, MC: FC530.01, Houston, TX 77030 (United States); Barrios, Roberto [Department of Pathology and Genomic Medicine, The Methodist Hospital Physician Organization, 6565 Fannin Street, Suite M227, Houston, TX 77030 (United States); Moorthy, Bhagavatula [Department of Pediatrics, Section of Neonatology, Texas Children' s Hospital, Baylor College of Medicine, 1102 Bates Avenue, MC: FC530.01, Houston, TX 77030 (United States)


    Sex-specific differences in pulmonary morbidity in humans are well documented. Hyperoxia contributes to lung injury in experimental animals and humans. The mechanisms responsible for sex differences in the susceptibility towards hyperoxic lung injury remain largely unknown. In this investigation, we tested the hypothesis that mice will display sex-specific differences in hyperoxic lung injury. Eight week-old male and female mice (C57BL/6J) were exposed to 72 h of hyperoxia (FiO{sub 2} > 0.95). After exposure to hyperoxia, lung injury, levels of 8-iso-prostaglandin F{sub 2} alpha (8-iso-PGF 2α) (LC–MS/MS), apoptosis (TUNEL) and inflammatory markers (suspension bead array) were determined. Cytochrome P450 (CYP)1A expression in the lung was assessed using immunohistochemistry and western blotting. After exposure to hyperoxia, males showed greater lung injury, neutrophil infiltration and apoptosis, compared to air-breathing controls than females. Pulmonary 8-iso-PGF 2α levels were higher in males than females after hyperoxia exposure. Sexually dimorphic increases in levels of IL-6 (F > M) and VEGF (M > F) in the lungs were also observed. CYP1A1 expression in the lung was higher in female mice compared to males under hyperoxic conditions. Overall, our results support the hypothesis that male mice are more susceptible than females to hyperoxic lung injury and that differences in inflammatory and oxidative stress markers contribute to these sex-specific dimorphic effects. In conclusion, this paper describes the establishment of an animal model that shows sex differences in hyperoxic lung injury in a temporal manner and thus has important implications for lung diseases mediated by hyperoxia in humans. - Highlights: • Male mice were more susceptible to hyperoxic lung injury than females. • Sex differences in inflammatory markers were observed. • CYP1A expression was higher in females after hyperoxia exposure.

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

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


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

    Ademola Emmanuel Fawibe


    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.

  17. Monitoring Cystic Fibrosis Lung Disease : Chest imaging and patient-related outcome measures

    L.A. Tepper (Leonie)


    markdownabstract__Abstract__ Cystic Fibrosis (CF) is a severe, life-shortening genetic disease with a wide spectrum of clinical manifestations, affecting 70,000 patients in the EU and USA. The most prevalent clinical manifestation is structural lung disease. Structural lung disease is the main caus

  18. Spirometry utilisation among Danish adults initiating medication targeting obstructive lung disease

    Koefoed, Mette


    with pharmacotherapy targeting obstructive lung disease and only few have additional tests conducted, although the predictive value of respiratory symptoms for diagnosing obstructive lung disease has proven to be low. Spirometry is recommended as the gold standard for confirming obstructive lung disease, and testing...... prescriptions for medication targeting obstructive lung disease are registered. All spirometry tests provided to the patient cohort in the time period 2007-2010 were extracted from the Danish National Health Service Register and the Danish National Patient Register and we assessed if patients had a spirometry...

  19. Inhaled adrenergics and anticholinergics in obstructive lung disease: do they enhance mucociliary clearance?

    Restrepo, Ruben D


    Pulmonary mucociliary clearance is an essential defense mechanism against bacteria and particulate matter. Mucociliary dysfunction is an important feature of obstructive lung diseases such as chronic obstructive pulmonary disease, asthma, cystic fibrosis, and bronchiectasis. This dysfunction in airway clearance is associated with accelerated loss of lung function in patients with obstructive lung disease. The involvement of the cholinergic and adrenergic neural pathways in the pathophysiology of mucus hypersecretion suggests the potential therapeutic role of bronchodilators as mucoactive agents. Although anticholinergics and adrenergic agonist bronchodilators have been routinely used, alone or in combination, to enhance mucociliary clearance in patients with obstructive lung disease, the existing evidence does not consistently show clinical effectiveness.

  20. High-resolution computed tomography in diffuse infiltrative diseases of the pulmonary parenchyma: A, B, C and quiz; La tomodensitometrie haute resolution dans les maladies infiltrantes diffuses du parenchyme pulmonaire: A, B, C et quiz

    Roy, M.C.; Cordeau, M.P.; Samson, L. [Universite de Montreal, Hotel-Dieu de Montreal, Quebec (Canada); Chartrand-Lefebvre, C. [Montreal Univ., PQ (Canada)


    High-resolution computed tomography (HRCT) provides precise imaging of lung parenchyma and is most useful in the investigation of diffuse interstitial lung disease (DILD). It involves thin collimation and high frequency reconstruction algorithm, and requires knowledge of the secondary pulmonary lobule anatomy. Its interpretation is facilitated by recognition of elementary signs (peri-broncho-vascular thickening, septal thickening, ground glass attenuation, etc.) which can be grouped in a HRCT pictorial glossary. In a quiz format, readers are asked to diagnose twelve cases of classical DILD with clinical correlation. (authors). 29 refs.

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

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


    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).

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

    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

  3. Spirometry utilisation among Danish adults initiating medication targeting obstructive lung disease

    Koefoed, Mette


    with pharmacotherapy targeting obstructive lung disease and only few have additional tests conducted, although the predictive value of respiratory symptoms for diagnosing obstructive lung disease has proven to be low. Spirometry is recommended as the gold standard for confirming obstructive lung disease, and testing...... can also rule out airway obstruction in patients with respiratory symptoms caused by other illnesses, such as heart failure or lung cancer. Initiating medication for obstructive lung disease without spirometry entails the risk of these patients experiencing unnecessary delay in the diagnostic process......-infectious dyspnoea, chronic cough and wheezing are common symptoms in the population. Patients often present with these symptoms in general practice and have a high probability of having obstructive lung diseases. However, there is an indication that the majority of these patients are treated empirically...

  4. Cellular interactions in the pathogenesis of interstitial lung diseases

    Gianluca Bagnato


    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.

  5. Opacidades em vidro fosco nas doenças pulmonares difusas: correlação da tomografia computadorizada de alta resolução com a anatomopatologia Ground-glass opacity in diffuse lung diseases: high-resolution computed tomography-pathology correlation

    Maria Lúcia de Oliveira Santos


    , association with other radiological, clinical and pathological findings must be considered for an accurate diagnostic interpretation. In this paper were reviewed 62 computed tomography examinations of patients with diffuse pulmonary diseases of 14 different etiologies in which ground-glass opacity was the only or the most remarkable finding, and correlated this findings with pathology abnormalities seen on specimens obtained from biopsies or necropsies. In pneumocystosis, ground-glass opacities correlated histologically with alveolar occupation by a foaming material containing parasites, in bronchioloalveolar cell carcinoma with thickening of the alveolar septa and occupation of the lumen by mucus and tumoral cells, in paracoccidioidomycosis with thickening of the alveolar septa, areas of fibrosis and alveolar bronchopneumonic exsudate, in sarcoidosis with fibrosis or clustering of granulomas and in idiopathic pulmonary fibrosis with alveolar septa thickening due to fibrosis. Alveolar occupation by blood was found in cases of leptospirosis, idiopathic hemosiderosis, metastatic kidney tumor and invasive aspergillosis whereas oily vacuoli were seen in lipoid pneumonia, proteinaceus and lipoproteinaceus material in silicoproteinosis and pulmonary alveolar proteinosis, and edematous fluid in cardiac failure.

  6. [Interpretation and use of routine pulmonary function tests: Spirometry, static lung volumes, lung diffusion, arterial blood gas, methacholine challenge test and 6-minute walk test].

    Bokov, P; Delclaux, C


    Resting pulmonary function tests (PFT) include the assessment of ventilatory capacity: spirometry (forced expiratory flows and mobilisable volumes) and static volume assessment, notably using body plethysmography. Spirometry allows the potential definition of obstructive defect, while static volume assessment allows the potential definition of restrictive defect (decrease in total lung capacity) and thoracic hyperinflation (increase in static volumes). It must be kept in mind that this evaluation is incomplete and that an assessment of ventilatory demand is often warranted, especially when facing dyspnoea: evaluation of arterial blood gas (searching for respiratory insufficiency) and measurement of the transfer coefficient of the lung, allowing with the measurement of alveolar volume to calculate the diffusing capacity of the lung for CO (DLCO: assessment of alveolar-capillary wall and capillary blood volume). All these pulmonary function tests have been the subject of an Americano-European Task force (standardisation of lung function testing) published in 2005, and translated in French in 2007. Interpretative strategies for lung function tests have been recommended, which define abnormal lung function tests using the 5th and 95th percentiles of predicted values (lower and upper limits of normal values). Thus, these recommendations need to be implemented in all pulmonary function test units. A methacholine challenge test will only be performed in the presence of an intermediate pre-test probability for asthma (diagnostic uncertainty), which is an infrequent setting. The most convenient exertional test is the 6-minute walk test that allows the assessment of walking performance, the search for arterial desaturation and the quantification of dyspnoea complaint.

  7. Associations of autophagy with lung diffusion capacity and oxygen saturation in severe COPD: effects of particulate air pollution.

    Lee, Kang-Yun; Chiang, Ling-Ling; Ho, Shu-Chuan; Liu, Wen-Te; Chen, Tzu-Tao; Feng, Po-Hao; Su, Chien-Ling; Chuang, Kai-Jen; Chang, Chih-Cheng; Chuang, Hsiao-Chi


    Although traffic exposure has been associated with the development of COPD, the role of particulate matter diffusion capacity of the lung for carbon monoxide levels (r=-0.914, P=0.029), changes in the pulse oxygen saturation (ΔSaO2; r=-0.973, P=0.005), receptor for advanced glycation end-products (r=-0.881, P=0.048), interleukin-6 (r=0.986, P=0.002), ubiquitin (r=0.940, P=0.017), and beclin 1 (r=0.923, P=0.025) in COPD. Next, we observed that ubiquitin was correlated with ΔSaO2 (r=-0.374, P=0.019). Beclin 1 was associated with diffusion capacity of the lung for carbon monoxide (r=-0.362, P=0.028), ΔSaO2 (r=-0.354, P=0.032), and receptor for advanced glycation end-products (r=-0.471, P=0.004). Autophagy may be an important regulator of the PM10-related pathogenesis of COPD, which could cause deterioration in the lung diffusion capacity and oxygen saturation.

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

    O'Dwyer, David N


    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.

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

    张佳林; 叶宝国; 陈宏浦


    common respiratory symptoms.The fall of activity tolerance and dyspnea were serious respiratory symptoms.64 patients had pulmonary function test and all of them showed a decreasing diffusing capacity.Among them,26 only showed diffusing capacity reduction,32 restrictive ventilation defect with diffusing capacity reduction,4 mixed ventilation defect with diffusing capacity reduction and 2 obstructive ventilation defect with diffusing capacity reduction.22 patients had pulmonary artery high pressure.In image,the features of interstitial lung diseases displayed as follows:nodus,steak,patchy sign,grid,ground-glass opacities and bullae of lung.The features of interstitial lung diseases displayed as grid and fibrosis in 15 of 32 restrictive ventilation defect with diffusing capacity reduction.Conclusions The respiratory symptoms of c onnective tissue diseases with interstitial lung disease are various.Pulmonary function of connective tissue diseases with interstitial lung disease primarily displays a restrictive ventilation defect with diffusing capacity reduction.Connective tissue diseases complicated with interstitial lung disease and of pulmonary arterial hypertension easily occur in mixed connective tissue disease (MCTD),overlap connective tissue disease,systemic lupus erythematosus (SLE) and systemic sclerosis(SSc).In image,the features of interstitial lung diseases is various.Among them,grid and fibrosis are related to pulmonary function of restrictive ventilation defect with diffusing capacity reduction.

  10. CT screening for lung cancer brings forward early disease. The Randomised Danish Lung Cancer Screening Trial (DLCST)

    Saghir, Zaigham; Dirksen, Asger; Ashraf, Haseem


    BackgroundThe effects of low-dose CT screening on disease stage shift, mortality and overdiagnosis are unclear. Lung cancer findings and mortality rates are reported at the end of screening in the Danish Lung Cancer Screening Trial.Methods4104 men and women, healthy heavy smokers/former smokers...... increase and/or volume doubling time15 mm were referred for diagnostic workup. In the control group, lung cancers were diagnosed and treated outside the study by the usual clinical practice.ResultsParticipation rates were high in both groups (screening: 95.5%; control: 93.0%; p...

  11. Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study.

    Khanna, Dinesh; Clements, Philip J; Furst, Daniel E; Chon, Yun; Elashoff, Robert; Roth, Michael D; Sterz, Mildred G; Chung, Joannie; FitzGerald, John D; Seibold, James R; Varga, John; Theodore, Arthur; Wigley, Fredrick M; Silver, Richard M; Steen, Virginia D; Mayes, Maureen D; Connolly, M Kari; Fessler, Barri J; Rothfield, Naomi F; Mubarak, Kamal; Molitor, Jerry; Tashkin, Donald P


    To determine whether baseline self-assessment measures of health status and physiologic indices of disease severity in alveolitis-positive patients with systemic sclerosis (SSc) correlate with the severity of their dyspnea, and to quantify functional impairment in patients with scleroderma lung disease and compare it with that in patients with chronic obstructive pulmonary disease (COPD). SSc patients (n = 138) with diffuse (n = 81) or limited (n = 57) cutaneous disease and active alveolitis (determined by bronchoalveolar lavage and/or high-resolution computed tomography) who participated in the National Heart, Lung, and Blood Institute-sponsored, multicenter, parallel-group, double-blind, randomized, placebo-controlled trial of oral cyclophosphamide for treatment of SSc-associated interstitial lung disease were evaluated. Pearson's univariate correlations were determined between the Short Form 36 (SF-36) physical component summary (PCS) and mental component summary (MCS) scales, functional questionnaires, and physiologic parameters of breathing (forced vital capacity [FVC] and single-breath diffusing capacity for carbon monoxide [DLCO]). Student's t-test was used to compare subgroups. Scores from 2 instruments for self-assessment of breathlessness, Mahler's baseline dyspnea index (BDI) and a visual analog scale (VAS) for breathing, were divided at the median. Values for the DLCO and FVC (% predicted) were divided based on the American Thoracic Society guidelines for mild (>70% of predicted), moderate (50-70% of predicted), and severe (alveolitis, all 8 domains of the SF-36 were significantly impaired as compared with the healthy population and were similar to those reported by patients with COPD. The SF-36 was able to discriminate between scleroderma lung disease patients with more severe and less severe breathlessness, the primary symptom of active alveolitis. The SF-36 complements the BDI and VAS scores for breathing in scleroderma lung disease and is variably

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

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


    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...

  13. Interstitial Lung Disease in India. Results of a Prospective Registry.

    Singh, Sheetu; Collins, Bridget F; Sharma, Bharat B; Joshi, Jyotsna M; Talwar, Deepak; Katiyar, Sandeep; Singh, Nishtha; Ho, Lawrence; Samaria, Jai Kumar; Bhattacharya, Parthasarathi; Gupta, Rakesh; Chaudhari, Sudhir; Singh, Tejraj; Moond, Vijay; Pipavath, Sudhakar; Ahuja, Jitesh; Chetambath, Ravindran; Ghoshal, Aloke G; Jain, Nirmal K; Devi, H J Gayathri; Kant, Surya; Koul, Parvaiz; Dhar, Raja; Swarnakar, Rajesh; Sharma, Surendra K; Roy, Dhrubajyoti J; Sarmah, Kripesh R; Jankharia, Bhavin; Schmidt, Rodney; Katiyar, Santosh K; Jindal, Arpita; Mangal, Daya K; Singh, Virendra; Raghu, Ganesh


    Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the clinical profile of ILDs in India. To characterize new-onset ILDs in India by creating a prospective ILD using multidisciplinary discussion (MDD) to validate diagnoses. Adult patients of Indian origin living in India with new-onset ILD (27 centers, 19 Indian cities, March 2012-June 2015) without malignancy or infection were included. All had connective tissue disease (CTD) serologies, spirometry, and high-resolution computed tomography chest. ILD pattern was defined by high-resolution computed tomography images. Three groups independently made diagnoses after review of clinical data including that from prompted case report forms: local site investigators, ILD experts at the National Data Coordinating Center (NDCC; Jaipur, India) with MDD, and experienced ILD experts at the Center for ILD (CILD; Seattle, WA) with MDD. Cohen's κ was used to assess reliability of interobserver agreement. A total of 1,084 patients were recruited. Final diagnosis: hypersensitivity pneumonitis in 47.3% (n = 513; exposure, 48.1% air coolers), CTD-ILD in 13.9%, and idiopathic pulmonary fibrosis in 13.7%. Cohen's κ: 0.351 site investigator/CILD, 0.519 site investigator/NDCC, and 0.618 NDCC/CILD. Hypersensitivity pneumonitis was the most common new-onset ILD in India, followed by CTD-ILD and idiopathic pulmonary fibrosis; diagnoses varied between site investigators and CILD experts, emphasizing the value of MDD in ILD diagnosis. Prompted case report forms including environmental exposures in prospective registries will likely provide further insight into the etiology and management of ILD worldwide.

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

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


    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.

  15. Rare lung disease research: strategies for improving identification and recruitment of research participants.

    Gupta, Samir; Bayoumi, Ahmed M; Faughnan, Marie E


    Research in rare lung diseases faces methodologic limitations by virtue of the small number of participants available to be studied. We explored several strategies that may improve researchers' ability to identify and recruit research participants with rare lung diseases. We provide an overview of strategies based on available evidence, previously used approaches, and reasoning. First, disease detection is generally poor and may be improved through strategies targeted at primary care practitioners or directly at patients, thus increasing the pool of patients available for research studies. Next, standardization of case definitions in rare lung diseases is often lacking, hindering research recruitment efforts because of confusion over appropriate recruitment criteria. Expert consensus statements can enhance both clinical care and research recruitment by standardizing definitions. Finally, recruitment strategies using rare lung disease registries, clinical research networks, novel Internet-based direct patient recruitment approaches, and patient organizations may facilitate recruitment of patients with rare lung diseases. In summary, although several strategies for improving the identification and recruitment of research participants with rare lung diseases have been proposed, published examples are few. Objective measurement and reporting of novel recruitment methods and collaboration among researchers facing the same limitations across various rare lung diseases are required. Advancements in this area are vital to the design and performance of much-needed robust clinical studies across the spectrum of rare lung diseases.

  16. Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis.

    Stahl, Mirjam; Wielpütz, Mark O; Graeber, Simon Y; Joachim, Cornelia; Sommerburg, Olaf; Kauczor, Hans-Ulrich; Puderbach, Michael; Eichinger, Monika; Mall, Marcus A


    Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for diagnostic monitoring and early intervention clinical trials. The lung clearance index (LCI) and chest magnetic resonance imaging (MRI) were shown to detect early lung disease in CF; however, the relationship between the two measures remains unknown. To correlate the LCI with abnormalities detected by MRI and compare the sensitivity of the two techniques to detect responses to therapy for pulmonary exacerbations in children with CF. LCI determined by age-adapted multiple breath washout techniques and MRI studies were performed in 97 clinically stable children with CF across the pediatric age range (0.2-21.1 yr). Furthermore, LCI (n = 26) or MRI (n = 10) were performed at the time of pulmonary exacerbation and after antibiotic therapy. MRI was evaluated using a dedicated morphofunctional score. The LCI correlated with the global MRI score as well as MRI-defined airway wall abnormalities, mucus plugging, and abnormal lung perfusion in infants and toddlers (P lung perfusion in early CF lung disease. Clinical trial registered with (NCT 02270476).

  17. Evaluation and Diagnosis of HIV-Associated Lung Disease.

    Maximous, Stephanie; Huang, Laurence; Morris, Alison


    There are myriad pulmonary conditions associated with HIV, ranging from acute infections to chronic noncommunicable diseases. The epidemiology of these diseases has changed significantly in the era of widespread antiretroviral therapy. Evaluation of the HIV-infected patient involves assessment of the severity of illness and a thorough yet efficient pursuit of definitive diagnosis, which may involve multiple etiologies simultaneously. Important clues to a diagnosis include medical and social history, demographic details such as travel and geography of residence, substance use, sexual practices, and domiciliary and incarceration status. CD4 cell count is a tremendously useful measure of immune function and risk for HIV-related diseases, and helps narrow down the differential. Careful history of current symptoms and physical examination with particular attention to extrapulmonary signs are crucial early steps. Many adjunctive laboratory studies can suggest or rule out particular diagnoses. Pulmonary function testing (PFT) may aid in characterization of several chronic noninfectious illnesses accelerated by HIV. Chest radiograph and computed tomography (CT) scan allow for classification of diseases by pathognomonic imaging patterns, although many infectious conditions present atypically, particularly with lower CD4 counts. Ultimately, definitive diagnosis with sputum, bronchoscopy with bronchoalveolar lavage, or lung tissue is often needed. It is of utmost importance to maintain a high degree of suspicion for HIV in otherwise undiagnosed patients, as the first presentation of HIV may be via an acute pulmonary illness.

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

    Amar, Rajeev K.


    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...

  19. Tomography patterns of lung disease in systemic sclerosis

    Andréa de Lima Bastos

    Full Text Available Abstract Currently, lung impairment is the leading factor responsible for the morbidity and mortality associated with systemic sclerosis. Therefore, the recognition of the various tomography patterns becomes decisive in the clinical management of these patients. In high-resolution computed tomography studies, the most common pattern is that of nonspecific interstitial pneumonia. However, there are other forms of lung involvement that must also be recognized. The aim of this study was to review the literature on the main changes resulting from pulmonary involvement in systemic sclerosis and the corresponding radiological findings, considering the current classification of interstitial diseases. We searched the Medline (PubMed, Lilacs, and SciELO databases in order to select articles related to pulmonary changes in systemic sclerosis and published in English between 2000 and 2015. The pulmonary changes seen on computed tomography in systemic sclerosis are varied and are divided into three main categories: interstitial, alveolar, and vascular. Interstitial changes constitute the most common type of pulmonary involvement in systemic sclerosis. However, alveolar and vascular manifestations must also be recognized and considered in the presence of atypical clinical presentations and inadequate treatment responses.

  20. Tomography patterns of lung disease in systemic sclerosis.

    Bastos, Andréa de Lima; Corrêa, Ricardo de Amorim; Ferreira, Gilda Aparecida


    Currently, lung impairment is the leading factor responsible for the morbidity and mortality associated with systemic sclerosis. Therefore, the recognition of the various tomography patterns becomes decisive in the clinical management of these patients. In high-resolution computed tomography studies, the most common pattern is that of nonspecific interstitial pneumonia. However, there are other forms of lung involvement that must also be recognized. The aim of this study was to review the literature on the main changes resulting from pulmonary involvement in systemic sclerosis and the corresponding radiological findings, considering the current classification of interstitial diseases. We searched the Medline (PubMed), Lilacs, and SciELO databases in order to select articles related to pulmonary changes in systemic sclerosis and published in English between 2000 and 2015. The pulmonary changes seen on computed tomography in systemic sclerosis are varied and are divided into three main categories: interstitial, alveolar, and vascular. Interstitial changes constitute the most common type of pulmonary involvement in systemic sclerosis. However, alveolar and vascular manifestations must also be recognized and considered in the presence of atypical clinical presentations and inadequate treatment responses.

  1. Tomography patterns of lung disease in systemic sclerosis

    Bastos, Andrea de Lima; Correa, Ricardo de Amorim; Ferreira, Gilda Aparecida, E-mail: [Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG (Brazil). Faculdade de Medicina


    Currently, lung impairment is the leading factor responsible for the morbidity and mortality associated with systemic sclerosis. Therefore, the recognition of the various tomography patterns becomes decisive in the clinical management of these patients. In high-resolution computed tomography studies, the most common pattern is that of nonspecific interstitial pneumonia. However, there are other forms of lung involvement that must also be recognized. The aim of this study was to review the literature on the main changes resulting from pulmonary involvement in systemic sclerosis and the corresponding radiological findings, considering the current classification of interstitial diseases. We searched the Medline (PubMed), Lilacs, and SciELO databases in order to select articles related to pulmonary changes in systemic sclerosis and published in English between 2000 and 2015. The pulmonary changes seen on computed tomography in systemic sclerosis are varied and are divided into three main categories: interstitial, alveolar, and vascular. Interstitial changes constitute the most common type of pulmonary involvement in systemic sclerosis. However, alveolar and vascular manifestations must also be recognized and considered in the presence of atypical clinical presentations and inadequate treatment responses. (author)

  2. Interstitial lung disease associated with human papillomavirus vaccination

    Yasushi Yamamoto


    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.

  3. Structure-Activity Association of Flavonoids in Lung Diseases

    João Henrique G. Lago


    Full Text Available Flavonoids are polyphenolic compounds classified into flavonols, flavones, flavanones, isoflavones, catechins, anthocyanidins, and chalcones according to their chemical structures. They are abundantly found in Nature and over 8,000 flavonoids have from different sources, mainly plant materials, have been described. Recently reports have shown the valuable effects of flavonoids as antiviral, anti-allergic, antiplatelet, antitumor, antioxidant, and anti-inflammatory agents and interest in these compounds has been increasing since they can be helpful to human health. Several mechanisms of action are involved in the biological properties of flavonoids such as free radical scavenging, transition metal ion chelation, activation of survival genes and signaling pathways, regulation of mitochondrial function and modulation of inflammatory responses. The anti-inflammatory effects of flavonoids have been described in a number of studies in the literature, but not frequently associated to respiratory disease. Thus, this review aims to discuss the effects of different flavonoids in the control of lung inflammation in some disorders such as asthma, lung emphysema and acute respiratory distress syndrome and the possible mechanisms of action, as well as establish some structure-activity relationships between this biological potential and chemical profile of these compounds.

  4. Tomography patterns of lung disease in systemic sclerosis*

    Bastos, Andréa de Lima; Corrêa, Ricardo de Amorim; Ferreira, Gilda Aparecida


    Currently, lung impairment is the leading factor responsible for the morbidity and mortality associated with systemic sclerosis. Therefore, the recognition of the various tomography patterns becomes decisive in the clinical management of these patients. In high-resolution computed tomography studies, the most common pattern is that of nonspecific interstitial pneumonia. However, there are other forms of lung involvement that must also be recognized. The aim of this study was to review the literature on the main changes resulting from pulmonary involvement in systemic sclerosis and the corresponding radiological findings, considering the current classification of interstitial diseases. We searched the Medline (PubMed), Lilacs, and SciELO databases in order to select articles related to pulmonary changes in systemic sclerosis and published in English between 2000 and 2015. The pulmonary changes seen on computed tomography in systemic sclerosis are varied and are divided into three main categories: interstitial, alveolar, and vascular. Interstitial changes constitute the most common type of pulmonary involvement in systemic sclerosis. However, alveolar and vascular manifestations must also be recognized and considered in the presence of atypical clinical presentations and inadequate treatment responses. PMID:27818546

  5. Prediction equations for diffusing capacity (transfer factor of lung for North Indians

    Sunil Kumar Chhabra


    Full Text Available Background: Prediction equations for diffusing capacity of lung for carbon monoxide (DLCO, alveolar volume (VA, and DLCO/VA using the current standardization guidelines are not available for Indian population. The present study was carried out to develop equations for these parameters for North Indian adults and examine the ethnic diversity in predictions. Materials and Methods: DLCO was measured by single-breath technique and VA by single-breath helium dilution using standardized methodology in 357 (258 males, 99 females normal nonsmoker adult North Indians and DLCO/VA was computed. The subjects were randomized into training and test datasets for development of prediction equations by multiple linear regressions and for validation, respectively. Results: For males, the following equations were developed: DLCO, −7.813 + 0.318 × ht −0.624 × age + 0.00552 × age 2 ; VA, −8.152 + 0.087 × ht −0.019 × wt; DLCO/VA, 7.315 − 0.037 × age. For females, the equations were: DLCO, −44.15 + 0.449 × ht −0.099 × age; VA, −6.893 + 0.068 × ht. A statistically acceptable prediction equation was not obtained for DLCO/VA in females. It was therefore computed from predicted DLCO and predicted VA. All equations were internally valid. Predictions of DLCO by Indian equations were lower than most Caucasian predictions in both males and females and greater than the Chinese predictions for males. Conclusion: This study has developed validated prediction equations for DLCO, VA, and DLCO/VA in North Indians. Substantial ethnic diversity exists in predictions for DLCO and VA with Caucasian equations generally yielding higher values than the Indian or Chinese equations. However, DLCO/VA predicted by the Indian equations is slightly higher than that by other equations.

  6. Reference values for lung function tests: III. Carbon monoxide diffusing capacity (transfer factor

    Neder J.A.


    Full Text Available Carbon monoxide diffusing capacity (DLCO or transfer factor (TLCO is a particularly useful test of the appropriateness of gas exchange across the lung alveolocapillary membrane. With the purpose of establishing predictive equations for DLCO using a non-smoking sample of the adult Brazilian population, we prospectively evaluated 100 subjects (50 males and 50 females aged 20 to 80 years, randomly selected from more than 8,000 individuals. Gender-specific linear prediction equations were developed by multiple regression analysis with single breath (SB absolute and volume-corrected (VA DLCO values as dependent variables. In the prediction equations, age (years and height (cm had opposite effects on DLCOSB (ml min-1 mmHg-1, independent of gender (-0.13 (age + 0.32 (height - 13.07 in males and -0.075 (age + 0.18 (height + 0.20 in females. On the other hand, height had a positive effect on DLCOSB but a negative one on DLCOSB/VA (P<0.01. We found that the predictive values from the most cited studies using predominantly Caucasian samples were significantly different from the actually measured values (P<0.05. Furthermore, oxygen uptake at maximal exercise (VO2max correlated highly to DLCOSB (R = 0.71, P<0.001; this variable, however, did not maintain an independent role to explain the VO2max variability in the multiple regression analysis (P>0.05. Our results therefore provide an original frame of reference for either DLCOSB or DLCOSB/VA in Brazilian males and females aged 20 to 80 years, obtained from the standardized single-breath technique.

  7. Effect of autoimmune diseases on risk and survival in histology-specific lung cancer.

    Hemminki, Kari; Liu, Xiangdong; Ji, Jianguang; Sundquist, Jan; Sundquist, Kristina


    Patients with autoimmune diseases are at an increased risk of cancer due to underlying dysregulation of the immune system or treatment. Data on cancer incidence, mortality and survival after autoimmune diseases would provide further information on the clinical implications. We systematically analysed data on lung cancer in patients diagnosed with 33 different autoimmune diseases. Standardised incidence ratios (SIRs), standardised mortality ratios (SMRs) and hazard ratios (HRs) were calculated for subsequent incident lung cancers or lung cancer deaths up to 2008 in patients hospitalised for autoimmune disease after 1964. Increased risks of lung cancer were recorded for SIRs after 12 autoimmune diseases, SMRs after 11 autoimmune diseases and HRs after two autoimmune diseases. The highest SIRs and SMRs, respectively, were seen after discoid lupus erythematosus (4.71 and 4.80), polymyosistis/dermatomyositis (4.20 and 4.17), systemic lupus erythematosus (2.47 and 2.69), rheumatic fever (2.07 and 2.07) and systemic sclerosis (2.19 and 1.98). Autoimmune disease did not influence survival overall but some autoimmune diseases appeared to impair survival in small cell carcinoma. All autoimmune diseases that had an SIR >2.0 are known to present with lung manifestations, suggesting that the autoimmune process contributes to lung cancer susceptibility. The data on survival are reassuring that autoimmune diseases do not influence prognosis in lung cancer.

  8. Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease.

    Montgomery, Gregory S; Sagel, Scott D; Taylor, Amy L; Abman, Steven H


    Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.

  9. Treatment of Systemic Sclerosis-related Interstitial Lung Disease: A Review of Existing and Emerging Therapies.

    Volkmann, Elizabeth R; Tashkin, Donald P


    Although interstitial lung disease accounts for the majority of deaths of patients with systemic sclerosis, treatment options for this manifestation of the disease are limited. Few high-quality, randomized, controlled trials exist for systemic sclerosis-related interstitial lung disease, and historically, studies have favored the use of cyclophosphamide. However, the benefit of cyclophosphamide for this disease is tempered by its complex adverse event profile. More recent studies have demonstrated the effectiveness of mycophenolate for systemic sclerosis-related interstitial lung disease, including Scleroderma Lung Study II. This review highlights the findings of this study, which was the first randomized controlled trial to compare cyclophosphamide with mycophenolate for the treatment of systemic sclerosis-related interstitial lung disease. The results reported in this trial suggest that there is no difference in treatment efficacy between mycophenolate and cyclophosphamide; however, mycophenolate appears to be safer and more tolerable than cyclophosphamide. In light of the ongoing advances in our understanding of the pathogenic mechanisms underlying interstitial lung disease in systemic sclerosis, this review also summarizes novel treatment approaches, presenting clinical and preclinical evidence for rituximab, tocilizumab, pirfenidone, and nintedanib, as well as hematopoietic stem cell transplantation and lung transplantation. This review further explores how reaching a consensus on appropriate study end points, as well as trial enrichment criteria, is central to improving our ability to judiciously evaluate the safety and efficacy of emerging experimental therapies for systemic sclerosis-related interstitial lung disease.

  10. Lack of spirometry use in Danish patients initiating medication targeting obstructive lung disease

    Koefoed, Mette; Christensen, René Depont; Søndergaard, Jens;


    Research indicates that a large proportion of patients using medication targeting obstructive lung disease have no history of spirometry testing.......Research indicates that a large proportion of patients using medication targeting obstructive lung disease have no history of spirometry testing....

  11. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis : A European consensus

    Heijerman, Harry; Westerman, Elsbeth; Conway, Steven; Touw, Daan; Döring, Gerd; Frijlink, Henderik

    In cystic fibrosis inhalation of drugs for the treatment of CF related lung disease has been proven to be highly effective. Consequently, an increasing number of drugs and devices have been developed for CF lung disease or are currently under development. In this European consensus document we

  12. Postmortem endogenous ethanol production and diffusion from the lung due to aspiration of wood chip dust in the work place.

    Furumiya, Junichi; Nishimura, Hiroyuki; Nakanishi, Akinori; Hashimoto, Yoshiaki


    We report an autopsy case of postmortem ethanol diffusion into the cardiac blood after aspiration of wood chips, although antemortem ethanol consumption was not evident. A man in his twenties, who was loading a truck with small wood chips in a hot, humid storehouse, was accidentally buried in a heap of chips. At the time the body was discovered, 20 h after the accident, rectal temperature was 36°C. Autopsy showed the cause of death to be asphyxia due to obstruction of the airway by aspiration of wood chips. The ethanol and n-propanol levels were significantly higher in the lungs (left, 0.603 and 0.009 mg/g; right, 0.571 and 0.006 mg/g) than in other tissues. A significant difference in ethanol concentration was observed between the left cardiac blood (0.243 mg/g) and the right femoral blood (0.042 mg/g). Low levels of ethanol and n-propanol were detected in the stomach contents (0.105 and 0.001 mg/g, respectively). In order to determine whether aspiration of wood chips affects postmortem ethanol production in the lung, we measured the ethanol and n-propanol levels of homogenized rabbit lung tissue incubated with autoclaved or non-autoclaved wood chips. Levels of ethanol and n-propanol were significantly higher in the homogenates incubated with non-autoclaved chips for 24h. The results of this animal experiment suggested that the ethanol detected in the lung was produced by putrefactive bacteria within the wood chips. After death, the ethanol produced endogenously in the lung appears to have diffused and affected the ethanol concentration of the left cardiac blood.

  13. Lung-function trajectories leading to chronic obstructive pulmonary disease

    Lange, Peter; Celli, B.; Agustí, A.;


    population norms. METHODS: We stratified participants in three independent cohorts (the Framingham Offspring Cohort, the Copenhagen City Heart Study, and the Lovelace Smokers Cohort) according to lung function (FEV1 ≥80% or age of patients, approximately...... at the end of the observation period had had a normal FEV1 before 40 years of age and had a rapid decline in FEV1 thereafter, with a mean (±SD) decline of 53±21 ml per year. The remaining half had had a low FEV1 in early adulthood and a subsequent mean decline in FEV1 of 27±18 ml per year (P...BACKGROUND: Chronic obstructive pulmonary disease (COPD) is thought to result from an accelerated decline in forced expiratory volume in 1 second (FEV1) over time. Yet it is possible that a normal decline in FEV1 could also lead to COPD in persons whose maximally attained FEV1 is less than...

  14. Erectile Dysfunction ia a common problem in Interstitial Lung Disease

    Fløe, Andreas; Hilberg, Ole; Wijsenbeek, Marlies

    .6%) had co-morbid heart disease and/or manifest atherosclerosis, and 6 (11.8%) had diabetes mellitus (DM). 39 (76.5%) had fibrosing ILD on high-resolution CT-scan or lung biopsy (IPF n=24, Fibrosing NSIP n=7, unclassifiable ILD with fibrosis n=8). 35 ILD patients (70%) had ED, and 22 (44%) hereof had...... severe ED. Having a co-morbidity was not associated with increased risk of ED (OR 0,94, P=0,74).  Conclusion: Our data clearly demonstrate that ED is a common problem in ILD. Almost half of all patients in this study had severe ED. This is, to our knowledge, the first study to report on the occurrence...... of sexual problems among male patients with ILD. The rate of ED was comparable to that found among COPD patients in prior studies. Further research is needed in order to identify specific risk factors for ED among ILD patients....

  15. Beyond pneumonoconiosis: Recently described occupational interstitial lung diseases

    Evangelia Nena


    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.

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

    Mark J. Hamblin


    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.


    Izidor Kern


    Full Text Available Background. By the bronchoscopic lavaging we obtain cytological specimen of the peripheral airways and lung parenchyma. Bronchoalveolar lavage fluid (BALF examination gives us information about inflammation in the interstitial lung diseases. BALF sampling and its laboratory processing are standardized in our hospital. Quality assurance practice requires that each institution establish disease characteristic BALF profiles. The aim of this study was to find common cytological characteristics of BALF specimens in sarcoidosis, extrinsic allergic bronchoalveolitis (EABA, asbestosis and idiopathic interstitial pneumonias (IIP.Material and methods. We included 135 BALF specimens of good quality from patients with one of the mentioned entities that were diagnosed clinically, radiographically and pathologically. Based on diferential cell counts and lymphocytic phenotypisation done by immunofluorescence, BALF specimens were categorized as normal type, lymphocytic, neutrophilic, eosinophilic, macrophagic and mixed cell alveolitis. Statistical comparison was performed by the analysis of variance which was done by SAS software.Results. BALF in sarcoidosis shown in 71% lymphocytic or mixed cell (lymphocytic and neutrophilic or eosinophilic type alveolitis with increased CD4/CD8 ratio (> 3.5 in 61.4% specimens. EABA patients had lymphocytic or mixed cell (lymphocytic and neutrophilic or eosinophilic type alveolitis in 53.3% of BALF specimens with decreased CD4/CD8 ratio (< 1 in 38.5% of specimens. Macrophagic alveolitis was the most common type found in asbestosis (52.9%. CD4/CD8 ratio was usually decreased or normal. Patients with IIP had all types of alveolitis and none of them prevailed, but neutrophils were increased often (53.3%. CD4/CD8 ratio was decreased in 65.5% of BALF specimens in IIP.Conclusions. Sarcoidosis has the most characteristic BALF profile (CD4 lymphocytic alveolitis. We often see macrophagic alveolitis in asbestosis. BALF specimens in

  18. Role of Innate Lymphoid Cells in Lung Disease

    SayedMehran Marashian


    Full Text Available  Innate lymphoid cells (ILCs are identified as novel population of hematopoietic cells which protect the body by coordinating the innate immune response against a wide range of threats including infections, tissue damages and homeostatic disturbances. ILCs, particularly ILC2 cells, are found throughout the body including the brain. ILCs are morphologically similar to lymphocytes, express and release high levels of T-helper (Th1, Th2 and Th17 cytokines but do not express classical cell-surface markers that are associated with other immune cell lineages.Three types of ILCs (ILC1, 2 & 3 have been reported depending upon the cytokines produced. ILC1 cells encompass natural killer (NK cells and interferon (IFN-g releasing cells; ILC2 cells release the Th2 cytokines, IL-5, IL-9 and IL-13 in response to IL-25 and IL-33; and ILC3 cells which release IL-17 and IL-22. ILC2 cells have been implicated inmucosal reactions occurring in animal models of allergic asthma and virus-induced lung disorders resulting in the regulation of airway remodeling and tissue homeostasis.There is evidence for increased ILC2 cell numbers in allergic responses in man but little is known about the role of ILCs in chronic obstructive pulmonary disease (COPD. Further understanding of the characteristics of ILCs such as their origin, location and phenotypes and function would help to clarify the role of these cells in the pathogenesis of various lung diseases.In this review we will focus on the role of ILC2 cells and consider their origin, function,location and possible role in the pathogenesis of the chronic inflammatory disorders such as asthma and COPD.   

  19. Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease.

    Lunt, Alan; McGhee, Emily; Robinson, Polly; Rees, David; Height, Susan; Greenough, Anne


    Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV. Post transfusion, the median Rrs5 had increased from 127.4 to 141.3% predicted (pvolume from 39.7 to 64.1 ml/m2 (pvolume in one second (p=0.0056) and vital capacity (p=0.0008) decreased. The increase in Rrs5 correlated with the increase in PCBV (r=0.50, p=0.0493). Increased pulmonary capillary blood volume may at least partially explain the lung function abnormalities in SCD children.

  20. Weight preserving image registration for monitoring disease progression in lung CT

    Gorbunova, Vladlena; Lo, Pechin Chien Pau; Haseem, Ashraf


    the density of lung tissue with respect to local expansion or compression such that the total weight of the lungs is preserved during deformation. Our method provides a good estimation of regional destruction of lung tissue for subjects with a significant difference in inspiration level between CT scans...... compared with intensities in the deformed baseline image indicate local loss of lung tissue that is associated with progression of emphysema. To account for differences in lung intensity owing to differences in the inspiration level in the two scans rather than disease progression, we propose to adjust...

  1. Disruption of the Hepcidin/Ferroportin Regulatory System Causes Pulmonary Iron Overload and Restrictive Lung Disease

    Joana Neves


    Full Text Available Emerging evidence suggests that pulmonary iron accumulation is implicated in a spectrum of chronic lung diseases. However, the mechanism(s involved in pulmonary iron deposition and its role in the in vivo pathogenesis of lung diseases remains unknown. Here we show that a point mutation in the murine ferroportin gene, which causes hereditary hemochromatosis type 4 (Slc40a1C326S, increases iron levels in alveolar macrophages, epithelial cells lining the conducting airways and lung parenchyma, and in vascular smooth muscle cells. Pulmonary iron overload is associated with oxidative stress, restrictive lung disease with decreased total lung capacity and reduced blood oxygen saturation in homozygous Slc40a1C326S/C326S mice compared to wild-type controls. These findings implicate iron in lung pathology, which is so far not considered a classical iron-related disorder.

  2. Lung Transplantation in Gaucher Disease: A Learning Lesson in Trying to Avoid Both Scylla and Charybdis.

    de Boer, Geertje M; van Dussen, Laura; van den Toorn, Leon M; den Bakker, Michael A; Hoek, Rogier A S; Hesselink, Dennis A; Hollak, Carla E M; van Hal, Peter Th W


    Gaucher disease (GD), a lysosomal storage disorder, may result in end-stage lung disease. We report successful bilateral lung transplantation in a 49-year-old woman with GD complicated by severe pulmonary hypertension and fibrotic changes in the lungs. Before receiving the lung transplant, the patient was undergoing both enzyme replacement therapy (imiglucerase) and triple pulmonary hypertension treatment (epoprostenol, bosentan, and sildenafil). She had a history of splenectomy, severe bone disease, and renal involvement, all of which were related to GD and considered as relative contraindications for a lung transplantation. In the literature, lung transplantation has been suggested for severe pulmonary involvement in GD but has been reported only once in a child. To our knowledge, until now, no successful procedure has been reported in adults, and no reports deal with the severe potential posttransplantation complications specifically related to GD.

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

    Lorenzo Cavagna


    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.

  4. Rheumatoid arthritis-associated interstitial lung disease: lung inflammation evaluated with high resolution computed tomography scan is correlated to rheumatoid arthritis disease activity.

    Pérez-Dórame, Renzo; Mejía, Mayra; Mateos-Toledo, Heidegger; Rojas-Serrano, Jorge


    To describe the association between rheumatoid arthritis disease activity (RA) and interstitial lung damage (inflammation and fibrosis), in a group of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). A retrospective study of RA patients with interstitial lung disease (restrictive pattern in lung function tests and evidence of interstitial lung disease in high resolution computed tomography (HRCT)). Patients were evaluated to exclude other causes of pulmonary disease. RA disease activity was measured with the CDAI index. Interstitial lung inflammation and fibrosis were determined by Kazerooni scale. We compared Kazerooni ground-glass score with the nearest CDAI score to HRCT date scan of the first medical evaluation at our institution. In nine patients, we compared the first ground-glass score with a second one after treatment with DMARDs and corticosteroids. Spearman's rank correlation coefficient was used to evaluate association between RA disease activity and the Kazerooni ground-glass and fibrosis scores. Thirty-four patients were included. A positive correlation between CDAI and ground-glass scores was found (rs=0.3767, PFibrosis and CDAI scores were not associated (rs=-0.0747, P<0.6745). After treatment, a downward tendency in the ground-glass score was observed (median [IQR]): (2.33 [2,3] vs. 2 [1.33-2.16]), P<0.056, along with a lesser CDAI score (27 [8-43] vs. 9 [5-12]), P<0.063. There is a correlation between RA disease activity and ground-glass appearance in the HRCT of RA-ILD patients. These results suggest a positive association between RA disease activity and lung inflammation in RA-ILD. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  5. Epidemiology of Lung Function and Chronic Obstructive Pulmonary Disease

    D.W. Loth (Daan)


    textabstractSpirometry is a technique to evaluate the pulmonary ventilatory function and is a reflection of several forces implied in lung volumes. The lung volume is dependent on the elastic recoil of the lungs and chest wall and the muscular efforts of the chest wall, diaphragm, and abdomen.

  6. Impact of lung disease on respiratory impedance in young children with cystic fibrosis.

    Ramsey, Kathryn A; Ranganathan, Sarath C; Gangell, Catherine L; Turkovic, Lidija; Park, Judy; Skoric, Billy; Stick, Stephen M; Sly, Peter D; Hall, Graham L


    This study aimed to evaluate the ability of the forced oscillation technique (FOT) to detect underlying lung disease in preschool children with cystic fibrosis (CF) diagnosed following newborn screening.184 children (aged 3-6 years) with CF underwent lung function testing on 422 occasions using the FOT to assess respiratory resistance and reactance at the time of their annual bronchoalveolar lavage collection and chest computed tomography scan. We examined associations between FOT outcomes and the presence and progression of respiratory inflammation, infection and structural lung disease.Children with CF who had pronounced respiratory disease, including free neutrophil elastase activity, infection with pro-inflammatory pathogens and structural lung abnormalities had similar FOT outcomes to those children without detectable lung disease. In addition, the progression of lung disease over 1 year was not associated with worsening FOT outcomes.We conclude that the forced oscillation technique is relatively insensitive to detect underlying lung disease in preschool children with CF. However, FOT may still be of value in improving our understanding of the physiological changes associated with early CF lung disease.

  7. Fetal cardiac disease and fetal lung volume: an in utero MRI investigation.

    Mlczoch, Elisabeth; Schmidt, Lisa; Schmid, Maximilian; Kasprian, Gregor; Frantal, Sophie; Berger-Kulemann, Vanessa; Prayer, Daniela; Michel-Behnke, Ina; Salzer-Muhar, Ulrike


    Magnetic resonance imaging (MRI) is a powerful, noninvasive tool to study fetal lung volumes after 18 weeks of gestation in vivo. In neonates with congenital heart disease (CHD), proper lung function is essential for postnatal survival. Antenatal detection of abnormal pulmonary development may help to optimize prenatal and perinatal management of at-risk fetuses. We aimed to investigate lung volumes in fetuses with prenatally diagnosed heart disease. A cross-sectional, retrospective study of 105 consecutive singleton pregnancies with CHD and a control, non-CHD group (n = 115), that underwent fetal MRI was performed. The heart defects detected were divided into four groups. Lung volumes of fetuses with heart disease were compared with control, non-CHD fetuses. In addition, z-scores of lung volumes were calculated for the CHD group (normal range z-scores from -2-+2). As a group, fetuses with CHD have significantly smaller lung volumes compared with control fetuses when corrected by gestational age (GA) (p = 0.049). Of the 105 CHD fetuses studied, 18 had lung volumes with a z-score < -2. Fetuses with different types of CHD showed similar lung volumes. Our data indicate that postpartum pulmonary symptoms and outcome in neonates with congenital heart disease may be attributed to the cardiac disease itself and in part to smaller lung volumes. © 2013 John Wiley & Sons, Ltd.

  8. Diffusion of hyperpolarized {sup 129}Xe in the lung: a simplified model of {sup 129}Xe septal uptake and experimental results

    Patz, Samuel; Muradyan, Iga; Dabaghyan, Mikayel; Washko, George R; Hatabu, Hiroto; Butler, James P [Center for Pulmonary Functional Imaging, Departments of Radiology and Pulmonary Medicine, Brigham and Women' s Hospital and Harvard Medical School, Boston, MA 02115 (United States); Hrovat, Mirko I, E-mail: [Mirtech, Inc., Brockton, MA 02301 (United States)


    We used hyperpolarized {sup 129}Xe NMR to measure pulmonary alveolar surface area per unit gas volume S{sub A}/V{sub gas}, alveolar septal thickness h and capillary transit time {tau}, three critical determinants of the lung's primary role as a gas exchange organ. An analytical solution for a simplified diffusion model is described, together with a modification of the xenon transfer contrast imaging technique utilizing 90{sup 0} radio-frequency pulses applied to the dissolved phase, rather than traditional 180{sup 0} pulses. With this approach, three-dimensional (3D) maps of S{sub A}/V{sub gas} were obtained. We measured global S{sub A}/V{sub gas}, h and {tau} in four normal subjects, two subjects with mild interstitial lung disease (ILD) and two subjects with mild chronic obstructive pulmonary disease (COPD). In normals, S{sub A}/V{sub gas} decreased with increasing lung volume from {approx}320 to 80 cm{sup -1}; both h{approx}13 {mu}m and {tau}{approx}1.5 s were relatively constant. For the two ILD subjects, h was, respectively, 36 and 97% larger than normal, quantifying an increased gas/blood tissue barrier; S{sub A}/V{sub gas} and {tau} were normal. The two COPD subjects had S{sub A}/V{sub gas} values {approx}25% that of normals, quantifying septal surface loss in emphysema; h and {tau} were normal. These are the first noninvasive, non-radiation-based, quantitative measurements of h and {tau} in patients with pulmonary disease.

  9. (18)FDG uptake associated with CT density on PET/CT in lungs with and without chronic interstitial lung diseases.

    Inoue, Kentaro; Okada, Ken; Taki, Yasuyuki; Goto, Ryoi; Kinomura, Shigeo; Fukuda, Hiroshi


    The dependent-density of computed tomography (CT) images of positron emission tomography (PET)/CT is sometimes difficult to distinguish from chronic interstitial lung disease (ILD) when it accompanies increased (18)F-fluorodeoxy-D: -glucose ((18)FDG) uptake. Though the possible utility of (18)FDG-PET for the diagnosis of active ILD has been reported, the clinical relevance of mild lung (18)FDG uptake in ILD cases without signs and symptoms suggesting acute progression has not been described. This study aimed to test relationships between (18)FDG uptake and lung density on CT using PET/CT in patients with normal lung as well as clinically stable chronic ILD. Thirty-six patients with normal lungs (controls) and 28 patients with chronic ILD (ILD cases) without acute exacerbation were retrospectively selected from (18)FDG-PET/CT scans performed in examination of malignant neoplasms. Elliptical regions of interest (ROIs) were placed on the lung. The relationships between CT density and (18)FDG uptake between the control and ILD cases were tested. The CT density and (18)FDG uptake had a linear correlation in both the controls and the ILD cases without a difference in their regression slopes, and they were overlapped between the controls and the ILD cases with higher mean values in the ILD cases. Lung (18)FDG uptake was considered to reflect a gravity-dependent tissue density in the normal lung. Though the lung (18)FDG uptake as well as the CT density tended to be higher in chronic ILD patients, it may be difficult to distinguish them in normal dependent regions from those related to chronic ILD in some cases.

  10. Serum B cell-activating factor (BAFF) level in connective tissue disease associated interstitial lung disease.

    Hamada, Tsutomu; Samukawa, Takuya; Kumamoto, Tomohiro; Hatanaka, Kazuhito; Tsukuya, Go; Yamamoto, Masuki; Machida, Kentaro; Watanabe, Masaki; Mizuno, Keiko; Higashimoto, Ikkou; Inoue, Yoshikazu; Inoue, Hiromasa


    Interstitial lung diseases (ILDs) are common in patients with connective tissue diseases (CTDs). Although the diagnosis of an underlying CTD in ILD (CTD-ILD) affects both prognosis and treatment, it is sometimes difficult to distinguish CTD-ILD from chronic fibrosing interstitial pneumonia (CFIP). B cell-activating factor belonging to the tumour necrosis factor family (BAFF) plays a crucial role in B cell development, survival, and antibody production. We examined serum levels of BAFF, surfactant protein D (SP-D), and Krebs von den Lungen-6 (KL-6) in 33 patients with CTD-ILD, 16 patients with undifferentiated CTD-ILD, 19 patients with CFIP, and 26 healthy volunteers. And we analysed the relationship between serum BAFF levels and pulmonary function, as well as the expression of BAFF in the lung tissue of patients with CTD-ILD. Serum levels of BAFF were significantly higher in CTD-ILD patients compared to healthy subjects and CFIP patients. However, there were no significant differences in serum levels of SP-D and KL-6. Furthermore, serum BAFF levels in CTD-ILD patients were inversely correlated with pulmonary function. BAFF was strongly expressed in the lungs of CTD-ILD patients, but weakly in normal lungs. This is the first study to demonstrate that serum BAFF levels were significantly higher in CTD-ILD patients compared to healthy subjects and CFIP patients. Furthermore, serum BAFF levels were correlated with pulmonary function. We consider that serum BAFF levels in patients with CTD-ILD reflect the presence of ILDs disease activity and severity. These finding suggest that BAFF may be a useful marker for distinguishing CTD-ILD from CFIP.

  11. Sex-specific lung diseases: effect of oestrogen on cultured cells and in animal models

    Bosung Shim


    Full Text Available Sex prevalence in lung disease suggests that sex-specific hormones may contribute to the pathogenesis and/or progression of at least some lung diseases, such as lung adenocarcinoma, lymphangioleiomyomatosis (LAM and benign metastasising leiomyoma (BML. Oestrogen is an important hormone in normal lung development and in the pathogenesis of female predominant pulmonary diseases. In vivo and in vitro studies have facilitated our understanding of disease pathogenesis and discovery of potential therapeutic targets. Oestrogen promoted disease progression in cell and animal models of lung adenocarcinoma, LAM and BML. Specifically, oestrogen enhanced tumour growth and metastasis in animal models of these diseases. Furthermore, 17β-estradiol (E2, the most abundant form of oestrogen in humans, increased the size and proliferation of cultured cells of lung adenocarcinoma and LAM. Coupled with the known mechanisms of oestrogen metabolism and signalling, these model systems may provide insights into the diverse effects of oestrogen and other hormones on lung diseases. Anti-oestrogen treatments that target key events of oestrogen synthesis or signalling, such as aromatase activity, oestrogen receptors and signalling pathways, may offer additional opportunities for clinical trials.

  12. Aging and Lung Disease. Clinical Impact and Cellular and Molecular Pathways.

    Rojas, Mauricio; Mora, Ana L; Kapetanaki, Maria; Weathington, Nathaniel; Gladwin, Mark; Eickelberg, Oliver


    With the expected rapid growth of the aging population worldwide, there is a clear need to understand the complex process of aging to develop interventions that might extend the health span in this group of patients. Aging is associated with increased susceptibility to a variety of chronic diseases, and lung pathologies are no exception. The prevalence of lung diseases such as idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease has been found to increase considerably with age. In October 2014, the Division of Pulmonary, Allergy, and Critical Care of the University of Pittsburgh cohosted the Pittsburgh-Munich Lung Conference focused in aging and lung disease with the Comprehensive Pneumology Center, Institute of Lung Biology and Disease, Ludwig-Maximilians University and Helmholtz Zentrum Munich Germany. The purpose of the conference was to disseminate novel concepts in aging mechanisms that have an impact in lung physiology and pathogenesis of pulmonary diseases that commonly occur in older populations. The conference included 28 presentations on diverse topics, which are summarized in this report. The participants identified priorities for future basic and translational investigations that will assist in the identification of molecular insights involved in the pathogenesis of age-related pulmonary diseases and the design of therapeutic interventions for these lung conditions.

  13. The potencial role of rare earths in the pathogenesis of interstitial lung disease: a case report of movie projectionist as investigated by neutron activation analysis.

    Porru, S; Placidi, D; Quarta, C; Sabbioni, E; Pietra, R; Fortaner, S


    A 60-year-old male subject who worked as a movie projectionist and who was exposed for 12 years to rare earths (RE) containing dusts from cored arc light carbon electrodes was investigated. Chest X-ray films and pulmonary function tests showed an interstitial lung disease, emphysema and a severe obstructive impairment with marked decrease of carbon monoxide diffusion capacity. The histological examination of a transbronchial biopsy confirmed the diffuse interstitial lung fibrosis. Neutron activation analysis (NAA) of the biopsy showed concentrations of cerium (Ce), lanthanum (La), neodimium (Nd), samarium (Sm), terbium (Tb) and ytterbium (Yb) which were high compared to the corresponding elements in the transbronchial biopsies of 5 unexposed subjects as a control group. Thorium (Th) (which is generally present as an impurity of the RE compounds) was also determined in order to estimate the radiation dose in the lung of the worker. On the basis of the clinical observations, of the analytical results by neutron activation analysis of RE and of the presence of Th in the transbronchial biopsy, as well as of the differential diagnosis, which tended to exclude other occupational or non-occupational lung diseases, a relation between the observed interstitial lung fibrosis and occupational exposure to RE is highly probable.

  14. [Continuous positive airway pressure and high-frequency independent lung ventilation in patients with chronic obstructive lung diseases].

    Fedorova, E A; Vyzhigina, M A; Gal'perin, Iu S; Zhukova, S G; Titov, V A; Godin, A V


    The original hypoxemia, hypercapnia, high pulmonary hypertension, high resistance of microcirculation vessels, right volumetric ventricular overload, persistent sub-edema of pulmonary intersticium as well as disparity of ventilation and perfusion between both lungs are the main problems in patients with chronic obstructive disease of the lungs (CODL). Such patients are, as a rule, intolerant to the independent lung collaboration or artificial single-stage ventilation (ASV). Patients with respiratory insufficiency, stages 2 and 3, and with a pronounced impaired type of ventilation have originally a deranged blood gas composition, like hypoxemia or hypercapnia. The application of volume-controllable bi-pulmonary ASV in such patients maintains an adequate gas exchange hemodynamics. However, ASV is accompanied by a significantly reduced gas-exchange function of the single ventilated lung and by essentially worsened intrapulmonary hemodynamics. Therefore, what is needed is to use alternative methods of independent lung ventilation in order to eliminate the gas-exchange impairments and to enable surgical interventions at thoracic organs in such patients (who are intolerant to ASV). A choice of a method and means of oxygen supply to the independent lung is of great importance. The possibility to avoid a high pressure in the airways, while maintaining, simultaneously, an adequate gas exchange, makes the method related with maintaining a constant positive pressure in the airways (CPPA) a priority one in case of CODL patients. The use of constant high-frequency ventilation in the independent lung in patients with obstructive pulmonary lesions does not improve the gas exchange or hemodynamics. Simultaneously, a growing total pulmonary resistance and an increasing pressure in the pulmonary artery are observed. Consequently, the discussed method must not be used for the ventilation support of the independent lung in patients with the obstructive type of the impaired external

  15. Diffusion tensor imaging for Alzheimer's disease: A review of concepts and potential clinical applicability

    Luciano de Gois Vasconcelos; Sonia Maria Dozzi Brucki; Andrea Parolin Jackowiski; Orlando Francisco Amodeo Bueno


    Abstract In view of the urgent need to identify an early and specific biomarker for Alzheimer's disease (AD), a PubMed database search was performed using the terms "Alzheimer disease" and "Diffusion Magnetic Resonance Imaging" to enable review of Diffusion tensor imaging (DTI) concepts and its potential clinical role in AD evaluation. Detailed analysis of selected abstracts showed that the main DTI measures, fractional anisotropy and apparent diffusion coefficient, indicators of fiber tract ...

  16. Chronic obstructive lung disease and posttraumatic stress disorder: current perspectives

    Abrams TE


    Full Text Available Thad E Abrams,1,2 Amy Blevins,1,3 Mark W Vander Weg1,2,4 1Department of Internal Medicine, University of Iowa, 2Center for Comprehensive Access and Delivery Research and Evaluation, Iowa City VA Health Care System, 3Hardin Health Sciences Library, 4Department of Psychological and Brain Sciences, University of Iowa, Iowa City, IA, USA Background: Several studies have reported on the co-occurrence of chronic obstructive pulmonary disease (COPD and psychiatric conditions, with the most robust evidence base demonstrating an impact of comorbid anxiety and depression on COPD-related outcomes. In recent years, research has sought to determine if there is a co-occurrence between COPD and posttraumatic stress disorder (PTSD as well as for associations between PTSD and COPD-related outcomes. To date, there have been no published reviews summarizing this emerging literature.Objectives: The primary objective of this review was to determine if there is adequate evidence to support a co-occurrence between PTSD and COPD. Secondary objectives were to: 1 determine if there are important clinical considerations regarding the impact of PTSD on COPD management, and 2 identify targeted areas for further research.Methods: A structured review was performed using a systematic search strategy limited to studies in English, addressing adults, and to articles that examined: 1 the co-occurrence of COPD and PTSD and 2 the impact of PTSD on COPD-related outcomes. To be included, articles must have addressed some type of nonreversible obstructive lung pathology.Results: A total of 598 articles were identified for initial review. Upon applying the inclusion and exclusion criteria, n=19 articles or abstracts addressed our stated objectives. Overall, there is inconclusive evidence to support the co-occurrence between PTSD and COPD. Studies finding a significant co-occurrence generally had inferior methods of identifying COPD; in contrast, studies that utilized more robust COPD

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

    Sfriso Paolo


    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.

  18. Discrimination of periodontal diseases using diffuse reflectance spectral intensity ratios

    Chandra Sekhar, Prasanth; Betsy, Joseph; Presanthila, Janam; Subhash, Narayanan


    This clinical study was to demonstrate the applicability of diffuse reflectance (DR) intensity ratio R620/R575 in the quantification and discrimination of periodontitis and gingivitis from healthy gingiva. DR spectral measurements were carried out with white-light illumination from 70 healthy sites in 30 healthy volunteers, and 63 gingivitis- and 58 periodontitis-infected sites in 60 patients. Clinical parameters such as probing pocket depth, clinical attachment level, and gingival index were recorded in patient population. Diagnostic accuracies for discrimination of gingivitis and periodontitis from healthy gingiva were determined by comparison of spectral signatures with clinical parameters. Divergence of average DR spectral intensity ratio between control and test groups was studied using analysis of variance. The mean DR spectrum on normalization at 620 nm showed marked differences between healthy tissue, gingivitis, and periodontitis. Hemoglobin concentration and apparent SO2 (oxygen saturation) were also calculated for healthy, gingivitis, and periodontitis sites. DR spectral intensities at 545 and 575 nm showed a decreasing trend with progression of disease. Among the various DR intensity ratios studied, the R620/R575 ratio provided a sensitivity of 90% and specificity of 94% for discrimination of healthy tissues from gingivitis and a sensitivity of 91% and specificity of 100% for discrimination of gingivitis from periodontitis.

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

    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


    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.

  20. Periodontal Disease and Incident Lung Cancer Risk: A Meta-Analysis of Cohort Studies.

    Zeng, Xian-Tao; Xia, Ling-Yun; Zhang, Yong-Gang; Li, Sheng; Leng, Wei-Dong; Kwong, Joey S W


    Periodontal disease is linked to a number of systemic diseases such as cardiovascular diseases and diabetes mellitus. Recent evidence has suggested periodontal disease might be associated with lung cancer. However, their precise relationship is yet to be explored. Hence, this study aims to investigate the association of periodontal disease and risk of incident lung cancer using a meta-analytic approach. PubMed, Scopus, and ScienceDirect were searched up to June 10, 2015. Cohort and nested case-control studies investigating risk of lung cancer in patients with periodontal disease were included. Hazard ratios (HRs) were calculated, as were their 95% confidence intervals (CIs) using a fixed-effect inverse-variance model. Statistical heterogeneity was explored using the Q test as well as the I(2) statistic. Publication bias was assessed by visual inspection of funnel plots symmetry and Egger's test. Five cohort studies were included, involving 321,420 participants in this meta-analysis. Summary estimates based on adjusted data showed that periodontal disease was associated with a significant risk of lung cancer (HR = 1.24, 95% CI = 1.13 to 1.36; I(2) = 30%). No publication bias was detected. Subgroup analysis indicated that the association of periodontal disease and lung cancer remained significant in the female population. Evidence from cohort studies suggests that patients with periodontal disease are at increased risk of developing lung cancer.

  1. The association between combined non-cystic fibrosis bronchiectasis and lung cancer in patients with chronic obstructive lung disease

    Kim YW


    Full Text Available Yeon Wook Kim,1 Kwang-Nam Jin,2 Eun Young Heo,3 Sung Soo Park,3 Hee Soon Chung,3 Deog Kyeom Kim31Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea; 2Department of Radiology, Seoul National University College of Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Republic of Korea; 3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Republic of KoreaBackground: Whereas the epidemiological association between lung cancer and chronic obstructive pulmonary disease (COPD, a chronic inflammatory respiratory disease, is well known, limited studies have examined the association between lung cancer and non-cystic fibrosis bronchiectasis, a representative chronic airway inflammatory disease. This study evaluated the association between bronchiectasis and lung cancer in patients with COPD.Methods: A matched case–control study was conducted in a referral hospital in South Korea. Among COPD patients with moderate to very severe airflow limitation (forced expiratory volume in one second/forced vital capacity <0.7 and forced expiratory volume in one second ≤70% [% predicted] who underwent chest computed tomography (CT between January 1, 2010 and May 30, 2013, patients with lung cancer and controls matched for age, sex, and smoking history were selected. The risk of lung cancer was assessed according to the presence of underlying bronchiectasis confirmed by chest CT.Results: The study enrolled 99 cases and 198 controls. Combined bronchiectasis on chest CT was inversely associated with the risk of lung cancer compared with controls (odds ratio [OR] 0.25, 95% confidence interval [CI] 0.12–0.52, P<0.001. Significant associations were found in

  2. Nontuberculous Mycobacterial Disease Is Not a Contraindication to Lung Transplantation in Patients With Cystic Fibrosis

    Qvist, Tavs; Pressler, Tanja; Thomsen, V O;


    Whether nontuberculous mycobacterial (NTM) disease is a contraindication to lung transplantation remains controversial. We conducted a nationwide study to evaluate the clinical importance of NTM infection among lung transplant patients with cystic fibrosis (CF) in Denmark and to determine if NTM...

  3. Identification of Chronic Obstructive Pulmonary Disease in Lung Cancer Screening Computed Tomographic Scans

    Mets, Onno M.; Buckens, Constantinus F. M.; Zanen, Pieter; Isgum, Ivana; van Ginneken, Bram; Prokop, Mathias; Gietema, Hester A.; Lammers, Jan-Willem J.; Vliegenthart, Rozemarijn; Oudkerk, Matthijs; van Klaveren, Rob J.; de Koning, Harry J.; Mali, Willem P. Th M.; de Jong, Pim A.


    Context Smoking is a major risk factor for both cancer and chronic obstructive pulmonary disease (COPD). Computed tomography (CT)-based lung cancer screening may provide an opportunity to detect additional individuals with COPD at an early stage. Objective To determine whether low-dose lung cancer

  4. Identification of chronic obstructive pulmonary disease in lung cancer screening computed tomographic scans

    Mets, O.M.; Buckens, C.F.; Zanen, P.; Isgum, I.; Ginneken, B. van; Prokop, M.; Gietema, H.A.; Lammers, J.W.; Vliegenthart, R.; Oudkerk, M.; Klaveren, R.J. van; Koning, H.J. de; Mali, W.P.Th.; Jong, P.A. de


    CONTEXT: Smoking is a major risk factor for both cancer and chronic obstructive pulmonary disease (COPD). Computed tomography (CT)-based lung cancer screening may provide an opportunity to detect additional individuals with COPD at an early stage. OBJECTIVE: To determine whether low-dose lung cancer

  5. Cross-sectional Assessment of Daily Physical Activity in Chronic Obstructive Pulmonary Disease Lung Transplant Patients

    Bossenbrock, Linda; ten Thicken, Nick H. T.; van der Bij, Wim; Verschuuren, Erik A. M.; Koeter, Gerard H.; de Greef, Mathieu H. G.

    Background: information about daily physical activity of chronic obstructive pulmonary disease (COPD) lung transplant patients is relevant for evaluation of the functional recovery of physical capacity after lung transplantation. The objective of this study was to cross-sectionally assess daily

  6. Epidemiological studies of the relationship between occupational exposures and chronic non-specific lung disease.

    Heederik, D.


    In this thesis the relationship between occupational exposures, lung function and Chronic Non-Specific Lung Disease is studied. The study comprises an epidemiological analysis of data from the British Pneumoconiosis Field Research among coal miners and an analysis of data gathered in the Zutphen

  7. Transient early wheeze and lung function in early childhood associated with chronic obstructive pulmonary disease genes

    Kerkhof, Marjan; Boezen, Hendrika; Granell, Raquel; Wijga, Alet H.; Brunekreef, Bert; Smit, Henriette A.; de Jongste, Johan C.; Thijs, Carel; Mommers, Monique; Penders, John; Henderson, John; Koppelman, Gerard H.; Postma, Dirkje S.


    Background: It has been hypothesized that a disturbed early lung development underlies the susceptibility to chronic obstructive pulmonary disease (COPD). Little is known about whether subjects genetically predisposed to COPD show their first symptoms or reduced lung function in childhood. Objective

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

    Fuat Sayır


    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

  9. Metabolomics provide new insights on lung cancer staging and discrimination from chronic obstructive pulmonary disease.

    Deja, Stanislaw; Porebska, Irena; Kowal, Aneta; Zabek, Adam; Barg, Wojciech; Pawelczyk, Konrad; Stanimirova, Ivana; Daszykowski, Michal; Korzeniewska, Anna; Jankowska, Renata; Mlynarz, Piotr


    Chronic obstructive pulmonary disease (COPD) and lung cancer are widespread lung diseases. Cigarette smoking is a high risk factor for both the diseases. COPD may increase the risk of developing lung cancer. Thus, it is crucial to be able to distinguish between these two pathological states, especially considering the early stages of lung cancer. Novel diagnostic and monitoring tools are required to properly determine lung cancer progression because this information directly impacts the type of the treatment prescribed. In this study, serum samples collected from 22 COPD and 77 lung cancer (TNM stages I, II, III, and IV) patients were analyzed. Then, a collection of NMR metabolic fingerprints was modeled using discriminant orthogonal partial least squares regression (OPLS-DA) and further interpreted by univariate statistics. The constructed discriminant models helped to successfully distinguish between the metabolic fingerprints of COPD and lung cancer patients (AUC training=0.972, AUC test=0.993), COPD and early lung cancer patients (AUC training=1.000, AUC test=1.000), and COPD and advanced lung cancer patients (AUC training=0.983, AUC test=1.000). Decreased acetate, citrate, and methanol levels together with the increased N-acetylated glycoproteins, leucine, lysine, mannose, choline, and lipid (CH3-(CH2)n-) levels were observed in all lung cancer patients compared with the COPD group. The evaluation of lung cancer progression was also successful using OPLS-DA (AUC training=0.811, AUC test=0.904). Based on the results, the following metabolite biomarkers may prove useful in distinguishing lung cancer states: isoleucine, acetoacetate, and creatine as well as the two NMR signals of N-acetylated glycoproteins and glycerol.

  10. Inhaler device preferences in older adults with chronic lung disease

    Ghazala L


    Full Text Available Introduction: Patient preferences are important for medication adherence and patient satisfaction, but little is known about older adult preferences for inhaler devices. Methods: We developed a 25-item written self-administered questionnaire assessing experience with inhalers, prior inhaler education, and preferences with respect to inhaler device features and inhaler device teaching. We then conducted a cross-sectional survey of patients at least 65 years of age with chronic lung disease who had experience using inhaler devices for at least six months in the ambulatory setting. Results: Fifty participants completed the questionnaire. The majority of participants (80% reported prior experience with a metered dose inhaler (MDI, but only 26% used an MDI with a spacer. Most patients (76% had received formal instruction regarding proper use of the inhaler, but only 34% had ever been asked to demonstrate their inhaler technique. Physician recommendation for an inhaler, cost of the inhaler device, and inhaler features related to convenience were important with respect to patient preferences. With regard to inhaler education, participants prefer verbal instruction and/or hands-on demonstration at the time a new inhaler is prescribed in the setting of the prescribing provider’s office. Conclusion: Patient preferences for inhaler devices and inhaler education among older adults indicate physician recommendation, cost, and convenience are important. The impact of patient preferences on inhaler adherence and clinical outcomes remains unknown.

  11. Rat models of asthma and chronic obstructive lung disease.

    Martin, James G; Tamaoka, Meiyo


    The rat has been extensively used to model asthma and somewhat less extensively to model chronic obstructive pulmonary disease (COPD). The features of asthma that have been successfully modeled include allergen-induced airway constriction, eosinophilic inflammation and allergen-induced airway hyperresponsiveness. T-cell involvement has been directly demonstrated using adoptive transfer techniques. Both CD4+ and CD8+ T cells are activated in response to allergen challenge in the sensitized rat and express Thelper2 cytokines (IL-4, IL-5 and IL-13). Repeated allergen exposure causes airway remodeling. Dry gas hyperpnea challenge also evokes increases in lung resistance, allowing exercise-induced asthma to be modeled. COPD is modeled using elastase-induced parenchymal injury to mimic emphysema. Cigarette smoke-induced airspace enlargement occurs but requires months of cigarette exposure. Inflammation and fibrosis of peripheral airways is an important aspect of COPD that is less well modeled. Novel approaches to the treatment of COPD have been reported including treatments aimed at parenchymal regeneration.

  12. Association of lung function genes with chronic obstructive pulmonary disease.

    Kim, Woo Jin; Lim, Myoung Nam; Hong, Yoonki; Silverman, Edwin K; Lee, Ji-Hyun; Jung, Bock Hyun; Ra, Seung Won; Choi, Hye Sook; Jung, Young Ju; Park, Yong Bum; Park, Myung Jae; Lee, Sei Won; Lee, Jae Seung; Oh, Yeon-Mok; Lee, Sang Do


    Spirometric measurements of pulmonary function are important in diagnosing and determining the severity of chronic obstructive pulmonary disease (COPD). We performed this study to determine whether candidate genes identified in genome-wide association studies of spirometric measurements were associated with COPD and if they interacted with smoking intensity. The current analysis included 1,000 COPD subjects and 1,000 controls recruited from 24 hospital-based pulmonary clinics. Thirteen SNPs, chosen based on genome-wide association studies of spirometric measurements in the Korean population cohorts, were genotyped. Genetic association tests were performed, adjusting for age, sex, and smoking intensity, using models including a SNP-by-smoking interaction term. PID1 and FAM13A were significantly associated with COPD susceptibility. There were also significant interactions between SNPs in ACN9 and FAM13A and smoking pack-years, and an association of ACN9 with COPD in the lowest smoking tertile. The risk allele of FAM13A was associated with increased expression of FAM13A in the lung. We have validated associations of FAM13A and PID1 with COPD. ACN9 showed significant interaction with smoking and is a potential candidate gene for COPD. Significant associations of genetic variants of FAM13A with gene expression levels suggest that the associated loci may act as genetic regulatory elements for FAM13A gene expression.

  13. Ethics and decision making in end stage lung disease.

    Simonds, A K


    Most physicians believe they do more good than harm, and these duties of helping and not harming the patient are rooted in the Hippocratic oath, the good Samaritan tradition, and the Order of the Knight Hospitallers founded in the 11th century to care for pilgrims and those wounded in the Crusades.(1) In recent times the simple principles of beneficence and non-maleficence have been augmented and sometimes challenged by a rising awareness of patient/consumer rights, and the public expectation of greater involvement in medical, social and scientific affairs which affect them. In a publicly funded healthcare system in which rationing (explicit or otherwise) is inevitable, the additional concepts of utility and distributive justice can easily come into conflict with the individual's right to autonomy. Possible treatment options for end stage lung disease include transplantation and long term invasive ventilation which are challenging in resource terms. Other interventions such as pulmonary rehabilitation and palliative care are relatively low cost but not uniformly accessible.

  14. Occupational lung diseases: from old and novel exposures to effective preventive strategies.

    Cullinan, Paul; Muñoz, Xavier; Suojalehto, Hille; Agius, Raymond; Jindal, Surinder; Sigsgaard, Torben; Blomberg, Anders; Charpin, Denis; Annesi-Maesano, Isabella; Gulati, Mridu; Kim, Yangho; Frank, Arthur L; Akgün, Metin; Fishwick, David; de la Hoz, Rafael E; Moitra, Subhabrata


    Occupational exposure is an important, global cause of respiratory disease. Unlike many other non-communicable lung diseases, the proximal causes of many occupational lung diseases are well understood and they should be amenable to control with use of established and effective approaches. Therefore, the risks arising from exposure to silica and asbestos are well known, as are the means of their prevention. Although the incidence of occupational lung disease has decreased in many countries, in parts of the world undergoing rapid economic transition and population growth-often with large informal and unregulated workforces-occupational exposures continue to impose a heavy burden of disease. The incidence of interstitial and malignant lung diseases remains unacceptably high because control measures are not implemented or exposures arise in novel ways. With the advent of innovative technologies, new threats are continually introduced to the workplace (eg, indium compounds and vicinal diketones). In developed countries, work-related asthma is the commonest occupational lung disease of short latency. Although generic control measures to reduce the risk of developing or exacerbating asthma are well recognised, there is still uncertainty, for example, with regards to the management of workers who develop asthma but remain in the same job. In this Review, we provide recommendations for research, surveillance, and other action for reducing the burden of occupational lung diseases.

  15. Lung MRI and impairment of diaphragmatic function in Pompe disease

    Wens, Stephan C A; Ciet, Pierluigi; Perez-Rovira, Adria;


    manually segmented. After normalization for lung size, changes in lung dimensions between inspiration and expiration were used for analysis; normalization was based on the cranial-caudal length ratio (representing vertical diaphragmatic displacement), and the anterior-posterior and left-right length ratios...

  16. Gastro-oesophageal reflux disease and non-asthma lung disease

    R. S. Morehead


    Full Text Available Gastro-oesophageal reflux disease (GERD is a common disorder in Western countries, and its relationship to airways disorders (e.g. asthma has been well established. Lung diseases other than asthma have also been associated with GERD, but the nature and scope of this relationship has not been fully defined. Diseases that have been associated with GERD include bronchiolitis syndromes, idiopathic pulmonary fibrosis, scleroderma and nontubercular mycobacterial infection. Diagnostic evaluation centres upon proving both reflux and pulmonary aspiration, which may be accomplished in some cases by lung biopsy. However, in many cases a compatible clinical and radiographic picture coupled with proof of proximal reflux by combined oesophageal probe testing may suffice for a provisional diagnosis and allow institution of anti-reflux measures. Proton-pump inhibitors are the medications of choice for GERD; other interventions shown to reduce reflux are weight loss, elevation of the head of the bed and avoidance of recumbency after meals. However, acid suppression therapy does not address non-acid reflux that may be important in disease pathogenesis in select patients, and lifestyle modifications often fail. Laparoscopic fundoplication is the procedure of choice for medically refractory GERD with excellent short-term results with respect to respiratory symptoms associated with GERD; however, long-term studies document a significant percentage of patients requiring ongoing acid suppression therapy.

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

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


    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.

  18. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy.

    Kurland, Geoffrey; Deterding, Robin R; Hagood, James S; Young, Lisa R; Brody, Alan S; Castile, Robert G; Dell, Sharon; Fan, Leland L; Hamvas, Aaron; Hilman, Bettina C; Langston, Claire; Nogee, Lawrence M; Redding, Gregory J


    There is growing recognition and understanding of the entities that cause interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD in older children and adults. A multidisciplinary panel was convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants under 2 years of age. Recommendations were formulated using a systematic approach. Outcomes considered important included the accuracy of the diagnostic evaluation, complications of delayed or incorrect diagnosis, psychosocial complications affecting the patient's or family's quality of life, and death. No controlled clinical trials were identified. Therefore, observational evidence and clinical experience informed judgments. These guidelines: (1) describe the clinical characteristics of neonates and infants (<2 yr of age) with diffuse lung disease (DLD); (2) list the common causes of DLD that should be eliminated during the evaluation of neonates and infants with DLD; (3) recommend methods for further clinical investigation of the remaining infants, who are regarded as having "childhood ILD syndrome"; (4) describe a new pathologic classification scheme of DLD in infants; (5) outline supportive and continuing care; and (6) suggest areas for future research. After common causes of DLD are excluded, neonates and infants with childhood ILD syndrome should be evaluated by a knowledgeable subspecialist. The evaluation may include echocardiography, controlled ventilation high-resolution computed tomography, infant pulmonary function testing, bronchoscopy with bronchoalveolar lavage, genetic testing, and/or lung biopsy. Preventive care, family education, and support are essential.

  19. Elemental analysis of occupational and environmental lung diseases by electron probe microanalyzer with wavelength dispersive spectrometer.

    Takada, Toshinori; Moriyama, Hiroshi; Suzuki, Eiichi


    Occupational and environmental lung diseases are a group of pulmonary disorders caused by inhalation of harmful particles, mists, vapors or gases. Mineralogical analysis is not generally required in the diagnosis of most cases of these diseases. Apart from minerals that are encountered rarely or only in specific occupations, small quantities of mineral dusts are present in the healthy lung. As such when mineralogical analysis is required, quantitative or semi-quantitative methods must be employed. An electron probe microanalyzer with wavelength dispersive spectrometer (EPMA-WDS) enables analysis of human lung tissue for deposits of elements by both qualitative and semi-quantitative methods. Since 1993, we have analyzed 162 cases of suspected occupational and environmental lung diseases using an EPMA-WDS. Our institute has been accepting online requests for elemental analysis of lung tissue samples by EPMA-WDS since January 2011. Hard metal lung disease is an occupational interstitial lung disease that primarily affects workers exposed to the dust of tungsten carbide. The characteristic pathological findings of the disease are giant cell interstitial pneumonia (GIP) with centrilobular fibrosis, surrounded by mild alveolitis with giant cells within the alveolar space. EPMA-WDS analysis of biopsied lung tissue from patients with GIP has demonstrated that tungsten and/or cobalt is distributed in the giant cells and centrilobular fibrosing lesion in GIP. Pneumoconiosis, caused by amorphous silica, and acute interstitial pneumonia, associated with the giant tsunami, were also elementally analyzed by EPMA-WDS. The results suggest that commonly found elements, such as silicon, aluminum, and iron, may cause occupational and environmental lung diseases.

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

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


    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.

  1. [Humidifier lung].

    Gerber, P; de Haller, R; Pyrozynski, W J; Sturzenegger, E R; Brändli, O


    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.

  2. Pulmonary lymphangioleiomyomatosis: Analysis of disease manifestation by region-based quantification of lung parenchyma

    Theilig, D., E-mail: [Charité, Universitätsmedizin Berlin, Department of Radiology, Charité Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin (Germany); Doellinger, F. [Charité, Universitätsmedizin Berlin, Department of Radiology, Charité Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin (Germany); Kuhnigk, J.M. [Fraunhofer MEVIS, Universitaetsallee 29, 28359 Bremen (Germany); Temmesfeld-Wollbrueck, B.; Huebner, R.H. [Charité, Department of Pneumology, Augustenburger Platz 1, 13353 Berlin (Germany); Schreiter, N.; Poellinger, A. [Charité, Universitätsmedizin Berlin, Department of Radiology, Charité Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin (Germany)


    Highlights: •The distribution of cystic lesions in LAM was evaluated with quantitative CT. •There were more cystic lesions in the central lung compared to peripheral areas. •Cystic changes were more frequent in apical two thirds compared to lower third. •Results might help to obviate the need for biopsy in more cases. -- Abstract: Purpose: Lymphangioleiomyomatosis (LAM) is characterized by proliferation of smooth muscle tissue that causes bronchial obstruction and secondary cystic destruction of lung parenchyma. The aim of this study was to evaluate the typical distribution of cystic defects in LAM with quantitative volumetric chest computed tomography (CT). Materials and methods: CT examinations of 20 patients with confirmed LAM were evaluated with region-based quantification of lung parenchyma. Additionally, 10 consecutive patients were identified who had recently undergone CT imaging of the lung at our institution, in which no pathologies of the lung were found, to serve as a control group. Each lung was divided into three regions (upper, middle and lower thirds) with identical number of slices. In addition, we defined a “peel” and “core” of the lung comprising the 2 cm subpleural space and the remaining inner lung area. Computerized detection of lung volume and relative emphysema was performed with the PULMO 3D software (v3.42, Fraunhofer MEVIS, Bremen, Germany). This software package enables the quantification of emphysematous lung parenchyma by calculating the pixel index, which is defined as the ratio of lung voxels with a density <−950 HU to the total number of voxels in the lung. Results: Cystic changes accounted for 0.1–39.1% of the total lung volume in patients with LAM. Disease manifestation in the central lung was significantly higher than in peripheral areas (peel median: 15.1%, core median: 20.5%; p = 0.001). Lower thirds of lung parenchyma showed significantly less cystic changes than upper and middle lung areas combined (lower

  3. Respiratory bronchiolitis-associated interstitial lung disease secondary to electronic nicotine delivery system use confirmed with open lung biopsy.

    Flower, Mark; Nandakumar, Lakshmy; Singh, Mahendra; Wyld, David; Windsor, Morgan; Fielding, David


    As a modern phenomenon, there is currently limited understanding of the possible toxic effects and broader implications of electronic nicotine delivery systems (ENDS). Large volumes of aerosolized particles are inhaled during "vaping" and there are now an increasing number of case reports demonstrating toxic effects of ENDS, as well as human studies demonstrating impaired lung function in users. This article presents a case of respiratory bronchiolitis interstitial lung disease (RB-ILD) precipitated by vaping in a 33-year-old male with 10 pack years of traditional cigarette and prior treatment for mixed germ cell tumour. The patient had started vaping 10-15 times per day while continuing to smoke 10 traditional cigarettes per day. After 3 months of exposure to e-cigarette vapour, chest computed tomography demonstrated multiple new poorly defined pulmonary nodules with fluffy parenchyma opacification centred along the terminal bronchovascular units. Video-assisted thoracoscopy with lung biopsy of the right upper and right middle lobes was undertaken. The microscopic findings were overall consistent with RB-ILD. This case demonstrates toxicity with use of ENDS on open lung biopsy with resolution of radiographic findings on cessation. We believe that this is the first case where open lung biopsy has demonstrated this and our findings are consistent with RB-ILD.

  4. Health outcomes associated with lung function decline and respiratory symptoms and disease in a community cohort

    Baughman, Penelope; Marott, Jacob L; Lange, Peter


    BACKGROUND: In workplace respiratory disease prevention, a thorough understanding is needed of the relative contributions of lung function loss and respiratory symptoms in predicting adverse health outcomes. METHODS: Copenhagen City Heart Study respiratory data collected at 4 examinations (1976...




    Background Short term treatment corticosteroids does not usually reduce airflow limitation and airway responsiveness in patients with chronic obstructive lung disease. We investigated whether corticosteroids modulate the effects of inhaled salbutamol and ipratropium bromide. Methods Ten non-allergic

  6. Association between chronic obstructive pulmonary disease and lung cancer: the missing link

    WANG Zeng-li


    Objective This review focuses on current knowledge of specific processes that drive chronic airway inflammation which are important in the pathogenesis of both chronic obstructive pulmonary disease (COPD) and lung cancer.Data sources The data used in this review were obtained mainly from studies reported in the PubMed database (1997-2012) using the terms of COPD and lung cancer.Study selection Data from published articles about prevalence of COPD-lung cancer overlap and mechanism involved in lung cancer development in COPD were identified,retrieved and reviewed.Results COPD prevalence,morbidity and mortality vary and are directly related to the prevalence of tobacco smoking except in developing countries where air pollution resulting from the burning of biomass fuels is also important.COPD is characterized by a chronic inflammation of lower airway and,importantly,the presence of COPD increases the risk of lung cancer up to 4.5 fold among long-term smokers.COPD is by far the greatest risk factor for lung cancer amongst smokers and is found in 50%-90% of patients with lung cancer.Conclusions Both COPD and lung cancer are tobacco smoking-associated chronic diseases that cluster in families and aggravate with age,and 50%-70% of patients diagnosed with lung cancer have declined spirometric evidence of COPD.Understanding and targeting common pathogenic mechanisms for lung cancer and COPD would have potential diagnostic and therapeutic implications for patients with these lung diseases and for people at risk.

  7. Magnetic resonance diffusion-weighted imaging in the diagnosis of diffuse liver diseases in rats

    GUAN Sheng; ZHOU Kang-rong; ZHAO Wei-dong; PENG Wei-jun; TANG Feng; MAO Jian


    Background The diagnosis of diffuse hepatic lesions in early stage is a tough task at any time for clinical conventional imaging Magnetic resonance diffusion-weighted imaging (MR DWI) can detect the changes of tissue structure at molecular level This study was designed to determine the value of DWI in the diagnosis of diffuse liver lesions in early stage.Methods Diffuse liver lesions were induced by diethylnitrosamine in 42 rats of test group. Fourteen rats in control group were fed with pure water. Dynamic changes of MR DWI were observed every week in both groups during the early stage of diffuse liver lesions (1 to 12 weeks after drug administration in the test group). Apparent diffusion coefficient (ADC) values of liver parenchyma in different stages and pathologic changes were analyzed.Results The process of diffuse hepatic lesions in the test group was classified into three stages according to pathological changes, namely hepatitis, hepatic fibrosis and cirrhosis. No obvious morphological changes were shown by conventional imaging in both groups during this stage. But MR DWI demonstrated heterogeneous signal changes in early stage of hepatic cirrhosis in the test group. No significant change of ADC values was found in the control group between different weeks (P>0.05). The ADC values of the test group declined from the fifth week, and after the tenth week the ADC values were significantly different between the test and control groups at gradient factor (b) value 300 sec/mm2 (P<0.05). At b value 600 and 1000 sec/mm2, significant difference was seen between the two groups from the sixth week onward. The range of ADC value of the groups was (1.7-0.9)±(0.40-0.04) mm2/sec (b=600) and (1.38-0.75)±(0.07-0.35) mm2/sec (b=1000), respectively. Dominant pathological changes included swelled hepatocytes within 1 to 4 weeks after the administration of diethylnitrosamine in the test group, hyperplasia of fibrous tissues in 5-8 weeks and formation of cirrhotic nodules in 9

  8. Monte-Carlo-Model for the aerosol bolus dispersion in the human lung. Part 2. Model predictions for the diseased lung; Monte-Carlo-Modell der Aerosolbolusdispersion in der menschlichen Lunge. Teil 2. Modellvorhersagen fuer die kranke Lunge

    Sturm, R.; Pawlak, E.; Hofmann, W. [Salzburg Univ. (Austria). Abt. fuer Physik und Biophysik


    After a mathematical extension of the existing model for the theoretical description of the aerosol bolus dispersion, the behavior of particle pulses in diseased lung structures was simulated. The geometry used for healthy lungs was modified in two aspects: First, a modelling of possible airway obstructions, which usually occur in patients with chronic bronchitis, chronic asthma or cystic fibrosis, was carried out and, second, a theoretical approximation of the emphysema, being observed in lungs of smokers, but also as an accompanying phenomenon in obstructive diseases, was established. According to the modified model, in lungs with airway obstructions the exhaled bolus exhibited a decreased dispersion with respect to healthy subjects, whereas in emphysematous lungs the respective half-width of the peak was increased. Standard deviation and skewness of the bolus were similarly influenced by the modified lung architecture. A combination of airway obstruction and emphysema caused an extensive compensation of individual dispersion effects, complicating a secure distinction from the healthy lung. According to the model, a special diagnostic value may be assigned to the bolus deposition, showing significant deviations from the normal case for all simulated diseases. (orig.)

  9. Effect of metabolic alkalosis on respiratory function in patients with chronic obstructive lung disease.

    Bear, R.; Goldstein, M.; Phillipson, E.; Ho, M.; Hammeke, M.; Feldman, R.; Handelsman, S.; Halperin, M.


    Eleven instances of a mixed acid-base disorder consisting of chronic respiratory acidosis and metabolic alkalosis were recognized in eight patients with chronic obstructive lung disease and carbon dioxide retention. Correction of the metabolic alkalosis led to substantial improvement in blood gas values and clinical symptoms. Patients with mixed chronic respiratory acidosis and metabolic alkalosis constitute a common subgroup of patients with chronic obstructive lung disease and carbon dioxide retention; these patients benefit from correction of the metabolic alkalosis. PMID:21028

  10. Diffuse reflectance spectroscopy: a new guidance tool for improvement of biopsy procedures in lung malignancies

    Evers, Daniel; Evers, D.J.; Nachabe, R.; Klomp, H.M.; van Sandick, J.W.; Wouters, M.W.; Lucassen, G.W.; Lucassen, Gerald; Hendriks, B.H.; Wesseling, J.; Ruers, Theo J.M.


    Background: A significant number of percutaneous intrathoracic biopsy procedures result in indeterminate cytologic or histologic diagnosis in clinical practice. Diffuse reflectance spectroscopy (DRS) is an optical technique that can distinguish different tissue types on a microscopic level. DRS may

  11. Diffuse reflectance spectroscopy: a new guidance tool for improvement of biopsy procedures in lung malignancies

    Evers, D.J.; Nachabe, R.; Klomp, H.M.; Sandick, van J.W.; Wouters, M.W.; Lucassen, G.W.; Hendriks, B.H.; Wesseling, J.; Ruers, T.J.M.


    Background: A significant number of percutaneous intrathoracic biopsy procedures result in indeterminate cytologic or histologic diagnosis in clinical practice. Diffuse reflectance spectroscopy (DRS) is an optical technique that can distinguish different tissue types on a microscopic level. DRS may

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

    Bonnefoy, Olivier; Ferretti, Gilbert; Calaque, Olivier; Coulomb, Max; Begueret, Hugues; Beylot-Barry, Marie; Laurent, Francois E-mail:


    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.

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

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


    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.

  14. Therapeutic prospects to treat skeletal muscle wasting in COPD (chronic obstructive lung disease).

    Hansen, Michelle J; Gualano, Rosa C; Bozinovski, Steve; Vlahos, Ross; Anderson, Gary P


    Chronic obstructive pulmonary disease (COPD) is an incurable group of lung diseases characterised by progressive airflow limitation and loss of lung function, which lead to profound disability. It is mostly caused by cigarette smoke. Although COPD is one of the most prevalent diseases worldwide and its incidence is increasing, current therapies do little to improve the condition. Much current research focuses on strategies to halt the accelerated rate of decline in lung function that occurs in the disease. However, as most symptoms occur when the lungs are already extensively and irreversibly damaged, it is uncertain whether an agent able to slow or halt decline in lung function would actually provide relief to COPD patients. As lung function worsens, systemic comorbidities contribute markedly to disability. Loss of lean body mass (skeletal muscle) has recently been identified as a major determinant of disability in COPD and an independent predictor of mortality. In contrast to lung structure damage, skeletal muscle retains regenerative capacity in COPD. In this review, we discuss mechanisms of wasting in COPD, focusing on therapeutic strategies that might improve the health and productive life expectancy of COPD patients by improving skeletal muscle mass and function. Single or combination approaches exploiting the suppression of procatabolic inflammatory mediators, inhibition of ubiquitin ligases, repletion of anabolic hormones and growth factors, inhibition of myoblast apoptosis, remediation of systemic oxidative stress and promotion of repair, and regeneration via stimulation of satellite cell differentiation hold considerable therapeutic promise.

  15. Role of lung surfactant in respiratory disease: current knowledge in large animal medicine.

    Christmann, U; Buechner-Maxwell, V A; Witonsky, S G; Hite, R D


    Lung surfactant is produced by type II alveolar cells as a mixture of phospholipids, surfactant proteins, and neutral lipids. Surfactant lowers alveolar surface tension and is crucial for the prevention of alveolar collapse. In addition, surfactant contributes to smaller airway patency and improves mucociliary clearance. Surfactant-specific proteins are part of the innate immune defense mechanisms of the lung. Lung surfactant alterations have been described in a number of respiratory diseases. Surfactant deficiency (quantitative deficit of surfactant) in premature animals causes neonatal respiratory distress syndrome. Surfactant dysfunction (qualitative changes in surfactant) has been implicated in the pathophysiology of acute respiratory distress syndrome and asthma. Analysis of surfactant from amniotic fluid allows assessment of fetal lung maturity (FLM) in the human fetus and exogenous surfactant replacement therapy is part of the standard care in premature human infants. In contrast to human medicine, use and success of FLM testing or surfactant replacement therapy remain limited in veterinary medicine. Lung surfactant has been studied in large animal models of human disease. However, only a few reports exist on lung surfactant alterations in naturally occurring respiratory disease in large animals. This article gives a general review on the role of lung surfactant in respiratory disease followed by an overview of our current knowledge on surfactant in large animal veterinary medicine.

  16. Qualitative aspects of exertional dyspnea in patients with restrictive lung disease

    Laveneziana Pierantonio


    Full Text Available Abstract Restrictive lung disease is a broad term encompassing a number of conditions in which lung volumes are reduced. Dyspnea is a common clinical manifestation of restrictive lung disease and frequently becomes a prominent and disabling symptom that undermines patients' ability to function and engage in activities of daily living (especially in those with more advanced restriction. Effective management of this disabling symptom awaits a better understanding of its underlying physiology. In recent decades, our understanding of the mechanisms of dyspnea in restrictive lung disease has been improved by a small, but significant, body of research. One approach to the study of dyspnea is to identify the major qualitative dimensions of the symptom in an attempt to uncover different underlying neurophysiologic mechanisms. This article will review the existing literature on the intensity and qualitative dimensions of dyspnea during exercise in patients with restrictive lung disease. The main focus will be on interstitial lung disease (ILD, since it is the prototypical restrictive disease.

  17. Immunogenetic basis of environmental lung disease: Lessons from the berylliosis model

    Saltini, C.; Richeldi, L. [Univ. di Modena, Dept. di Scienze Mediche, Modena (Italy); Amicosante, M. [Univ. di Roma `Tor Vergata`, Dept. di Biologia, Roma (Italy); Franchi, A. [Univ. de Modena, Dept. Medicina Interna, Modena (Italy); Lombardi, G. [Hammersmith Hospital, Dept. of Immunology, London (United Kingdom)


    The role of genetic factors has been hypothesized in the pathogenesis of a number of chronic inflammatory lung diseases. The genes of the major histocompatibility complex (MHC) locus on human chromosome 6 have been identified as important determinants in diseases caused both by inorganic and organic compounds such as beryllium, gold, acid anhydrides, isocyanates and grass pollens. Since many environmental factors are the determinants of the immunopathogenesis of asthma, pulmonary granulomatous disorders, hypersensitivity pneumonitis and fibrotic lung disorders, an understanding of the interaction between environmental factors is crucial to epidemiology, prevention and treatment of these disorders. Berylliosis is an environmental chronic inflammatory disorder of the lung caused by inhalation of beryllium dusts. A human leukocyte antigen class II marker (HLA-DP Glu69) has been found to be strongly associated with the disease. In in vitro studies, the gene has been shown to play a direct role in the immunopathogenesis of the disease. In human studies, the gene has been shown to confer increased susceptibility to beryllium in exposed workers, thus suggesting that HLA gene markers may be used as epidemiological probes to identify population groups at higher risk of environmental lung diseases, to identify environmental levels of lung immunotoxicants that would be safe for the entire population and the prevent disease risk associated with occupation, manufactured products and the environment. Studies on the associations between human leukocyte antigens and chronic inflammatory lung disorders are reviewed in the context of the berylliosis model. (au) 123 refs.

  18. Chest physiotherapy in preterm infants with lung diseases

    Cota Francesco


    Full Text Available Abstract Background In neonatology the role of chest physiotherapy is still uncertain because of the controversial outcomes. Methods The aim of this study was to test the applicability in preterm infants of 'reflex rolling', from the Vojta method, in preterm neonates with lung pathology, with particular attention to the effects on blood gases and oxygen saturation, on the spontaneous breathing, on the onset of stress or pain. The study included 34 preterm newborns with mean gestational age of 30.5 (1.6 weeks - mean (DS - and birth weight of 1430 (423 g - mean (DS -, who suffered from hyaline membrane disease, under treatment with nasal CPAP (continuous positive airways pressure, or from pneumonia, under treatment with oxygen-therapy. The neonates underwent phase 1 of 'reflex rolling' according to Vojta method three times daily. Respiratory rate, SatO2, transcutaneous PtcCO2 e PtcO2 were monitored; in order to evaluate the onset of stress or pain following the stimulations, the NIPS score and the PIPP score were recorded; cerebral ultrasound scans were performed on postnatal days 1-3-5-7, and then weekly. Results In this population the first phase of Vojta's 'reflex rolling' caused an increase of PtcO2 and SatO2 values. No negative effects on PtcCO2 and respiratory rate were observed, NIPS and PIPP stress scores remained unmodified during the treatment; in no patient the intraventricular haemorrhage worsened in time and none of the infants developed periventricular leucomalacia. Conclusions Our experience, using the Vojta method, allows to affirm that this method is safe for preterm neonates, but further investigations are necessary to confirm its positive effects and to evaluate long-term respiratory outcomes.

  19. Potential contribution of Type I lung epithelial cells to chronic neonatal lung disease

    Henry J. Rozycki


    Full Text Available The alveolar surface is covered by large flat Type I cells (alveolar epithelial cells 1, AEC1. The normal physiological function of AEC1s involves gas exchange, based on their location in approximation to the capillary endothelium and their thinness, and in ion and water flux, as shown by the presence of solute active transport proteins, water channels, and impermeable tight junctions between cells. With the recent ability to produce relatively pure cultures of AEC1 cells, new functions have been described. These may be relevant to lung injury, repair and the abnormal development that characterizes bronchopulmonary dysplasia. To hypothesize a potential role for AEC1 in the development of lung injury and abnormal repair/development in premature lungs, evidence is presented for their presence in the developing lung, how their source may not be the Type II cell (AEC2 as has been assumed for forty years, and how the cell can be damaged by same type of stressors as those which lead to bronchopulmonary dysplasia (BPD. Recent work shows that the cells are part of the innate immune response, capable of producing pro-inflammatory mediators, which could contribute to the increase in inflammation seen in early bronchopulmonary dysplasia. One of the receptors found exclusively on AEC1 cells in the lung, called RAGE, may also have a role in increased inflammation, and to alveolar simplification. While the current evidence for AEC1 involvement in BPD is circumstantial and limited at present, the accumulating data supports several hypotheses and questions regarding potential differences in the behavior of AEC1 cells from newborn and premature lung compared with the adult lung.

  20. Role of thalamic diffusion for disease differentiation between multiple sclerosis and ischemic cerebral small vessel disease

    Oeztoprak, Bilge; Oeztoprak, Ibrahim; Salk, Ismail [Cumhuriyet University School of Medicine, Department of Radiology, Sivas (Turkey); Topalkara, Kamil [Bayindir Hospital, Department of Neurology, Ankara (Turkey); Erkoc, Mustafa F. [Bozok University School of Medicine, Department of Radiology, Yozgat (Turkey)


    Cerebral small vessel disease (CSVD) and multiple sclerosis (MS) both harbor multiple, T2-hyperintense white matter lesions on conventional magnetic resonance imaging (MRI).We aimed to determine the microstructural changes via diffusion-weighted imaging (DWI) in normal appearing thalami. We hypothesized that the apparent diffusion coefficient (ADC) values would be different in CSVD and MS, since the extent of arterial involvement is different in these two diseases. DWI was performed for 50 patients with CSVD and 35 patients with MS along with gender- and age-matched controls whose conventional MRI revealed normal findings. DWI was done with 1.5 Tesla MR devices using echo planar imaging (EPI) for b = 0, 1000 s/mm{sup 2}. ADC values were obtained from the thalami which appeared normal on T2-weighted and FLAIR images. Standard oval regions of interest (ROIs) of 0.5 cm{sup 2} which were oriented parallel to the long axis of the thalamus were used for this purpose. The mean ADC value of the thalamus was (0.99 ± 0.16) x 10{sup -3} mm{sup 2}/s in patients with CSVD, whereas the mean ADC value was (0.78 ± 0.06) x 10{sup -3} mm{sup 2}/s in the control group. The mean ADC value was significantly higher in patients with CSVD compared to the controls (p < 0.001). The mean ADC values of the thalamus were (0.78 ± 0.08) x 10{sup -3} mm{sup 2}/s in MS patients, and (0.75 ± 0.08) x 10{sup -3} mm{sup 2}/s in the control group, which are not significantly different (p > 0.05). Our study revealed a difference in the diffusion of the thalami between CSVD and MS. DWI may aid in the radiological disease differentiation. (orig.)

  1. Improved identification of peripheral lung tumors by using diffuse reflectance and fluorescence spectroscopy

    Spliethoff, J.W.; Spliethoff, Jarich; Evers, Daniel; Evers, D.J.; Klomp, H.M.; van Sandick, J.W.; Wouters, M.W.J.M.; Nachabe, R.; Lucassen, G.W.; Lucassen, Gerald; Hendriks, B.H.W.; Wesseling, J.; Ruers, Theo J.M.


    Introduction: A significant number of transthoracic diagnostic biopsy procedures for lung lesions show indeterminate results. Such failures are potentially due to inadequate recognition of vital tumor tissue. The objective of this study was to evaluate whether optical spectroscopy at the tip of a

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

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


    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.

  3. Stem cells, cell therapies, and bioengineering in lung biology and diseases. Comprehensive review of the recent literature 2010-2012.

    Weiss, Daniel J


    A conference, "Stem Cells and Cell Therapies in Lung Biology and Lung Diseases," was held July 25 to 28, 2011 at the University of Vermont to review the current understanding of the role of stem and progenitor cells in lung repair after injury and to review the current status of cell therapy and ex vivo bioengineering approaches for lung diseases. These are rapidly expanding areas of study that provide further insight into and challenge traditional views of mechanisms of lung repair after injury and pathogenesis of several lung diseases. The goals of the conference were to summarize the current state of the field, to discuss and debate current controversies, and to identify future research directions and opportunities for basic and translational research in cell-based therapies for lung diseases. The goal of this article, which accompanies the formal conference report, is to provide a comprehensive review of the published literature in lung regenerative medicine from the last conference report through December 2012.

  4. A novel mechanical lung model of pulmonary diseases to assist with teaching and training

    Shaw Geoffrey M


    Full Text Available Abstract Background A design concept of low-cost, simple, fully mechanical model of a mechanically ventilated, passively breathing lung is developed. An example model is built to simulate a patient under mechanical ventilation with accurate volumes and compliances, while connected directly to a ventilator. Methods The lung is modelled with multiple units, represented by rubber bellows, with adjustable weights placed on bellows to simulate compartments of different superimposed pressure and compliance, as well as different levels of lung disease, such as Acute Respiratory Distress Syndrome (ARDS. The model was directly connected to a ventilator and the resulting pressure volume curves recorded. Results The model effectively captures the fundamental lung dynamics for a variety of conditions, and showed the effects of different ventilator settings. It was particularly effective at showing the impact of Positive End Expiratory Pressure (PEEP therapy on lung recruitment to improve oxygenation, a particulary difficult dynamic to capture. Conclusion Application of PEEP therapy is difficult to teach and demonstrate clearly. Therefore, the model provide opportunity to train, teach, and aid further understanding of lung mechanics and the treatment of lung diseases in critical care, such as ARDS and asthma. Finally, the model's pure mechanical nature and accurate lung volumes mean that all results are both clearly visible and thus intuitively simple to grasp.

  5. Macrophage phenotype is associated with disease severity in preterm infants with chronic lung disease.

    Lynne R Prince

    Full Text Available BACKGROUND: The etiology of persistent lung inflammation in preterm infants with chronic lung disease of prematurity (CLD is poorly characterized, hampering efforts to stratify prognosis and treatment. Airway macrophages are important innate immune cells with roles in both the induction and resolution of tissue inflammation. OBJECTIVES: To investigate airway innate immune cellular phenotypes in preterm infants with respiratory distress syndrome (RDS or CLD. METHODS: Bronchoalveolar lavage (BAL fluid was obtained from term and preterm infants requiring mechanical ventilation. BAL cells were phenotyped by flow cytometry. RESULTS: Preterm birth was associated with an increase in the proportion of non-classical CD14(+/CD16(+ monocytes on the day of delivery (58.9 ± 5.8% of total mononuclear cells in preterm vs 33.0 ± 6.1% in term infants, p = 0.02. Infants with RDS were born with significantly more CD36(+ macrophages compared with the CLD group (70.3 ± 5.3% in RDS vs 37.6 ± 8.9% in control, p = 0.02. At day 3, infants born at a low gestational age are more likely to have greater numbers of CD14(+ mononuclear phagocytes in the airway (p = 0.03, but fewer of these cells are functionally polarized as assessed by HLA-DR (p = 0.05 or CD36 (p = 0.05 positivity, suggesting increased recruitment of monocytes or a failure to mature these cells in the lung. CONCLUSIONS: These findings suggest that macrophage polarization may be affected by gestational maturity, that more immature macrophage phenotypes may be associated with the progression of RDS to CLD and that phenotyping mononuclear cells in BAL could predict disease outcome.

  6. Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease

    Gøtzsche, Peter C; Johansen, Helle Krogh


    Alpha-1 antitrypsin deficiency is an inherited disorder that can cause lung disease. People who smoke are more seriously affected and have a greater risk of dying from the disease.......Alpha-1 antitrypsin deficiency is an inherited disorder that can cause lung disease. People who smoke are more seriously affected and have a greater risk of dying from the disease....

  7. Fibrocytes Regulate Wilms Tumor 1-Positive Cell Accumulation in Severe Fibrotic Lung Disease.

    Sontake, Vishwaraj; Shanmukhappa, Shiva K; DiPasquale, Betsy A; Reddy, Geereddy B; Medvedovic, Mario; Hardie, William D; White, Eric S; Madala, Satish K


    Collagen-producing myofibroblast transdifferentiation is considered a crucial determinant in the formation of scar tissue in the lungs of patients with idiopathic pulmonary fibrosis. Multiple resident pulmonary cell types and bone marrow-derived fibrocytes have been implicated as contributors to fibrotic lesions because of the transdifferentiation potential of these cells into myofibroblasts. In this study, we assessed the expression of Wilms tumor 1 (WT1), a known marker of mesothelial cells, in various cell types in normal and fibrotic lungs. We demonstrate that WT1 is expressed by both mesothelial and mesenchymal cells in idiopathic pulmonary fibrosis lungs but has limited or no expression in normal human lungs. We also demonstrate that WT1(+) cells accumulate in fibrotic lung lesions, using two different mouse models of pulmonary fibrosis and WT1 promoter-driven fluorescent reporter mice. Reconstitution of bone marrow cells into a TGF-α transgenic mouse model demonstrated that fibrocytes do not transform into WT1(+) mesenchymal cells, but they do augment accumulation of WT1(+) cells in severe fibrotic lung disease. Importantly, the number of WT1(+) cells in fibrotic lesions was correlated with severity of lung disease as assessed by changes in lung function, histology, and hydroxyproline levels in mice. Finally, inhibition of WT1 expression was sufficient to attenuate collagen and other extracellular matrix gene production by mesenchymal cells from both murine and human fibrotic lungs. Thus, the results of this study demonstrate a novel association between fibrocyte-driven WT1(+) cell accumulation and severe fibrotic lung disease. Copyright © 2015 by The American Association of Immunologists, Inc.

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

    Silvia Quadrelli


    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

  9. Spectrum of high-resolution computed tomography imaging in occupational lung disease

    Bhawna Satija


    Full Text Available Damage to the lungs caused by dusts or fumes or noxious substances inhaled by workers in certain specific occupation is known as occupational lung disease. Recognition of occupational lung disease is especially important not only for the primary worker, but also because of the implications with regard to primary and secondary disease prevention in the exposed co-workers. Although many of the disorders can be detected on chest radiography, high-resolution computed tomography (HRCT is superior in delineating the lung architecture and depicting pathology. The characteristic radiological features suggest the correct diagnosis in some, whereas a combination of clinical features, occupational history, and radiological findings is essential in establishing the diagnosis in others. In the presence of a history of exposure and consistent clinical features, the diagnosis of even an uncommon occupational lung disease can be suggested by the characteristic described HRCT findings. In this article, we briefly review the HRCT appearance of a wide spectrum of occupational lung diseases.

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

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


    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.

  11. A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

    Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im [Seoul Medical Center, Seoul (Korea, Republic of)


    Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

  12. Comparison of Serum Lipid Levels in Chronic Obstructive Pulmonary Disease and Lung Cancer

    Mehmet Kos


    Full Text Available Aim: Relationship between serum lipid level in chronic obstructive lung disease (COLD and lung cancer was not well documented. In our study we planned to compare serum lipid levels (Total Cholesterol-TC, low density lipoprotein cholesterol-LDL-C, trigliseride-TGL, and high density lipoprotein cholesterol-HDL-C in these common diseases. Material and Method: We evaluated 100 patients and 50 control group retrospectively. We enrolled the lipid parameters before any medical treatment start. Student%u2019s t-test and one-way ANOVA test was used for comparison of the patient characteristics and mean cholesterol level. Results: TC levels were higher in COLD disease than lung cancer group but not statistically significant. TGL levels were higher in lung cancer group than COLD and control group but this was also not statistically significant. Mild-moderate degree COLD patients had lower HDL-C than severe COLD patients (p=0.02. But TC and TGL levels were lower in severe COLD pateints. Small cell lung sancer and non-small lung cancers had statistically significantly lower TC and TGL levels (respectively p=0.04 and p=0.02. Discussion: We estimated that lipid leves of at the beginning of COLD were decreased to provide lipid necessity in cancer tissue due to tumor rapid cell proliferation in cancer, tumor cachexia and increased nutrition problems when developed lung cancer. Larger prospective studies are required to