Update on new muscle glycogenosis

DEFF Research Database (Denmark)

Laforêt, Pascal; Malfatti, Edoardo; Vissing, John

2017-01-01

PURPOSE OF REVIEW: The field of muscle glycogenoses has progressed in recent years by the identification of new disorders, and by reaching a better understanding of pathophysiology of the disorders and the physiology of glycogen metabolism. RECENT FINDINGS: In this review, we describe the clinical...... and pathological features of the three most recently described muscle glycogenoses caused by recessive mutations in GYG1, RBCK1 and PGM1. The three involved enzymes play different roles in glycogen metabolism. Glycogenin-1 (GYG1) is involved in the initial steps of glycogen synthesis, whereas phosphoglucomutase...... with abnormal storage material in the heart, but most cases present with a polyglucosan body myopathy without cardiac involvement. SUMMARY: The recent identification of new glycogenosis not only allows to improve the knowledge of glycogen metabolism, but also builds bridges with protein glycosylation and immune...

Pulmonary interstitial emphysema presenting in a woman on the intensive care unit: case report and review of literature

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Jovaisa Tomas

2011-06-01

Full Text Available Abstract Introduction Pulmonary interstitial emphysema is a life-threatening form of ventilator-induced lung injury. We present one of the few reported adult cases of pulmonary interstitial emphysema in a woman with respiratory failure admitted to our intensive care unit. Case presentation An 87-year-old Caucasian woman with a diagnosis of community-acquired pneumonia was admitted to our intensive care unit requiring invasive ventilation. The combination of a poor oxygenation index and bilateral alveolar/interstitial infiltrates on a chest radiograph fulfilled the criteria for adult respiratory distress syndrome; the cause was thought to be a combination of the direct pneumonic pulmonary injury and extrapulmonary severe sepsis. By day seven, the fraction of inspired oxygen, peak airway and positive end expiratory pressures weaned sufficiently to allow an uncomplicated percutaneous tracheostomy. On day 10, problems with ventilation necessitated recruitment maneuvers with a Mapleson C circuit, after which dramatic surgical emphysema was noted. An upper airway bronchoscopy showed no obvious tracheal wall injury, and computed tomography of her chest showed extensive surgical emphysema, perivascular emphysema and peribronchial emphysema, which were consistent with a diagnosis of pulmonary interstitial emphysema. Over the following days, despite protective ventilatory strategies and intercostal tube thoracostomy, lung compliance along with oxygenation deteriorated and our patient died on day 14. Conclusion The development of pulmonary interstitial emphysema is a rare but real risk when caring for patients with worsening lung compliance on the intensive care unit. Improved awareness of the condition, early protective ventilation strategies and timely treatment of any of the lethal complications will hopefully result in improved survival from the condition in adults.

Familial Interstitial Pulmonary Fibrosis: A Large Family with Atypical Clinical Features

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Ranji Chibbar

2010-01-01

Full Text Available A large kindred of familial pulmonary fibrosis is reported. Six members from the first two generations of this particular kindred were described more than 40 years previously; six more individuals from the third and fourth generations have also been evaluated. The proband, now 23 years of age, has mild disease; the other 11 documented affected family members all died from their disease at an average age of 37 years (range 25 to 50 years. The pathology was that of usual interstitial pneumonia, as is typical in idiopathic pulmonary fibrosis. However, the initial radiographic pattern in many of these individuals was upper lobe and nodular and, along with the young age, was atypical for idiopathic pulmonary fibrosis. Several genetic abnormalities have been associated with familial pulmonary fibrosis. The present study examined the genes coding for surfactant protein-C, ATP-binding cassette protein A3 and telomerase, and found no abnormalities.

Comparison of Active Drug Concentrations in the Pulmonary Epithelial Lining Fluid and Interstitial Fluid of Calves Injected with Enrofloxacin, Florfenicol, Ceftiofur, or Tulathromycin

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Foster, Derek M.; Martin, Luke G.; Papich, Mark G.

2016-01-01

Bacterial pneumonia is the most common reason for parenteral antimicrobial administration to beef cattle in the United States. Yet there is little information describing the antimicrobial concentrations at the site of action. The objective of this study was to compare the active drug concentrations in the pulmonary epithelial lining fluid and interstitial fluid of four antimicrobials commonly used in cattle. After injection, plasma, interstitial fluid, and pulmonary epithelial lining fluid concentrations and protein binding were measured to determine the plasma pharmacokinetics of each drug. A cross-over design with six calves per drug was used. Following sample collection and drug analysis, pharmacokinetic calculations were performed. For enrofloxacin and metabolite ciprofloxacin, the interstitial fluid concentration was 52% and 78% of the plasma concentration, while pulmonary fluid concentrations was 24% and 40% of the plasma concentration, respectively. The pulmonary concentrations (enrofloxacin + ciprofloxacin combined) exceeded the MIC90 of 0.06 μg/mL at 48 hours after administration. For florfenicol, the interstitial fluid concentration was almost 98% of the plasma concentration, and the pulmonary concentrations were over 200% of the plasma concentrations, exceeding the breakpoint (≤ 2 μg/mL), and the MIC90 for Mannheimia haemolytica (1.0 μg/mL) for the duration of the study. For ceftiofur, penetration to the interstitial fluid was only 5% of the plasma concentration. Pulmonary epithelial lining fluid concentration represented 40% of the plasma concentration. Airway concentrations exceeded the MIC breakpoint for susceptible respiratory pathogens (≤ 2 μg/mL) for a short time at 48 hours after administration. The plasma and interstitial fluid concentrations of tulathromcyin were lower than the concentrations in pulmonary fluid throughout the study. The bronchial concentrations were higher than the plasma or interstitial concentrations, with over 900

Comparison of Active Drug Concentrations in the Pulmonary Epithelial Lining Fluid and Interstitial Fluid of Calves Injected with Enrofloxacin, Florfenicol, Ceftiofur, or Tulathromycin.

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Derek M Foster

Full Text Available Bacterial pneumonia is the most common reason for parenteral antimicrobial administration to beef cattle in the United States. Yet there is little information describing the antimicrobial concentrations at the site of action. The objective of this study was to compare the active drug concentrations in the pulmonary epithelial lining fluid and interstitial fluid of four antimicrobials commonly used in cattle. After injection, plasma, interstitial fluid, and pulmonary epithelial lining fluid concentrations and protein binding were measured to determine the plasma pharmacokinetics of each drug. A cross-over design with six calves per drug was used. Following sample collection and drug analysis, pharmacokinetic calculations were performed. For enrofloxacin and metabolite ciprofloxacin, the interstitial fluid concentration was 52% and 78% of the plasma concentration, while pulmonary fluid concentrations was 24% and 40% of the plasma concentration, respectively. The pulmonary concentrations (enrofloxacin + ciprofloxacin combined exceeded the MIC90 of 0.06 μg/mL at 48 hours after administration. For florfenicol, the interstitial fluid concentration was almost 98% of the plasma concentration, and the pulmonary concentrations were over 200% of the plasma concentrations, exceeding the breakpoint (≤ 2 μg/mL, and the MIC90 for Mannheimia haemolytica (1.0 μg/mL for the duration of the study. For ceftiofur, penetration to the interstitial fluid was only 5% of the plasma concentration. Pulmonary epithelial lining fluid concentration represented 40% of the plasma concentration. Airway concentrations exceeded the MIC breakpoint for susceptible respiratory pathogens (≤ 2 μg/mL for a short time at 48 hours after administration. The plasma and interstitial fluid concentrations of tulathromcyin were lower than the concentrations in pulmonary fluid throughout the study. The bronchial concentrations were higher than the plasma or interstitial concentrations

Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings.

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Maglione, Paul J; Overbey, Jessica R; Cunningham-Rundles, Charlotte

2015-01-01

Common variable immunodeficiency may be complicated by interstitial lung disease, which leads to worsened morbidity and mortality in some. Although immunomodulatory treatment has efficacy, choice of patient, duration of treatment, and long-term follow-up are not available. Interstitial lung disease appears stable in certain instances, so it is not known whether all patients will develop progressive disease or require immunomodulatory therapy. This study aims to determine if all common variable immunodeficiency patients with interstitial lung disease have physiological worsening, and if clinical and/or laboratory parameters may correlate with disease progression. A retrospective review of medical records at Mount Sinai Medical Center in New York was conducted for referred patients with common variable immunodeficiency, CT scan-confirmed interstitial lung disease, and periodic pulmonary function testing covering 20 or more months before immunomodulatory therapy. Fifteen patients were identified from the retrospective review and included in this study. Of the 15 patients with common variable immunodeficiency, 9 had physiological worsening of interstitial lung disease adapted from consensus guidelines, associated with significant reductions in forced expiratory volume in 1 second, forced vital capacity, and diffusion capacity of the lung for carbon monoxide. Those with progressive lung disease also had significantly lower mean immunoglobulin G levels, greater increases and highest levels of serum immunoglobulin M (IgM), and more significant thrombocytopenia. Interstitial lung disease resulted in physiological worsening in many, but not all subjects, and was associated with suboptimal immunoglobulin G replacement. Those with worsening pulmonary function tests, elevated IgM, and severe thrombocytopenic episodes appear to be at highest risk for progressive disease. Such patients may benefit from immunomodulatory treatment. Copyright © 2015 American Academy of Allergy

Early detection of interstitial pneumonia by WXGa-citrate scintigraphy. Cases of abnormal pulmonary WXGa uptake with normal chest radiographs

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Ito, Shinsaku; Mikami, Riichiro; Ryujin, Yoshitada

1985-04-01

In this paper we report our recent experience indicating usefulness of WXGa-citrate scintigraphy in 4 cases with inflammatory pulmonary diseases. These cases showed abnormal pulmonary WXGa uptake with normal chest radiographs. The first case with malignant lymphoma and the second one with lung cancer suffered from pulmonary infection following secondary immuno-insufficiency due to radiotherapy and chemotherapy. Pneumocystis carinii was suspected as causative agent in the first case, and gram negative bacilli in the second case. The third case with lung cancer developed radiation pneumonia after radiotherapy. The fourth case with acute bronchitis developed drug induced interstitial pneumonia presumably due to minocycline administration. It is concluded that WXGa-citrate scintigraphy is more sensitive for early detection of interstitial pneumonia than routine chest radiography.

Diffuse interstitial pulmonary infiltrate associated with retroperitoneal mass: report of two cases

International Nuclear Information System (INIS)

Teixeira, A.A.; Ramos, M.V.; Natal, M.R.C.

1990-01-01

The authors describe two cases of diffuse pulmonary interstitial infiltrate associated with retroperitoneal mass. Both patients were females and presented spontaneous pneumothorax. One of them had a typical hystological presentation of lymphangiomyomatosis and in the other on the retroperitoneal mass was classified as an angiomyolipoma. Angiomyomatosis are often associated with tuberous sclerosis and lymphangiomyomatosis. A review of the literature and the differential diagnosis are presented. (author)

Identification of Clinical Phenotypes in Idiopathic Interstitial Pneumonia with Pulmonary Emphysema.

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Sato, Suguru; Tanino, Yoshinori; Misa, Kenichi; Fukuhara, Naoko; Nikaido, Takefumi; Uematsu, Manabu; Fukuhara, Atsuro; Wang, Xintao; Ishida, Takashi; Munakata, Mitsuru

2016-01-01

Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis.

Pulmonary hypertension in childhood interstitial lung disease: A systematic review of the literature.

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Bromley, Susan; Vizcaya, David

2017-05-01

Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systematically review the literature regarding the occurrence of pulmonary hypertension (PH) in chILD, its effect on prognosis and healthcare use, and its treatment in clinical practice. Searches of PubMed and EMBASE databases (up to February 2016), and American Thoracic Society conference abstracts (2009-2015) were conducted using relevant keywords. References from selected articles and review papers were scanned to identify further relevant articles. A total of 20 articles were included; estimates of PH in chILD ranged from 1% to 64% with estimates among specific chILD entities ranging from 0% to 43%. Comparisons between studies were limited by differences in the study populations, including the size, age range, and heterogeneous composition of the ILD case series in terms of the nature and severity of the clinical entities, and also the methods used to diagnose PH. Three studies found that among patients with chILD, those with PH had a significantly higher risk (up to sevenfold) of death compared with those without PH. Information on the treatment of pulmonary hypertension in chILD or the effect of PH on healthcare use was not available. Data on the use and effectiveness of treatments for pulmonary hypertension in chILD are required to address this area of unmet need. Pediatr Pulmonol. 2017;52:689-698. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Pulmonary hypertension due to unclassified interstitial lung disease in a Pembroke Welsh corgi.

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Morita, Tomoya; Nakamura, Kensuke; Tatsuyuki, Osuga; Kobayashi, Atsushi; Ichii, Osamu; Yabuki, Akira; Takiguchi, Mitsuyoshi

2018-04-23

A 12 year-old intact male Pembroke Welsh corgi weighing 10.8 kg was presented for evaluation of a 3-month history of dyspnea, and a 1-week history of exercise intolerance and anorexia. Severe hypoxemia (PaO 2 56 mmHg), diffuse lung alveolar infiltration, and severe pulmonary hypertension (tricuspid regurgitation pressure gradient was 81 mmHg) were identified. A tentative diagnosis of severe PH due to lung disease or pulmonary thromboembolism was made and treated intensively. After 5 days of hospitalization, the dog died despite oxygen supplementation and anticoagulant therapy. This dog was diagnosed as unclassified interstitial lung disease based on histopathological findings.

Radiographic and microscopic correlation of diffuse interstitial and bronchointerstitial pulmonary patterns in the caudodorsal lung of adult Thoroughbred horses in race training

International Nuclear Information System (INIS)

Wisner, E.R.; O'Brien, T.R.; Lakritz, J.; Pascoe, J.R.; Wilson, D.W.; Tyler, W.S.

1993-01-01

Complete thoracic radiographic examinations were performed on 7 horses ranging in age from 24 to 60 months, followed by in-situ lung fixation. Radiographs were examined by 3 radiologists for the presence, degree and distribution of generalised pulmonary patterns within a region of interest in the caudodorsal lung. Pulmonary tissue was obtained from 12 sites within a designated volume of interest in the caudodorsal lung, corresponding to the area of interest evaluated radiographically, and examined for the presence, character and severity of microscopic lesions. Radiographic findings within the volume of interest consisted of mild to moderate bronchial, bronchointerstitial, or interstitial pulmonary patterns. Interstitial and bronchointerstitial radiographic findings were related to severity of peribronchiolar mononuclear cell infiltrates, the degree of bronchiolar mucosal plication, and alveolar capillary and peribronchial blood vessel erythrocyte content. The severity of the interstitial radiographic pattern was inversely associated with the perceived diagnostic quality of the radiographic examinations. There was no evidence of spatial variation in the severity of the microscopic changes examined in this limited pulmonary region. Inter-rater reliability between radiologists was good in the assessment of diagnostic quality of the radiographic examinations but poor in assessing severity of the primary generalised pulmonary patterns within the radiographic region of interest

Radionuclide study for the interstitial lung disease

International Nuclear Information System (INIS)

Kawakami, Kenji; Mori, Yutaka; Ujita, Masuo

1991-01-01

The contribution of pulmonary nuclear medicine was evaluated in 105 patients with interstitial pulmonary diseases (IPD). Ventilation study (V) with 81m Kr, distribution of compliance in thoraco-pulmonary system (C) by 81m Kr gas bolus inhalation method, perfusion study (Q) with 99m Tc-MAA, 67 Ga scintigraphy and an assessment of pulmonary epithelial permeability with 99m Tc-DTPA aerosol were performed as nuclear medicine procedures. Pulmonary function test (%DLco, vital capacity and functional residual capacity) and blood gas analysis were also examined. Abnormalities in V were larger than that in Q which was high V/Q mismatch finding, in the interstitial pneumonia. Correlation between V/Q mismatch and PaO 2 was, therefore, not significant. %DLco was decreased in cases with larger V/Q mismatches. 67 Ga accumulated in the early stage of interstitial pneumonia when CT or chest X-ray did not show any finding. %DLco was decreased in cases with strong accumulation of 67 Ga. 67 Ga might be useful to evaluate activity of the diseases. Pulmonary epithelial permeability was assessed by 99m Tc-DTPA inhalation study. This permeability accelerated in idiopathic interstitial fibrosis and sarcoidosis. Pulmonary epithelial permeability may be useful as an indicator for epithelial cell injury. (author)

Acute lyme infection presenting with amyopathic dermatomyositis and rapidly fatal interstitial pulmonary fibrosis: a case report

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Nguyen Hanh

2010-06-01

Full Text Available Abstract Introduction Dermatomyositis has been described in the setting of lyme infection in only nine previous case reports. Although lyme disease is known to induce typical clinical findings that are observed in various collagen vascular diseases, to our knowledge, we believe that our case is the first presentation of acute lyme disease associated with amyopathic dermatomyositis, which was then followed by severe and fatal interstitial pulmonary fibrosis only two months later. Case presentation We present a case of a 64-year-old African-American man with multiple medical problems who was diagnosed with acute lyme infection after presenting with the pathognomonic rash and confirmatory serology. In spite of appropriate antimicrobial therapy for lyme infection, he developed unexpected amyopathic dermatomyositis and then interstitial lung disease. Conclusions This case illustrates a potential for lyme disease to produce clinical syndromes that may be indistinguishable from primary connective tissue diseases. An atypical and sequential presentation (dermatomyositis and interstitial lung disease of a common disease (lyme infection is discussed. This case illustrates that in patients who are diagnosed with lyme infection who subsequently develop atypical muscular, respiratory or other systemic complaints, the possibility of severe rheumatological and pulmonary complications should be considered.

Radionuclide study for the interstitial lung disease

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Kawakami, Kenji; Mori, Yutaka; Ujita, Masuo (Jikei Univ., Tokyo (Japan). School of Medicine)

1991-07-01

The contribution of pulmonary nuclear medicine was evaluated in 105 patients with interstitial pulmonary diseases (IPD). Ventilation study (V) with {sup 81m}Kr, distribution of compliance in thoraco-pulmonary system (C) by {sup 81m}Kr gas bolus inhalation method, perfusion study (Q) with {sup 99m}Tc-MAA, {sup 67}Ga scintigraphy and an assessment of pulmonary epithelial permeability with {sup 99m}Tc-DTPA aerosol were performed as nuclear medicine procedures. Pulmonary function test (%DLco, vital capacity and functional residual capacity) and blood gas analysis were also examined. Abnormalities in V were larger than that in Q which was high V/Q mismatch finding, in the interstitial pneumonia. Correlation between V/Q mismatch and PaO{sub 2} was, therefore, not significant. %DLco was decreased in cases with larger V/Q mismatches. {sup 67}Ga accumulated in the early stage of interstitial pneumonia when CT or chest X-ray did not show any finding. %DLco was decreased in cases with strong accumulation of {sup 67}Ga. {sup 67}Ga might be useful to evaluate activity of the diseases. Pulmonary epithelial permeability was assessed by {sup 99m}Tc-DTPA inhalation study. This permeability accelerated in idiopathic interstitial fibrosis and sarcoidosis. Pulmonary epithelial permeability may be useful as an indicator for epithelial cell injury. (author).

The diffuse interstitial lung disease - with emphasis in the idiopathic interstitial pneumonias

International Nuclear Information System (INIS)

Bustillo P, Jose G; Pacheco, Pedro M; Matiz, Carlos; Ojeda, Paulina; Carrillo B, Jorge A.

2003-01-01

The term diffuse interstitial lung disease, it refers to those diseases that commit the interstice basically, the space between the membrane basal epithelial and endothelial, although the damage can also commit the outlying air spaces and the vessels; the supplement is centered in the diffuse interstitial lung illness of unknown cause; well-known as idiopathic interstitial pneumonias, making emphasis in the more frequents, the pulmonary fibrosis idiopathic or cryptogenic fibrosant alveolitis

[Clinical and radiological features of pulmonary tuberculosis manifested as interstitial lung diseases.].

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Shi, Ju-Hong; Feng, Rui-E; Tian, Xin-Lun; Xu, Wen-Bing; Xu, Zuo-Jun; Liu, Hong-Rui; Zhu, Yuan-Jue

2009-12-01

The purpose of this paper was to investigate the clinical and radiological features of pulmonary tuberculosis presenting as interstitial lung diseases (ILD). We analyzed the data of cases suspected of diffuse parenchyma lung diseases at this hospital between October 2003 and October 2007. The diagnosis of active pulmonary tuberculosis was based on epithelioid granuloma or positive acid-fast bacilli in lung biopsy and changes on serial radiographs obtained during treatment. The data of a series of 230 consecutive patients with suspected ILD were retrospectively analyzed. The diagnosis was confirmed by lung biopsy. Twelve patients were confirmed to have pulmonary tuberculosis. There were 5 males and 7 females with a mean age of 38 +/- 11 years (range, 17 - 68). The median course of disease in these patients was 3 months (range, 0.5 - 18 months). Patients with pulmonary tuberculosis presented with fever (11/12), cough (9/12), weight loss (7/12), dyspnea (7/12), lymphadenopathy (4/12), and splenohepatomegaly (2/12). On chest CT scan, ground-glass attenuation was identified in 4, bilateral patchy infiltration in 5, tree-in-bud appearance 1, and centrilobular lesions in 2 of the 12 patients. During the follow-up period (median, 9 month, range from 3 to 12 month), 11 patients improved, but 1 died of diabetic ketoacidosis. The diagnosis of pulmonary tuberculosis should be considered in suspected ILD patients presenting with fever, splenohepatomegaly and lymphadenopathy.

Interstitial inflammatory lesions of the pulmonary allograft: a retrospective analysis of 2697 transbronchial biopsies

DEFF Research Database (Denmark)

Burton, C.M.; Iversen, M.; Carlsen, J.

2008-01-01

. The strongest histological correlations were between ACR and lymphocytic bronchiolitis (OR 5.1, Pfibrosis (OR 3.2, Ppulmonary hemosiderosis were also more likely to demonstrate the finding of interstitial fibrosis (OR 3.0 and 3.7, P..., incidence and possible associations between commonly identified inflammatory and fibrotic lesions in the pulmonary allograft. METHODS: Retrospective chart review of all transbronchial biopsies performed within the first 2 years of 299 lung-transplanted patients in the period 1996 to 2006. RESULTS: A total...... (BOOP) and interstitial pneumonitis occurred at 4 to 6 weeks, and 6 to 12 weeks, respectively. There was a steady increase in the cumulative proportion of patients with fibrosis and bronchiolitis obliterans, at each successive scheduled surveillance time point beyond 3 months posttransplantation...

Advanced sickle cell associated interstitial lung disease presenting ...

African Journals Online (AJOL)

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 ...

Clinical use of pulmonary function tests and high-resolution tomography in interstitial lung diseases

International Nuclear Information System (INIS)

Garcia C, Clara P; Mejia M, Luis F

2010-01-01

Diagnosis of interstitial lung diseases is generally arrived at by clinical history, physical examination, and radiologic images, especially high-resolution CT-scanning. It is important to note that, while these diseases have different clinical and histological characteristics, they share a basic pattern of abnormal lung function. With regard to high-resolution tomography, the characteristics of these diseases are similar, although there are specific differences that can be helpful for correct diagnosis. These diseases have severe consequences on respiratory gas exchange. These alterations, combined with other abnormalities of lung function, cause the signs and symptoms and have an impact on quality of life. The use of physiologic parameters is not only helpful for diagnosis, but can also assess severity, help to define the consequences of treatment, and aid in the follow-up. Although some pulmonary function tests can remain completely normal with severe radiographic findings, 10% of patients have impaired lung function before radiologic changes. High-resolution tomography is an essential imaging tool for the study of these patients. This is true not only for diagnosis, but also with regard to clinical parameters and follow-up. Its prognostic use is continually gaining importance. In this article we assess the clinical use of pulmonary function tests and high-resolution tomography in interstitial lung diseases.

Thin-slice high-resolution CT study of pulmonary asbestosis and idiopathic interstitial pneumonia

International Nuclear Information System (INIS)

Hatakeyama, Masayuki; Maeda, Munehiro; Ohmura, Takuya

1987-01-01

Thin-slice high-resolution CT findings were compared between 36 patients with pulmonary asbestos exposure (AS) and 33 patients with idiopathic interstitial pneumonia (IIP). The CT scans of these patients were classified into 5 types (0-IV) by the subpleural curvilinear shadow (SCLS) and honey-comb shadow (HS). SCLS was detected in 22 (62 %) patients with AS and 7 (21 %) with IIP. HS was detected in 14 (39 %) patients with AS and 33 (100 %) with IIP. In both the diseases, SCLS was distributed mainly in the lower lobe in CT types I and II, and in mildly fibrotic segments in types III and IV. In CT types II, III and IV, SCLS was always communicated with HS. Thin-slice high-resolution CT is considered very helpful in diagnosis and staging of not only AS and IIP but also pulmonary fibrosis. (author)

Smoking-related interstitial lung diseases

International Nuclear Information System (INIS)

Marten, K.

2007-01-01

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

Pulmonary epithelial permeability in normal subjects and patients with idiopathic interstitial pneumonia

International Nuclear Information System (INIS)

Anazawa, Yoshiki; Isawa, Toyoharu; Teshima, Takeo; Miki, Makoto; Motomiya, Masakichi

1991-01-01

99m Tc-DTPA is a low molecular weight substance, which is believed to pass through the pulmonary epithelium when it is inhaled as an aerosol. We performed 99m Tc-DTPA inhalation studies in 10 nonsmoking normal subjects and 10 patients with biopsy proven idiopathic interstitial pneumonia prior to therapy. 99m Tc-DTPA aerosol was inhaled for 3 min with the subject in the supine position and radioactivity was measured anteriorly with a gamma camera and recorded on computer. Measurements were performed for 3 min with the subject inhaling aerosol and for the subsequent 30 min with the subject in the same position. Time activity curves from the five regions of interest (ROIs) including the entire left lung, the entire right lung, and the upper, middle and lower third of the right lung were separately fitted to a single exponential function for the initial 7 min following cessation of inhalation, and the respective clearance half life (t1/2) in min was calculated. Lung function data, arterial blood gas tensions and blood chemistry were also obtained for comparison with the t1/2 values. The t1/2 values were significantly smaller in all ROIs in patients with idiopathic interstitial pneumonia than in normal subjects, indicating an increased pulmonary epithelial permeability in these patients. There was no relationship between t1/2 and %DL co , %DL co /V A , PaO 2 , or LDH. Although the true pathophysiologic significance of t1/2 measured using 99m Tc-DTPA aerosol is still not known, we consider that this measurement may be an important indicator of nonrespiratory lung function, in particular the degree of alveolar epithelial damage. (author)

Persistent diffuse pulmonary interstitial emphysema mimicking pulmonary emphysema

OpenAIRE

Demura, Y; Ishizaki, T; Nakanishi, M; Ameshima, S; Itoh, H

2009-01-01

A 69-year-old male non-smoker with a history of atopic asthma presented with symptoms suggestive of chronic obstructive pulmonary disease and this appeared to be corroborated by lung function testing and a chest radiograph. However, a chest CT showed no evidence of pulmonary emphysema and instead demonstrated free air along the bronchovascular sheaths indicative of pulmonary interstistial emphysema, possibly caused by repeated prior exacerbations of asthma. His lung function tests and symptom...

Smoking-related interstitial lung diseases: histopathological and imaging perspectives

Energy Technology Data Exchange (ETDEWEB)

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

2003-04-01

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

Smoking-related interstitial lung diseases: histopathological and imaging perspectives

International Nuclear Information System (INIS)

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

2003-01-01

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

Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis.

Science.gov (United States)

Aggarwal, Rohit; McBurney, Christine; Schneider, Frank; Yousem, Samuel A; Gibson, Kevin F; Lindell, Kathleen; Fuhrman, Carl R; Oddis, Chester V

2017-03-01

To compare the survival outcomes between myositis-associated usual interstitial pneumonia (MA-UIP) and idiopathic pulmonary fibrosis (IPF-UIP). Adult MA-UIP and IPF-UIP patients were identified using CTD and IPF registries. The MA-UIP cohort included myositis or anti-synthetase syndrome patients with interstitial lung disease while manifesting UIP on high-resolution CT chest and/or a lung biopsy revealing UIP histology. IPF subjects met American Thoracic Society criteria and similarly had UIP histopathology. Kaplan-Meier survival curves compared cumulative and pulmonary event-free survival (event = transplant or death) between (i) all MA-UIP and IPF-UIP subjects, (ii) MA-UIP with biopsy proven UIP (n = 25) vs IPF-UIP subjects matched for age, gender and baseline forced vital capacity (±10%). Cox proportional hazards ratios compared the survival controlling for co-variates. Eighty-one IPF-UIP and 43 MA-UIP subjects were identified. The median cumulative and event-free survival time in IPF vs MA-UIP was 5.25/1.8 years vs 16.2/10.8 years, respectively. Cumulative and event-free survival was significantly worse in IPF-UIP vs MA-UIP [hazards ratio of IPF-UIP was 2.9 (95% CI: 1.5, 5.6) and 5.0 (95% CI: 2.8, 8.7) (P < 0.001), respectively]. IPF-UIP event-free survival (but not cumulative) remained significantly worse than MA-UIP with a hazards ratio of 6.4 (95% CI: 3.0, 13.8) after controlling for age at interstitial lung disease diagnosis, gender, ethnicity and baseline forced vital capacity%. Respiratory failure was the most common cause of death in both groups. A sub-analysis of 25 biopsy-proven MA-UIP subjects showed similar results. MA-UIP patients demonstrated a significant survival advantage over a matched IPF cohort, suggesting that despite similar histological and radiographic findings at presentation, the prognosis of MA-UIP is superior to that of IPF-UIP. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology

Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease.

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Caron, Melissa; Hoa, Sabrina; Hudson, Marie; Schwartzman, Kevin; Steele, Russell

2018-06-30

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression ( i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD ( i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide ( D LCO ) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted D LCO Only five studies specifically aimed to validate the PFTs: two concluded that D LCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that D LCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression. Copyright ©ERS 2018.

Venous Thromboembolism and Risk of Idiopathic Interstitial Pneumonia A Nationwide Study

DEFF Research Database (Denmark)

Sode, Birgitte Margareta; Dahl, Morten; Nielsen, Sune Fallgaard

2010-01-01

Rationale: Idiopathic interstitial pneumonia is characterized by pulmonary fibrosis and high mortality. Objectives: We examined the association between ever-diagnosed venous thromboembolism and risk of incident idiopathic interstitial pneumonia. Venous thromboembolism was taken as a proxy...... Danish registries. Measurements and Main Results: Age-standardized incidence rates per 10,000 person-years for idiopathic interstitial pneumonia were higher among those ever diagnosed with venous thromboembolism (1.8; n = 158,676), pulmonary embolism (2.8; n = 70,586), and deep venous thrombosis only (1.......2; n = 88,090), than among control subjects (0.8; n = 7,260,278). Multivariate-adjusted hazard ratios for idiopathic interstitial pneumonia were 1.8 (95% confidence interval [Cl], 1.7-1.9) in those ever diagnosed with venous thromboembolism, 2.4 (95% CI, 2.3-2.6) in those ever diagnosed with pulmonary...

Interstitial lung diseases with fibrosis - the pattern at high resolution

International Nuclear Information System (INIS)

Jarzemska, A.; Lasek, W.; Nawrocka, E.; Meder, G.; Zapala, M.

2003-01-01

Surgical lung biopsy, either open thoracotomy or video-assisted thoracoscopy is recommended in the diagnosis of interstitial lung diseases (ILD). In some cases, however, the repetitive pattern of radiological features in high-resolution computed tomography is often sufficient to confirm the diagnosis in a non-invasive manner. The purpose of the study was to determine whether patients with ILD can be selected on the basis of the HRCT pattern. Thin-section CT scans were performed in 40 patients with histologically proven idiopathic interstitial pneumonia (26 patients with usual interstitial pneumonia UIP, 2 patients with desquamative interstitial pneumonia DIP, 2 patients with bronchiolitis obliterans organizing pneumonia BOOP, 2 patients with non-specific interstitial pneumonia NSIP, 11 patients with hypersensitivity pneumonitis, and 3 patients with pulmonary histiocytosis X). The location and the intensity of lesions were taken into consideration. Clinical and histopathological findings were compared. HRCT features of interstitial lung diseases such as nodules and cystic spaces in hypersensitivity pneumonitis and pulmonary histiocytosis, and ground-glass opacities in idiopathic interstitial pneumonias (IIP) were statistically significant for differential diagnosis in ILD cases. Combination of honeycombing and ground-glass opacities found in UIP and nodules found in DIP were also statistically significant features in IIP subtypes diagnosis. In some cases, HRCT patterns of hypersensitivity pneumonitis, pulmonary histiocytosis X and IPF combined with clinical findings allowed for the accurate diagnosis without resorting to lung biopsy. Within a group of idiopathic interstitial pneumonia only in usual interstitial pneumonia characteristic pattern in thin-section CT can be defined. In other subgroups some typical features can imply a diagnosis. (author)

Hypothalamic digoxin, hemispheric chemical dominance, and interstitial lung disease.

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Kurup, Ravi Kumar; Kurup, Parameswara Achutha

2003-10-01

The isoprenoid pathway produces three key metabolites--endogenous digoxin, dolichol, and ubiquinone. This was assessed in patients with idiopathic pulmonary fibrosis and in individuals of differing hemispheric dominance to find out the role of hemispheric dominance in the pathogenesis of idiopathic pulmonary fibrosis. All 15 cases of interstitial lung disease were right-handed/left hemispheric dominant by the dichotic listening test. The isoprenoidal metabolites--digoxin, dolichol, and ubiquinone, RBC membrane Na(+)-K+ ATPase activity, serum magnesium, tyrosine/tryptophan catabolic patterns, free radical metabolism, glycoconjugate metabolism, and RBC membrane composition--were assessed in idiopathic pulmonary fibrosis as well as in individuals with differing hemispheric dominance. In patients with idiopathic pulmonary fibrosis there was elevated digoxin synthesis, increased dolichol and glycoconjugate levels, and low ubiquinone and elevated free radical levels. There was also an increase in tryptophan catabolites and a reduction in tyrosine catabolites. There was an increase in cholesterol phospholipid ratio and a reduction in glycoconjugate level of RBC membrane in patients with idiopathic pulmonary fibrosis. Isoprenoid pathway dysfunction con tributes to the pathogenesis of idiopathic pulmonary fibrosis. The biochemical patterns obtained in interstitial lung disease are similar to those obtained in left-handed/right hemispheric chemically dominant individuals by the dichotic listening test. However, all the patients with interstitial lung disease were right-handed/left hemispheric dominant by the dichotic listening test. Hemispheric chemical dominance has no correlation with handedness or the dichotic listening test. Interstitial lung disease occurs in right hemispheric chemically dominant individuals and is a reflection of altered brain function.

Idiopathic pulmonary fibrosis.

Science.gov (United States)

Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

2017-02-23

Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

The classification, natural history and radiological/histological appearance of idiopathic pulmonary fibrosis and the other idiopathic interstitial pneumonias

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G. Raghu

2008-12-01

Full Text Available The idiopathic interstitial pneumonias (IIPs are a heterogeneous group of rare interstitial lung diseases (ILDs or diffuse parenchymal lung diseases, which, as their name implies, are of unknown aetiology. The past 10 yrs have seen important advances in the classification of the IIPs into idiopathic pulmonary fibrosis (IPF and its corresponding histopathological pattern of usual interstitial pneumonia (UIP, plus six non-IPF IIP subtypes. The present article will look at the current classification of IIPs, arising from the Consensus Statement of the American Thoracic Society and European Respiratory Society, and discusses the importance of differential diagnosis of IPF from the non-IPF IIP subtypes, especially nonspecific interstitial pneumonia. Diagnosis of IIPs is a dynamic process involving close collaboration between pulmonologists, radiologists and pathologists. Increasingly accurate diagnosis of IPF has been made possible by the use of high-resolution computed tomography (HRCT and refinements in surgical lung biopsy. In IPF, a lung HRCT will typically reveal irregular reticular opacities, traction bronchiestasis and, most importantly, peripheral honeycombing. In contrast, histological examination shows evidence of UIP manifesting as typically subpleural and paraseptal established fibrosis, often with honeycomb changes, associated with mild chronic inflammation and varying numbers of fibroblastic foci in continuity with the edges of areas of established fibrosis. Despite these advances, obtaining a consistent and uniform diagnosis of idiopathic interstitial pneumonias is difficult, with studies showing significant disagreement in the diagnosis of interstitial lung diseases between academic centres of expertise and community-based clinicians. Greater interaction between academic and community clinicians, together with improved education, is needed to bridge this gap.

Familial Pulmonary Fibrosis

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... Education & Training Home Conditions Familial Pulmonary Fibrosis Familial Pulmonary Fibrosis Make an Appointment Find a Doctor Ask a ... more members within the same family have Idiopathic Pulmonary Fibrosis (IPF) or any other form of Idiopathic Interstitial ...

Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function

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Jeong, Yeon Joo; Lee, Kyung Soo; Chung, Man Pyo; Chung Myung Jin; Han, Joung Ho [Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Mueller, Nestor L. [University of British Columbia, Vancouver (Canada); Colby, Thomas V. [Mayo Clinic Scottsdale, Scottsdale (United States); Kim, Seon Woo [Samsung Medical Center, Seoul (Korea, Republic of)

2005-09-15

We wanted to demonstrate and compare the serial high-resolution CTs (HRCT) and the pulmonary function test (PFT) findings of the usual interstitial pneumonia (UIP) and the non-specific interstitial pneumonia (NSIP). The serial HRCT scans and the PFT results were retrospectively analysed and compared for 35 patients having UIP without significant honeycombing (UIP-w/o hc, < 5% of honeycombing at CT), 35 patients having UIP with honeycombing (UIP-w/i hc, {>=} 5% of honeycombing), and 25 patients with NSIP. The mortality rates were also compared. Follow-up CT scans were available in 75 patients (29 UIP-w/o hc patients, 22 UIP-w/i hc patients and 24 NSIP patients) and the follow-up periods ranged from 150 to 2,370 days. The initial and follow-up PFT data were available for 71 patients. On the initial CT, significant differences were present between the UIP-w/i hc patients and both the UIP-w/o hc patients and the NSIP patients in the overall extent, ground-glass opacity (GGO) away from the reticulation, reticulation and honeycombing (all {rho} < 0.05). Improvement was noticed in five (17%) of 29 UIP-w/o hc patients, none of 22 UIP-w/i hc patients, and 9 (37%) of 24 NSIP patients; deterioration was noted in six (21%) UIP-w/o hc patients, two (9%) UIP-w/i hc patients and three (13%) NSIP patients ({rho} 0.044 between UIP-w/o and UIP-w/i hc; {rho} = 0.637 between UIP-w/o hc and NSIP; {rho} = 0.007 between UIP-w/i hc and NSIP). The serial changes of the pulmonary function in the NSIP patients were different from those noted for the UIP-w/i hc and UIP-w/o hc patients ({rho} = 0.440 between UIP-w/o and UIP-w/i hc; {rho} = 0.022 between UIP-w/o hc and NSIP; {rho} =0.003 between UIP-w/i hc and NSIP). Five (14%) of the 35 patients with UIP-w/o hc, 16 (46%) of the 35 patients with UIP-w/i hc and three (12%) of the 25 patients with NSIP died ({rho} = 0.002, comparison for the three groups). On CT, NSIP and UIP-w/o hc patients have similar patterns of parenchymal abnormalities and

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

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Zarir F Udwadia

2015-01-01

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

The contribution of pulmonary nuclear medicine

International Nuclear Information System (INIS)

Kawakami, Kenji

1991-01-01

The contribution of pulmonary nuclear medicine was evaluated in 115 patients with interstitial pulmonary diseases (IPD). Ventilation study (V) with 81m Kr or 133 Xe, distribution of compliance in thoraco-pulmonary system (C) by 81m Kr gas bolus inhalation method, perfusion study (Q) with 99m Tc-MAA, 67 Ga scintigraphy and an assessment of pulmonary epithelial permeability with 99m Tc-DTPA aerosol were performed as nuclear medicine procedures. Pulmonary function test (%DLco, vital capacity, and functional residual capacity) and blood gas analysis were also examined. Abnormalities in V were larger than that in Q, which was high V/Q mismatch finding, in interstitial pneumonia. Correlation between V/Q mismatch and PaO 2 was, therefore, not significant. %DLco was decreased in cases with larger V/Q mismatches. 67 Ga accumulated in the early stage of interstitial pneumonia when CT or chest X-ray did not show any finding. %DLco was decreased in cases with strong accumulation of 67 Ga. 67 Ga might be useful to evaluate activity of the disease. Pulmonary epithelial permeability was assessed by 99m Tc-DTPA inhalation study. This permeability became accelerated in idiopathic interstitial fibrosis and sarcoidosis. Pulmonary epithelial permeability may be useful as an indicator for epithelial cell injury. (author)

Chlorambucil-Induced Acute Interstitial Pneumonitis

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Hammad Shafqat

2014-01-01

Full Text Available Chlorambucil is an alkylating agent commonly used in treatment of chronic lymphocytic leukemia (CLL. We report a case of interstitial pneumonitis developing in an 83-year-old man 1.5 months after completing a six-month course of chlorambucil for CLL. The interstitial pneumonitis responded to therapy with prednisone. We performed a systematic review of literature and identified 13 other case reports of chlorambucil-induced pulmonary toxicity, particularly interstitial pneumonitis. No unifying risk factor could be discerned and the mechanism of injury remains unknown. In contrast, major randomized trials of chlorambucil therapy in CLL have not reported interstitial pneumonitis as an adverse effect, which may be due to the rarity of the phenomenon or due to underreporting of events occurring after completion of treatment. Clinicians should consider drug-induced interstitial pneumonitis in the differential diagnosis of a suggestive syndrome developing even after discontinuation of chlorambucil.

Pulmonary adenocarcinoma mutation profile in smokers with smoking-related interstitial fibrosis

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Primiani A

2014-05-01

Full Text Available Andrea Primiani,1 Dora Dias-Santagata,1 A John Iafrate,1 Richard L Kradin1,2 1Pathology Service, 2Pulmonary Medicine/Critical Care Unit, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA Abstract: Cigarette smoking is an established cause of lung cancer. However, pulmonary fibrosis is also an independent risk factor for the development of lung cancer. Smoking-related interstitial fibrosis (SRIF has recently been reported. We hypothesized that adenocarcinomas in lungs with SRIF might show distinct molecular changes and examined the molecular phenotype of 168 resected lung adenocarcinomas in lungs with and without SRIF. The diagnosis of SRIF was determined by histological examination, based on the presence of alveolar septal thickening, due to pauci-inflamed, hyalinized, “ropy” collagen, in areas of lung greater than 1 cm away from the tumor. Tumors were concomitantly examined genotypically for mutations in genes frequently altered in cancer, including EGFR and KRAS, by SNaPshot and by fluorescence in situ hybridization for possible ALK rearrangements. Fluorescence in situ hybridization for ROS1 rearrangement (n=36 and/or MET amplification (n=31 were performed when no mutation was identified by either SNaPshot or ALK analysis. Sixty-five cases (38.7% showed SRIF, which was distributed in all lobes of the lungs examined. No differences were observed in sex, average age, or smoking history in patients with and without SRIF. There was no difference in either the percent or types of adenocarcinoma genetic mutations in patients with SRIF versus those without. This data suggests that SRIF does not represent an independent risk factor for the development of the major known and targeted mutations seen in pulmonary adenocarcinoma. However, additional research is required to investigate the potential significance of SRIF in the pathogenesis of lung cancer. Keywords: lung, cancer, smoking, SRIF

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

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Sandra de Barros Cobra

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

Esophageal involvement and interstitial lung disease in mixed connective tissue disease.

Science.gov (United States)

Fagundes, M N; Caleiro, M T C; Navarro-Rodriguez, T; Baldi, B G; Kavakama, J; Salge, J M; Kairalla, R; Carvalho, C R R

2009-06-01

Mixed connective tissue disease is a systemic inflammatory disorder that results in both pulmonary and esophageal manifestations. We sought to evaluate the relationship between esophageal dysfunction and interstitial lung disease in patients with mixed connective tissue disease. We correlated the pulmonary function data and the high-resolution computed tomography findings of interstitial lung disease with the results of esophageal evaluation in manometry, 24-hour intraesophageal pH measurements, and the presence of esophageal dilatation on computed tomography scan. Fifty consecutive patients with mixed connective tissue disease, according to Kasukawa's classification criteria, were included in this prospective study. High-resolution computed tomography parenchymal abnormalities were present in 39 of 50 patients. Esophageal dilatation, gastroesophageal reflux, and esophageal motor impairment were also very prevalent (28 of 50, 18 of 36, and 30 of 36, respectively). The presence of interstitial lung disease on computed tomography was significantly higher among patients with esophageal dilatation (92% vs. 45%; pmotor dysfunction (90% vs. 35%; pesophageal and pulmonary involvement, our series revealed a strong association between esophageal motor dysfunction and interstitial lung disease in patients with mixed connective tissue disease.

Tumorous interstitial lung disease

International Nuclear Information System (INIS)

Dinkel, E.; Meyer, E.; Mundinger, A.; Helwig, A.; Blum, U.; Wuertemberger, G.

1990-01-01

The radiological findings in pulmonary lymphangitic carcinomatosis and in leukemic pulmonary infiltrates mirror the tumor-dependent monomorphic interstitial pathology of lung parenchyma. It is a proven fact that pulmonary lymphangitic carcinomatosis is caused by hematogenous tumor embolization to the lungs; pathogenesis by contiguous lymphangitic spread is the exception. High-resolution CT performed as a supplement to the radiological work-up improves the sensitivity for pulmonary infiltrates in general and thus makes the differential diagnosis decided easier. Radiological criteria cannot discriminate the different forms of leukemia. Plain chest X-ray allows the diagnosis of pulmonary involvement in leukemia due to tumorous infiltrates and of tumor- or therapy-induced complications. It is essential that the radiological findings be interpreted with reference to the stage of tumor disease and the clinical parameters to make the radiological differential diagnosis of opportunistic infections more reliable. (orig.) [de

Estimation of pulmonary water distribution and pulmonary congestion by computed tomography

International Nuclear Information System (INIS)

Morooka, Nobuhiro; Watanabe, Shigeru; Masuda, Yoshiaki; Inagaki, Yoshiaki

1982-01-01

Computed tomography (CT) of the lung in normal subjects and patients with congestive heart failure was performed in the supine position with deep inspiration to obtain pulmonary CT values and images. The mean CT value in normal subjects was higher in the posterior than anterior lung field, presumably because blood vessels were more dilated in the former than the latter due to the effects of gravity. The mean pulmonary CT value in patients with congestive heart failure was significantly increased possibly due to an increase in blood flow per unit lung volume arising from either pulmonary congestion or pulmonary interstitial and alveolar edema. The mean pulmonary CT value increased parallel to the severity of pulmonary congestion, interstitial or alveolar edema and was well correlated with the pulmonary arterial wedge pressure, indicating that such a correlation was a valuable tool in assessing therapeutic effects. The results of the present study indicatethat pulmonary CT is useful for the noninvasive estimation of intrapulmonary water content and its distribution, thereby providing an effective diagnostic clue to various conditions in congestive heart failure. (author)

Smoking-related interstitial lung diseases; Interstitielle Lungenerkrankungen bei Rauchern

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

2007-03-15

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

Radiographic and high resolution CT findings of non-specific interstitial pneumonia/fibrosis

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Kondoh, Yasuhiro; Taniguchi, Hiroyuki; Nishiyama, Satoshi [Tosei General Hospital, Seto, Aichi (Japan); Yokoi, Toyoharu; Suzuki, Ryujiro; Noda, Yasunobu; Kato, Toshiyuki; Kaneko, Michie

1999-01-01

We evaluated the radiographic and high resolution CT findings in fifteen patients with biopsy proven nonspecific interstitial pneumonia. The most common radiographic findings in NSIP were bilateral infiltrates involving alveolar pattern, interstitial pattern, and mixed alveolar-interstitial pattern, which distributed mainly in the middle and lower lung zones. Loss of lung volumes were common. The predominant findings of linear and reticular opacities on HRCT were peribronchovascular interstitial thickening, parenchymal bands, intralobular interstitial thickening, and traction bronchiectasis. Honeycombing was not noted in any patient on initial CT scans. The predominant findings of increased lung opacity were mixed pattern of ground glass opacity and consolidation. Because these findings mimic those of idiopathic pulmonary fibrosis/usual interstitial pneumonia, distinction between NSIP and IPF/UIP seems to be difficult by radiographic and HRCT findings. The response to corticosteroid therapy was good. At follow up HRCT, the pulmonary abnormalities observed on initial scans had disappeared or were diminished in most cases. Intralobular interstitial thickening and traction bronchiectasis, that have been considered to be an indicator of irreversible fibrosis, occasionally disappeared after corticosteroid therapy. (author)

Dynamic Gd-DTPA enhanced breath-hold 1.5 t MRI of normal lungs and patients with interstitial lung disease and pulmonary nodules: preliminary results

International Nuclear Information System (INIS)

Semelka, R.C.; Maycher, B.; Shoenut, J.P.; Kroeker, R.; Griffin, P.; Lertzman, M.

1992-01-01

A FLASH technique was used, which encompassed the entire thorax in the transverse plane, before and after dynamic intravenous injection of godalinium DTPA (Gd-DTPA) to study 7 patients with normal lungs, 12 patients with interstitial lung disease (ILD), and 11 patients with pulmonary nodules. Comparative CT studies were obtained within 2 weeks of the MRI study in the patients with lung disease. Quantitative signal intensity (SI) measurements were performed. Qualitative evaluation of lung parenchyma was determined in a prospective blinded fashion, and in the normal group comparison was made with the CT images. In normal patients, SI of lung parenchyma increased by 7.7±1.3%. On precontrast images, second-order pulmonary branchings were visible while post-contrast, fifth- to sixth-order branches were apparent. In patients with ILD, interstitial changes enhanced to a variable extent, increases in SI ranging from minimal (49.9%) to substantial (308.4%). Detection of pulmonary nodules improved following contrast injection. The minimum lesion size detectable decreased from 8 mm precontrast to 5 mm post-contrast. Percentage contrast enhancement was greater for malignant nodules (124.2±79.7%) than benign nodules (5.8±4.7%) (p<0.01). (orig.)

Pulmonary manifestations of malaria

International Nuclear Information System (INIS)

Rauber, K.; Enkerlin, H.L.; Riemann, H.; Schoeppe, W.; Frankfurt Univ.

1987-01-01

We report on the two different types of pulmonary manifestations in acute plasmodium falciparum malaria. The more severe variant shows long standing interstitial pulmonary infiltrates, whereas in the more benign courses only short-term pulmonary edemas are visible. (orig.) [de

Mild pulmonary emphysema a risk factor for interstitial lung disease when using cetuximab for squamous cell carcinoma of the head and neck.

Science.gov (United States)

Okamoto, Isaku; Tsukahara, Kiyoaki; Sato, Hiroki; Motohashi, Ray; Yunaiyama, Daisuke; Shimizu, Akira

2017-12-01

Interstitial lung disease (ILD) is an occasionally fatal adverse event associated with cetuximab (Cmab) therapy. Our objective was to clarify to what degree pulmonary emphysema is a risk factor in the treatment of head and neck cancer with Cmab through a retrospective analysis. Subjects were 116 patients who were administered Cmab for head and neck squamous cell carcinoma. The degree of pulmonary emphysema before initiating treatment with Cmab was visually assessed retrospectively, with scoring according to the Goddard classification used in Japanese chronic obstructive pulmonary disease (COPD) guidelines for chest computed tomography (CT). Scoring was conducted by two diagnostic radiologists and mean scores were used. Cutoffs for the development and nondevelopment of ILD were examined by receiver operating characteristic (ROC) analysis and Fisher's exact test. Values of p pulmonary emphysema would represent a risk factor for ILD when using Cmab.

Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis.

Science.gov (United States)

Juge, Pierre-Antoine; Borie, Raphaël; Kannengiesser, Caroline; Gazal, Steven; Revy, Patrick; Wemeau-Stervinou, Lidwine; Debray, Marie-Pierre; Ottaviani, Sébastien; Marchand-Adam, Sylvain; Nathan, Nadia; Thabut, Gabriel; Richez, Christophe; Nunes, Hilario; Callebaut, Isabelle; Justet, Aurélien; Leulliot, Nicolas; Bonnefond, Amélie; Salgado, David; Richette, Pascal; Desvignes, Jean-Pierre; Lioté, Huguette; Froguel, Philippe; Allanore, Yannick; Sand, Olivier; Dromer, Claire; Flipo, René-Marc; Clément, Annick; Béroud, Christophe; Sibilia, Jean; Coustet, Baptiste; Cottin, Vincent; Boissier, Marie-Christophe; Wallaert, Benoit; Schaeverbeke, Thierry; Dastot le Moal, Florence; Frazier, Aline; Ménard, Christelle; Soubrier, Martin; Saidenberg, Nathalie; Valeyre, Dominique; Amselem, Serge; Boileau, Catherine; Crestani, Bruno; Dieudé, Philippe

2017-05-01

Despite its high prevalence and mortality, little is known about the pathogenesis of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Given that familial pulmonary fibrosis (FPF) and RA-ILD frequently share the usual pattern of interstitial pneumonia and common environmental risk factors, we hypothesised that the two diseases might share additional risk factors, including FPF-linked genes. Our aim was to identify coding mutations of FPF-risk genes associated with RA-ILD.We used whole exome sequencing (WES), followed by restricted analysis of a discrete number of FPF-linked genes and performed a burden test to assess the excess number of mutations in RA-ILD patients compared to controls.Among the 101 RA-ILD patients included, 12 (11.9%) had 13 WES-identified heterozygous mutations in the TERT , RTEL1 , PARN or SFTPC coding regions . The burden test, based on 81 RA-ILD patients and 1010 controls of European ancestry, revealed an excess of TERT , RTEL1 , PARN or SFTPC mutations in RA-ILD patients (OR 3.17, 95% CI 1.53-6.12; p=9.45×10 -4 ). Telomeres were shorter in RA-ILD patients with a TERT , RTEL1 or PARN mutation than in controls (p=2.87×10 -2 ).Our results support the contribution of FPF-linked genes to RA-ILD susceptibility. Copyright ©ERS 2017.

CSF1R inhibition prevents radiation pulmonary fibrosis by depletion of interstitial macrophages.

Science.gov (United States)

Meziani, Lydia; Mondini, Michele; Petit, Benoît; Boissonnas, Alexandre; Thomas de Montpreville, Vincent; Mercier, Olaf; Vozenin, Marie-Catherine; Deutsch, Eric

2018-03-01

Radiation-induced lung fibrosis (RIF) is a delayed side-effect of chest radiotherapy, frequently associated with macrophage infiltration.We aimed to characterise the role of pulmonary macrophages in RIF using human lung biopsies from patients receiving radiotherapy for thorax malignancies and a RIF model developed in C57BL/6 mice after 16-Gy thorax irradiation.High numbers of macrophages (both interstitial and alveolar) were detected in clinical and preclinical RIF. In the preclinical model, upregulation of T-helper (Th)2 cytokines was measured, whereas Th1 cytokines were downregulated in RIF tissue lysate. Bronchoalveolar lavage demonstrated upregulation of both types of cytokines. At steady state, tissue-infiltrating macrophages (IMs) expressed 10-fold more arginase (Arg)-1 than alveolar macrophages (AMs), and a 40-fold upregulation of Arg-1 was found in IMs isolated from RIF. IMs, but not AMs, were able to induce myofibroblast activation in vitro In addition, whereas depletion of AMs using Clodrosome didn't affect RIF score, depletion of IMs using a clinically available colony-stimulating factor receptor-1 (CSF1R) neutralising antibody was antifibrotic.These findings suggest differential contributions of alveolar versus interstitial macrophages in RIF, highlighting the fibrogenic role of IMs. The CSF1/CSF1R pathway was identified as a new therapeutic target to inhibit RIF. Copyright ©ERS 2018.

HRCT of diffuse interstitial pneumonia during treatment

International Nuclear Information System (INIS)

Takahashi, Masashi; Sano, Akira; Imanaka, Kazufumi

1989-01-01

HRCT was carried out in twenty patients with diffuse interstitial pneumonia: 13 cases of IIP, 3 of BOOP, 2 of drug-induced pneumonia, 1 of rheumatoid lung and acute interstitial pneumonia of unknown origin. With special attention to inflammatory activity, the patients underwent HRCT periodically during the treatment. Correlative investigation between HRCT image and grade of accumulation in 67 Ga scintigraphy was also performed. Response to steroid therapy was clearly reflected on HRCT image, that was shown as decreasing pulmonary density or thinning of honeycomb wall. HRCT is considered to be useful in assessing the activity of diffuse interstitial pneumonia. (author)

Interstitial lung involvement in rheumatoid arthritis

Directory of Open Access Journals (Sweden)

David Vladimirovich Bestaev

2014-01-01

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

Smoking-related interstitial lung diseases: radiologic-pathologic correlation

International Nuclear Information System (INIS)

Hidalgo, Alberto; Franquet, Tomas; Gimenez, Ana; Pineda, Rosa; Madrid, Marta; Bordes, Ramon

2006-01-01

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.)

Smoking-related interstitial lung diseases: radiologic-pathologic correlation

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

2006-11-15

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

The pulmonary histopathology of anti-KS transfer RNA synthetase syndrome.

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Schneider, Frank; Aggarwal, Rohit; Bi, David; Gibson, Kevin; Oddis, Chester; Yousem, Samuel A

2015-01-01

The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

Pulmonary complications after bone marrow transplantation in chest radiography

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Schuster, J.; Sailer, M.; Schmeiser, T.; Schumacher, K.A.; Heit, W.

1988-01-01

In a retrospective study chest radiographs of 87 bone marrow transplant recipients were analysed. 36 patients had pulmonary complications with lung opacifications. Interstitial changes were more frequent than air-space pneumonias. The latter were caused by bacteria and fungi only. The most common cause of pulmonary complications was cytomegalovirus pneumonia. It was characterised uniformly by a bilateral diffuse interstitial pattern. Idiopathic interstitial pneumonias were indistinguishable from CMV infection. Pneumonias caused by Epstein-Barr virus and protozoa, diffuse radiation pneumonitis and leukaemic infiltrates were rare and also associated with interstitial changes.

Pulmonary complications after bone marrow transplantation in chest radiography

International Nuclear Information System (INIS)

Schuster, J.; Sailer, M.; Schmeiser, T.; Schumacher, K.A.; Heit, W.; Ulm Univ.

1988-01-01

In a retrospective study chest radiographs of 87 bone marrow transplant recipients were analysed. 36 patients had pulmonary complications with lung opacifications. Interstitial changes were more frequent than air-space pneumonias. The latter were caused by bacteria and fungi only. The most common cause of pulmonary complications was cytomegalovirus pneumonia. It was characterised uniformly by a bilateral diffuse interstitial pattern. Idiopathic interstitial pneumonias were indistinguishable from CMV infection. Pneumonias caused by Epstein-Barr virus and protozoa, diffuse radiation pneumonitis and leukaemic infiltrates were rare and also associated with interstitial changes. (orig.) [de

Early detection of interstitial pneumonia by 67Ga-citrate scintigraphy

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Ito, Shinsaku; Mikami, Riichiro; Ryujin, Yoshitada

1985-01-01

In this paper we report our recent experience indicating usefulness of 67 Ga-citrate scintigraphy in 4 cases with inflammatory pulmonary diseases. These cases showed abnormal pulmonary 67 Ga uptake with normal chest radiographs. The first case with malignant lymphoma and the second one with lung cancer suffered from pulmonary infection following secondary immuno-insufficiency due to radiotherapy and chemotherapy. Pneumocystis carinii was suspected as causative agent in the first case, and gram negative bacilli in the second case. The third case with lung cancer developed radiation pneumonia after radiotherapy. The fourth case with acute bronchitis developed drug induced interstitial pneumonia presumably due to minocycline administration. It is concluded that 67 Ga-citrate scintigraphy is more sensitive for early detection of interstitial pneumonia than routine chest radiography. (author)

Dyskeratosis Congenita Associated Non-Specific Interstitial Pneumonia

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Unnati D. Desai

2017-01-01

Full Text Available Dyskeratosis Congenita (DC is a rare inherited disorder of ectodermal dysplasia. It consists of a classical mucocutaneous triad of abnormal skin pigmentation, nail dystrophy and leukoplakia. Pulmonary disease is seen in 10-15%. It is characterized by Idiopathic Pulmonary Fibrosis (IPF, or Idiopathic Familial Pulmonary Fibrosis (IFPF. Non-specific Interstitial Pneumonia (NSIP has been reported rarely in children with DC and in an isolated adult patient. Our patient had classical clinical presentation of DC with pancytopenia and portal hypertension and clinic-radiological features of NSIP which is a rare association.

Influence of chest background on pulmonary 99m Tc-DTPA clearance in interstitial lung disease

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Ishizaka, Akitoshi; Kanazawa, Minoru; Suzuki, Yukio; Hasegawa, Naoki; Kubo, Atsushi; Kawashiro, Takeo

1992-01-01

The authors examined the effect of chest extracellular 99m T c -diethylenetriamine pentaacetate (DTPA) as a background in the measurement of pulmonary 99m T c -DTPA clearance in patients with interstitial lung disease (ILD). Eight healthy nonsmokers (HN) and eight patients with ILD were studied. They monitored changes in gamma counts after the inhalation of 99m T c -DTPA aerosol by using a gamma camera placed over the anterior chest. The rate constant of pulmonary 99m T c -DTPA clearance (k; %/min) was assessed by calculating the slope of the decrease in the gamma counts. The chest background, estimated by 99m T c -DTPA intravenous injection, was subtracted from the original data to obtain the corrected DTPA clearance (k c ; %/min). In patients with ILD, k was significantly greater [2.19 ± 1.03 (SD) %/min; n = 8] compared with HN (0.86 ± 0.17%/min; n = 8; P c was also greater (2.80 ± 1.15%/min; n = 8; P c among all subjects (r = 0.987, P 99m T c -DTPA clearance. 16 refs., 2 figs., 2 tabs

67Gallium citrate lung scans in interstitial lung disease

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Niden, A.H.; Mishkin, F.S.; Khurana, M.M.L.

1976-01-01

Patients with diffuse interstitial lung disease often require a lung biopsy to determine the diagnosis and proper therapy. However, once the diagnosis is established, clinical evaluation of symptoms, chest roentgenogram and pulmonary function testing are the only noninvasive means currently available to assess activity of the disease process and response to the therapy. Although these measures appear adequate in the presence of acute active disease in which response to therapy results in readily demonstrable changes in the above parameters, they may be insensitive to subtle changes that can occur in minimally active disease with slowly progressive interstitial pulmonary fibrosis over a period of years. A more sensitive noninvasive technique for identifying these cases with a smoldering diffuse interstitial inflammatory process might greatly improve our ability to effectively manage such patients. With this in mind, the value of gallium lung scan was investigated to assess its ability to predict inflammatory activity in such a clinical setting

The contribution of pulmonary nuclear medicine; Imaging and physiology

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Kawakami, Kenji (Jikei Univ., Tokyo (Japan). School of Medicine)

1991-07-01

The contribution of pulmonary nuclear medicine was evaluated in 115 patients with interstitial pulmonary diseases (IPD). Ventilation study (V) with {sup 81m}Kr or {sup 133}Xe, distribution of compliance in thoraco-pulmonary system (C) by {sup 81m}Kr gas bolus inhalation method, perfusion study (Q) with {sup 99m}Tc-MAA, {sup 67}Ga scintigraphy and an assessment of pulmonary epithelial permeability with {sup 99m}Tc-DTPA aerosol were performed as nuclear medicine procedures. Pulmonary function test (%DLco, vital capacity, and functional residual capacity) and blood gas analysis were also examined. Abnormalities in V were larger than that in Q, which was high V/Q mismatch finding, in interstitial pneumonia. Correlation between V/Q mismatch and PaO{sub 2} was, therefore, not significant. %DLco was decreased in cases with larger V/Q mismatches. {sup 67}Ga accumulated in the early stage of interstitial pneumonia when CT or chest X-ray did not show any finding. %DLco was decreased in cases with strong accumulation of {sup 67}Ga. {sup 67}Ga might be useful to evaluate activity of the disease. Pulmonary epithelial permeability was assessed by {sup 99m}Tc-DTPA inhalation study. This permeability became accelerated in idiopathic interstitial fibrosis and sarcoidosis. Pulmonary epithelial permeability may be useful as an indicator for epithelial cell injury. (author).

31P-NMR studies of a case of type III glycogenosis

International Nuclear Information System (INIS)

Kawai, Mitsuru; Aizawa, Hitoshi; Itoh, Masamitsu; Yoshikawa, Kohki; Murase, Toshio

1988-01-01

31 P-NMR spectra of skeletal muscles were obtained from a patient of type III glycogenosis (33 y.o. man, reported by one of the authors, T. Murase, in 1973) and the control subject (32 y.o. man), using a superconducting whole body MR (Magnetom, Siemens). Two parameters, 1. muscle pH calculated from the chemical shift of Pi (inorganic phosphate) and PCr (creatine phosphate) and 2. PCr/Pi ratio were monitored before and after the aerobic or ischemic exercise. In resting state, the spectra were normal except for the muscle pH of thigh extensors (7.3), which was obviously higher than that of the control subject (7.0). Significant reduction of PCr/Pi ratio (from 7.0 to 4.1) was observed after the aerobic exercise in thigh extensors. Such a reduction was not recognized in the control subject. The ischemic exercise of forearm muscles revealed slight decrease in muscle pH (from 7.1 to 6.9), which was less prominent than that of the control subject. These results were compatible with the abnormality in the energy metabolism of this disorder, the block in the pathway of glycogenolysis. (author)

Pulmonary Artery Size in Interstitial Lung Disease and Pulmonary Hypertension: Association with Interstitial Lung Disease Severity and Diagnostic Utility

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Matthew Chin

2018-06-01

Full Text Available PurposeIt is postulated that ILD causes PA dilatation independent of the presence of pulmonary hypertension (PH, so the use of PA size to screen for PH is not recommended. The aims of this study were to investigate the association of PA size with the presence and severity of ILD and to assess the diagnostic accuracy of PA size for detecting PH.MethodsIncident patients referred to a tertiary PH centre underwent baseline thoracic CT, MRI and right heart catheterisation (RHC. Pulmonary artery diameter was measured on CT pulmonary angiography and pulmonary arterial areas on MRI. A thoracic radiologist scored the severity of ILD on CT from 0 to 4, 0 = absent, 1 = 1–25%, 2 = 26–50%, 3 = 51–75%, and 4 = 76–100% extent of involvement. Receiver operating characteristic analysis and linear regression were employed to assess diagnostic accuracy and independent associations of PA size.Results110 had suspected PH due to ILD (age 65 years (SD 13, M:F 37:73 and 379 had suspected PH without ILD (age 64 years (SD 13, M:F 161:218. CT derived main PA diameter was accurate for detection of PH in patients both with and without ILD - AUC 0.873, p =< 0.001, and AUC 0.835, p =< 0.001, respectively, as was MRI diastolic PA area, AUC 0.897, p =< 0.001, and AUC 0.857, p =< 0.001, respectively Significant correlations were identified between mean pulmonary arterial pressure (mPAP and PA diameter in ILD (r = 0.608, p < 0.001, and non-ILD cohort (r = 0.426, p < 0.001. PA size was independently associated with mPAP (p < 0.001 and BSA (p = 0.001, but not with forced vital capacity % predicted (p = 0.597, Transfer factor of the lungs for carbon monoxide (TLCO % predicted (p = 0.321 or the presence of ILD on CT (p = 0.905. The severity of ILD was not associated with pulmonary artery dilatation (r = 0.071, p = 0.459.ConclusionsPulmonary arterial pressure elevation leads to pulmonary arterial dilation, which is not independently influenced by the presence or severity of

The correlation of symptoms, pulmonary function tests and exercise testing with high-resolution computed tomography in patients with idiopathic interstitial pneumonia in a tertiary care hospital in South India.

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Isaac, Barney Thomas Jesudason; Thangakunam, Balamugesh; Cherian, Rekha A; Christopher, Devasahayam Jesudas

2015-01-01

For the follow-up of patients with idiopathic interstitial pneumonias (IIP), it is unclear which parameters of pulmonary function tests (PFT) and exercise testing would correlate best with high-resolution computed tomography (HRCT).. To find out the correlation of symptom scores, PFTs and exercise testing with HRCT scoring in patients diagnosed as idiopathic interstitial pneumonia. Cross-sectional study done in pulmonary medicine outpatients department of a tertiary care hospital in South India. Consecutive patients who were diagnosed as IIP by a standard algorithm were included into the study. Cough and dyspnea were graded for severity and duration. Pulmonary function tests and exercise testing parameters were noted. HRCT was scored based on an alveolar score, an interstitial score and a total score. The HRCT was correlated with each of the clinical and physiologic parameters. Pearson's/Spearman's correlation coefficient was used for the correlation of symptoms and parameters of ABG, PFT and 6MWT with the HRCT scores. A total of 94 patients were included in the study. Cough and dyspnea severity (r = 0.336 and 0.299), FVC (r = -0.48), TLC (r = -0.439) and DLCO and distance saturation product (DSP) (r = -0.368) and lowest saturation (r = -0.324) had significant correlation with total HRCT score. Among these, DLCO, particularly DLCO corrected % of predicted, correlated best with HRCT score (r = -0.721).. Symptoms, PFT and exercise testing had good correlation with HRCT. DLCO corrected % of predicted correlated best with HRCT.

Serial changes and prognostic implications of CT findings in combined pulmonary fibrosis and emphysema: comparison with fibrotic idiopathic interstitial pneumonias alone.

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Lee, Geewon; Kim, Ki Uk; Lee, Ji Won; Suh, Young Ju; Jeong, Yeon Joo

2017-05-01

Background Although fibrotic idiopathic interstitial pneumonias (IIPs) alone and those combined with pulmonary emphysema are naturally progressive diseases, the process of deterioration and outcomes are variable. Purpose To evaluate and compare serial changes of computed tomography (CT) abnormalities and prognostic predictive factors in fibrotic IIPs alone and those combined with pulmonary emphysema. Material and Methods A total of 148 patients with fibrotic IIPs alone (82 patients) and those combined with pulmonary emphysema (66 patients) were enrolled. Semi-quantitative CT analysis was used to assess the extents of CT characteristics which were evaluated on initial and follow-up CT images. Univariate and multivariate analyses were performed to assess the effects of clinical and CT variables on survival. Results Significant differences were noted between fibrotic scores, as determined using initial CT scans, in the fibrotic IIPs alone (21.22 ± 9.83) and those combined with pulmonary emphysema groups (14.70 ± 7.28) ( P pulmonary emphysema group. Multivariate Cox proportional hazards analysis showed changes in the extent of GGO (hazard ratio, 1.056) and the presence of lung cancer (hazard ratio, 4.631) were predictive factors of poor survivals. Conclusion Although patients with fibrotic IIPs alone and those combined with pulmonary emphysema have similar mortalities, lung cancer was more prevalent in patients with fibrotic IIPs combined with pulmonary emphysema. Furthermore, changes in the extent of GGO and the presence of lung cancer were independent prognostic factors of poor survivals.

Pulmonary hypertension in interstitial lung disease: Limitations of echocardiography compared to cardiac catheterization.

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Keir, Gregory J; Wort, S John; Kokosi, Maria; George, Peter M; Walsh, Simon L F; Jacob, Joseph; Price, Laura; Bax, Simon; Renzoni, Elisabetta A; Maher, Toby M; MacDonald, Peter; Hansell, David M; Wells, Athol U

2018-01-12

In interstitial lung disease (ILD), pulmonary hypertension (PH) is a major adverse prognostic determinant. Transthoracic echocardiography (TTE) is the most widely used tool when screening for PH, although discordance between TTE and right heart catheter (RHC) measured pulmonary haemodynamics is increasingly recognized. We evaluated the predictive utility of the updated European Society of Cardiology/European Respiratory Society (ESC/ERS) TTE screening recommendations against RHC testing in a large, well-characterized ILD cohort. Two hundred and sixty-five consecutive patients with ILD and suspected PH underwent comprehensive assessment, including RHC, between 2006 and 2012. ESC/ERS recommended tricuspid regurgitation (TR) velocity thresholds for assigning high (>3.4 m/s), intermediate (2.9-3.4 m/s) and low (3.4 m/s, and excluded PH in 60% of ILD subjects with a TR velocity <2.8 m/s. Thus, the ESC/ERS guidelines misclassified 40% of subjects as 'low probability' of PH, when PH was confirmed on subsequent RHC. Evaluating alternative TR velocity thresholds for assigning a low probability of PH did not significantly improve the ability of TR velocity to exclude a diagnosis of PH. In patients with ILD and suspected PH, currently recommended ESC/ERS TR velocity screening thresholds were associated with a high positive predictive value (86%) for confirming PH, but were of limited value in excluding PH, with 40% of patients misclassified as low probability when PH was confirmed at subsequent RHC. © 2018 Asian Pacific Society of Respirology.

Smoking-related interstitial fibrosis combined with pulmonary emphysema: computed tomography-pathologic correlative study using lobectomy specimens.

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Otani, Hideji; Tanaka, Tomonori; Murata, Kiyoshi; Fukuoka, Junya; Nitta, Norihisa; Nagatani, Yukihiro; Sonoda, Akinaga; Takahashi, Masashi

2016-01-01

To evaluate the incidence and pathologic correlation of thin-section computed tomography (TSCT) findings in smoking-related interstitial fibrosis (SRIF) with pulmonary emphysema. Our study included 172 consecutive patients who underwent TSCT and subsequent lobectomy. TSCT findings including clustered cysts with visible walls (CCVW) and ground-glass attenuation with/without reticulation (GGAR) were evaluated and compared in nonsmokers and smokers and among lung locations. TSCT findings, especially CCVW, were also compared with histological findings using lobectomy specimens. The incidence of CCVW and GGAR was significantly higher in smokers than in nonsmokers (34.1% and 40.7%, respectively, vs 2.0% and 12.2%). CCVW and GGAR were frequently found in the lower and peripheral zones. Histologically, CCVW corresponded more often with SRIF with emphysema than usual interstitial pneumonia (UIP, 63.3% vs 30%). CCVW of irregular size and shape were seen in 19 of 20 SRIF with emphysema and in seven of nine UIP-manifested areas with similar round cysts. A less-involved subpleural parenchyma was observed more frequently in SRIF with emphysema. SRIF with emphysema is a more frequent pathological finding than UIP in patients with CCVW on TSCT. The irregular size and shape of CCVW and a less-involved subpleural parenchyma may be a clue suggesting the presence of SRIF with emphysema.

/sup 67/Gallium citrate lung scans in interstitial lung disease

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Niden, A.H.; Mishkin, F.S.; Khurana, M.M.L.

1976-02-01

Patients with diffuse interstitial lung disease often require a lung biopsy to determine the diagnosis and proper therapy. However, once the diagnosis is established, clinical evaluation of symptoms, chest roentgenogram and pulmonary function testing are the only noninvasive means currently available to assess activity of the disease process and response to the therapy. Although these measures appear adequate in the presence of acute active disease in which response to therapy results in readily demonstrable changes in the above parameters, they may be insensitive to subtle changes that can occur in minimally active disease with slowly progressive interstitial pulmonary fibrosis over a period of years. A more sensitive noninvasive technique for identifying these cases with a smoldering diffuse interstitial inflammatory process might greatly improve our ability to effectively manage such patients. With this in mind, the value of gallium lung scan was investigated to assess its ability to predict inflammatory activity in such a clinical setting.

Risk of acute exacerbation of interstitial pneumonia after pulmonary resection for lung cancer in patients with idiopathic pulmonary fibrosis based on preoperative high-resolution computed tomography

International Nuclear Information System (INIS)

Suzuki, Hidemi; Sekine, Yasuo; Yoshida, Shigetoshi

2011-01-01

In patients with lung cancer accompanied by idiopathic pulmonary fibrosis (IPF), acute exacerbation of the IPF often occurs after pulmonary resection; however, few studies have been done to identify its preexisting risk factors. We analyzed the high-resolution computed tomography (HRCT) findings of IPF to identify the radiological characteristics of IPF susceptible to acute exacerbation after lung cancer surgery. We reviewed retrospectively 28 lung cancer patients with IPF who underwent pulmonary resection. Clinical data, respiratory function, HRCT findings, and historical features were compared between the acute exacerbation (n=9) and nonexacerbation (n=19) groups. The classification of radiological findings of IPF on HRCT was done using a scoring system of seven factors related to the interstitial shadow, including fibrosis, ground-glass opacity, and low-attenuation area. There were no significant differences in clinical background, respiratory function, composite physiologic index, or pathological features between the groups; however, the degree of fibrosis on preoperative HRCT was significantly higher in the exacerbation group (P<0.003). The fibrosis score was higher on the opposite side to the lung cancer in the exacerbation group (P<0.05). Although it is difficult to predict postoperative acute IPF exacerbation, the degree and laterality of co-existing fibrosis seem to be predictors. (author)

Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

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Isah, Muhammad Danasabe; Abbas, Aminu; Abba, Abdullahi A; Umar, Mohammed

2016-01-01

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

Interstitial pulmonary alterations in visceral leishmaniasis: evaluation with high-resolution computed tomography; Alteracoes pulmonares intersticiais na leishmaniose visceral: avaliacao pela tomografia computadorizada de alta resolucao

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Costa, Norma Selma Santos; Cerri, Giovanni Guido [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Dept. de Radiologia

1999-08-01

Visceral leishmaniasis, also called kala-azar, is a disease caused by a protozoan, the Leishmania donovani chagasi, that comprises reticuloendothelial system with involvement of the liver, spleen and bone marrow. It is endemic in some areas of northeastern Brazil and other countries of Latin America and Africa. The pathogenesis is related to the immunologic system of patients that present with the inability to activate the phagocytosis of the macrophages. As occurs in the liver and kidneys, the lungs are also involved with interstitial abnormalities caused by Leishmania that are not dependent upon the presence of the parasite. The histopathologic changes described are the involvement of inter alveolar septal in three different phases, irregularly and diffusely throughout the whole pulmonary parenchyma. This work analyzed high-resolution computed tomography (HRCT) of the thorax in 17 patients with visceral leishmaniasis in order to detect and characterize the abnormalities described in the anatomo pathologic findings reported in the literature. The HRCT is being used to evaluate chronic interstitial lung disease in a good correlation with histologic findings. The most common findings detected by HRCT were the reticular opacities that include peribronchovascular interstitial thickening and interlobular septal thickening an ground-glass opacity. The HRCT suggests that similar changes to that found in alveolar structures may occur in the secondary pulmonary lobule and that the involvement in the parenchymal interstitium represents the findings reported by pathological studies in visceral leishmaniasis. (author)

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

International Nuclear Information System (INIS)

Araki, Tetsuro; Nishino, Mizuki; Zazueta, Oscar E.; Gao, Wei; Dupuis, Josée; Okajima, Yuka; Latourelle, Jeanne C.; Rosas, Ivan O.; Murakami, Takamichi; O’Connor, George T.; Washko, George R.; Hunninghake, Gary M.

2015-01-01

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

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

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

2015-07-15

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

CT of diffuse pulmonary diseases

International Nuclear Information System (INIS)

Itoh, Harumi; Murata, Kiyoshi; Todo, Giro

1987-01-01

While the theory of chest radiographic interpretation in diagnosing diffuse pulmonary diseases has not yet been established, X-ray computed tomography (CT), having intrinsic high contrast resolution and improved spatial resolution, has proved to offer important imformation concerning the location and invasion of diffuse pulmonary lesions. This study related to CT-pathologic correlation, focusing on perivascular interstitial space and secondary pulmonary lobule at macroscopic levels. The perivascular interstitial space was thickened as a result of the infiltration of cancer, granulomas, and inflammatory cells. This finding appeared as irregular contour of the blood vessel on CT. Centrilobular nodules were distributed at the tip of the bronchus or pulmonary artery on CT. The distance from the terminal and respiratory bronchioles to the lobular border was 2 to 3 mm. Lobular lesions were delineated as clear margin on CT. Contribution of these CT features to chest radiographic interpretation must await further studies. (Namekawa, K.)

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

OpenAIRE

Khalil, Nasreen; O'Connor, Robert

2004-01-01

IDIOPATHIC PULMONARY FIBROSIS (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomogra...

Interstitial Lung Disease in Rheumatoid Arthritis in the Era of Biologics

Directory of Open Access Journals (Sweden)

A. Picchianti Diamanti

2011-01-01

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

/sup 31/P-NMR studies of a case of type III glycogenosis

Energy Technology Data Exchange (ETDEWEB)

Kawai, Mitsuru; Aizawa, Hitoshi; Itoh, Masamitsu; Yoshikawa, Kohki; Murase, Toshio

1988-05-01

/sup 31/P-NMR spectra of skeletal muscles were obtained from a patient of type III glycogenosis (33 y.o. man, reported by one of the authors, T. Murase, in 1973) and the control subject (32 y.o. man), using a superconducting whole body MR (Magnetom, Siemens). Two parameters, 1. muscle pH calculated from the chemical shift of Pi (inorganic phosphate) and PCr (creatine phosphate) and 2. PCr/Pi ratio were monitored before and after the aerobic or ischemic exercise. In resting state, the spectra were normal except for the muscle pH of thigh extensors (7.3), which was obviously higher than that of the control subject (7.0). Significant reduction of PCr/Pi ratio (from 7.0 to 4.1) was observed after the aerobic exercise in thigh extensors. Such a reduction was not recognized in the control subject. The ischemic exercise of forearm muscles revealed slight decrease in muscle pH (from 7.1 to 6.9), which was less prominent than that of the control subject. These results were compatible with the abnormality in the energy metabolism of this disorder, the block in the pathway of glycogenolysis.

Paraseptal Emphysema: Prevalence and Distribution on CT and Association with Interstitial Lung Abnormalities

Science.gov (United States)

Araki, Tetsuro; Nishino, Mizuki; Zazueta, Oscar E.; Gao, Wei; Dupuis, Josée; Okajima, Yuka; Latourelle, Jeanne C.; Rosas, Ivan O.; Murakami, Takamichi; O’Connor, George T.; Washko, George R.; Hunninghake, Gary M.; Hatabu, Hiroto

2015-01-01

Objective To investigate the prevalence and distribution of paraseptal emphysema on chest CT images in the Framingham Heart Study (FHS) population, and assess its impact on pulmonary function. Also pursued was the association with interstitial lung abnormalities. Materials and Methods We assessed 2633 participants in the FHS for paraseptal emphysema on chest CT. Characteristics of participants, including age, sex, smoking status, clinical symptoms, and results of pulmonary function tests, were compared between those with and without paraseptal emphysema. The association between paraseptal emphysema and interstitial lung abnormalities was investigated. Results Of the 2633 participants, 86 (3%) had pure paraseptal emphysema (defined as paraseptal emphysema with no other subtypes of emphysema other than paraseptal emphysema or a very few centrilobular emphysema involved) in at least one lung zone. The upper zone of the lungs was almost always involved. Compared to the participants without paraseptal emphysema, those with pure paraseptal emphysema were significantly older, and were more frequently male and smokers (mean 64 years, 71% male, mean 36 pack-years, pemphysema and interstitial lung abnormalities (pemphysema was 3% in the FHS population, predominantly affects the upper lung zone, and contributes to decreased pulmonary function. Cigarette smoking, aging, and male gender were the factors associated with the presence of paraseptal emphysema. Significant association between paraseptal emphysema and interstitial lung abnormalities was observed. PMID:25868675

High altitude pulmonary edema: case report

International Nuclear Information System (INIS)

Garcia P, Edward; Contreras Zuniga, Eduardo; Zuluaga, Sandra Ximena

2006-01-01

Acute pulmonary edema is characterized by the accumulation of liquid in the pulmonary interstice, the alveoli, the bronchi and bronchioles; it is from the excessive circulation from the pulmonary vascular system towards extra vascular and the respiratory spaces. The Liquid filters first at the interstitial space to soon perivascular and peri bronchial and, gradually, towards the alveoli and bronchi

Diffuse pulmonary ossification. A case report

International Nuclear Information System (INIS)

Torres D, Carlos A; Ojeda L, Paulina

1997-01-01

The diffuse pulmonary ossification (DPO) is a rare disease characterized by metaplastic formation of bony tissue in the lung parenchyma. Generally it is associated with other disorders as mitral stenosis and interstitial fibrosis. Sometimes the DPO is idiopathic. We report the case of a 49-year-old man who presented with cough and hemoptysis. The radiological findings suggested an interstitial lung disease. The fiber optic bronchoscopy was normal. The pulmonary function tests showed a mild airway obstruction. The bacteriological and serological studies for tuberculosis, mycosis, and collagen-vascular disease were negatives. An open lung biopsy was performed and the pathologic findings were interpreted as diffuse pulmonary ossification (DPO). Any other disease was found; so, in this case the DPO was classified as idiopathic

Treatment of giant pulmonary interstitial emphysema by ipsilateral bronchial occlusion with a Swan-Ganz catheter

International Nuclear Information System (INIS)

Rastogi, Shantanu; Gupta, Archana; Wung, Jen-Tien; Berdon, Walter E.

2007-01-01

Unilateral giant pulmonary interstitial emphysema (PIE) can be seen as a complication of chronic ventilation in extremely low-birth-weight babies. Many can be managed by conventional pulmonary care which includes positioning, suctioning, chest physiotherapy, gentle conventional ventilation and high-frequency ventilation. Some may need invasive procedures such as lung puncture, pleurotomies and excisional surgery. This is the group in which single-lung ventilation may be beneficial and circumvent the need for an invasive procedure. We describe the technique of single-lung ventilation using a Swan-Ganz catheter to block the main stem bronchus on the diseased side in air-leak syndromes. A retrospective chart review was done on 17 newborns undergoing single-lung ventilation using this technique at the Children's Hospital of New York, Columbia University, from 1986 to 2000. The technique was successful in the management of severe, neonatal unilateral lung disease not responsive to conventional modes of therapy in all but two neonates as seen by a significant improvement in pH and a decrease in PaCO 2 levels. In one neonate malpositioning of the Swan-Ganz catheter balloon could have contributed to the development of pneumothorax. The described technique of single-lung ventilation provides a safe, minimally invasive and economically feasible method of management of unilateral giant PIE in newborns not responsive to conventional modes of therapy with minimal complications. (orig.)

Treatment of giant pulmonary interstitial emphysema by ipsilateral bronchial occlusion with a Swan-Ganz catheter

Energy Technology Data Exchange (ETDEWEB)

Rastogi, Shantanu [College of Physicians and Surgeons, Columbia University, Division of Neonatology, Department of Pediatrics, The Children' s Hospital of New York, New York, NY (United States); Maimonides Medical Center, Department of Pediatrics, Brooklyn, NY (United States); Gupta, Archana; Wung, Jen-Tien [College of Physicians and Surgeons, Columbia University, Division of Neonatology, Department of Pediatrics, The Children' s Hospital of New York, New York, NY (United States); Berdon, Walter E. [College of Physicians and Surgeons, Columbia University, Pediatric Radiology, The Children' s Hospital of New York, New York, NY (United States)

2007-11-15

Unilateral giant pulmonary interstitial emphysema (PIE) can be seen as a complication of chronic ventilation in extremely low-birth-weight babies. Many can be managed by conventional pulmonary care which includes positioning, suctioning, chest physiotherapy, gentle conventional ventilation and high-frequency ventilation. Some may need invasive procedures such as lung puncture, pleurotomies and excisional surgery. This is the group in which single-lung ventilation may be beneficial and circumvent the need for an invasive procedure. We describe the technique of single-lung ventilation using a Swan-Ganz catheter to block the main stem bronchus on the diseased side in air-leak syndromes. A retrospective chart review was done on 17 newborns undergoing single-lung ventilation using this technique at the Children's Hospital of New York, Columbia University, from 1986 to 2000. The technique was successful in the management of severe, neonatal unilateral lung disease not responsive to conventional modes of therapy in all but two neonates as seen by a significant improvement in pH and a decrease in PaCO{sub 2} levels. In one neonate malpositioning of the Swan-Ganz catheter balloon could have contributed to the development of pneumothorax. The described technique of single-lung ventilation provides a safe, minimally invasive and economically feasible method of management of unilateral giant PIE in newborns not responsive to conventional modes of therapy with minimal complications. (orig.)

A stepwise composite echocardiographic score predicts severe pulmonary hypertension in patients with interstitial lung disease.

Science.gov (United States)

Bax, Simon; Bredy, Charlene; Kempny, Aleksander; Dimopoulos, Konstantinos; Devaraj, Anand; Walsh, Simon; Jacob, Joseph; Nair, Arjun; Kokosi, Maria; Keir, Gregory; Kouranos, Vasileios; George, Peter M; McCabe, Colm; Wilde, Michael; Wells, Athol; Li, Wei; Wort, Stephen John; Price, Laura C

2018-04-01

European Respiratory Society (ERS) guidelines recommend the assessment of patients with interstitial lung disease (ILD) and severe pulmonary hypertension (PH), as defined by a mean pulmonary artery pressure (mPAP) ≥35 mmHg at right heart catheterisation (RHC). We developed and validated a stepwise echocardiographic score to detect severe PH using the tricuspid regurgitant velocity and right atrial pressure (right ventricular systolic pressure (RVSP)) and additional echocardiographic signs. Consecutive ILD patients with suspected PH underwent RHC between 2005 and 2015. Receiver operating curve analysis tested the ability of components of the score to predict mPAP ≥35 mmHg, and a score devised using a stepwise approach. The score was tested in a contemporaneous validation cohort. The score used "additional PH signs" where RVSP was unavailable, using a bootstrapping technique. Within the derivation cohort (n=210), a score ≥7 predicted severe PH with 89% sensitivity, 71% specificity, positive predictive value 68% and negative predictive value 90%, with similar performance in the validation cohort (n=61) (area under the curve (AUC) 84.8% versus 83.1%, p=0.8). Although RVSP could be estimated in 92% of studies, reducing this to 60% maintained a fair accuracy (AUC 74.4%). This simple stepwise echocardiographic PH score can predict severe PH in patients with ILD.

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

Science.gov (United States)

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

2014-09-15

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.

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

LENUS (Irish Health Repository)

O'Dwyer, David N

2013-10-01

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

Pulmonary disease in patients with human immunodeficiency virus infection

DEFF Research Database (Denmark)

Lundgren, J D; Orholm, Marianne; Lundgren, B

1989-01-01

cause pulmonary disease alone or in combination. Bilateral interstitial infiltrates are the most frequent chest x-ray abnormality and are most frequently caused by infection with Pneumocystis carinii. Cytomegalovirus, Mycobacterium tuberculosis, nonspecific interstitial pneumonitis and pulmonary Kaposi......Pulmonary disease is the most important cause of morbidity and mortality in patients infected with human immunodeficiency virus (HIV). All parts of the hospital system are expected to be involved in the diagnosis and treatment of HIV infected patients in the coming years. Many different processes......'s sarcoma are the most important parts of the differential diagnosis. An aggressive approach to the diagnosis of pulmonary disease in this patient population is indicated in order to provide optimal care and assess new therapies....

Interstitial pneumonia and pulmonary hypertension associated with suspected ehrlichiosis in a dog

NARCIS (Netherlands)

Toom, Marjolein Lisette den; Dobak, Tetyda Paulina; Broens, Els Marion; Valtolina, Chiara

2016-01-01

BACKGROUND: In dogs with canine monocytic ehrlichiosis (CME), respiratory signs are uncommon and clinical and radiographic signs of interstitial pneumonia are poorly described. However, in human monocytic ehrlichiosis, respiratory signs are common and signs of interstitial pneumonia are well known.

Arterial Carboxyhemoglobin Measurement Is Useful for Evaluating Pulmonary Inflammation in Subjects with Interstitial Lung Disease.

Science.gov (United States)

Hara, Yu; Shinkai, Masaharu; Kanoh, Soichiro; Fujikura, Yuji; K Rubin, Bruce; Kawana, Akihiko; Kaneko, Takeshi

2017-01-01

Objective The arterial concentration of carboxyhemoglobin (CO-Hb) in subjects with inflammatory pulmonary disease is higher than that in healthy individuals. We retrospectively analyzed the relationship between the CO-Hb concentration and established markers of disease severity in subjects with interstitial lung disease (ILD). Methods The CO-Hb concentration was measured in subjects with newly diagnosed or untreated ILD and the relationships between the CO-Hb concentration and the serum biomarker levels, lung function, high-resolution CT (HRCT) findings, and the uptake in gallium-67 ( 67 Ga) scintigraphy were evaluated. Results Eighty-one non-smoking subjects were studied (mean age, 67 years). Among these subjects, (A) 17 had stable idiopathic pulmonary fibrosis (IPF), (B) 9 had an acute exacerbation of IPF, (C) 44 had stable non-IPF, and (D) 11 had an exacerbation of non-IPF. The CO-Hb concentrations of these subjects were (A) 1.5±0.5%, (B) 2.1±0.5%, (C) 1.2±0.4%, and (D) 1.7±0.5%. The CO-Hb concentration was positively correlated with the serum levels of surfactant protein (SP)-A (r=0.38), SP-D (r=0.39), and the inflammation index (calculated from HRCT; r=0.57) and was negatively correlated with the partial pressure of oxygen in the arterial blood (r=-0.56) and the predicted diffusion capacity of carbon monoxide (r=-0.61). The CO-Hb concentrations in subjects with a negative heart sign on 67 Ga scintigraphy were higher than those in subjects without a negative heart sign (1.4±0.5% vs. 1.1±0.3%, p=0.018). Conclusion The CO-Hb levels of subjects with ILD were increased, particularly during an exacerbation, and were correlated with the parameters that reflect pulmonary inflammation.

An approach to interstitial lung disease in India

Directory of Open Access Journals (Sweden)

J N Pande

2014-07-01

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

Flock worker's lung: chronic interstitial lung disease in the nylon flocking industry.

Science.gov (United States)

Kern, D G; Crausman, R S; Durand, K T; Nayer, A; Kuhn, C

1998-08-15

Two young men working at a nylon flocking plant in Rhode Island developed interstitial lung disease of unknown cause. Similar clusters at the same company's Canadian plant were reported previously. To define the extent, clinicopathologic features, and potential causes of the apparent disease outbreak. Case-finding survey and retrospective cohort study. Academic occupational medicine program. All workers employed at the Rhode Island plant on or after 15 June 1990. Symptomatic employees had chest radiography, pulmonary function tests, high-resolution computed tomography, and serologic testing. Those with unexplained radiographic or pulmonary function abnormalities underwent bronchoalveolar lavage, lung biopsy, or both. The case definition of "flock worker's lung" required histologic evidence of interstitial lung disease (or lavage evidence of lung inflammation) not explained by another condition. Eight cases of flock worker's lung were identified at the Rhode Island plant. Three cases were characterized by a high proportion of eosinophils (25% to 40%) in lavage fluid. Six of the seven patients who had biopsy had histologic findings of nonspecific interstitial pneumonia, and the seventh had bronchiolitis obliterans organizing pneumonia. All seven of these patients had peribronchovascular interstitial lymphoid nodules, usually with germinal centers, and most had lymphocytic bronchiolitis and interstitial fibrosis. All improved after leaving work. Review of the Canadian tissue specimens showed many similar histologic findings. Among the 165-member study cohort, a 48-fold or greater increase was seen in the sex-adjusted incidence rate of all interstitial lung disease. Work in the nylon flocking industry poses substantial risk for a previously unrecognized occupational interstitial lung disease. Nylon fiber is the suspected cause of this condition.

Interstitial lung abnormalities are associated with increased mortality in smokers

DEFF Research Database (Denmark)

Hoyer, Nils; Wille, Mathilde M W; Thomsen, Laura H

2018-01-01

OBJECTIVE: The aim of this study was to investigate whether smokers with incidental findings of interstitial lung abnormalities have an increased mortality during long-term follow-up, and review the contributing causes of death. METHODS: Baseline CT scans of 1990 participants from the Danish Lung...... in this lung cancer screening population of relatively healthy smokers and were associated with mortality regardless of the interstitial morphological phenotype. The increased mortality was partly due to an association with lung cancer and non-pulmonary malignancies....

Nuclear scan of pulmonary hemorrhage in radiopathic pulmonary hemosiderosis

International Nuclear Information System (INIS)

Miller, T.; Tanaka, T.

1979-01-01

Idiopathic pulmonary hemosiderosis, a disease of unknown etiology most often occuring in children, is characterized by recurring episodes of alveolar consolidation. Exacerbations of pulmonary hemorrhage coincide with episodes of alveolar filling; repeated episodes lead to progressive interstitial fibrosis and eventually to corpulmonale. Serial nuclear scans of the lungs after injection of radiolabeled red blood cells should parallel the pathologic and radiographic findings. We observed the accumulation of radiolabeled red blood cells in the lungs on scan images, a finding not previously reported

Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis.

Science.gov (United States)

Agrawal, Varun; Crisi, Giovanna M; D'Agati, Vivette D; Freda, Benjamin J

2012-02-01

Among the various renal manifestations of sarcoidosis, granulomatous inflammation confined to the tubulointerstitial compartment is the most commonly reported finding. We present the case of a 66-year-old man with acute kidney injury, hypercalcemia, mild restrictive pulmonary disease, and neurologic signs of parietal lobe dysfunction. Kidney biopsy showed diffuse interstitial inflammation with noncaseating granulomas that exhibited the unusual feature of infiltrating the walls of small arteries with destruction of the elastic lamina, consistent with granulomatous vasculitis. The findings of granulomatous interstitial nephritis on kidney biopsy, hypercalcemia, and possible cerebral and pulmonary involvement in the absence of other infectious, drug-induced, or autoimmune causes of granulomatous disease established the diagnosis of sarcoidosis. Pulse methylprednisolone followed by maintenance prednisone therapy led to improvement in kidney function, hypercalcemia, and neurologic symptoms. Vasculocentric granulomatous interstitial nephritis with granulomatous vasculitis is a rare and under-recognized manifestation of renal sarcoidosis. Copyright © 2012 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Pulmonary abnormalities in mitral valve disease. Comparison between pulmonary wedge pressure, regional pulmonary blood flow and chest films

Energy Technology Data Exchange (ETDEWEB)

Andersen, L H; Andersen, Jr, P E [Odense Univ. (Denmark)

1978-01-01

Chest films, right sided heart catheterization, and measurement of the regional lung perfusion, using /sup 133/Xe, were carried out 31 times on patients with mitral valve disease. A relationship was found between the radiologic evaluation in 3 grades, and the values of pulmonary wedge pressure and the apical and basal perfusion. Changes in flow distribution as reflected in altered appearance of the vessels and the presence of interstitial edema were found to be the most sensitive factors in the evaluation of pulmonary wedge pressure. Chest radiography was thus found suitable for the evaluation of pulmonary wedge pressure in mitral valve disease.

CT Analysis of the Anterior Mediastinum in Idiopathic Pulmonary Fibrosis and Nonspecific Interstitial Pneumonia

International Nuclear Information System (INIS)

Lee, Chang Hyun; Lee, Hyun Ju; Son, Kyu Ri; Chun, Eun Ju; Lim, Kun Young; Goo, Jin Mo; Im, Jung Gi; Heo, Jeong Nam; Song, Jae Woo

2006-01-01

We wanted to determine whether the amount and shape of the anterior mediastinal fat in the patients suffering with usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) was different from those of the normal control group. We selected patients who suffered with UIP (n 26) and NSIP (n = 26) who had undergone CT scans. Twenty-six controls were selected from individuals with normal CT findings and normal pulmonary function tests. All three groups (n = 78) were individually matched for age and gender. The amounts of anterior mediastinal fat, and the retrosternal anteroposterior (AP) and transverse dimensions of the anterior mediastinal fat were compared by one-way analysis of variance and Bonferroni's test. The shapes of the anterior mediastinum were compared using the Chi-square test. Exact logistic regression analysis and polychotomous logistic regression analysis were employed to assess whether the patients with NSIP or UIP had a tendency to show a convex shape of their anterior mediastinal fat. The amount of anterior mediastinal fat was not different among the three groups (p 0.175). For the UIP patients, the retrosternal AP dimension of the anterior mediastinal fat was shorter (p = 0.037) and the transverse dimension of the anterior mediastinal fat was longer (p = 0.001) than those of the normal control group. For the NSIP patients, only the transverse dimension was significantly longer than those of the normal control group (p < 0.001). The convex shape of the anterior mediastinum was predictive of NSIP (OR = 19.7, CI 3.32-∞, p < 0.001) and UIP (OR = 24.42, CI 4.06-∞, p < 0.001). For UIP patients, the retrosternal AP and transverse dimensions are different from those of normal individuals, whereas the amounts of anterior mediastinal fat are similar. UIP and NSIP patients have a tendency to have a convex shape of their anterior mediastinal fat

Non-infective pulmonary disease in HIV-positive children

International Nuclear Information System (INIS)

Theron, Salomine; Andronikou, Savvas; George, Reena; Plessis, Jaco du; Hayes, Murray; Mapukata, Ayanda; Goussard, Pierre; Gie, Robert

2009-01-01

It is estimated that over 90% of children infected with human immunodeficiency virus (HIV) live in the developing world and particularly in sub-Saharan Africa. Pulmonary disease is the most common clinical feature of acquired immunodeficiency syndrome (AIDS) in infants and children causing the most morbidity and mortality, and is the primary cause of death in 50% of cases. Children with lung disease are surviving progressively longer because of earlier diagnosis and antiretroviral treatment and, therefore, thoracic manifestations have continued to change and unexpected complications are being encountered. It has been reported that 33% of HIV-positive children have chronic changes on chest radiographs by the age of 4 years. Lymphocytic interstitial pneumonitis is common in the paediatric HIV population and is responsible for 30-40% of pulmonary disease. HIV-positive children also have a higher incidence of pulmonary malignancies, including lymphoma and pulmonary Kaposi sarcoma. Immune reconstitution inflammatory syndrome is seen after highly active antiretroviral treatment. Complications of pulmonary infections, aspiration and rarely interstitial pneumonitis are also seen. This review focuses on the imaging findings of non-infective chronic pulmonary disease. (orig.)

Drug-induced interstitial lung diseases. Often forgotten

International Nuclear Information System (INIS)

Poschenrieder, F.; Stroszczynski, C.; Hamer, O.W.

2014-01-01

Drug-induced interstitial lung diseases (DILD) are probably more common than diagnosed. Due to their potential reversibility, increased vigilance towards DILD is appropriate also from the radiologist's point of view, particularly as these diseases regularly exhibit radiological correlates in high-resolution computed tomography (HRCT) of the lungs. Based on personal experience typical relatively common manifestations of DILD are diffuse alveolar damage (DAD), eosinophilic pneumonia (EP), hypersensitivity pneumonitis (HP), organizing pneumonia (OP), non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). These patterns are presented based on case studies, whereby emphasis is placed on the clinical context. This is to highlight the relevance of interdisciplinary communication and discussion in the diagnostic field of DILD as it is a diagnosis of exclusion or of probability in most cases. Helpful differential diagnostic indications for the presence of DILD, such as an accompanying eosinophilia or increased attenuation of pulmonary consolidations in amiodarone-induced pneumopathy are mentioned and the freely available online database http://www.pneumotox.com is presented. (orig.) [de

Association between HCV induced mixed cryoglobulinemia and pulmonary affection: The role of TNF-alpha in the pathogenesis of pulmonary changes

Directory of Open Access Journals (Sweden)

AbdelBaset M. Saleh

2014-01-01

Conclusions: The results of this study suggest that pulmonary involvement is common in patients with chronic HCV infection and mixed cryoglobulinemia. Cryoglobulinemia may lead to pulmonary involvement through vascular and interstitial deposition of cryoglobulins, which results in impaired gas exchange and airway affection.

Disease activity of idiopathic pulmonary fibrosis -value of high resolution CT-

International Nuclear Information System (INIS)

Lee, Jin Seong; Im, Jung Gi; Han, Man Chung; Kim, Chu Wan; Suh, Jin Suk

1991-01-01

Idiopathic pulmonary fibrosis (IPF) has characteristic clinical and pathologic features. In patients with uniform intra-alveolar cellularity, the process is often referred to as desquamative interstitial pneumonia. When alveolar septal fibrosis predominate, the process is known as usual interstitial pneumonia. Recently most investigators believe that desquamative interstitial pneumonia is the early stage and usual interstitial pneumonia is the late stage of the same disease process. The lone-term survival and the best response to treatment with corticosteroids is found in patients with marked disease activity and little fibrosis. Since disease activity is reflected by interstitial and intraalveolar cellularity, activity of idiopathic pulmonary fibrosis might result in opacification of air spaces on CT scans. There was no significant difference in estimating the visual HRCT scores of active area between two observers (p>0.05). Activity score of HRCT scan correlated significantly with improvement of DLCO/VA after corticosteroids treatment

Disease activity of idiopathic pulmonary fibrosis -value of high resolution CT-

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Lee, Jin Seong; Im, Jung Gi; Han, Man Chung; Kim, Chu Wan; Suh, Jin Suk [Seoul National University College of Medicine, Seoul (Korea, Republic of)

1991-01-15

Idiopathic pulmonary fibrosis (IPF) has characteristic clinical and pathologic features. In patients with uniform intra-alveolar cellularity, the process is often referred to as desquamative interstitial pneumonia. When alveolar septal fibrosis predominate, the process is known as usual interstitial pneumonia. Recently most investigators believe that desquamative interstitial pneumonia is the early stage and usual interstitial pneumonia is the late stage of the same disease process. The lone-term survival and the best response to treatment with corticosteroids is found in patients with marked disease activity and little fibrosis. Since disease activity is reflected by interstitial and intraalveolar cellularity, activity of idiopathic pulmonary fibrosis might result in opacification of air spaces on CT scans. There was no significant difference in estimating the visual HRCT scores of active area between two observers (p>0.05). Activity score of HRCT scan correlated significantly with improvement of DLCO/VA after corticosteroids treatment.

Pulmonary lymphoproliferative disorders with affinity to lymphoma: a clinicopathoradiologic study of 16 cases

International Nuclear Information System (INIS)

Fernandez Cruz, J.; Gonzalez Garcia, A.; Escobar Casas, P.; Gomez Benitez, S.; Gonzalez Guirao, M.A.; Borderas, F.

1993-01-01

Pulmonary lymphoproliferative disorders include plasma cell granuloma, Castleman's disease, pseudolymphoma, lymphocytic interstitial pneumonia, angioimmunoblastic lymphadenopathy and lymphomatoid granulomatosis. We carried out a retrospective study for the purpose of analysing the clinical and radiological findings of 16 cases of pulmonary lymphoproliferative disorders seen during the decade 1980-1990. The cases comprised 8 lymphocytic interstitial pneumonia, 5 lymphomatoid granulomatosis, 2 plasma cell granuloma and 1 angioimmunoblastic lymphadenopathy. Owing to the overlap and low specificity of the radiological patterns in these processes, histopathological examination is required. In view of the frequent evolution of pulmonary lymphoproliferative disorders to malignant lymphoma (4 cases, 1 of lymphocytic interstitial pneumonia and 3 of lymphomatoid granulomatosis, in our series) we provide a description of the radiological changes that occur during this process. (orig.)

[Asymptomatic polymyositis with pulmonary disease].

Science.gov (United States)

Acosta Fernández, O; Alfonso Déniz, J; Morales Umpiérrez, A; Rodríguez de Castro, F; Esparza Morera, R

1994-02-01

We present the case of a medium-aged patient with a alveolo-interstitial pulmonary affection after 5 months of evolution, characterized by cough and progressive dyspnea accompanied by sustained febricula, slight constitutional syndrome and dermatological and articular manifestations. It was finally concluded that the patient had a polymyositis, relating form of affection a secondary interstitial pneumopathy. The aim of this study has been to highlight a rare case of diffuse interstitial disease in the context of a polymyositis in which we obtained an excellent therapeutical response, as well as to make some considerations regarding its etiology and its clinical and pathological expression.

Humane metapneumovirus (HMPV) associated pulmonary infections in immunocompromised adults—Initial CT findings, disease course and comparison to respiratory-syncytial-virus (RSV) induced pulmonary infections

International Nuclear Information System (INIS)

Syha, R.; Beck, R.; Hetzel, J.; Ketelsen, D.; Grosse, U.; Springer, F.; Horger, M.

2012-01-01

Aim: To describe computed tomography (CT)-imaging findings in human metapneumovirus (HMPV)-related pulmonary infection as well as their temporal course and to analyze resemblances/differences to pulmonary infection induced by the closely related respiratory-syncytial-virus (RSV) in immunocompromised patients. Materials and methods: Chest-CT-scans of 10 HMPV PCR-positive patients experiencing pulmonary symptoms were evaluated retrospectively with respect to imaging findings and their distribution and results were then compared with data acquired in 13 patients with RSV pulmonary infection. Subsequently, we analyzed the course of chest-findings in HMPV patients. Results: In HMPV, 8/10 patients showed asymmetric pulmonary findings, whereas 13/13 patients with RSV-pneumonia presented more symmetrical bilateral pulmonary infiltrates. Image analysis yielded in HMPV patients following results: ground-glass-opacity (GGO) (n = 6), parenchymal airspace consolidations (n = 5), ill-defined nodular-like centrilobular opacities (n = 9), bronchial wall thickening (n = 8). In comparison, results in RSV patients were: GGO (n = 10), parenchymal airspace consolidations (n = 9), ill-defined nodular-like centrilobular opacities (n = 10), bronchial wall thickening (n = 4). In the course of the disease, signs of acute HMPV interstitial pneumonia regressed transforming temporarily in part into findings compatible with bronchitis/bronchiolitis. Conclusions: Early chest-CT findings in patients with HMPV-related pulmonary symptoms are compatible with asymmetric acute interstitial pneumonia accompanied by signs of bronchitis; the former transforming with time into bronchitis and bronchiolitis before they resolve. On the contrary, RSV-induced pulmonary infection exhibits mainly symmetric acute interstitial pneumonia.

Humane metapneumovirus (HMPV) associated pulmonary infections in immunocompromised adults—Initial CT findings, disease course and comparison to respiratory-syncytial-virus (RSV) induced pulmonary infections

Energy Technology Data Exchange (ETDEWEB)

Syha, R., E-mail: roland.syha@med.uni-tuebingen.de [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str.3, 72076 Tübingen (Germany); Beck, R. [Institute of Medical Virology, Eberhard-Karls-University, Elfriede-Authorn-Str. 6, 72076 Tübingen (Germany); Hetzel, J. [Department of Medical Oncology and Hematology, Eberhard-Karls-University, Otfried-Müller-Str. 10, 72070 Tübingen (Germany); Ketelsen, D.; Grosse, U.; Springer, F.; Horger, M. [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str.3, 72076 Tübingen (Germany)

2012-12-15

Aim: To describe computed tomography (CT)-imaging findings in human metapneumovirus (HMPV)-related pulmonary infection as well as their temporal course and to analyze resemblances/differences to pulmonary infection induced by the closely related respiratory-syncytial-virus (RSV) in immunocompromised patients. Materials and methods: Chest-CT-scans of 10 HMPV PCR-positive patients experiencing pulmonary symptoms were evaluated retrospectively with respect to imaging findings and their distribution and results were then compared with data acquired in 13 patients with RSV pulmonary infection. Subsequently, we analyzed the course of chest-findings in HMPV patients. Results: In HMPV, 8/10 patients showed asymmetric pulmonary findings, whereas 13/13 patients with RSV-pneumonia presented more symmetrical bilateral pulmonary infiltrates. Image analysis yielded in HMPV patients following results: ground-glass-opacity (GGO) (n = 6), parenchymal airspace consolidations (n = 5), ill-defined nodular-like centrilobular opacities (n = 9), bronchial wall thickening (n = 8). In comparison, results in RSV patients were: GGO (n = 10), parenchymal airspace consolidations (n = 9), ill-defined nodular-like centrilobular opacities (n = 10), bronchial wall thickening (n = 4). In the course of the disease, signs of acute HMPV interstitial pneumonia regressed transforming temporarily in part into findings compatible with bronchitis/bronchiolitis. Conclusions: Early chest-CT findings in patients with HMPV-related pulmonary symptoms are compatible with asymmetric acute interstitial pneumonia accompanied by signs of bronchitis; the former transforming with time into bronchitis and bronchiolitis before they resolve. On the contrary, RSV-induced pulmonary infection exhibits mainly symmetric acute interstitial pneumonia.

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

International Nuclear Information System (INIS)

Hwang, Kyu Won; Woo, Ok Hee; Yong, Hwan Seok; Shin, Bong Kyung; Shim, Jae Jeong; Kang, Eun Young

2008-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

2008-04-15

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

Interstitial lung disease pattern turned out to be a predominantly lepidic lung adenocarcinoma

Directory of Open Access Journals (Sweden)

Irena Hammen, Dr. Med

2017-01-01

Full Text Available We report a case of a 46-year-old woman without any medical history who presented to our Respiratory Department with exertional dyspnoea for the last 6 weeks associated with non-productive cough. Chest radiography showed bilateral diffuse interstitial opacity. Bronchoalveolar lavage and transbronchial biopsies performed during flexible bronchoscopy as a step in the diagnostic workup of idiopathic interstitial pneumonia showed cells of pulmonary adenocarcinoma.

Usual interstitial pneumonia in adult-onset still's disease

International Nuclear Information System (INIS)

Rodelo, Joaquin; Gonzalez, Luis Alonso; Velasquez, Monica Patricia; Vasquez, Gloria; Uribe, Oscar; Perez, Maria del Pilar; Ramirez, Luis Alberto

2005-01-01

Adult-onset still's disease (AOSD) is a multi-system inflammatory disorder of unknown origin, characterized by high spiking fevers, evanescent salmon colored rash, arthralgias or arthritis, hepatospleno-megaly, Iymphadenopathy and sore throat. It is not uncommon for AOSD to involve other organs, such as the liver, the kidney; the bone marrow and less often the lungs. Pulmonary involvement ranges from 30 to 40 % (0 to 53 %), the pulmonary manifestations of AOSD include pleurisy, acute pneumonitis and even the acute respiratory distress syndrome. We present a case of a patient with AOSD who developed an interstitial lung disease and reviewed the literature on it

Interstitial Features at Chest CT Enhance the Deleterious Effects of Emphysema in the COPDGene Cohort.

Science.gov (United States)

Ash, Samuel Y; Harmouche, Rola; Ross, James C; Diaz, Alejandro A; Rahaghi, Farbod N; Sanchez-Ferrero, Gonzalo Vegas; Putman, Rachel K; Hunninghake, Gary M; Onieva, Jorge Onieva; Martinez, Fernando J; Choi, Augustine M; Bowler, Russell P; Lynch, David A; Hatabu, Hiroto; Bhatt, Surya P; Dransfield, Mark T; Wells, J Michael; Rosas, Ivan O; San Jose Estepar, Raul; Washko, George R

2018-06-05

Purpose To determine if interstitial features at chest CT enhance the effect of emphysema on clinical disease severity in smokers without clinical pulmonary fibrosis. Materials and Methods In this retrospective cohort study, an objective CT analysis tool was used to measure interstitial features (reticular changes, honeycombing, centrilobular nodules, linear scar, nodular changes, subpleural lines, and ground-glass opacities) and emphysema in 8266 participants in a study of chronic obstructive pulmonary disease (COPD) called COPDGene (recruited between October 2006 and January 2011). Additive differences in patients with emphysema with interstitial features and in those without interstitial features were analyzed by using t tests, multivariable linear regression, and Kaplan-Meier analysis. Multivariable linear and Cox regression were used to determine if interstitial features modified the effect of continuously measured emphysema on clinical measures of disease severity and mortality. Results Compared with individuals with emphysema alone, those with emphysema and interstitial features had a higher percentage predicted forced expiratory volume in 1 second (absolute difference, 6.4%; P < .001), a lower percentage predicted diffusing capacity of lung for carbon monoxide (DLCO) (absolute difference, 7.4%; P = .034), a 0.019 higher right ventricular-to-left ventricular (RVLV) volume ratio (P = .029), a 43.2-m shorter 6-minute walk distance (6MWD) (P < .001), a 5.9-point higher St George's Respiratory Questionnaire (SGRQ) score (P < .001), and 82% higher mortality (P < .001). In addition, interstitial features modified the effect of emphysema on percentage predicted DLCO, RVLV volume ratio, 6WMD, SGRQ score, and mortality (P for interaction < .05 for all). Conclusion In smokers, the combined presence of interstitial features and emphysema was associated with worse clinical disease severity and higher mortality than was emphysema alone. In addition, interstitial features

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

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Takafumi Suda

2015-01-01

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

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment.

Science.gov (United States)

Khalil, Nasreen; O'Connor, Robert

2004-07-20

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.

FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study

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Jacquelin, V. [AP-HP, Hosp. Avicenne, Department of Nuclear Medicine, Bobigny (France); Mekinian, A. [AP-HP, Hosp. Saint-Antoine, Department of Internal Medicine and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris (France); Brillet, P.Y. [AP-HP, Hosp. Avicenne, Department of Radiology, Bobigny (France); Univ. Paris 13, Sorbonne Paris Cité, Bobigny (France); Nunes, H. [AP-HP, Hosp. Avicenne, Department of Pneumology, Bobigny (France); Univ. Paris 13, Sorbonne Paris Cité, Bobigny (France); Fain, O. [AP-HP, Hosp. Saint-Antoine, Department of Internal Medicine and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris (France); Valeyre, D. [AP-HP, Hosp. Avicenne, Department of Pneumology, Bobigny (France); Univ. Paris 13, Sorbonne Paris Cité, Bobigny (France); Soussan, M., E-mail: michael.soussan@aphp.fr [AP-HP, Hosp. Avicenne, Department of Nuclear Medicine, Bobigny (France); Univ. Paris 13, Sorbonne Paris Cité, Bobigny (France)

2016-12-15

Purpose: Our study aimed to analyse the characteristics of nonspecific interstitial pneumonia (NSIP) using FDG-PET/CT (PET) and to evaluate its ability to predict the therapeutic response. Procedures: Eighteen NSIP patients were included. Maximum standardized uptake value (SUV{sub max}), FDG uptake extent (in percentage of lung volume), high resolution CT scan (HRCT) elementary lesions, and HRCT fibrosis score were recorded. The predictive value of the parameters for lung function improvement was evaluated using logistic regression and Receiver Operating Characteristic (ROC) curve analysis (n = 13/18). Results: All patients had an increased pulmonary FDG uptake (median SUV{sub max} = 3.1 [2–7.6]), with a median extent of 19% [6–67]. Consolidations, ground-glass opacities, honeycombing and reticulations showed uptake in 90%, 89%, 85% and 76%, respectively. FDG uptake extent was associated with improvement of pulmonary function under treatment (increase in forced vital capacity > 10%, p = 0.03), whereas SUV{sub max} and HRCT fibrosis score were not (p > 0.5). For FDG uptake extent, ROC analysis showed an area under the curve at 0.85 ± 0.11 and sensitivity/specificity was 88%/80% for a threshold fixed at 21%. Conclusions: Increased FDG uptake was observed in all NSIP patients, both in inflammatory and fibrotic HRCT lesions. The quantification of FDG uptake extent might be useful to predict functional improvement under treatment.

FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study

International Nuclear Information System (INIS)

Jacquelin, V.; Mekinian, A.; Brillet, P.Y.; Nunes, H.; Fain, O.; Valeyre, D.; Soussan, M.

2016-01-01

Purpose: Our study aimed to analyse the characteristics of nonspecific interstitial pneumonia (NSIP) using FDG-PET/CT (PET) and to evaluate its ability to predict the therapeutic response. Procedures: Eighteen NSIP patients were included. Maximum standardized uptake value (SUV max ), FDG uptake extent (in percentage of lung volume), high resolution CT scan (HRCT) elementary lesions, and HRCT fibrosis score were recorded. The predictive value of the parameters for lung function improvement was evaluated using logistic regression and Receiver Operating Characteristic (ROC) curve analysis (n = 13/18). Results: All patients had an increased pulmonary FDG uptake (median SUV max = 3.1 [2–7.6]), with a median extent of 19% [6–67]. Consolidations, ground-glass opacities, honeycombing and reticulations showed uptake in 90%, 89%, 85% and 76%, respectively. FDG uptake extent was associated with improvement of pulmonary function under treatment (increase in forced vital capacity > 10%, p = 0.03), whereas SUV max and HRCT fibrosis score were not (p > 0.5). For FDG uptake extent, ROC analysis showed an area under the curve at 0.85 ± 0.11 and sensitivity/specificity was 88%/80% for a threshold fixed at 21%. Conclusions: Increased FDG uptake was observed in all NSIP patients, both in inflammatory and fibrotic HRCT lesions. The quantification of FDG uptake extent might be useful to predict functional improvement under treatment.

A complex craniovertebral junction malformation in a patient with late onset glycogenosis 2

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Mariasofia Cotelli

2014-01-01

Full Text Available Glycogenosis II (GSDII is an autosomal recessive lysosomal storage disorder resulting from deficiency of acid alpha-glucosidase and subsequent lysosomal accumulation of glycogen in skeletal, cardiac and smooth muscles. The late-onset form is characterized by wide variability of the phenotypical spectrum. Clinical findings may include muscle weakness, respiratory insufficiency, vascular abnormalities, low bone mineral density and higher risk of developing osteoporosis. Craniovertebral junction (CVJ malformations have never been described so far. We here report on a GSDII 43-year-old woman who harbored the mutations IVS1-13T>G and c.2237G>A in the acid alpha-glucosidase gene. She recurrently suffered from headache, neck pain and dizziness. Brain MRI and CT scan showed the presence of a very rare complex CVJ malformation composed of basilar invagination, basiocciput hypoplasia, partial C1 assimilation, C1 posterior arch aplasia and C1 lateral mass hypoplasia and offset. Although we cannot rule out their coincidental occurrence, the rarity of multiple CVJ malformations in the general population as well as the well-known GSDII multisystem involvement should suggest to study the CVJ in the diagnostic process of GSDII patients in order to assess the CVJ malformation frequency in GSDII population and verify a possible relationship between these two conditions.

Pulmonary mineralization in four dogs with Cushing's syndrome

International Nuclear Information System (INIS)

Berry, C.R.; Ackerman, N.; Monce, K.

1994-01-01

The clinical and imaging features of four dogs with Cushing's syndrome and pulmonary mineralization are reviewed. Three dogs presented with a primary complaint of respiratory distress/dyspnea. Three dogs had pituitary dependent Cushing's syndrome, while the remaining one dog had iatrogenic Cushing's syndrome. Each dog had clinical features typical for Cushing's syndrome. Two of the dogs were euthanized due to progressive hypoxemia. In each dog, the serum calcium, phosphorous, blood urea nitrogen and creatinine were normal. A generalized increase in unstructured interstitial pulmonary opacity with diffuse mineralization was noted on thoracic radiographs of all dogs. In one dog, an ill-defined nodular interstitial pattern of mineralization was present. Delayed bone phase scintigraphy using 99m Technetium methylene diphosphonate documented generalized pulmonary uptake in two dogs. 99m Technetium labeled microaggregated albumin lung perfusion scans were normal in these two dogs. 99m Tc-MDP scintigraphy can provide useful information in diagnosing pulmonary mineralization in Cushingoid dogs

Angiotensin II type 1 and 2 receptors and lymphatic vessels modulate lung remodeling and fibrosis in systemic sclerosis and idiopathic pulmonary fibrosis.

Science.gov (United States)

Parra, Edwin Roger; Ruppert, Aline Domingos Pinto; Capelozzi, Vera Luiza

2014-01-01

To validate the importance of the angiotensin II receptor isotypes and the lymphatic vessels in systemic sclerosis and idiopathic pulmonary fibrosis. We examined angiotensin II type 1 and 2 receptors and lymphatic vessels in the pulmonary tissues obtained from open lung biopsies of 30 patients with systemic sclerosis and 28 patients with idiopathic pulmonary fibrosis. Their histologic patterns included cellular and fibrotic non-specific interstitial pneumonia for systemic sclerosis and usual interstitial pneumonia for idiopathic pulmonary fibrosis. We used immunohistochemistry and histomorphometry to evaluate the number of cells in the alveolar septae and the vessels stained by these markers. Survival curves were also used. We found a significantly increased percentage of septal and vessel cells immunostained for the angiotensin type 1 and 2 receptors in the systemic sclerosis and idiopathic pulmonary fibrosis patients compared with the controls. A similar percentage of angiotensin 2 receptor positive vessel cells was observed in fibrotic non-specific interstitial pneumonia and usual interstitial pneumonia. A significantly increased percentage of lymphatic vessels was present in the usual interstitial pneumonia group compared with the non-specific interstitial pneumonia and control groups. A Cox regression analysis showed a high risk of death for the patients with usual interstitial pneumonia and a high percentage of vessel cells immunostained for the angiotensin 2 receptor in the lymphatic vessels. We concluded that angiotensin II receptor expression in the lung parenchyma can potentially control organ remodeling and fibrosis, which suggests that strategies aimed at preventing high angiotensin 2 receptor expression may be used as potential therapeutic target in patients with pulmonary systemic sclerosis and idiopathic pulmonary fibrosis.

A case of idiopathic interstitial pneumonia

OpenAIRE

豊見山, 寛; 兼島, 洋; 下地, 克佳; 金城, 勇徳; 中富, 昌夫; 小張, 一峰; 松井, 克明; Tomiyama, Hiroshi; Kaneshima, Hiroshi; Shimoji, Katsuyoshi; Kinjo, Yutoku; Nakatomi, Masao; Kobari, Kazumine; Matsui, Katsuaki; 琉球大学医学部第一内科

1982-01-01

A case of idiopathic interstitial pneumonia was reported. A 53-year-old man was admitted to our hospital with his complaint of shortness of breath. On physical examination clubbing of the fingers was noticed and velcro rales was heard on the bilateral lower back. On laboratory data no remarkable finding was revealed. Chest roentgenogram showed diffuse reticulonodular shadow and small ring shadow of the bilateral lower lung fields. On pulmonary function test decline of VC and DLco was revealed...

Pathophysiology of Pulmonary Hypertension in Chronic Parenchymal Lung Disease.

Science.gov (United States)

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

2016-04-01

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.

Pulmonary manifestation of AIDS

International Nuclear Information System (INIS)

Blum, U.; Dinkel, E.; Laaff, H.; Wuertemberger, G.; Senn, H.; Vaith, P.; Kroepelin, T.; Freiburg Univ.; Freiburg Univ.; Freiburg Univ.; Freiburg Univ.

1989-01-01

We reviewed retrospectively the clinical records of 28 patients with AIDS staged group IV according to CDC-criteria. Among these, 19 had pulmonary disease: most of them (n=17) had pneumocystis carinii pneumonia (Pcp). 12/17 patients with proven Pcp displayed typical X-ray findings with diffuse perihilar interstitial infiltration sparing lung periphery. 3/17 had atypical features and 2 normal chest x-ray findings. These data are important to identify patients with pulmonary complications of AIDS. (orig.) [de

CT Analysis of the Anterior Mediastinum in Idiopathic Pulmonary Fibrosis and Nonspecific Interstitial Pneumonia

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Lee, Chang Hyun; Lee, Hyun Ju; Son, Kyu Ri; Chun, Eun Ju; Lim, Kun Young; Goo, Jin Mo; Im, Jung Gi [Seoul National University College of Medicine, Seoul (Korea, Republic of); Heo, Jeong Nam; Song, Jae Woo [University of Ulsan College of Medicine, Seoul (Korea, Republic of)

2006-09-15

We wanted to determine whether the amount and shape of the anterior mediastinal fat in the patients suffering with usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) was different from those of the normal control group. We selected patients who suffered with UIP (n 26) and NSIP (n = 26) who had undergone CT scans. Twenty-six controls were selected from individuals with normal CT findings and normal pulmonary function tests. All three groups (n = 78) were individually matched for age and gender. The amounts of anterior mediastinal fat, and the retrosternal anteroposterior (AP) and transverse dimensions of the anterior mediastinal fat were compared by one-way analysis of variance and Bonferroni's test. The shapes of the anterior mediastinum were compared using the Chi-square test. Exact logistic regression analysis and polychotomous logistic regression analysis were employed to assess whether the patients with NSIP or UIP had a tendency to show a convex shape of their anterior mediastinal fat. The amount of anterior mediastinal fat was not different among the three groups (p 0.175). For the UIP patients, the retrosternal AP dimension of the anterior mediastinal fat was shorter (p = 0.037) and the transverse dimension of the anterior mediastinal fat was longer (p = 0.001) than those of the normal control group. For the NSIP patients, only the transverse dimension was significantly longer than those of the normal control group (p < 0.001). The convex shape of the anterior mediastinum was predictive of NSIP (OR = 19.7, CI 3.32-{infinity}, p < 0.001) and UIP (OR = 24.42, CI 4.06-{infinity}, p < 0.001). For UIP patients, the retrosternal AP and transverse dimensions are different from those of normal individuals, whereas the amounts of anterior mediastinal fat are similar. UIP and NSIP patients have a tendency to have a convex shape of their anterior mediastinal fat.

Optical techniques in pulmonary medicine. SPIE photonics West.

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Suter, Melissa J; Lam, Stephen; Brenner, Matthew

2012-04-01

There is ongoing interest in the emerging field of pulmonary photonic-based diagnostics. Potential clinical need areas that are being actively investigated at this time include airway and peripheral lung cancer diagnostics, pulmonary parenchymal and interstitial disorders, alveolar structure function, inhalation injury, ciliary function analysis, asthma and obstructive lung diseases.

Modeling pulmonary fibrosis by abnormal expression of telomerase/apoptosis/collagen V in experimental usual interstitial pneumonia

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Parra, E.R.; Pincelli, M.S.; Teodoro, W.R.; Velosa, A.P.P.; Martins, V.; Rangel, M.P.; Barbas-Filho, J.V.; Capelozzi, V.L.

2014-01-01

Limitations on tissue proliferation capacity determined by telomerase/apoptosis balance have been implicated in pathogenesis of idiopathic pulmonary fibrosis. In addition, collagen V shows promise as an inductor of apoptosis. We evaluated the quantitative relationship between the telomerase/apoptosis index, collagen V synthesis, and epithelial/fibroblast replication in mice exposed to butylated hydroxytoluene (BHT) at high oxygen concentration. Two groups of mice were analyzed: 20 mice received BHT, and 10 control mice received corn oil. Telomerase expression, apoptosis, collagen I, III, and V fibers, and hydroxyproline were evaluated by immunohistochemistry, in situ detection of apoptosis, electron microscopy, immunofluorescence, and histomorphometry. Electron microscopy confirmed the presence of increased alveolar epithelial cells type 1 (AEC1) in apoptosis. Immunostaining showed increased nuclear expression of telomerase in AEC type 2 (AEC2) between normal and chronic scarring areas of usual interstitial pneumonia (UIP). Control lungs and normal areas from UIP lungs showed weak green birefringence of type I and III collagens in the alveolar wall and type V collagen in the basement membrane of alveolar capillaries. The increase in collagen V was greater than collagens I and III in scarring areas of UIP. A significant direct association was found between collagen V and AEC2 apoptosis. We concluded that telomerase, collagen V fiber density, and apoptosis evaluation in experimental UIP offers the potential to control reepithelization of alveolar septa and fibroblast proliferation. Strategies aimed at preventing high rates of collagen V synthesis, or local responses to high rates of cell apoptosis, may have a significant impact in pulmonary fibrosis

Modeling pulmonary fibrosis by abnormal expression of telomerase/apoptosis/collagen V in experimental usual interstitial pneumonia

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Parra, E.R.; Pincelli, M.S. [Departamento de Patologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Teodoro, W.R.; Velosa, A.P.P. [Disciplina de Reumatologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Martins, V.; Rangel, M.P.; Barbas-Filho, J.V.; Capelozzi, V.L. [Departamento de Patologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil)

2014-06-04

Limitations on tissue proliferation capacity determined by telomerase/apoptosis balance have been implicated in pathogenesis of idiopathic pulmonary fibrosis. In addition, collagen V shows promise as an inductor of apoptosis. We evaluated the quantitative relationship between the telomerase/apoptosis index, collagen V synthesis, and epithelial/fibroblast replication in mice exposed to butylated hydroxytoluene (BHT) at high oxygen concentration. Two groups of mice were analyzed: 20 mice received BHT, and 10 control mice received corn oil. Telomerase expression, apoptosis, collagen I, III, and V fibers, and hydroxyproline were evaluated by immunohistochemistry, in situ detection of apoptosis, electron microscopy, immunofluorescence, and histomorphometry. Electron microscopy confirmed the presence of increased alveolar epithelial cells type 1 (AEC1) in apoptosis. Immunostaining showed increased nuclear expression of telomerase in AEC type 2 (AEC2) between normal and chronic scarring areas of usual interstitial pneumonia (UIP). Control lungs and normal areas from UIP lungs showed weak green birefringence of type I and III collagens in the alveolar wall and type V collagen in the basement membrane of alveolar capillaries. The increase in collagen V was greater than collagens I and III in scarring areas of UIP. A significant direct association was found between collagen V and AEC2 apoptosis. We concluded that telomerase, collagen V fiber density, and apoptosis evaluation in experimental UIP offers the potential to control reepithelization of alveolar septa and fibroblast proliferation. Strategies aimed at preventing high rates of collagen V synthesis, or local responses to high rates of cell apoptosis, may have a significant impact in pulmonary fibrosis.

Clinical features of usual interstitial pneumonia with anti-neutrophil cytoplasmic antibody in comparison with idiopathic pulmonary fibrosis.

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Hosoda, Chiaki; Baba, Tomohisa; Hagiwara, Eri; Ito, Hiroyuki; Matsuo, Norikazu; Kitamura, Hideya; Iwasawa, Tae; Okudela, Koji; Takemura, Tamiko; Ogura, Takashi

2016-07-01

Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP). We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy. There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP. ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP. © 2016 Asian Pacific Society of Respirology.

Jaccoud's arthropathy and pulmonary fibrosis in CREST syndrome

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Spinel B, Nestor; Montenegro, Pablo; Rondon Federico; Restrepo, Jose F; Iglesias G, Antonio

2010-01-01

We report a case of a 48 years old patient with diagnosis of incomplete CREST syndrome (variant limited systemic sclerosis) in who we documented the presence of Jaccoud's arthropathy of the hands and pulmonary involvement by pulmonary fibrosis type usual interstitial pneumonia, with positivity for rheumatoid factor and anti-cyclic citrullinated peptide antibody.

Desquamative interstitial pneumonia: A case report

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Lovrenski Aleksandra

2014-01-01

Full Text Available Introduction. Desquamative interstitial pneumonia is one of the rarest idiopathic interstitial pneumonias and the rarest form of smoking-related interstitial lung diseases. It was first described by Liebow in 1965. Histologically, it is characterized by the presence of eosinophilic macrophages uniformly filling airspaces which often contain a finely granular light-brown pigment that does not stain for hemosiderin. The alveolar walls are usually mildly thickened by fibrous tissue and infiltrated by a moderate number of lymphocytes. Case Outline. Our patient was a 56-year-old male, heavy smoker, with bilateral lung infiltrations of unknown etiology and several months of discomfort in the form of dry cough and shortness of breath. Lung function tests showed a moderate restrictive ventilation disorder and a severe reduction of diffusing capacity. Since bronchoscopic specimens did not reveal lung lesion etiology, an open lung biopsy of the lower left pulmonary lobe was performed, and based on the obtained surgical material the pathohistologically diagnosis of desquamative interstitial pneumonia was established. The patient was started on corticosteroid and immunosuppressive therapy, and he ceased smoking. At the last control examination, two years after the onset of symptoms, the patient was feeling well, and high-resolution computed tomography (HRCT scan of the thorax showed regression of pathological changes. Conclusion. Although, as in our case, the majority of DIP patients improve on treatment, some patients still develop progressive irreversible fibrosis despite therapy.

Assessment of pulmonary ventilation patterns by nonradioactive Xe enhanced CT

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Shiraishi, Tomokuni; Mizuno, Masayoshi; Harima, Yoko; Kobayashi, Akitomo; Hasegawa, Takeo

1984-01-01

To assess the patterns of regional pulmonary ventilation in conjunction with pulmonary blood flow, pulmonary Xe enhanced CT was performed in 26 patients with interstitial disorders including 3 with chest irradiation and 23 with old myocardial infarction and in 2 control patients. Washout time was short and its patterns were not influenced by an increased or decreased blood flow in the control group. In the group with chest irradiation, a decreased blood flow and slight disturbance were seen in the affected lung, while an increased blood flow and a slightly increased resistance of the pulmonary periphery were suspected in the opposite lung. Furthermore, a prolonged washout and relative reduction of the washout seemed to be reflective of the interstitial degeneration and decreased blood flow, respectively. In the group with old myocardial infarction, the prolongation of washout became marked because of a decreased blood flow caused by venous congestion. (Namekawa, K.)

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

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William N. Rom

2011-01-01

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

Clinical Outcomes in Interstitial Lung Diseases : Measuring and improving quality of life

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M.J.G. Van Manen (Mirjam)

2017-01-01

markdownabstractInterstitial lung diseases (ILDs) contain a wide variety of disorders, usually affecting both lungs diffusely. The most common ILDs are idiopathic pulmonary fibrosis and sarcoidosis. ILDs have a major impact on quality of life. Although it is well-known that quality of life is

Pulmonary Hypertension in Parenchymal Lung Disease

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Tsangaris, Iraklis; Tsaknis, Georgios; Anthi, Anastasia; Orfanos, Stylianos E.

2012-01-01

Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases. PMID:23094153

Pulmonary tissue volume, cardiac output, and diffusing capacity in sustained microgravity

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Verbanck, S.; Larsson, H.; Linnarsson, D.; Prisk, G. K.; West, J. B.; Paiva, M.

1997-01-01

In microgravity (microG) humans have marked changes in body fluids, with a combination of an overall fluid loss and a redistribution of fluids in the cranial direction. We investigated whether interstitial pulmonary edema develops as a result of a headward fluid shift or whether pulmonary tissue fluid volume is reduced as a result of the overall loss of body fluid. We measured pulmonary tissue volume (Vti), capillary blood flow, and diffusing capacity in four subjects before, during, and after 10 days of exposure to microG during spaceflight. Measurements were made by rebreathing a gas mixture containing small amounts of acetylene, carbon monoxide, and argon. Measurements made early in flight in two subjects showed no change in Vti despite large increases in stroke volume (40%) and diffusing capacity (13%) consistent with increased pulmonary capillary blood volume. Late in-flight measurements in four subjects showed a 25% reduction in Vti compared with preflight controls (P volume, to the extent that it was no longer significantly different from preflight control. Diffusing capacity remained elevated (11%; P pulmonary perfusion and pulmonary capillary blood volume, interstitial pulmonary edema does not result from exposure to microG.

Study on the deposition patterns of aerosol inhalation scintigraphy, 1; Comparison of the deposition patterns of aerosol inhalation scintigraphy with lung function tests in pulmonary diseases

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Watanabe, Hiroyuki [Nara Medical Univ., Kashihara (Japan)

1989-06-01

The deposition patterns of aerosol inhalation scintigraphies and lung function tests were studied in 102 cases; 64 cases of obstructive pulmonary diseases (19 pulmonary emphysema, 27 diffuse panbronchiolitis, 18 chronic bronchitis) and 38 restrictive pulmonary disease (15 idiopathic interstitial pneumonia, 16 pulmonary asbestosis, 7 interstitial pneumonia due to collagen vascular disease). The deposition patterns were classified into 5 patterns (Type A:normal homogenous distribution; Type B: mildly unhomogenous distribution; Type C: severely unhomogenous distribution mingled with hot spots; Type D: non-hilar hot spots; and Type E: hilar hot spots). The deposition patterns of restrictive pulmonary diseases were markedly abnormal as well as obstructive pulmonary diseases. The deposition patterns showed mainly Types C, D and E in obstructive pulmonary diseases, Type B in restrictive pulmonary diseases. The deposition patterns showed mainly Type E in pulmonary emphysema, Types C and D in diffuse panbronchiolitis, Types A, B and C in chronic bronchitis, Type B in idiopathic interstitial pneumonia interstitial pneumonia due to collagen vascular disease, Types B and C in pulmonary asbestosis. The deposition patterns correlated well with %FEV{sub 1.0} which was a good indicator of the severity of obstructive pulmonary diseases and restrictive pulmonary diseases. Furthermore, the mean %FEV{sub 1.0} in obstructive pulmonary diseases was nearly equal to the mean %FEV{sub 1.0} in restrictive pulmonary diseases in each type of the deposition patterns. (J.P.N.).

Pulmonary manifestations in anaphylactoid purpura (Henoch-Schoenlein syndrome)

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Fiegler, W.; Siemoneit, K.D.

1981-01-01

Pulmonary manifestations in anaphylactoid purpura (Henoch-Schoenlein-syndrome). Radiological observations are reported on 2 female adults with Henoch-Schoenlein-syndrome accompanied by reversible pulmonary disease. This form of anaphylaktoid purpura has rarely been reported until now. The pulmonary opacities are regarded as intraalveolar bleeding probably with edem and interstitial perivascular infiltrations. They developed at the same time as the skin lesions and are probably part of the disease. These pulmonary changes are characterized by the following radiological criteria: 1. parahilar, butterfly-shaped opacities, 2. air-bronchograms, 3. reticulo-nodular pattern, 4. rapid change. (orig.) [de

Pulmonary Talcosis in an Immunocompromised Patient

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Thanh-Phuong Nguyen

2016-01-01

Full Text Available The first case of pulmonary talcosis or talc pneumoconiosis related to inhalation of talc during its extraction and processing in mines was described by Thorel in 1896. Pulmonary talcosis is most commonly seen secondary to occupational exposure or intravenous (IV drug abuse and, occasionally, in excessive use of cosmetic talc. Based on literature review, there has been an increase in reported incidents of pulmonary talcosis due to various forms of exposure to the mineral. We report an 82-year-old man who is diagnosed with Philadelphia chromosome positive pre-B cell acute lymphoblastic leukemia (ALL treated with palliative imatinib who presented with chronic hemoptysis and dyspnea shortly after his diagnosis. His symptoms were initially thought to be due to an infectious etiology due to his malignancy, immunocompromised state, and radiographic findings until high-resolution computerized tomographic (HRCT findings showed a diffuse pulmonary fibrosis picture that prompted further questioning and a more thorough history inquiry on his exposure to causative agents of interstitial lung disease. Very often, patients do not recognize their exposure, especially in those whose exposure is unrelated to their occupation. Our case emphasizes the need for thorough and careful history taking of occupational and nonoccupational exposure to known causative agents of interstitial lung disease.

Radiological case. Pulmonary Lymphangioleiomyomatosis

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Rivera Bernal, Aura Lucia; Carrillo Bayona, Jorge Alberto; Ojeda Leon, Paulina

2004-01-01

Lymphangioleiomyomatosis is a rare disorder, which affects principally the pulmonary parenchyma of young women at a reproductive age, and is pathologically characterized by the interstitial proliferation of smooth muscle and formation of cysts in the lung. We present the case of a 35-year-old woman that has a lymphangioleiomyomatosis diagnosis

A syndrome of severe idiopathic pulmonary parenchymal disease with pulmonary hypertension in Pekingese

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Köster LS

2016-02-01

Full Text Available Liza S Köster,1 Robert M Kirberger2 1Section of Medicine, Department of Clinical Sciences, Integrative Mammalian Research (IMR Center, Ross University School of Veterinary Medicine (RUSVM, Basseterre, St Kitts, West Indies; 2Diagnostic Imaging Section, Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Onderstepoort, South Africa Abstract: This paper describes 35 Pekingese dogs with a syndrome characterized by dyspnea, cyanosis, episodic syncope, soft pulmonary “Velcro” crackles, pulmonary hypertension (PH, and computed tomography and radiographic changes consistent with pulmonary parenchymal disease. The medical data base was searched with the criteria “Pekingese” and “syncope” or “dyspnea” or “tachypnea” or “pulmonary hypertension”, over a 36-month period. Inclusion criteria were echocardiographic changes consistent with noninvasive diagnosis of PH, either subjectively by B-mode or objectively by Doppler. Dogs were excluded (n=106 if there were insufficient or poor-quality radiographic or echocardiographic records or if diseases other than chronic pulmonary disease were found to be the etiology. The records of 35 dogs met these criteria and presented with a respiratory crises preceded by a history of chronic exercise intolerance and episodic syncope. The average age was 14.5 years (range: 7–19 years, with 21 males and 14 females. Most of the dogs had an interstitial lung pattern with radiographic evidence of right heart enlargement. There was a 77% (n=27 mortality and a median survival of 60 days (interquartile range: 9–210 days. This study highlights a cor pulmonale syndrome from PH due to chronic pulmonary parenchymal disease, with a grave prognosis, in middle-aged to geriatric population of Hong Kong Pekingese. Keywords: computed tomography, interstitial lung disease, dog, syncope

Clinical Management of Acute Interstitial Pneumonia: A Case Report

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Yang Xia

2012-01-01

Full Text Available We describe a 51-year-old woman who was admitted to hospital because of cough and expectoration accompanied with general fatigue and progressive dyspnea. Chest HRCT scan showed areas of ground glass attenuation, consolidation, and traction bronchiectasis in bilateral bases of lungs. BAL fluid test and transbronchial lung biopsy failed to offer insightful evidence for diagnosis. She was clinically diagnosed with acute interstitial pneumonia (AIP. Treatment with mechanical ventilation and intravenous application of methylprednisolone (80 mg/day showed poor clinical response and thus was followed by steroid pulse therapy (500 mg/day, 3 days. However, she died of respiratory dysfunction eventually. Autopsy showed diffuse alveolar damage associated with hyaline membrane formation, pulmonary interstitial, immature collagen edema, and focal type II pneumocyte hyperplasia.

Effects of irradiation on the pulmonary hemodynamics and the pulmonary vascular permeability

International Nuclear Information System (INIS)

Ohkuda, Kazuhiro; Watanabe, Shinkichi; Okada, Shinichiroh

1982-01-01

In 4 sheeps, base lines of hemodynamics and lymph dynamics were observed for 2 hours, and then 1,000 rad of 60 Co was irradiated to the inferior lobes of the lung. Pulmonary hemodynamics and lymph dynamics were continuously observed, and water and protein permeability of the irradiated pulmonary vessels was evaluated. In 4 control sheeps, no change in pulmonary hemodynamics and lymph dynamics was noted. In the irradiated group, there was no remarkable change in pulmonary hemodynamics for 6 to 8 hours after 60 Co irradiation. Pulmonary lymph flow began to increase 2 hours after irradiation to about 1.7 times the base line level after 4 hours. The increase in pulmonary lymph flow was accompanied by decrease in plasma protein concentration and increase in protein concentration of the lung lymph, resulting in an apparent increase in the ratio of lymph/plasma protein concentration. Water and protein leak from the pulmonary vessels increased. A photomicroscopic observation revealed dilatation of the lymphatic vessels in the lung interstice and a mild pulmonary interstitial edema. Vascular damage, especially due to increased water and protein permeability of the lung capillary vessels, occurred immediately after 60 Co irradiation. (Ueda, J.)

Idiopathic pulmonary fibrosis in a Staffordshire bull terrier with hypothyroidism.

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Corcoran, B M; Dukes-McEwan, J; Rhind, S; French, A

1999-04-01

Radiographic evidence of chronic interstitial lung changes, usually believed to be attributable to lung fibrosis, is readily recognised in canine practice. Furthermore, there is a body of anecdotal evidence suggesting that a specific clinical entity consistent with chronic lung fibrosis occurs in specific breeds of terrier dogs. However, there is little pathological data to confirm these radiographic and clinical findings and, therefore, chronic interstitial lung disease of dogs is poorly characterised. In this report, a case of chronic pulmonary fibrosis is described in which histopathological confirmation was possible, and suggested that the condition might be analogous to idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) in humans.

Pulmonary fibrosis in rheumatoid arthritis: a review of clinical features and therapy.

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Roschmann, R A; Rothenberg, R J

1987-02-01

During the past four decades there has been a growing appreciation of the frequency of pulmonary abnormalities associated with RA. Approximately 30% to 40% of patients with RA demonstrate either radiographic or pulmonary function abnormalities indicative of interstitial fibrosis or restrictive lung disease. The severity of pulmonary fibrosis is not associated with rheumatologic symptoms or the duration of the associated RA, nor is there any clear relation to the extraarticular features of RA or serologic findings. Survival rates in patients with coexisting RA and pulmonary fibrosis are similar to those of patients with idiopathic pulmonary fibrosis. However, the spectrum of disease activity is quite variable. The majority of patients with progressive pulmonary symptomatology, when treated with corticosteroids, will have equivocal results. Some patients appear to respond to immunosuppressive or cytotoxic medications. The role of macrophages may be central to the injury to lung. Recent studies suggest a potential treatment role for cyclosporine, which may be able to interrupt lymphocyte-stimulated macrophage activation, and thus, fibroblast-mediated fibrosis in patients with pulmonary interstitial fibrosis. Bronchoalveolar lavage studies may delineate subgroups of patients who are more likely to respond to immunosuppressive agents, especially when treatment is started early.

Negative-Pressure Pulmonary Edema.

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Bhattacharya, Mallar; Kallet, Richard H; Ware, Lorraine B; Matthay, Michael A

2016-10-01

Negative-pressure pulmonary edema (NPPE) or postobstructive pulmonary edema is a well-described cause of acute respiratory failure that occurs after intense inspiratory effort against an obstructed airway, usually from upper airway infection, tumor, or laryngospasm. Patients with NPPE generate very negative airway pressures, which augment transvascular fluid filtration and precipitate interstitial and alveolar edema. Pulmonary edema fluid collected from most patients with NPPE has a low protein concentration, suggesting hydrostatic forces as the primary mechanism for the pathogenesis of NPPE. Supportive care should be directed at relieving the upper airway obstruction by endotracheal intubation or cricothyroidotomy, institution of lung-protective positive-pressure ventilation, and diuresis unless the patient is in shock. Resolution of the pulmonary edema is usually rapid, in part because alveolar fluid clearance mechanisms are intact. In this review, we discuss the clinical presentation, pathophysiology, and management of negative-pressure or postobstructive pulmonary edema. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

The Evaluation of Interstitial Abnormalities in Group B of the 2011 Global Initiative for Chronic Obstructive Lung Disease (GOLD) Classification of Chronic Obstructive Pulmonary Disease (COPD).

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Ohgiya, Masahiro; Matsui, Hirotoshi; Tamura, Atsuhisa; Kato, Takafumi; Akagawa, Shinobu; Ohta, Ken

2017-10-15

Objective In 2011, the Global Initiative for Chronic Obstructive Lung Disease (GOLD) classification categorized chronic obstructive pulmonary disease (COPD) patients into 4 groups. A report demonstrated that the mortality in Group B was higher than that in Group C. Ischemic heart disease and cancer were suggested to be the cause. The aim of the present study was to test the hypothesis that interstitial lung abnormalities (ILAs) are more prevalent in Group B than Group C and that they may be responsible for the higher mortality in Group B. Methods Patients were selected based on their pulmonary function test results. The inclusion criterion was a forced expiratory volume in 1 second (FEV 1 )/forced vital capacity (FVC) of COPD patients, ILAs were detected in 10.3% of the patients in Group A, 22.5% of the patients in Group B, 5.6% of the patients in Group C, and 23.1% of the patients in Group D. In Group B, the frequency of ILAs was significantly higher and the area affected by the ILAs was significantly greater in comparison to Group C. Among the patterns of interstitial abnormalities, the area of honeycombing in Group B was significantly greater than that in Group C. Furthermore, among the patients in Group B, the LAA in the ILA-positive patients was significantly greater than that in the ILA-negative patients. Conclusion In Group B, the area occupied by ILAs-especially honeycombing-was greater than that in Group C. This contributed to the preserved %FEV 1 and possibly to the poorer prognosis of the patients in Group B.

Spontaneous pneumothorax associated with pulmonary fibrosis in a patient with neurofibromatosis type 2

International Nuclear Information System (INIS)

Alcala Cerra, Gabriel; Moscote-Salazar, Luis Rafael; Lozano Tagua, Carlos Fernando; Sabogal Barrios, Ruben

2010-01-01

Pulmonary involvement in patients with neurofibromatosis has been repetitively reported as a very rare complication in type 1 variety. It is characterized by pulmonary interstitial disease, pulmonary fibrosis and bullaes, the last with high risk of rupture. We described a case of spontaneous pneumothorax in a patient with type 2 neurofibromatosis, as consequence of pulmonary fibrotic changes. To our knowledge this association had not been reported.

Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design?

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Nathan SD

2014-07-01

Full Text Available Steven D Nathan, Christopher S King Advanced Lung Disease Clinic, Inova Fairfax Hospital, Falls Church, VA, USA Abstract: Idiopathic pulmonary fibrosis (IPF is a disease that carries a high mortality. Pulmonary hypertension (PH frequently complicates the course of patients with IPF and is associated with significantly worse outcomes. Whether PH is a surrogate or driver of these worse outcomes remains unanswered, but the presence of PH represents an attractive target for therapy. This review delves into the various pulmonary vasoactive agents that have been subjected to study in IPF, the pitfalls of some of these prior studies, and attempts to lay a foundation for future study designs targeting PH in IPF. Keywords: phenotype, interstitial lung disease

Purine Bases in Blood Plasma of Patients with Chronic Pulmonary Diseases

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Larissa E. Muravluyova

2012-09-01

Full Text Available The article is focused on the study of purine bases and intermediates of purine catabolism in plasma of patients with chronic obstructive bronchitis and idiopathic interstitial pneumonia. Decrease of adenine and hypoxantine in plasma of patients with idiopathic interstitial pneumonia was registered. Increase of guanine in plasma of patients with chronic obstructive pulmonary disease was established.

Idiopathic pulmonary fibrosis: evolving concepts.

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Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H

2014-08-01

Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000. Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

Pulmonary Alveolar Proteinosis in Association with Congenital Dyserythropoietic Anemia: A Case Report

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Marcus A. Carden

2012-01-01

Full Text Available A two-year-old girl with congenital dyserythropoietic anemia (CDA acutely developed fever, tachypnea, and increased oxygen requirement. Chest X-ray revealed bilateral interstitial infiltrates and mild cardiomegaly. Blood cultures grew no infectious agents, while pulmonary specimens grew cytomegalovirus (CMV. Treatment with intravenous ganciclovir was initiated but without response. Final cytologic preparations of bronchoalveolar lavage (BAL fluid revealed eosinophilic amorphous material consistent with pulmonary alveolar proteinosis (PAP. CDA and PAP are extremely rare disorders in pediatrics. PAP should be considered in patients with hematological disorders who present with acute interstitial pneumonia, after infectious causes are ruled out.

Sirolimus-associated interstitial pneumonitis in a liver transplant recipient

International Nuclear Information System (INIS)

Claire Berrouet, Marie; Aristizabal, Julian Miguel; Restrepo, Juan Carlos; Correa, Gonzalo

2005-01-01

Sirolimus is an immunosuppressive drug that has been used during the past few years. Sirolimus is indicated in rescue therapies and to reduce the secondary toxic effects of calcineurin inhibitors. This drug has been associated with infrequent but severe pulmonary toxicity. Cases of interstitial pneumonitis, bronchiolitis obliterans with organizing pneumonia, and alveolar proteinosis have been described. We describe a case of pulmonary toxicity associated with the use of sirolimus in a 59-yr-old liver transplant recipient. We also review all reported cases of sirolimus-associated lung toxicity among liver transplantation recipients, with the intention of understanding the risk factors, the clinical picture and the outcomes of this complication. Five cases have been reported since January 2000, including the present case. Clinical presentation is similar, with fever, dyspnoea, fatigue, cough, and hemoptysis. Discontinuation of the drug led to resolution of clinical and radiographic findings. Sirolimus-induced pulmonary toxicity is a serious condition and should be considered in the differential diagnosis of liver recipients presenting with respiratory findings. Discontinuation of the drug is associated with resolution of the pulmonary compromise

Subclinical interstitial lung involvement in rheumatic diseases. Correlations of high-resolution Computed Tomography patterns with functional and cytologic findings

International Nuclear Information System (INIS)

Salaffi, F.; Baldelli, S.

1999-01-01

The aims of this study were to quantify the severity and extent of subclinical interstitial lung disease as depicted on HRCT and to study the relationship between the patterns of lung disease quantified by HRCT and the functional parameters and bronchoalveolar lavage findings in patients with rheumatic diseases. The results confirm that HRCT is a sensitive tool in detecting interstitial lung disease in patients with rheumatic diseases with no signs and symptoms of pulmonary involvement. The relationship between the different HRCT patterns and bronchoalveolar lavage cell profiles can identify patients at higher risk of developing irreversible lung fibrosis. A long-term, prospective follow-up study is needed to determine whether these patients will develop over pulmonary disease [it

020. Coexistence of lung adenocarcinoma and usual interstitial pneumonia: a case report

Science.gov (United States)

Baliaka, Aggeliki; Papaemmanouil, Styliani; Spyratos, Dionysis; Zarogoulidis, Paul; Sakkas, Leonidas

2015-01-01

Background Usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia of unknown cause. The most common symptoms are progressively increased shortness of breath and dry cough. Some studies suggest an association between usual interstitial pneumonia and lung cancer through different pathogenetic mechanisms. Objective The case presentation of a patient with lung adenocarcinoma and UIP. Methods A 66-year-old male presented with persistent dry cough, hemoptysis and dyspnea. The chest radiographs revealed a mass in the lower lobe of the left lung, measuring 3 cm, as well as diffuse interstitial changes in the same lobe. Two partial lobectomies were performed. Results Histological examination of the mass showed moderately differentiated adenocarcinoma, focally with bronchoalveolar pattern (Immunohistochemical detection of EGFR: positive). The rest lung parenchyma presented histological appearance of UIP. Conclusions According to clinicopathological studies, the prevalence of lung cancer among patients with UIP/IPF varies between 4% and 9%. The overall median survival of IPF-Ca patients is seven months in comparison with IPF only patients (14 months).

Neonatal Pulmonary Hemosiderosis

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Boris Limme

2014-01-01

Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage. The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images is strongly suggestive.

Increased mortality by septicemia, interstitial pneumonitis and pulmonary fibrosis among bone marrow transplant recipients receiving an increased mean dose rate of total irradiation

International Nuclear Information System (INIS)

Ringden, O.; Baaryd, I.; Johansson, B.

1983-01-01

Seven bone marrow transplant recipients with acute lymphoblastic leukemia receiving a mean dose rate of 0.07 Gy/min of total body irradiation towards the pelvic midpoint and the lungs had an increased (p<0.01) overall death rate of 86 per cent compared with 33 per cent among 27 patients with acute non-lymphoblastic leukemia or acute lymphoblastic leukemia treated with a mean dose rate of 0.04 Gy/min. Among the patients receiving the higher dose rate there was an increased mortality in causes related to radiation toxicity like early septicemia, interstitial pneumonitis and pulmonary fibrosis, compared with all patients receiving the lower dose rate (p<0.01) and also with 10 patients from this group with acute lymphoblastic leukemia (p<0.02). (Auth.)

Need for a cooperative study: Pulmonary Langerhans cell histiocytosis and its management in adults

NARCIS (Netherlands)

McClain, Kenneth L.; Gonzalez, Jorge Mario; Jonkers, Rene; de Juli, Emanuela; Egeler, Maarten

2002-01-01

BACKGROUND: Pulmonary involvement with Langerhans cell histiocytosis (LCH, formerly known as histiocytosis-X) presents as an interstitial process in children and adults either with or without symptoms. In contrast to other manifestations of LCH, most patients with pulmonary disease are adults.

Interstitial lung illness: Discoveries in x-ray of the thorax vs computerized tomography of high resolution

International Nuclear Information System (INIS)

Patino O, Maria Olga; Gomez Ramirez, Rafael

1993-01-01

This study wants to prove the advantages of the computerized axial tomography (TAC) of high resolution on the conventional radiography and the follow-up of the patients with interstitial pulmonary disease (IPD). 29 patients were studied with conventional RX and TAC with 17. $ % of high resolution which had normal radiography with clear abnormalities in the TAC of high resolution, the type of interstitial pulmonary disease (IPD). In the 68,9% of the cases it showed abnormality as well in TAC as in conventional RX. In the fact, that the TAC does not allow a specific etiologic diagnosis of the IPD type, as it is done in the biopsy. It allows identifying abnormality in those patients apparently normal by the radiography of the thorax and when it already exists the histopathologic diagnosis it allows the follow-up in a no invasive way

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

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Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

2015-01-01

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

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José Baddini-Martinez

2015-10-01

Full Text Available Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

Pleuro-pulmonary abnormalities in patients with systemic lupus erythematosus assessment with high resolution computed tomography, chest radiography and pulmonary function tests

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Sant, S.M.; Doran, M.; Fenelon, H.M.; Breatnach, E.S. [University of Michigan (United States)

1998-04-01

The objective has to assess the nature of pleuro-pulmonary abnormalities, with particular reference to interstitial lung disease (ILD), in patients with systemic lupus erythematosus (SLE) In conclusion, HRCT is more sensitive than PFTs or CXR in the evaluation of pleuro-pulmonary disease in SLE. We report an unusually high prevalence of HRCT appearances suggestive of ILD in patients with SLE. subclinical lung disease is common in patients with SLE. (author)

Pleuro-pulmonary abnormalities in patients with systemic lupus erythematosus assessment with high resolution computed tomography, chest radiography and pulmonary function tests

International Nuclear Information System (INIS)

Sant, S.M.; Doran, M.; Fenelon, H.M.; Breatnach, E.S.

1998-01-01

The objective has to assess the nature of pleuro-pulmonary abnormalities, with particular reference to interstitial lung disease (ILD), in patients with systemic lupus erythematosus (SLE) In conclusion, HRCT is more sensitive than PFTs or CXR in the evaluation of pleuro-pulmonary disease in SLE. We report an unusually high prevalence of HRCT appearances suggestive of ILD in patients with SLE. subclinical lung disease is common in patients with SLE. (author)

Bosutinib induced pleural effusions: Case report and review of tyrosine kinase inhibitors induced pulmonary toxicity

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Natalia I. Moguillansky, MD

2017-01-01

Full Text Available Tyrosine kinase inhibitors are known to cause pulmonary complications. We report a case of bosutinib related bilateral pleural effusions in a patient with chronic myeloid leukemia. Characteristics of the pleural fluid are presented. We also discuss other tyrosine kinase inhibitors induced pulmonary toxicities, including pulmonary hypertension and interstitial lung disease.

Lung lobar volume in patients with chronic interstitial pneumonia

International Nuclear Information System (INIS)

Harada, Hisao; Koba, Hiroyuki; Saitoh, Tsukasa; Abe, Shosaku.

1997-01-01

We measured lung lobar volume by using helical computed tomography (HCT) in 23 patients with idiopathic interstitial pneumonia (IIP), 7 patients with chronic interstitial pneumonia associated with collagen vascular disease (CVD-IP), and 5 healthy volunteers HCT scanning was done at the maximal inspiratory level and the resting end-expiratory level. To measure lung lobar volume, we traced the lobar margin on HCT images with a digitizer and calculated the lobar volume with a personal computer. The lower lobar volume and several factors influencing it in chronic interstitial pneumonia were studied. At the maximal inspiratory level, the lower lobar volume as a percent of the whole lung volume was 46.8±4.13% (mean ± SD) in the volunteers, 39.5±6.19% in the patients with IIP, and 27.7±7. 86% in the patients with CVD-IP. The lower lobar volumes in the patients were significantly lower than in the volunteers. Patients with IIP in whom autoantibody tests were positive had lower lobar volumes that were very low and were similar to those of patients with CVD-IP. These data suggest that collagen vascular disease may develop in patients with interstitial pneumonia. The patients with IIP who had emphysematous changes on the CT scans had smaller decreases in total lung capacity and lower ratios of forced expiratory volume in one second to forced vital capacity than did those who had no emphysematous changes, those two groups did not differ in the ratio of lower lobar volume to whole lung volume. This suggests that emphysematous change is not factor influencing lower lobar volume in patients with chronic interstitial pneumonia. We conclude that chronic interstitial pneumonia together with very low values for lower lobar volume may be a pulmonary manifestation of collagen vascular disease. (author)

Pulmonary scintigraphy using 197HgCl2 and pulmonary perfusion scintigraphy in bronchopulmonary diseases

International Nuclear Information System (INIS)

Fujii, Tadashige; Kanai, Hisakata; Handa, Kenjiro; Kusama, Shozo

1981-01-01

75 patients with pulmonary tuberculosis and 106 patients with bronchopulmonary diseases whose chest x-rays showed diffuse shadows were studied. Pulmonary scintigraphy using 197 HgCl 2 was useful for the diagnosis of the localization and the activity of pulmonary tuberculosis, because 197 HgCl 2 readily accumulated in the foci, and its accumulation rate was related to the activity of the foci. 197 HgCl 2 also accumulated markedly in foci of pneumoconiosis, especially, in areas showing large shadows and foci suspected to be tuberculosis. 197 HgCl 2 also accumulated in areas of chronic bronchitis, diffuse interstitial pneumonia and bronchiectasis. Its accumulation was considered to have a relation to the activity of inflammation. In primary pulmonary carcinoma, 197 HgCl 2 accumulated most markedly, in the primary lesions. 197 HgCl 2 also accumulated in metastatic or invasion areas of the hilus and the mediastinum. It accumulated in intrapulmonary metastatic foci of pulmonary carcinoma and multiple metastatic pulmonary tumors, but it was difficult to differentiate these diseases from other pulmonary diseases. In selected cases, it was useful to use pulmonary scintigraphy using 197 HgCl 2 together with pulmonary perfusion scintigraphy for the diagnosis of diffuse bronchopulmonary diseases. (Tsunoda, M.)

Childhood AIDS: pulmonary involvement. Clinico radiological correlation

International Nuclear Information System (INIS)

Cinta, C.; Muro, D.; Perez, A.; Otero, M.C.

1997-01-01

To determine the different radiological findings in the lung of children presenting human ummunodeficiency virus (HIV) infection. We assess the different radiological patterns and their prevalence. The study deals with 58 children presenting HIV infection. All underwent plain chest radiography at some time during the course of their disease to determine the cause of different respiratory symptoms (dyspnea, fever, cough, etc.).Bronchial lavage was performed in all the children who required intubation due to severe respiratory distress. The results of chest X-ray were normal in 29 children despite the existence of pulmonary symptomatology. The other 29 patients presented pulmonary, including 21 cases of pneumonia (36,2%) in 16 patients, in seven of whom the pathogen was determined by blood culture. Four cases of Pneumocystis carinii (6.89%) were diagnosed by means of bronchial lavage. There were nine children with interstitial pneumonitis, five of the lym-phoid variant (8.62%) and four of unknown origin (6.89%). In contrast to the findings in adults, we have had no cases of pulmonary tuberculosis among our patients The rates of morbidity and mortality among HIV-infected children are associated with lung involvement. However, the prevalence has decreased progressively since the introduction of antiviral agents. Recurrent bacterial pneumonia and opportunistic Pneumocystis carinii infection are the most common types of acute pulmonary involvement. Interstitial pneumonitis usually develops a chronic course. (Author) 15 refs

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

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

2006-03-01

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

Interstitial lung disease induced by fluoxetine: Systematic review of literature and analysis of Vigiaccess, Eudravigilance and a national pharmacovigilance database.

Science.gov (United States)

Deidda, Arianna; Pisanu, Claudia; Micheletto, Laura; Bocchetta, Alberto; Del Zompo, Maria; Stochino, Maria Erminia

2017-06-01

We investigated a pulmonary adverse drug reaction possibly induced by fluoxetine, the Interstitial Lung Disease, by performing a systematic review of published case reports on this subject, a review of the World Health Organization VigiAccess database, of the European EudraVigilance database and of a national Pharmacovigilance database (Italian Pharmacovigilance Network). The research found a total of seven cases linking fluoxetine to Interstitial Lung Disease in the literature. 36 cases of interstitial lung disease related to fluoxetine were retrieved from the VigiAccess database (updated to July 2016), and 36 reports were found in EudraVigilance database (updated to June 2016). In the Italian Pharmacovigilance database (updated to August 2016), we found only one case of Interstitial Lung Disease, codified as "pulmonary disease". Our investigation shows that fluoxetine might be considered as a possible cause of Interstitial Lung Disease. In particular, although here we do not discuss the assessment of benefits and harms of fluoxetine, since this antidepressant is widely used, our review suggests that fluoxetine-induced Interstitial Lung Disease should be considered in patients with dyspnea, associated or not with dry cough, who are treated with this drug. An early withdrawn of fluoxetine could be useful to obtain a complete remission of this adverse drug reaction and special attention should be particularly devoted to long-term therapy, and to female and elderly patients. Although the spontaneous reporting system is affected by important limitations, drug post- marketing surveillance represents an important tool to evaluate the real world effectiveness and safety of drugs. Copyright © 2017 Elsevier Ltd. All rights reserved.

Interstitial nephritis.

Science.gov (United States)

Papper, S

1980-01-01

There are many causes of interstitial nephritis other than pyelonephritis. The term interstitial nephritis does not connote a single etiologic or pathogenetic mechanism; it rather arbitrarily places together a wider variety of renal diseases that have a predilection for early and major involvement of the renal interstitium. The prototype of acute interstitial nephritis is acute pyelonephritis. In addition, there is a drug-related acute interstitial disease that is probably of immunological nature and usually reverses with discontinuance of the offending drug. Chronic interstitial nephritis includes many diverse illnesses. Nonobstructive pyelonephritis occurs but its prevalence is debated. Analgesic abuse nephropathy is not rare and is potentially reversible. Papillary necrosis has many causes and a wide spectrum of clinical presentations. Heavy metals, such as lead, cause interstitial nephritis. Balkan nephropathy occurs in an endemic area and although not bacterial in origin is of unknown cause.

The COPD Assessment Test as a Prognostic Marker in Interstitial Lung Disease

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Fujiko Someya

2016-01-01

Full Text Available The chronic obstructive pulmonary disease (COPD Assessment Test (CAT, which was developed to measure the health status of patients with COPD, was applied to patients with interstitial lung disease, aiming to examine the CAT as a predictor of outcome. Over a follow-up period of more than one year, 101 consecutive patients with interstitial lung disease were evaluated by the CAT. The CAT scores of 40 in total were categorized into four subsets according to the severity. Patients with higher (more severe scores exhibited lower forced vital capacity and lung diffusion capacity for carbon monoxide. The survival rate was significantly lower in patients with higher scores (log-rank test, P = 0.0002, and the hazard ratios for death of the higher scores and lower lung diffusion capacity for carbon monoxide were independently significant. These findings suggest that CAT can indicate the risk of mortality in patients with interstitial lung disease.

Effect of radioiodine therapy on pulmonary metastases from well-differentiated thyroid cancer

International Nuclear Information System (INIS)

Yu Yongli; Jin Changqing; Chen Libo; Lu Hankui; Zhu Ruisen

2002-01-01

Objective: To investigate effect of radioiodine therapy on pulmonary metastases from well-differentiated thyroid cancer. Methods: Fifty-eight cases of pulmonary metastases were studied utilizing chest X-ray, pulmonary function test and 99 Tc m -DTPA aerosol clearance examination. Results: The data of pulmonary function test were of no difference between groups of various radiation doses cumulation (P > 0.05). The differences of vital capacity (VC), fast vital capacity (FVC) and forced expiratory volume in one second (FEV 1 ) were not remarkable between the two groups of male or female patients (P > 0.05), but the difference of maximal volume ventilation (MVV) was P 99 Tc m -DTPA aerosol clearance rates between various groups were of no difference (P > 0.05). Only for one female, 43 years old who had received an accumulative dose of 51.8 GBq radioiodine over an 8-yr period, her pulmonary function test showed evidence of restrictive lung disease, chest X-ray showed haziness and DTPA half-time clearance was 26.4 min, which is suggestive of interstitial fibrosis. Conclusion: The cumulative dose of radioiodine and gender of the patients had not statistically significant effects on the pulmonary function. Interstitial fibrosis must be carefully watched when the cumulative radioiodine dose is over 37 GBq

The relationship between ventilatory lung motion and pulmonary perfusion shown by ventilatory lung motion imaging

International Nuclear Information System (INIS)

Fujii, Tadashige; Tanaka, Masao; Nakatsuka, Tatsuya; Yoshimura, Kazuhiko; Hirose, Yoshiki; Hirayama, Jiro; Kobayashi, Toshio; Handa, Kenjiro

1991-01-01

Using ventilatory lung motion imaging, which was obtained from two perfusion lung scintigrams with 99m Tc-macroaggregated albumin taken in maximal inspiration and maximal expiration, the lung motion (E-I/I) of the each unilateral lung was studied in various cardiopulmonary diseases. The sum of (E-I)/I(+) of the unilateral lung was decreased in the diseased lung for localized pleuropulmonary diseases, including primary lung cancer and pleural thickening, and in both lungs for heart diseases, and diffuse pulmonary diseases including diffuse interstitial pneumonia and diffuse panbronchiolitis. The sum of (E-I)/I(+) of the both lungs, which correlated with vital capacity and PaO 2 , was decreased in diffuse interstitial pneumonia, pulmonary emphysema, diffuse panbronchiolitis, primary lung cancer, pleural diseases and so on. (E-I)/I(+), correlated with pulmonary perfusion (n=49, r=0.51, p 81m Kr or 133 Xe (n=49, r=0.61, p<0.001) than pulmonary perfusion. The ventilatory lung motion imaging, which demonstrates the motion of the intra-pulmonary areas and lung edges, appears useful for estimating pulmonary ventilation of the perfused area as well as pulmonary perfusion. (author)

Pulmonary complications in renal transplantation

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Choi, Jung Bin; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Lee, Seung Rho; Hahm, Chang Kok; Joo, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of)

2003-04-01

To evaluate the radiographic and CT findings of pulmonary complications other than pulmonary edema arising from renal transplantation. Among 393 patients who had undergone renal transplantation at our hospital during a previous ten-year period, 23 with pulmonary complications other than pulmonary edema were included in this study. The complications involved were infection caused by CMV (n=6), bacteria (n=4), fungus (n=4), tuberculosis (n=2), varicella (n=1) or chlamydia (n=1), and malignancy involving lung cancer (n=4) or Kaposi's sarcoma (n=1). Two chest radiologists reviewed all images. The complications manifesting mainly as pulmonary nodules were lung cancer (4/4), tuberculosis (1/2), and Kaposi's sarcoma (1/1). Pulmonary consolidation was a main feature in bacterial infection (4/4), fungal infection (3/4), tuberculosis (1/2), chlamydial infection (1/1), and varicellar pneumonia (1/1). Ground-glass attenuation was a main CT feature in CMV pneumonia (4/6), and increased interstitial making was a predominant radiographic feature in CMV pneumonia (2/6). The main radiologic features described above can be helpful for differential diagnosis of the pulmonary complications of renal transplantation.

Surfactant gene polymorphisms and interstitial lung diseases

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Pantelidis Panagiotis

2001-11-01

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

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

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

1997-03-31

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

High resolution computed tomographic features of pulmonary alveolar microlithiasis

International Nuclear Information System (INIS)

Deniz, Omer; Ors, Fatih; Tozkoparan, Ergun; Ozcan, Ayhan; Gumus, Seyfettin; Bozlar, Ugur; Bilgic, Hayati; Ekiz, Kudret; Demirci, Necmettin

2005-01-01

Background: Pulmonary alveolar microlithiasis (PAM) is a rare, chronic lung disease with unknown etiology and with a nonuniform clinical course. Nonuniformity of clinical course might be related to the degree of pulmonary parenchymal alterations, which can be revealed with high resolution computed tomography (HRCT). However, HRCT findings of PAM were not fully described in the current literature. Aim: The aim of this study was to interpret and to contribute to describe HRCT findings of PAM and to investigate a correlation between profusion of micro nodules (MN) and pulmonary parenchymal alterations in patients with PAM. Material and methods: Ten male patients with PAM (mean age: 22 ± 3.2) were included into the study. HRCT images were assessed for patterns, distribution, and profusion of pulmonary abnormalities. Dividing the lungs into three zones, profusion of abnormalities was assessed. A profusion score (1-4) was given and the scores of each zone were then summed to obtain a global profusion score for HRCT ranging from 0 to 12. Also a parenchymal alteration score (PAS) was defined with respect to profusion of abnormalities. Chest X-rays were also scored. Results: All of ten patients with PAM had findings of interstitial lung disease in varying degrees on their HRCTs. HRCT findings of patients with PAM were as following: MN, parenchymal bands (PB), ground glass opacity (GGO) and, sub pleural interstitial thickening (SPIT) in 10 patients; interlobular septal thickening (ILST), in 9 patients; paraseptal emphysema (PSA) in 8 patients; centrilobular emphysema (CLA) in 7 patients; bronchiectasis (BE), confluent micro nodules (CMN) in 6 patients; peri bronchovascular interstitial thickening (PBIT) in 5 patients; panacinar emphysema (PANAA) in 3 patients; pleural calcification (PC) in 2 patients. A significant correlation between MN scores and PAS (r = 0.68, p = 0.031, MN scores and GGO scores (r = 0.69, p = 0.027) and, MN scores and CLA scores (r = 0.67, p = 0

Unilateral lung transplantation for pulmonary fibrosis.

Science.gov (United States)

1986-05-01

Improvements in immunosuppression and surgical techniques have made unilateral lung transplantation feasible in selected patients with end-stage interstitial lung disease. We report two cases of successful unilateral lung transplantation for end-stage respiratory failure due to pulmonary fibrosis. The patients, both oxygen-dependent, had progressive disease refractory to all treatment, with an anticipated life expectancy of less than one year on the basis of the rate of progression of the disease. Both patients were discharged six weeks after transplantation and returned to normal life. They are alive and well at 26 months and 14 months after the procedure. Pulmonary-function studies have shown substantial improvement in their lung volumes and diffusing capacities. For both patients, arterial oxygen tension is now normal and there is no arterial oxygen desaturation with exercise. This experience shows that unilateral lung transplantation, for selected patients with end-stage interstitial lung disease, provides a good functional result. Moreover, it avoids the necessity for cardiac transplantation, as required by the combined heart-lung procedure, and permits the use of the donor heart for another recipient.

The radiographic findings in diagnosis of pulmonary lymphoma

International Nuclear Information System (INIS)

Song Wei; Wang Li; Yan Hongzhen

2001-01-01

Objective: To study the radiographic findings in the diagnosis of pulmonary lymphoma and pseudo lymphoma. Methods: Eight patients with pulmonary lymphoma and 2 with pseudo lymphoma were examined by X-ray film, tomography, and CT. Results: Single or multiple nodules or masses were observed in 8 patients with pulmonary lymphoma, shaggy borders or halo of ground-glass attenuation in 7 patients, 2 patients had multiple patchy infiltrates bilaterally, 2 had diffuse interstitial infiltrates and 1 had miliary nodules. Multiple consolidations with air bronchogram and without hilar and mediastinal lymphadenopathy were observed in 2 patients with pulmonary pseudo lymphoma. Conclusion: Radiographic findings of pulmonary lymphoma were varied, the most common findings were the nodules or masses with shaggy borders or halo of ground-glass attenuation. The specific findings of pulmonary pseudo lymphoma were multiple consolidations with air bronchogram and without hilar and mediastinal lymphadenopathy. The final diagnosis relied on pathology

Role of bronchoscopy in evaluation of cases with sputum smear negative pulmonary tuberculosis, interstitial lung disease and lung malignancy: A retrospective study of 712 cases.

Science.gov (United States)

Kumar, Raj; Gupta, Nitesh

2015-01-01

The introduction of flexible bronchoscope has revolutionized the field of pulmonary medicine and is a standard instrument used for diagnostic purpose. A retrospective analysis of the clinico-radiological profile, indication, biopsy procedure and complications, for patients undergoing bronchoscopy at one of the respiratory unit at a tertiary care center in India. Retrospective analysis of 712 bronchoscopies was done in regard to demographic profile, clinical and radiological presentation and diagnostic indication. The results were analyzed on basis of bronchoscopy inspection and histopathological specimen obtained from transbronchial (TBLB), endobronchial biopsy (EBLB) and cytology specimen by transbronchial needle aspiration (TBNA). Furthermore, diagnostic yield of each biopsy procedure and their combination was evaluated. Of 712 patients undergoing bronchoscopy, the pathological diagnosis was achieved in 384 (53.93%). Of 384 diagnosed cases, the clinic-radio-pathological diagnosis of pulmonary tuberculosis in 88 (22.19%), interstitial lung disease (ILDs) in 226 (58.85%), and lung cancer in 70 (18.22%) cases. Of 116 sputum smear negative tuberculosis patients, 88 (75.86%) were diagnosed to be pulmonary tuberculosis; the contribution of BAL being 71.59%. Of 226 ILDs, sarcoidosis was most common 148/226 (65.48%). Among 70 lung cancer diagnosed cases, squamous cell carcinoma was most common (54.28%). The results from current study reemphasizes on the diagnostic utility as well as safety of the bronchoscopy procedure. Copyright © 2015 Tuberculosis Association of India. Published by Elsevier B.V. All rights reserved.

Pneumocystis jirovecii colonization in chronic pulmonary disease

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Gutiérrez S.

2011-05-01

Full Text Available Pneumocystis jirovecii causes pneumonia in immunosuppressed individuals. However, it has been reported the detection of low levels of Pneumocystis DNA in patients without signs and symptoms of pneumonia, which likely represents colonization. Several studies performed in animals models and in humans have demonstrated that Pneumocystis induces a local and a systemic response in the host. Since P. jirovecii colonization has been found in patients with chronic pulmonary diseases it has been suggested that P. jirovecii may play a role in the physiopathology and progression of those diseases. In this report we revise P. jirovecii colonization in different chronic pulmonary diseases such us, chronic obstructive pulmonary disease, interstitial lung diseases, cystic fibrosis and lung cancer.

rheumatoid arthritis associated with pulmonary fibrosis in nigerians

African Journals Online (AJOL)

Various pulmonary manifestations have been reported in the developed world, these include diffuse interstitial fibrosis ... system revealed pulse of 70bpm, BP 110/70 mmHg,. Apex beat not displaced, heart sounds I, II and normal. ... Other systems were essentially normal. Laboratory investigations showed elevated ESR.

Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center

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Geetakiran Arakkal

2017-01-01

Conclusions: In our patients, pulmonary involvement was more common than cardiac involvement. Interstitial lung disease and cardiac involvement were more commonly seen in diffuse systemic sclerosis whereas pulmonary hypertension was more frequent in limited systemic sclerosis. Hence, it is important to screen the patients for cardiopulmonary involvement for early diagnosis and treatment and a better prognostic outcome.

A young man with multiple pulmonary cysts | Ibrahim | Libyan ...

African Journals Online (AJOL)

Several diseases cause cystic or cyst-like parenchymal lung abnormalities including adult pulmonary Langerhan\\'s cell histiocytosis (PLCH), lymphangioleiomyomatosis (LAM), emphysema, end-stage interstitial lung disease, and cystic bronchiectasis. Many of these diseases can now be diagnosed with high accuracy by ...

Radiologic observations on pulmonary radiation injury

International Nuclear Information System (INIS)

Liang Yong

1992-01-01

Based on the data of pulmonary radiation injury in 16 cases, the relationship among the radiation dosage and field, the development and onset time of the pulmonary radiation injury were discussed, and the dynamic changes of pulmonary radiation injury in X-ray films were analysed. The author found that: (1) there was a close relationship between the development of radiation injury and radiation dosages and the size of radiation fields, i.e. for the large radiation field, a relatively small dosage was needed for developing radiation injury ; (2) most off acute radiation injury of the lungs appeared within one month of postirradiation therapy, and the chronic pulmonary fibrosis appeared at 4.23 months after radiation therapy, with a fibrosis rate of about 85.7% within a half year; (3) the clinical manifestations of pulmonary radiation injury were not parallel to the X-ray signs, namely the X-ray changes were more severe than clinical manifestations. On the basis of X-ray signs and the dynamic changes of pulmonary radiation injury, the differentiation of radiation injury from interstitial pulmonary metastasis, primary tumor, common pneumonia, and tumor recurrence after radiation therapy were discussed

Exertional dyspnoea in interstitial lung diseases: the clinical utility of cardiopulmonary exercise testing

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Matteo Bonini

2017-02-01

Full Text Available Interstitial lung diseases (ILDs represent a heterogeneous group of pathologies characterised by alveolar and interstitial damage, pulmonary inflammation (usually associated with fibrosis, decreased lung function and impaired gas exchange, which can be attributed to either a known or an unknown aetiology. Dyspnoea is one of the most common and disabling symptoms in patients with ILD, significantly impacting quality of life. The mechanisms causing dyspnoea are complex and not yet fully understood. However, it is recognised that dyspnoea occurs when there is an imbalance between the central respiratory efferent drive and the response of the respiratory musculature. The respiratory derangement observed in ILD patients at rest is even more evident during exercise. Pathophysiological mechanisms responsible for exertional dyspnoea and reduced exercise tolerance include altered respiratory mechanics, impaired gas exchange, cardiovascular abnormalities and peripheral muscle dysfunction. This review describes the respiratory physiology of ILD, both at rest and during exercise, and aims to provide comprehensive and updated evidence on the clinical utility of the cardiopulmonary exercise test in the assessment and management of these pathological entities. In addition, the role of exercise training and pulmonary rehabilitation programmes in the ILD population is addressed.

Ossificação pulmonar dendriforme Dendriform pulmonary ossification

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Andrezza Araújo de Oliveira Duarte

2006-06-01

Full Text Available A ossificação pulmonar difusa é uma condição rara, de etiologia desconhecida, na qual osso maduro é encontrado no parênquima pulmonar. É quase sempre descoberta como um achado incidental de autópsias. Freqüentemente afeta homens de meia-idade e é assintomática. Relata-se o caso de um paciente de 75 anos, que apresentou uma radiografia torácica com comprometimento pulmonar difuso e cujo diagnóstico foi baseado no exame histopatológico de fragmento pulmonar obtido através da biópsia a céu aberto, o qual demonstrou fibrose intersticial acentuada com ossificação do parênquima pulmonar.Diffuse pulmonary ossification is a rare condition of unknown pathogenesis in which mature bone is found in the pulmonary parenchyma. It is almost invariably discovered as an incidental finding at autopsy. Most commonly, it affects middle-aged men and is asymptomatic. We present the case of a 75-year-old man in which the chest X-ray showed diffuse interstitial infiltrate. Diagnosis was based on histopathological examination by open-lung biopsy, which revealed interstitial fibrosis with pulmonary ossification.

Pathogenesis of Idiopathic Pulmonary Fibrosis

Science.gov (United States)

Wolters, Paul J.; Collard, Harold R.; Jones, Kirk D.

2014-01-01

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with aging that is characterized by the histopathological pattern of usual interstitial pneumonia. Although an understanding of the pathogenesis of IPF is incomplete, recent advances delineating specific clinical and pathologic features of IPF have led to better definition of the molecular pathways that are pathologically activated in the disease. In this review we highlight several of these advances, with a focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelial cells. We then discuss the pathologic changes within IPF fibroblasts and the extracellular matrix, and we conclude with a summary of how these profibrotic pathways may be interrelated. PMID:24050627

A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis.

Science.gov (United States)

Oliveira, Rudolf K F; Pereira, Carlos A C; Ramos, Roberta P; Ferreira, Eloara V M; Messina, Carolina M S; Kuranishi, Lilian T; Gimenez, Andrea; Campos, Orlando; Silva, Célia M C; Ota-Arakaki, Jaquelina S

2014-08-01

Chronic hypersensitivity pneumonitis is a common fibrotic interstitial lung disease. The prevalence of pulmonary hypertension diagnosed by right heart catheterisation and its cardiopulmonary function findings in patients with chronic hypersensitivity pneumonitis are unknown. Consecutive symptomatic patients with chronic hypersensitivity pneumonitis were prospectively evaluated. All patients were submitted to right heart catheterisation, pulmonary function testing, a 6-min walk test, echocardiography, blood gas determination and N-terminal pro-brain natriuretic peptide analyses. Nonhypoxaemic patients also underwent incremental cardiopulmonary exercise testing. 50 patients underwent right heart catheterisation; 25 (50%) of these had pulmonary hypertension and 22 (44%) had a pre-capillary haemodynamic pattern. The patients with pre-capillary pulmonary hypertension had lower forced vital capacity (mean ± sd 50 ± 17% versus 69 ± 22% predicted, p<0.01), carbon monoxide diffusing capacity (37 ± 12% versus 47 ± 14% predicted, p<0.01), arterial oxygen tension (median (interquartile range) 59.0 (47.8-69.3) versus 73.0 (62.2-78.5) mmHg, p<0.01) and saturation after the 6-min walk test (78 ± 8% versus 86 ± 7%, p<0.01). In pre-capillary pulmonary hypertension, oxygen uptake was also lower at the anaerobic threshold (41 ± 11% versus 50 ± 8% predicted, p=0.04) and at peak exercise (12.8 ± 1.6 versus 15.0 ± 2.5 mL · kg(-1) · min(-1), p=0.02). Pre-capillary pulmonary hypertension is common in symptomatic chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity. Additionally, pulmonary hypertension is more prevalent in hypoxaemic patients with impaired lung function and exercise capacity. ©ERS 2014.

Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: A report from NRITLD

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Tafti Saeid

2008-01-01

Full Text Available Background: Ever since Katzenstein and Fiorelli introduced the term nonspecific interstitial pneumonia (NSIP to denote those cases of interstitial pneumonia that cannot be categorized as any of the other types of idiopathic interstitial pneumonias (IIP, there has been continuing debate on whether it is a real clinical entity or not. The American Thoracic Society/European Respiratory Symposium task group tried to identify idiopathic NSIP as a separate disease and exclude it from the category of IIP. However, it appears that the clinical presentation of NSIP and usual interstitial pneumonia (UIP are the same. Objective : To show that the radiologic features of NSIP and UIP should be relied upon, instead of clinical presentation and pathologic findings, to differentiate between the two. Materials and Methods: Consecutive patients who had received a diagnosis of either NSIP or UIP on the basis of open lung biopsy between January 2001 and December 2007 were identified for inclusion in this retrospective review. The study included 61 subjects: 32 men and 29 women with a mean age of 59.39 ± 14.5 years. Chest computed tomography images of all the cases were collected for a review. High resolution computed tomography (HRCT and all pathologic specimens were also evaluated. A weighted kappa coefficient was used to evaluate whether radiology can be used instead of biopsy for the diagnosis of NSIP and UIP. Comparison of the mean ages and the time intervals (i.e., interval between symptom onset and the time of diagnosis in the UIP and NSIP groups was done using the Mann-Whitney U test. Association between gender and biopsy result was evaluated by the Fisher exact test. Data were evaluated using SPSS, v.13. Results : Sixty-one patients were included in this study, 32 were male and 29 were female. On the basis of biopsy findings, 50 (82% patients had UIP and 11 (18% had NSIP. Thirty (60% of the 50 patients who had UIP were male and 20 (40% were female; 2 (18

Idiopathic pulmonary fibrosis: treatment update.

LENUS (Irish Health Repository)

O'Connell, Oisin J

2011-11-01

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

Tc-99m-MIBI Uptake in Active Pulmonary Tuberculosis: A Case Report

International Nuclear Information System (INIS)

Lee, Chang Ho; Park, Chan Hee; Hwang, Hee Sung; Bae, Moon Sun

1996-01-01

Technetium-99m MIBI was developed as a myocardiac perfusion imagine agent and has been used effectively in the detection and post-therapeutic evaluation of various neoplasm such as thyroid, lung, bone and breast tumors. As an infrequent findings, Tc-99m MIBI agent has shown in non-neoplastic pulmonary conditions including fibroding alveolitis, pulmonary actinomycosis, active pulmonary sarcoidosis, pulmonary interstitial fibrosis in progressive systemic sclerosis and active osteomyelitis. In a recent report conducted by Cetin Oncel, Tc-99m MIBI imaging is an effective method in the detection and follow-up of pulmonary tuberculosis. We have also experienced Tc-99m-MIBI uptake in active pulmonary tuberculosis incidentally found in a patient with suspected proliferative villonodular synovitis of the left ankle.

Radiological diagnosis of pulmonary edema in chronic renal failure

International Nuclear Information System (INIS)

Tret'yakov, A.E.

1983-01-01

Pulmonary edema has been revealed in 132 patients (51.6 %) during radiologic examination of 256 patients with chronic renal failure. The performance of anterio-posterior chest radiographs was in most cases necessary and quite sufficient for making diagnostic conclusions. Follow up study of patients with pulmonary edema and analysis of radiologic picture of the alterations permitted physicians to distinguish approximately 3 stages of the process development, which transit from one into another. Stage 1 involves early disorders and prodromes of pulmonary edema; Stage 2 interstitial lung edema; Stage 3 alveolar edema. The circulation enforcement of the upper lobar vessels has been the main feature of stage 1. Radiogramometry provided additional information for the pulmonary edema diagnosis. For instance, cardioradiometric data are useful for pulmonary edema diagnosis and evidence in favour of its close connection with heart disorders

Pulmonary fibrosis and exposure to steel welding fume.

Science.gov (United States)

Cosgrove, M P

2015-12-01

Arc welders who have been exposed to high concentrations of steel welding fume for prolonged periods of time may develop pulmonary fibrosis but the nature of the fibrotic changes has been debated over the last 80 years without any clear international consensus. To characterize the nature of the pulmonary fibrosis that develops in response to steel welding fume exposure and to provide a working hypothesis that would explain the findings of the existing research, to provide a platform for future research and to inform future occupational and clinical management of welders with pulmonary effects from welding fume. Review of the world literature on pulmonary fibrosis and welding of steel in all languages using PubMed, with further secondary search of references in the articles found in the primary search. Google and Reference Manager were used as further confirmatory search tools. Only case series and case reports were found but these provided consistent evidence that the consequence of exposure to steel welding fume at high levels for a prolonged period of time is a type of pulmonary fibrosis similar to, and possibly the same as, respiratory bronchiolitis which eventually develops into desquamative interstitial pneumonia with ongoing exposure. Steel welding fume may cause an occupational respiratory bronchiolitis which may develop into de squamative interstitial pneumonia with ongoing exposure. This concept may explain the difficulties in interpreting the wider literature on welding fume and lung function at lower exposures and may also explain the increased risk of lung cancer in welders. © The Author 2015. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Correlation of tomographic findings with pulmonary function parameters in nonsmoking patients with idiopathic pulmonary fibrosis

International Nuclear Information System (INIS)

Lopes, Agnaldo Jose; Capone, Domenico; Mogami, Roberto; Jansen, Jose Manoel .E mail: phel.lop@uol.com.br; Cunha, Daniel Leme da; Melo, Pedro Lopes de

2007-01-01

Objective: To correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF). Methods: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography findings were quantified: total interstitial disease (TID), reticular abnormality/honeycombing, and ground-glass opacity (GGO). The functional variables were measured by spirometry, forced oscillation technique (FOT), helium dilution method, as well as the single-breath method of measuring diffusion capacity of the lung for carbon monoxide (DLCO). Results: Of the 30 patients studied, 18 were female, and 12 were male, with a mean age of 70.9 years. We found that TID and reticular abnormality and honeycombing correlated significantly (negative correlations) with the measurements of forced vital capacity (FVC), total lung capacity (TLC), DLCO, and dynamic respiratory compliance were found, as well as that GGO correlated significantly (and positively) with residual volume/TLC. The ratio of forced expiratory flow between 25 and 75% of FVC to FVC (FEF25-75%/FVC) correlated positively with TID, reticular abnormality/honeycombing, and GGO. Conclusion: In IPF patients, the measurements of volume, diffusion, and dynamic compliance are the physiological variables which best reflect the extent of the interstitial disease on HRCT scans. (author)

Correlation of tomographic findings with pulmonary function parameters in nonsmoking patients with idiopathic pulmonary fibrosis

Energy Technology Data Exchange (ETDEWEB)

Lopes, Agnaldo Jose; Capone, Domenico; Mogami, Roberto; Jansen, Jose Manoel [Universidade do Estado do Rio de Janeiro (UERJ), RJ (Brazil). School of Medical Sciences].E mail: phel.lop@uol.com.br; Cunha, Daniel Leme da [Universidade do Estado do Rio de Janeiro (UERJ), RJ (Brazil). Pedro Ernesto University Hospital. Dept. of Radiology and Diagnostic Imaging; Melo, Pedro Lopes de [Universidade do Estado do Rio de Janeiro (UERJ), RJ (Brazil). Inst. of Biology

2007-11-15

Objective: To correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF). Methods: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography findings were quantified: total interstitial disease (TID), reticular abnormality/honeycombing, and ground-glass opacity (GGO). The functional variables were measured by spirometry, forced oscillation technique (FOT), helium dilution method, as well as the single-breath method of measuring diffusion capacity of the lung for carbon monoxide (DLCO). Results: Of the 30 patients studied, 18 were female, and 12 were male, with a mean age of 70.9 years. We found that TID and reticular abnormality and honeycombing correlated significantly (negative correlations) with the measurements of forced vital capacity (FVC), total lung capacity (TLC), DLCO, and dynamic respiratory compliance were found, as well as that GGO correlated significantly (and positively) with residual volume/TLC. The ratio of forced expiratory flow between 25 and 75% of FVC to FVC (FEF25-75%/FVC) correlated positively with TID, reticular abnormality/honeycombing, and GGO. Conclusion: In IPF patients, the measurements of volume, diffusion, and dynamic compliance are the physiological variables which best reflect the extent of the interstitial disease on HRCT scans. (author)

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

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Federica Novelli

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

IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report.

Science.gov (United States)

Chen, Chiu-Fan; Chu, Kuo-An; Tseng, Yen-Chiang; Wu, Chang-Che; Lai, Ruay-Sheng

2017-12-01

IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. A 43-year-old man had chronic cough and dyspnea on exertion for 4 years. Initial chest radiography showed diffuse interstitial infiltration. Follow-up chest computed tomography 4 years later revealed bilateral diffuse centrilobular nodules with tree-in-bud pattern, bronchial wall thickening, and mediastinal lymph nodes. Bilateral diffuse multifocal ground-glass opacities and mosaic attenuation were also observed. Pulmonary function test revealed mixed restrictive and obstructive ventilatory impairment. Video-assisted thoracoscopic surgery (VATS) lung biopsy showed interstitial fibrosis with lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Serum IgG4 level also showed remarkable elevation. Therefore, IgG4-RLD is confirmed. VATS wedge resection of right upper lobe and mediastinal lymph node. The patient responded well to steroid and immunosuppression therapy, and was regular followed-up in outpatient clinic. IgG4-RLD should be considered not only in ILD, but also in small airway disease. Serum IgG4 level may be a useful tool for screening.

Rituximab in the Treatment of Interstitial Lung Disease Associated with Antisynthetase Syndrome: A Multicenter Retrospective Case Review.

Science.gov (United States)

Doyle, Tracy J; Dhillon, Namrata; Madan, Rachna; Cabral, Fernanda; Fletcher, Elaine A; Koontz, Diane C; Aggarwal, Rohit; Osorio, Juan C; Rosas, Ivan O; Oddis, Chester V; Dellaripa, Paul F

2018-06-01

To assess clinical outcomes including imaging findings on computed tomography (CT), pulmonary function testing (PFT), and glucocorticoid (GC) use in patients with the antisynthetase syndrome (AS) and interstitial lung disease (ILD) treated with rituximab (RTX). We retrospectively identified all patients at 2 institutions with AS-ILD who were treated with RTX. Baseline demographics, PFT, and chest CT were assessed before and after RTX. Two radiologists independently evaluated CT using a standardized scoring system. Twenty-five subjects at the Brigham and Women's Hospital (n = 13) and University of Pittsburgh Medical Center (n = 12) were included. Antisynthetase antibodies were identified in all patients (16 Jo1, 6 PL-12, 3 PL-7). In 21 cases (84%), the principal indication for RTX use was recurrent or progressive ILD, owing to failure of other agents. Comparing pre- and post-RTX pulmonary variables at 12 months, CT score and forced vital capacity were stable or improved in 88% and 79% of subjects, respectively. Total lung capacity (%) increased from 56 ± 13 to 64 ± 13 and GC dose decreased from 18 ± 9 to 12 ± 12 mg/day. Although DLCO (%) declined slightly at 1 year, it increased from 42 ± 17 to 70 ± 20 at 3 years. The most common imaging patterns on CT were nonspecific interstitial pneumonia (NSIP; n = 13) and usual interstitial pneumonia/fibrotic NSIP (n = 5), of which 5 had concurrent elements of cryptogenic organizing pneumonia. Stability or improvement in pulmonary function or severity of ILD on CT was seen in most patients. Use of RTX was well tolerated in the majority of patients. RTX may play a therapeutic role in patients with AS-ILD, and further clinical investigation is warranted.

Glycyrrhizic acid alleviates bleomycin-induced pulmonary fibrosis in rats

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Lili eGao

2015-10-01

Full Text Available Idiopathic pulmonary fibrosis is a progressive and lethal form of interstitial lung disease that lacks effective therapies at present. Glycyrrhizic acid (GA, a natural compound extracted from a traditional Chinese herbal medicine Glycyrrhiza glabra, was recently reported to benefit lung injury and liver fibrosis in animal models, yet whether GA has a therapeutic effect on pulmonary fibrosis is unknown. In this study, we investigated the potential therapeutic effect of GA on pulmonary fibrosis in a rat model with bleomycin (BLM-induced pulmonary fibrosis. The results indicated that GA treatment remarkably ameliorated BLM-induced pulmonary fibrosis and attenuated BLM-induced inflammation, oxidative stress, epithelial-mesenchymal transition and activation of tansforming growth factor-beta signaling pathway in the lungs. Further, we demonstrated that GA treatment inhibited proliferation of 3T6 fibroblast cells, induced cell cycle arrest and promoted apoptosis in vitro, implying that GA-mediated suppression of fibroproliferation may contribute to the anti-fibrotic effect against BLM-induced pulmonary fibrosis. In summary, our study suggests a therapeutic potential of GA in the treatment of pulmonary fibrosis.

Diagnostic imaging of pulmonary lymphangiosis carcinomatosa

International Nuclear Information System (INIS)

Rehbock, B.; Hieckel, H.G.

2004-01-01

The diagnosis of pulmonary lymphangiosis carcinomatosa (PLC) is of great importance for the prognostically-oriented therapy stratification of tumor patients. In this field, high-resolution computed tomography (HRCT) is the state of the art in imaging. Using HRCT, it is possible to identify pulmonary parenchymal structures in a detailed fashion to evaluate interstitial patterns. This step is preceded by an x-ray of the thorax that detects pathological findings and rules out other diseases. The typical characteristics of PLC are described with particular attention to HR-phenomenology, and discussed in comparison with the literature regarding anatomy and pathogenesis. Finally, conclusions are drawn for differential diagnosis and supported by characteristic cases. (orig.) [de

Bronchoalveolar lavage: role in the pathogenesis, diagnosis, and management of interstitial lung disease

International Nuclear Information System (INIS)

Daniele, R.P.; Elias, J.A.; Epstein, P.E.; Rossman, M.D.

1985-01-01

Bronchoalveolar lavage has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of information are provided by the evaluation of lavage fluid. First, the identification of cellular constituents helps to separate inflammatory processes in which lymphocytes predominate (for example, sarcoidosis, hypersensitivity pneumonitis, and berylliosis) from those in which neutrophils or macrophages predominate (for example, idiopathic pulmonary fibrosis and histiocytosis X). Second, the cells removed during lavage can be studied for their immune properties and function; tested with specific antigens, in diseases such as berylliosis and hypersensitivity pneumonitis; and examined for the presence of unique surface antigens with monoclonal antibodies (for example, histiocytosis X). Third, in conjunction with scanning electron microscopy and electron probe analysis, lavage makes possible the identification of inorganic particles in alveolar macrophages of patients with pneumoconiotic lung disease. Finally, although lavage is still an investigative procedure for most pulmonary disorders, it has an established role in the diagnosis of opportunistic infections in the immunocompromised patient

Management of Myositis-Related Interstitial Lung Disease.

Science.gov (United States)

Morisset, Julie; Johnson, Cheilonda; Rich, Eric; Collard, Harold R; Lee, Joyce S

2016-11-01

Interstitial lung disease (ILD) is a frequent pulmonary manifestation and an important cause of morbidity and mortality in patients with idiopathic inflammatory myopathy. Myositis-related ILD presents a therapeutic challenge for clinicians, as there are no available guidelines to help with management decisions. This review covers the existing evidence on the pharmacologic and nonpharmacologic management of myositis-related ILD, highlighting the lack of randomized controlled data to guide treatment. Given the absence of existing guidelines to inform treatment decisions, we provide a comprehensive summary, including dosing, side effects, and suggested monitoring of the commonly used immunosuppressive agents and a proposed treatment algorithm based on the existing literature. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

Science.gov (United States)

Xaubet, Antoni; Molina-Molina, María; Acosta, Orlando; Bollo, Elena; Castillo, Diego; Fernández-Fabrellas, Estrella; Rodríguez-Portal, José Antonio; Valenzuela, Claudia; Ancochea, Julio

2017-05-01

Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

Radiation enhances silica translocation to the pulmonary interstitium and increases fibrosis in mice

International Nuclear Information System (INIS)

Adamson, I.Y.R.

1992-01-01

The effects of whole body irradiation (WBR) on particle clearance and the development of pulmonary fibrosis have been investigated. Using carbon, clearance is accomplished by polymorphonuclear leukocytes (PMN) and alveolar macrophages (AM), and only a few particles reach the interstitum. However, in preirradiated mice, the usual eflux of inflammatory cells is much delayed so that more free carbon remains in the alveoli, and by 1 week, many particles cross the epithelium to be phagocytized by interstitial macrophages. Carbon is found in the peribronchiolar interstitium 6 months later with no evidence of fibrosis. In the present study, mice received 1 mg silica intratracheally 2 days after 6.5 Gy WBR when the white blood cell count was low. A much-reduced Am and PMN response was found in the following 2 weeks compared to the reaction to silica alone, and many silica particles reached interstitial macrophages. In this case, macrophage activation by silica was associated with fibroblast proliferation, and by 16 weeks, much more pulmonary fibrosis was produced than after silica or irradiation only. This was measured biochemically and correlated with a large increase in retained silica in the irradiation-silica group. The results indicate that radiation inhibits the inflammatory response to particle instillation, resulting in greater translocation of free particles to the pulmonary interstitium. In the case of silica, the greater, prolonged interaction with interstitial macrophages leads to a much exaggerated fibrotic reaction. 17 refs., 11 figs

Crackle pitch and rate do not vary significantly during a single automated-auscultation session in patients with pneumonia, congestive heart failure, or interstitial pulmonary fibrosis.

Science.gov (United States)

Vyshedskiy, Andrey; Ishikawa, Sadamu; Murphy, Raymond L H

2011-06-01

To determine the variability of crackle pitch and crackle rate during a single automated-auscultation session with a computerized 16-channel lung-sound analyzer. Forty-nine patients with pneumonia, 52 with congestive heart failure (CHF), and 18 with interstitial pulmonary fibrosis (IPF) performed breathing maneuvers in the following sequence: normal breathing, deep breathing, cough several times; deep breathing, vital-capacity maneuver, and deep breathing. From the auscultation recordings we measured the crackle pitch and crackle rate. Crackle pitch variability, expressed as a percentage of the average crackle pitch, was small in all patients and in all maneuvers: pneumonia 11%, CHF 11%, pulmonary fibrosis 7%. Crackle rate variability was also small: pneumonia 31%, CHF 32%, IPF 24%. Compared to the first deep-breathing maneuver (100%), the average crackle pitch did not significantly change following coughing (pneumonia 100%, CHF 103%, IPF 100%), the vital-capacity maneuver (pneumonia 100%, CHF 92%, IPF 104%), or during quiet breathing (pneumonia 97%, CHF 100%, IPF 104%). Similarly, the average crackle rate did not change significantly following coughing (pneumonia 105%, CHF 110%, IPF 90%) or the vital-capacity maneuver (pneumonia 102%, CHF 101%, IPF 99%). However, during normal breathing the crackle rate was significantly lower in the patients with pneumonia (74%, P auscultation session suggests that crackle rate can be used to follow the course of cardiopulmonary illnesses such as pneumonia, IPF, and CHF.

Treatment of pulmonary brucellosis: a systematic review.

Science.gov (United States)

Solera, Javier; Solís García Del Pozo, Julián

2017-01-01

Pulmonary involvement is a rare, focal complication of human brucellosis. The aim of this review is to describe clinical and radiologic features, treatment administered and clinical course of these patients. Areas covered: We conducted a systematic search of scientific reports of brucellosis with pulmonary involvement published from January 1985 to July 2016. Four main patterns of disease were observed: pneumonia, pleural effusion, nodules and interstitial pattern. Cough and fever were the most common symptoms. Brucella spp. culture was obtained from blood (50%) or pleural fluid. Treatment is based on the same antibiotics and combinations of antibiotics as for patients with acute no complicated brucellosis. The most frequent antimicrobial combination was doxycycline and rifampin for six weeks. The clinical course was favorable in most reports, and mortality was remarkably low (Brucellosis from other pulmonary infections, such as tuberculosis, sometimes posed an added diagnostic challenge.

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

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C. Giacomelli

2011-09-01

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

Endoarterial pulmonary metastasis of malignant trophoblast associated with a term intrauterine pregnancy.

Science.gov (United States)

Carlson, J A; Day, T G; Kuhns, J G; Howell, R S; Masterson, B J

1984-02-01

A previously healthy gravida 4, para 3, developed preclampsia and progressive dyspnea at the 37th gestational week and had bilateral pulmonary infiltrates on chest roentgenogram. She delivered a healthy, term, male infant with a normal appearing placenta. Postpartum, her respiratory status gradually worsened. A lung biopsy on the 20th postpartum day revealed intravascular trophoblasts, diffuse arteriolar thrombosis with pulmonary infarction, and subacute interstitial pneumonitis. Combination chemotherapy was instituted, but the patient died from respiratory insufficiency.

IDENTIFICATION AND CHARACTERIZATION OF AN IDIOPATHIC PULMONARY FIBROSIS-LIKE CONDITION IN CATS

Science.gov (United States)

Interstitial lung diseases are a heterogeneous group of disorders due to a variety of causes. In veterinary medicine, those with a prominent fibrotic component of unknown etiology are often called idiopathic pulmonary fibrosis (IPF). In human medicine, this term is reserved for ...

Changes of bronchoalveolar cell pattern and lecithin content in experimental interstitial pneumonia

International Nuclear Information System (INIS)

Manabe, Hideki; Yasuoka, Susumu; Tsubura, Eiro

1978-01-01

The pathogenesis of diffuse interstitial fibrosing pneumonitis (DIFP) was studied by histological observations and analysis of the cells and lecithin content of bronchoalveolar lavage of rats with cyclophosphamide (CY)-induced pneumonitis or irradiation pneumonitis. The rats developed diffuse interstitial pneumonitis one week after the last of 5 intraperitoneal injections of 50 mg/kg of CY and gradually recovered in the next 14 weeks. The number of alveolar macrophages and the lecithin content in the bronchoalveolar lavage from these rats corresponded to the degree of inflammatory change of the lung tissue. The results of cell counts and analysis of the bronchoalveolar lavage from rats with irradiated pneumonitis were similar to those on rats with CY-induced pneumonitis, except that in irradiated rats the lecithin content of the lavage decreased with increase in severity of pulmonary fibrosis. These results indicate that the cell number and lecithin content of bronchoalveolar lavage are good parameters for use in diagnosis of DIFP. (auth.)

Energetics of formation and migration of self-interstitials and self-interstitial clusters in α-iron

International Nuclear Information System (INIS)

Wirth, B.D.; Odette, G.R.; California Univ., Santa Barbara, CA; Maroudas, D.; Lucas, G.E.; California Univ., Santa Barbara, CA

1997-01-01

Energetic primary recoil atoms from fast neutron irradiation generate both isolated point defects and clusters of vacancies and interstitials. Self-interstitial mobility as well as defect cluster stability and mobility play key roles in the subsequent fate of defects and, hence, in the overall microstructural evolution under irradiation. Self-interstitials and two, three and four-member self-interstitial clusters are highly mobile at low temperatures as observed in molecular-dynamics simulations and high mobility probably also extends to larger clusters. In this study, the morphology, energetics and mobility of self-interstitials and small self-interstitial clusters in α-iron are studied by molecular-statics and molecular-dynamics simulations using a Finnis-Sinclair many-body interatomic potential. Self-interstitial migration is found to be a two-step process consisting of a rotation out of the split-dumbbell configuration into the split-dumbbell configuration and translational jumps through the crowdion configuration before returning to the dumbbell configuration. Self-interstitial clusters of type split-interstitials assembled on adjacent {110} planes migrate along directions in an amoeba-like fashion by sequential local dissociation and re-association processes. (orig.)

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

Science.gov (United States)

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

2013-12-01

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

Do We Need Exercise Tests to Detect Gas Exchange Impairment in Fibrotic Idiopathic Interstitial Pneumonias?

Directory of Open Access Journals (Sweden)

Benoit Wallaert

2012-01-01

Full Text Available In patients with fibrotic idiopathic interstitial pneumonia (f-IIP, the diffusing capacity for carbon monoxide (DLCO has been used to predict abnormal gas exchange in the lung. However, abnormal values for arterial blood gases during exercise are likely to be the most sensitive manifestations of lung disease. The aim of this study was to compare DLCO, resting PaO2, P(A-aO2 at cardiopulmonary exercise testing peak, and oxygen desaturation during a 6-min walk test (6MWT. Results were obtained in 121 patients with idiopathic pulmonary fibrosis (IPF, n=88 and fibrotic nonspecific interstitial pneumonias (NSIP, n=33. All but 3 patients (97.5% had low DLCO values (35 mmHg and 100 (83% demonstrated significant oxygen desaturation during 6MWT (>4%. Interestingly 27 patients had low DLCO and normal P(A-aO2, peak and/or no desaturation during the 6MWT. The 3 patients with normal DLCO also had normal PaO2, normal P(A-aO2, peak, and normal oxygen saturation during 6MWT. Our results demonstrate that in fibrotic IIP, DLCO better defines impairment of pulmonary gas exchange than resting PaO2, exercise P(A-aO2, peak, or 6MWT SpO2.

[Diagnosis of pulmonary hemorrhage of the newborn infants using lung ultrasonography].

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Liu, J; Fu, W; Chen, S W; Wang, Y

2017-01-02

Objective: To investigate the accuracy and reliability of lung ultrasound in diagnosis of pulmonary hemorrhage of the newborn infants. Method: From January 2014 to May 2016, 142 neonates from the Army General Hospital of the Chinese PLA were enrolled in the study. They were divided into two groups: a study group of 42 neonates, who were diagnosed with pulmonary hemorrhage according to their medical history, clinical manifestations and chest X-ray findings, and a control group of 100 neonates with no lung disease. All subjects underwent bedside lung ultrasound in a quiet state in a supine, lateral or prone posture, performed by a single experienced physician. The ultrasound findings were compared between the two groups.Fisher's exact test was uesd for comparison between two groups. Result: The lung ultrasound main findings associated with pulmonary hemorrhage included: (1) Shred sign: which was seen in 40 patients(95%). (2) Lung consolidation with air bronchograms: which were seen in 35 patients(83%). (3) Pleural effusion: which was seen in 34 infants(81%), pleurocentesis confirmed that the fluid was really bleeding.(4)Atelectasis: which was seen in 14 cases(33%). (5) Pleural line abnormalities and disappearing A-lines with an incidence of 100%. (6) Alveolar-interstitial syndrome: 5 patients(12%)had the main manifestations of alveolar-interstitial syndrome. The above signs were not seen in normal controls (all P hemorrhage, which is suitable for routine application for the diagnosis of pulmonary hemorrhage in the neonatal intensive care unit.

August 2017 pulmonary case of the month

Directory of Open Access Journals (Sweden)

Wesselius LJ

2017-08-01

Full Text Available No abstract available. Article truncated after the first page. History of Present Illness: The patient is a 60-year-old woman with dyspnea on exertion when she had a pulmonary embolism following knee surgery 3 years earlier. She smoked 1 pack per day for the past 40 years. She was seen at another hospital and had pulmonary function testing which showed only a DLco which was 66% of predicted. Serologic studies were negative for a rheumatologic disorder. A CT scan was also performed (Figure 1. The CT scan was interpreted as showing a few small nodules and possible very early interstitial lung disease. Which of the following are true? 1. A pulmonary embolism can reduce the DLco; 2. Her CT scan is characteristic of Langerhans cell histiocytosis; 3. Smoking can reduce the DLco; 4. 1 and 3; 5.\tAll of the above …

Interstitial lung disease during trimethoprim/sulfamethoxazole administration

International Nuclear Information System (INIS)

Yuzurio, Syota; Horita, Naokatsu; Shiota, Yutaro; Kanehiro, Arihiko; Tanimoto, Mitsune

2010-01-01

We studied clinical and radiographic features of interstitial lung disease (ILD) during trimethoprim/sulfamethoxazole (TMP/SMX) administration. Ten patients who had received prednisolone treatment for underlying diffuse pulmonary disease showed various ILDs after introduction of TMP/SMX. The radiographic features of the ILDs were not consistent with infectious disease or exacerbation of the underlying disease, and these diagnoses were excluded radiographically and on clinical grounds during the differential diagnosis of the ILDs. These ILDs emerged relatively early after introduction of TMP/SMX, which is consistent with the former case report of drug-induced ILD (DI-ILD) caused by TMP/SMX. Therefore DI-ILDs caused by TMP/SMX were suspected in these cases. In most of these cases, the ILDs were clinically mild and disappeared immediately although administration of TMP/SMX was continued. (author)

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

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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

2016-04-01

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.

Radiological findings of pulmonary Kaposi's sarcoma. Manifestaciones radiologicas del sarcoma de Kaposi pulmonar

Energy Technology Data Exchange (ETDEWEB)

Rosello, J A; Hernandez, S; Arranz, M; Jareo, J; Ancoechea, J

1994-01-01

Kaposi's sarcoma (KS) is the most common neoplasm in AIDS patients. The incidence of pulmonary involvement is approximately 20%. The radiological findings are reported in plain chest x-ray and computed tomography (CT) in 15 patients diagnosed as having pulmonary Kaposi's sarcoma, in whom concomitant pulmonary infection was ruled out. The most common radiological pattern was that of bilateral perihilar interstitial involvement (86%), while poorly defined multiple nodules seldom presented (13%). In 40% of cases, the pulmonary parenchymal lesion was accompanied by pleural effusion. This sign is useful in the differential diagnosis involving opportunistic P. carinii pneumonia, a very common process in these patients which rarely presents with pleural effusion. The chest CT finding that was most characteristic of pulmonary Kaposi's sarcoma was bilateral perihilar peribronchovascular enlargement. (Author)

Morphometric changes of pulmonary tissues after 20 Gy external irradiation of rat chest

International Nuclear Information System (INIS)

Cao Zhenshan; Ye Changqing; Yuan Lizhen

1996-01-01

The changes in the main parameters of the lungs at different periods of early stage after local 20 Gy external irradiation of the lungs were measured with morphometric method. The results indicated that the walls of pulmonary arterioles and venules thickened and the vascular permeability index (area of vascular lumen/total area of blood vessel) decreased 7 days after irradiation (P 2 , r = -0.919), indicating that narrowing of the vascular lumen was the result of thickening of the vascular wall. Fifteen days after irradiation, the pulmonary alveolar wall thickened, the area of alveolar cavity decreased and the area of pulmonary interstitial space increased (P<0.01). Electron microscopic examination demonstrated profuse exudation surrounding the microvessels, obvious evacuation of pulmonary type-II cells and increase in cellular types and quantity of pulmonary tissues

Evaluation of the relationship between radiological abnormalities and both pulmonary function and pulmonary hypertension in coal workers' pneumoconiosis

Energy Technology Data Exchange (ETDEWEB)

Yildiz, O.A.; Gulbay, B.E.; Saryal, S.; Karabiylkoglu, G. [Ankara University, Ankara (Turkey). School of Medicine

2007-05-15

The aim of this study was to investigate the effect of the radiological evidence of emphysema, and the extent of interstitial involvement, on lung function and pulmonary arterial pressure (PAP) in patients with coal workers' pneumoconiosis (CWP). All patients showed a mild decrease in FEV1/FVC and a mild increase in FRC. Forty-four per cent of patients developed mild to moderate pulmonary hypertension. Emphysema scores correlated significantly with airflow rates, including FEV1%, FEV1/FVC and FEF25-75%, and with carbon monoxide diffusing capacity (DLCO)% predicted as well as FRC% predicted and the ratio RV/TLC, which are indices of air trapping. Additionally, profusion and global profusion scores showed significant correlation with FEV1/FVC, DLCO% predicted, specific airway conductance and smoking. Mean PAP showed a significant negative correlation with FEF50% predicted, DLCO% predicted and profusion score. The impairment of pulmonary function (mainly disturbance in airflow rates and air trapping) and pulmonary hypertension may be present, even in a simple form of CWP. The pulmonary function impairment in patients with CWP is likely to be attributable to the occurrence of emphysema. However, pulmonary hypertension was directly related to the profusion of pneumoconiotic nodules, which may result in obliteration of the vascular bed.

Pulmonary functional MR imaging for COPD

International Nuclear Information System (INIS)

Ohno, Yoshiharu

2008-01-01

Chronic obstructive pulmonary disease (COPD) is a slowly progressive disease characterized by airflow limitation, cough, sputum production, and, at later stages, dyspnea. COPD is currently the fourth-leading cause of mortality and the twelfth-leading cause of disability, and by the year 2020 it is expected to be the third-leading cause of death and the fifth-leading cause of disability worldwide. The diagnosis of COPD largely relies on a history of exposure to noxious stimuli and abnormal lung function test results. Since the pathology of COPD varies and the molecular mechanisms are only slightly understood, the diagnosis and stage assessment of COPD have relied on the results of pulmonary function test. In addition, CT and nuclear medicine study are utilized for assessment of regional morphological and functional abnormalities. Recently, pulmonary functional MR imaging is suggested as a new technique for assessment of regional physiopathologic information in various pulmonary diseases including COPD, pulmonary thromboembolism, lung cancer and interstitial lung diseases. This review article covers the brief description of theory and clinical application of contrast-enhanced perfusion MR imaging; hyperpolarized noble gas MR imaging and oxygen-enhanced MR imaging in COPD subjects. We believe that further basic studies as well as clinical applications of this new technique will define the real significance of pulmonary functional MR imaging for the future of pulmonary functional imaging and its usefulness for diagnosis and patients' management in COPD. (author)

Lung uptake of thallium-201 on resting myocardial imaging in assessment of pulmonary edema

Energy Technology Data Exchange (ETDEWEB)

Tamaki, N.; Yonekura, Y.; Yamamoto, K. (Kyoto Univ. (Japan). Hospital)

1981-03-01

We have noted increased lung uptake of thallium-201 on resting myocardial images in patients with congestive heart failure. To evaluate this phenomenon, lung uptake of thallium on resting myocardial imaging was examined in 328 patients with various cardiovascular diseases. Increased lung uptake was observed in 117 cases (78%) with myocardial infarction, 32 (37%) with angina pectoris, 6 (27%) with hypertensive heart disease, 7 (30%) with hypertrophic cardiomyopathy, 6 (100%) with congestive cardiomyopathy, 11 (100%) with valvular heart disease, and 7 (71%) with congenital heart disease, however, only one (5%) of normal subjects revealed increased uptake. Left ventricular ejection fraction was evaluated in 32 cases with ischemic heart disease on the same day and it was significantly decreased as the lung uptake of thallium increased. Increased thallium activity in the lung seemed to be another noninvasive marker of lift heart failure in ischemic heart disease. Lung uptake of thallium was compared with pulmonary congestive signs on chest X-ray in 29 cases. The uptake was well correlated with the degree of pulmonary edema, and thallium myocardial image revealed remarkably increased lung uptake in all the patients accompanied with pulmonary interstitial edema on chest X-ray. Therefore, this phenomenon will demonstrate pulmonary edema, since thallium may be extracted to the increased interstitial distribution space of the lung as well as the myocardium in a patient with pulmonary edema. We conclude that thallium myocardial scintigraphy is useful not only in identification and localization of myocardial ischemia or infarction, but also in evaluation of pulmonary edema at the same time.

Lung uptake of thallium-201 on resting myocardial imaging in assessment of pulmonary edema

International Nuclear Information System (INIS)

Tamaki, Nagara; Yonekura, Yoshiharu; Yamamoto, Kazutaka

1981-01-01

We have noted increased lung uptake of thallium-201 on resting myocardial images in patients with congestive heart failure. To evaluate this phenomenon, lung uptake of thallium on resting myocardial imaging was examined in 328 patients with various cardiovascular diseases. Increased lung uptake was observed in 117 cases (78%) with myocardial infarction, 32 (37%) with angina pectoris, 6 (27%) with hypertensive heart disease, 7 (30%) with hypertrophic cardiomyopathy, 6 (100%) with congestive cardiomyopathy, 11 (100%) with valvular heart disease, and 7 (71%) with congenital heart disease, however, only one (5%) of normal subjects revealed increased uptake. Left ventricular ejection fraction was evaluated in 32 cases with ischemic heart disease on the same day and it was significantly decreased as the lung uptake of thallium increased. Increased thallium activity in the lung seemed to be another noninvasive marker of lift heart failure in ischemic heart disease. Lung uptake of thallium was compared with pulmonary congestive signs on chest X-ray in 29 cases. The uptake was well correlated with the degree of pulmonary edema, and thallium myocardial image revealed remarkably increased lung uptake in all the patients accompanied with pulmonary interstitial edema on chest X-ray. Therefore, this phenomenon will demonstrate pulmonary edema, since thallium may be extracted to the increased interstitial distribution space of the lung as well as the myocardium in a patient with pulmonary edema. We conclude that thallium myocardial scintigraphy is useful not only in identification and localization of myocardial ischemia or infarction, but also in evaluation of pulmonary edema at the same time. (author)

Pulmonary Lymphangioleiomyomatosis. Presentation of 3 cases and revision of the literature

International Nuclear Information System (INIS)

Pedrozo Pupo, John C; Robinson, Eugenia; Gaviria, Alejandro; Ojeda Leon, Paulina; Pacheco, Pedro M

2000-01-01

We present three cases of women with pulmonary Iymphangioleiomyomatosis diagnosis, a 47-year-old woman whose initial manifestation was pneumothorax, a 40-year-old woman with progressive dyspnoea and other a 45-year-old woman with pneumothorax. Lymphangioleiomyomatosis is a rare lung disease that afflicts young women childbearing age and premenopausal. It is characterized pathologically by the proliferation of atypical pulmonary interstitial smooth muscle and by cyst formation. Unknown origin usually leads to progressive deterioration of lung function and eventual death from failure. It occurs in women of reproductive age and people with tuberous sclerosis

Morphological analysis and pathological basis of the fine pulmonary reticulation at high-resolution CT

International Nuclear Information System (INIS)

Guan Chunshuang; Ma Daqing; Guan Yansheng; Chen Budong; Zhang Yansong

2010-01-01

Objective: To study the morphological appearance and pathological basis of the fine pulmonary reticulation at HRCT. Methods: One hundred and seven patients were analyzed about the morphology findings and dynamic changes on pulmonary HRCT. Twenty-four coal worker's pneumoconiosis (CWP) specimens were examined to make comparison between CT and pathology. The data was analyzed by using the Chi-square test. Results: The reticular gap was less than 3 mm in diameter. The morphology of reticulation was round or irregular. Pulmonary parenchyma was seen between the gaps. The reticular wall was smooth or coarse. The thickness was less than 1 mm. One hundred and seven patients had accompanying signs including ground-glass opacity (68.2%, 73 patients), crazy paving (23.4%, 25 patients), interlobular septal thickening (84.1%, 90 patients), emphysema (32.7%, 35 patients), interface sign (58.9%, 63 patients), traction bronchiolectasis (41.1%, 44 patients) and honeycombing (26.2%, 28 patients). The differences of the honeycomb, traction bronchiolectosis, interbobular septal thickening, interface sign and paving were statistically significant between the fibrotic group and pneunonia (P <0.01). Pneumonia showed extensive area of ground-glass opacity (GGO) with fine reticulation. Fine reticculation with both interlobular septal thickening and small nodules were observed more frequently in lmphangitic carcinomatosis. Idiopathic pulmonary fibrosis (IPF) showed fine reticulation among the honeycombing. Connective tissue disease (CTD) showed fine reticulation with rarely honeycombing and it could be partly absorbed. Fine reticulation with emphysema was seen in chronic bronchitis. In the 58 follow-up patients, the fine reticulation increased in 26 patients, decreased or disappeared in 22 patients and showed no change in 10 patients. The major pathological basis of the fine reticulation was intralobular interstitial thickening, including fibrosis hyperplasia, inflammatory cells and tumor

Chronic interstitial lung disease in nylon flocking industry workers--Rhode Island, 1992-1996.

Science.gov (United States)

1997-09-26

Interstitial lung disease (ILD) occurs infrequently; some cases are attributed to sarcoidosis, pulmonary hemorrhage syndromes, connective tissue diseases, hypersensitivity pneumonitis, drugs, radiation, and mineral dusts (e.g., silica or asbestos). However, most cases of ILD are of uncertain classification or etiology. This report describes preliminary findings of the investigation in Rhode Island of an outbreak of ILD among workers involved in the manufacture of finely cut nylon (flock) and flocked fabric (used for upholstery, clothing, and automobiles); the findings provide evidence of a newly recognized occupational illness.

Lysyl oxidases in idiopathic pulmonary fibrosis: A key participant in collagen I matrix remodelling

NARCIS (Netherlands)

Tjin, Gavin; Mahar, Annabelle; Kable, Eleanor; Burgess, Janette

2015-01-01

Introduction: The fibrotic element in Idiopathic Pulmonary Fibrosis (IPF) is a key feature and is associated with Usual Interstitial Pneumonia (UIP) pattern. Fibrillar collagen I (COL1) has second harmonic generation (SHG) properties, with signals both in the forward (F) (organized collagen) &

Normal expiratory flow rate and lung volumes in patients with combined emphysema and interstitial lung disease: a case series and literature review.

Science.gov (United States)

Heathcote, Karen L; Cockcroft, Donald W; Fladeland, Derek A; Fenton, Mark E

2011-01-01

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.

A rare case of pulmonary toxoplasmosis in a patient with undifferentiated inflammatory arthritis on chronic methotrexate and corticosteroid therapy.

Science.gov (United States)

Abdulkareem, Abdullateef; D'Souza, Ryan Steven; Patel, Nitin; Donato, Anthony A

2017-08-23

Pulmonary toxoplasmosis is a serious pulmonary condition caused by the protozoan Toxoplasma gondii It typically affects immunocompromised patients presenting acutely with cough, fever, myalgias, arthralgias and lymphadenopathy, and chronically with persistent cough and dyspnoea. Because of its protean features, it can mimic many more common lung conditions in the immunocompromised patient, including atypical pneumonia, Pneumocystis pneumonia and interstitial lung disease. In this article, we present the case of a 55-year-old woman who presented to our hospital with persistent dyspnoea and cough, initially suspected to have an arthritis-related interstitial lung disease. She received a final diagnosis of pulmonary toxoplasmosis after lung biopsy demonstrated Toxoplasma cysts, later confirmed by serology. Treatment with trimethoprim-sulfamethoxazole resulted in significant improvement of her respiratory symptoms after 3 months. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Chymase: a multifunctional player in pulmonary hypertension associated with lung fibrosis.

Science.gov (United States)

Kosanovic, Djuro; Luitel, Himal; Dahal, Bhola Kumar; Cornitescu, Teodora; Janssen, Wiebke; Danser, A H Jan; Garrelds, Ingrid M; De Mey, Jo G R; Fazzi, Gregorio; Schiffers, Paul; Iglarz, Marc; Fischli, Walter; Ghofrani, Hossein Ardeschir; Weissmann, Norbert; Grimminger, Friedrich; Seeger, Werner; Reiss, Irwin; Schermuly, Ralph Theo

2015-10-01

Limited literature sources implicate mast-cell mediator chymase in the pathologies of pulmonary hypertension and pulmonary fibrosis. However, there is no evidence on the contribution of chymase to the development of pulmonary hypertension associated with lung fibrosis, which is an important medical condition linked with increased mortality of patients who already suffer from a life-threatening interstitial lung disease.The aim of this study was to investigate the role of chymase in this particular pulmonary hypertension form, by using a bleomycin-induced pulmonary hypertension model.Chymase inhibition resulted in attenuation of pulmonary hypertension and pulmonary fibrosis, as evident from improved haemodynamics, decreased right ventricular remodelling/hypertrophy, pulmonary vascular remodelling and lung fibrosis. These beneficial effects were associated with a strong tendency of reduction in mast cell number and activity, and significantly diminished chymase expression levels. Mechanistically, chymase inhibition led to attenuation of transforming growth factor β1 and matrix-metalloproteinase-2 contents in the lungs. Furthermore, chymase inhibition prevented big endothelin-1-induced vasoconstriction of the pulmonary arteries.Therefore, chymase plays a role in the pathogenesis of pulmonary hypertension associated with pulmonary fibrosis and may represent a promising therapeutic target. In addition, this study may provide valuable insights on the contribution of chymase in the pulmonary hypertension context, in general, regardless of the pulmonary hypertension form. Copyright ©ERS 2015.

Pulmonary manifestations of Sjögren's syndrome

Directory of Open Access Journals (Sweden)

Thomas Flament

2016-06-01

Full Text Available In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

Lung adenocarcinoma mimicking pulmonary fibrosis-a case report

International Nuclear Information System (INIS)

Mehić, Bakir; Duranović Rayan, Lina; Bilalović, Nurija; Dohranović Tafro, Danina; Pilav, Ilijaz

2016-01-01

Lung cancer is usually presented with cough, dyspnea, pain and weight loss, which is overlapping with symptoms of other lung diseases such as pulmonary fibrosis. Pulmonary fibrosis shows characteristic reticular and nodular pattern, while lung cancers are mostly presented with infiltrative mass, thick-walled cavitations or a solitary nodule with spiculated borders. If the diagnosis is established based on clinical symptoms and CT findings, it would be a misapprehension. We report a case of lung adenocarcinoma whose symptoms as well as clinical images overlapped strongly with pulmonary fibrosis. The patient’s non-productive cough, progressive dyspnea, restrictive pattern of pulmonary function test and CT scans (showing reticular interstitial opacities) were all indicative of pulmonary fibrosis. The patient underwent a treatment consisting of corticosteroids and antibiotics, to no avail. Histopathology of the lung showed that the patient suffered from mucinous adenocarcinoma. Albeit the immunohistochemical staining was not consistent with lung adenocarcinoma, tumor’s morphological characteristics were consistent, and were used to make the definitive diagnosis. Given the fact that radiography cannot always make a clear-cut difference between pulmonary fibrosis and lung adenocarcinomas, and that clinical symptoms often overlap, histological examination should be considered as gold standard for diagnosis of lung adenocarcinoma

Significance of various pulmonary and extrapulmonary abnormalities on HRCT of the chest in scleroderma lung

International Nuclear Information System (INIS)

Pandey, Anoop Kumar; Wilcox, Pearce; O’ Brien, Julie; Ellis, Jennifer; Brown, Jacquie; Leipsic, Jonathon

2013-01-01

Patients with systemic sclerosis (SSc) are routinely investigated with high-resolution computed tomography (HRCT) chest for early detection and accurate characterization of complicating interstitial lung diseases. Though the primary aim of HRCT is to delineate the burden of pulmonary involvement and to characterize the nature of fibrosis to potentially help guide management, it provides an opportunity to evaluate extrapulmonary manifestations, particularly the dilated pulmonary artery, esophageal dilatation, and pericardial abnormalities which have their own clinical significance. The aim of this article is to discuss the significance of various pulmonary and extrapulmonary abnormalities that may be identified on HRCT chest of SSc patients

Esophageal motor disease and reflux patterns in patients with advanced pulmonary disease undergoing lung transplant evaluation.

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Seccombe, J; Mirza, F; Hachem, R; Gyawali, C P

2013-08-01

Advanced pulmonary disorders are linked to esophageal hypomotility and reflux disease. However, characterization of esophageal function using high resolution manometry (HRM) and ambulatory pH monitoring, segregation by pulmonary pathology, and comparison to traditional reflux disease are all limited in the literature. Over a 4 year period, 73 patients (55.2 ± 1.3 years, 44F) were identified who underwent esophageal function testing as part of lung transplant evaluation for advanced pulmonary disease (interstitial lung disease, ILD = 47, obstructive lung disease, OLD = 24, other = 2). Proportions of patients with motor dysfunction (≥ 80% failed sequences = severe hypomotility) and/or abnormal reflux parameters (acid exposure time, AET ≥ 4%) were determined, and compared to a cohort of 1081 patients (48.4 ± 0.4 years, 613F) referred for esophageal function testing prior to antireflux surgery (ARS). The proportion of esophageal body hypomotility was significantly higher within advanced pulmonary disease categories (35.6%), particularly ILD (44.7%), compared to ARS patients (12.1%, P esophageal motor pattern or reflux evidence. Interstitial lung disease has a highly significant association with esophageal body hypomotility. Consequently, prevalence of abnormal esophageal acid exposure is high, but implications for post lung transplant chronic rejection remain unclear. © 2013 John Wiley & Sons Ltd.

The reliability of lung ultrasound in assessment of idiopathic pulmonary fibrosis

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Manolescu D

2018-03-01

Full Text Available Diana Manolescu,1 Lavinia Davidescu,2 Daniel Traila,3 Cristian Oancea,3 Voicu Tudorache3 1Radiology Department, University of Medicine and Pharmacy “Victor Babes”, Timişoara, Romania; 2Department of Pulmonology, University of Medicine and Pharmacy Oradea, Oradea, Romania; 3Department of Pulmonology, University of Medicine and Pharmacy “Victor Babes”, Timişoara, Romania Abstract: Idiopathic pulmonary fibrosis (IPF is the severest form of idiopathic interstitial pneumonia, with a median survival time estimated at 2–5 years from the time of diagnosis. It occurs mainly in elderly adults, suggesting a strong link between the fibrosis process and aging. Although chest high-resolution computed tomography (HRCT is currently the method of choice in IPF assessment, diagnostic imaging with typical usual interstitial pneumonia (UIP provides definitive results in only 55%, requiring an invasive surgical procedure such as lung biopsy or cryobiopsy for the final diagnostic analysis. Lung ultrasound (LUS as a noninvasive, non-radiating examination is very sensitive to detect subtle changes in the subpleural space. The evidence of diffuse, multiple B-lines defined as vertical, hyperechoic artifacts is the hallmark of interstitial syndrome. A thick, irregular, fragmented pleura line is associated with subpleural fibrotic scars. The total numbers of B-lines are correlated with the extension of pulmonary fibrosis on HRCT, being an LUS marker of severity. The average distance between two adjacent B-lines is an indicator of a particular pattern on HRCT. It is used to appreciate a pure reticular fibrotic pattern as in IPF compared with a predominant ground glass pattern seen in fibrotic nonspecific interstitial pattern. The distribution of the LUS artifacts has a diagnostic value. An upper predominance of multiple B-lines associated with the thickening of pleura line is an LUS feature of an inconsistent UIP pattern, excluding the IPF diagnosis. LUS is a

Histopathological Study of Cyclosporine Pulmonary Toxicity in Rats

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Said Said Elshama

2016-01-01

Full Text Available Cyclosporine is considered one of the common worldwide immunosuppressive drugs that are used for allograft rejection prevention. However, articles that address adverse effects of cyclosporine use on the vital organs such as lung are still few. This study aims to investigate pulmonary toxic effect of cyclosporine in rats by assessment of pulmonary histopathological changes using light and electron microscope examination. Sixty male adult albino rats were divided into three groups; each group consists of twenty rats. The first received physiological saline while the second and third groups received 25 and 40 mg/kg/day of cyclosporine, respectively, by gastric gavage for forty-five days. Cyclosporine reduced the lung and body weight with shrinkage or pyknotic nucleus of pneumocyte type II, degeneration of alveoli and interalveolar septum beside microvilli on the alveolar surface, emphysema, inflammatory cellular infiltration, pulmonary blood vessels congestion, and increase of fibrous tissues in the interstitial tissues and around alveoli with negative Periodic Acid-Schiff staining. Prolonged use of cyclosporine induced pulmonary ultrastructural and histopathological changes with the lung and body weight reduction depending on its dose.

Chest radiographic features of lymphocytic interstitial pneumonitis in HIV-infected children

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Pitcher, R.D., E-mail: pitcher@iafrica.co [Division of Paediatric Radiology, Red Cross War Memorial Children' s Hospital, Department of Radiation Medicine, University of Cape Town (South Africa); Beningfield, S.J. [Division of Radiology, New Groote Schuur Hospital and University of Cape Town (South Africa); Zar, H.J. [Department of Paediatric Pulmonology, Red Cross War Memorial Children' s Hospital, School of Child and Adolescent Health, University of Cape Town (South Africa)

2010-02-15

Aim: To review the radiological features of biopsy-proven lymphocytic interstitial pneumonitis (LIP) in human immunodeficiency virus (HIV)-infected children and establish whether these are based on systematic radiological analysis, and to investigate whether more specific radiological diagnostic criteria can be developed. Materials and methods: A Medline search of English-language articles on the radiological features of biopsy-proven LIP in HIV-infected children was conducted for the period 1982 to 2007 inclusive. Radiological findings were compared with the Centers for Disease Control and Prevention (CDC) criteria for a presumptive diagnosis of LIP. Results: Pulmonary pathology was recorded as 'diffuse' and 'bilateral' in 125 (97.6%) of 128 reported cases of LIP. Twenty-five different terms were used to describe the pulmonary parenchyma. In 96 (75%), the terminology was consistent with CDC diagnostic criteria. Radiological evolution was documented in 43 (33.5%). Persistent focal opacification superimposed on diffuse pulmonary nodularity was demonstrated in 10 (7.8%). The method of radiological evaluation was described in six (4.6%). In no instance was the terminology defined. Conclusion: The radiological features of LIP have not been systematically analysed. However, CDC criteria remain reliable, allowing diagnosis of at least 75% of cases. The sensitivity of these criteria may be increased by including cases with persistent focal pulmonary opacification superimposed on diffuse nodularity. Longitudinal studies utilizing standardized radiographic analysis are needed to elucidate the natural history of LIP.

Chest radiographic features of lymphocytic interstitial pneumonitis in HIV-infected children

International Nuclear Information System (INIS)

Pitcher, R.D.; Beningfield, S.J.; Zar, H.J.

2010-01-01

Aim: To review the radiological features of biopsy-proven lymphocytic interstitial pneumonitis (LIP) in human immunodeficiency virus (HIV)-infected children and establish whether these are based on systematic radiological analysis, and to investigate whether more specific radiological diagnostic criteria can be developed. Materials and methods: A Medline search of English-language articles on the radiological features of biopsy-proven LIP in HIV-infected children was conducted for the period 1982 to 2007 inclusive. Radiological findings were compared with the Centers for Disease Control and Prevention (CDC) criteria for a presumptive diagnosis of LIP. Results: Pulmonary pathology was recorded as 'diffuse' and 'bilateral' in 125 (97.6%) of 128 reported cases of LIP. Twenty-five different terms were used to describe the pulmonary parenchyma. In 96 (75%), the terminology was consistent with CDC diagnostic criteria. Radiological evolution was documented in 43 (33.5%). Persistent focal opacification superimposed on diffuse pulmonary nodularity was demonstrated in 10 (7.8%). The method of radiological evaluation was described in six (4.6%). In no instance was the terminology defined. Conclusion: The radiological features of LIP have not been systematically analysed. However, CDC criteria remain reliable, allowing diagnosis of at least 75% of cases. The sensitivity of these criteria may be increased by including cases with persistent focal pulmonary opacification superimposed on diffuse nodularity. Longitudinal studies utilizing standardized radiographic analysis are needed to elucidate the natural history of LIP.

Idiopathic pulmonary fibrosis

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Noble Paul W

2008-03-01

Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

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

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

2011-12-01

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

Short-term Automated Quantification of Radiologic Changes in the Characterization of Idiopathic Pulmonary Fibrosis Versus Nonspecific Interstitial Pneumonia and Prediction of Long-term Survival.

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De Giacomi, Federica; Raghunath, Sushravya; Karwoski, Ronald; Bartholmai, Brian J; Moua, Teng

2018-03-01

Fibrotic interstitial lung diseases presenting with nonspecific and overlapping radiologic findings may be difficult to diagnose without surgical biopsy. We hypothesized that baseline quantifiable radiologic features and their short-term interval change may be predictive of underlying histologic diagnosis as well as long-term survival in idiopathic pulmonary fibrosis (IPF) presenting without honeycombing versus nonspecific interstitial pneumonia (NSIP). Forty biopsy-confirmed IPF and 20 biopsy-confirmed NSIP patients with available high-resolution chest computed tomography 4 to 24 months apart were studied. CALIPER software was used for the automated characterization and quantification of radiologic findings. IPF subjects were older (66 vs. 48; P<0.0001) with lower diffusion capacity for carbon monoxide and higher volumes of baseline reticulation (193 vs. 83 mL; P<0.0001). Over the interval period, compared with NSIP, IPF patients experienced greater functional decline (forced vital capacity, -6.3% vs. -1.7%; P=0.02) and radiologic progression, as noted by greater increase in reticulation volume (24 vs. 1.74 mL; P=0.048), and decrease in normal (-220 vs. -37.7 mL; P=0.045) and total lung volumes (-198 vs. 58.1 mL; P=0.03). Older age, male gender, higher reticulation volumes at baseline, and greater interval decrease in normal lung volumes were predictive of IPF. Both baseline and short-term changes in quantitative radiologic findings were predictive of mortality. Baseline quantitative radiologic findings and assessment of short-term disease progression may help characterize underlying IPF versus NSIP in those with difficult to differentiate clinicoradiologic presentations. Our study supports the possible utility of assessing serial quantifiable high-resolution chest computed tomographic findings for disease differentiation in these 2 entities.

Radiological findings of pulmonary Kaposi's sarcoma. Manifestaciones radiologicas del sarcoma de Kaposi pulmonar

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Rosello, J.A.; Hernandez, S.; Arranz, M.; Jareo, J.; Ancoechea, J.

1994-01-01

Kaposi's sarcoma (KS) is the most common neoplasm in AIDS patients. The incidence of pulmonary involvement is approximately 20%. The radiological findings are reported in plain chest x-ray and computed tomography (CT) in 15 patients diagnosed as having pulmonary Kaposi's sarcoma, in whom concomitant pulmonary infection was ruled out. The most common radiological pattern was that of bilateral perihilar interstitial involvement (86%), while poorly defined multiple nodules seldom presented (13%). In 40% of cases, the pulmonary parenchymal lesion was accompanied by pleural effusion. This sign is useful in the differential diagnosis involving opportunistic P. carinii pneumonia, a very common process in these patients which rarely presents with pleural effusion. The chest CT finding that was most characteristic of pulmonary Kaposi's sarcoma was bilateral perihilar peribronchovascular enlargement. (Author)

Interstitial pregnancy: role of MRI

International Nuclear Information System (INIS)

Filhastre, M.; Lesnik, A.; Dechaud, H.; Taourel, P.

2005-01-01

We report the MRI features of two cases of interstitial pregnancy. In both cases, MRI was able to localize the ectopic pregnancy by showing a gestational structure surrounded by a thick wall in the upper part of the uterine wall separated from the endometrium by an uninterrupted junctional zone. Because US may confuse angular and interstitial pregnancies and because interstitial pregnancy has a particular evolutive course, MR imaging may play a key role in the diagnosis and management of women with interstitial pregnancy. (orig.)

Comorbid Conditions in Idiopathic Pulmonary Fibrosis: Recognition and Management

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Justin M. Oldham

2017-08-01

Full Text Available Idiopathic pulmonary fibrosis (IPF, a fibrosing interstitial pneumonia of unknown etiology, primarily affects older adults and leads to a progressive decline in lung function and quality of life. With a median survival of 3–5 years, IPF is the most common and deadly of the idiopathic interstitial pneumonias. Despite the poor survivorship, there exists substantial variation in disease progression, making accurate prognostication difficult. Lung transplantation remains the sole curative intervention in IPF, but two anti-fibrotic therapies were recently shown to slow pulmonary function decline and are now approved for the treatment of IPF in many countries around the world. While the approval of these therapies represents an important first step in combatting of this devastating disease, a comprehensive approach to diagnosing and treating patients with IPF remains critically important. Included in this comprehensive assessment is the recognition and appropriate management of comorbid conditions. Though IPF is characterized by single organ involvement, many comorbid conditions occur within other organ systems. Common cardiovascular processes include coronary artery disease and pulmonary hypertension (PH, while gastroesophageal reflux and hiatal hernia are the most commonly encountered gastrointestinal disorders. Hematologic abnormalities appear to place patients with IPF at increased risk of venous thromboembolism, while diabetes mellitus (DM and hypothyroidism are prevalent metabolic disorders. Several pulmonary comorbidities have also been linked to IPF, and include emphysema, lung cancer, and obstructive sleep apnea. While the treatment of some comorbid conditions, such as CAD, DM, and hypothyroidism is recommended irrespective of IPF, the benefit of treating others, such as gastroesophageal reflux and PH, remains unclear. In this review, we highlight common comorbid conditions encountered in IPF, discuss disease-specific diagnostic

Pulmonary imaging techniques in the diagnosis of occupational interstitial lung disease

International Nuclear Information System (INIS)

Leonard, J.F.; Templeton, P.A.

1992-01-01

The chest radiograph is extensively used in evaluating workers at risk for developing occupational lung disease. Other pulmonary imaging techniques used in conjunction with the initial chest radiograph include conventional computed tomography, high resolution computed tomography, and gallium scintigraphy. This chapter evaluates the use of these techniques and their appropriate applications in the pneumoconioses, hypersensitivity pneumonitis, berylliosis, and hard metal diseases.65 references

Tomographic findings of acute pulmonary toxoplasmosis in immunocompetent patients.

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de Souza Giassi, Karina; Costa, Andre Nathan; Apanavicius, Andre; Teixeira, Fernando Bin; Fernandes, Caio Julio Cesar; Helito, Alfredo Salim; Kairalla, Ronaldo Adib

2014-11-25

Toxoplasmosis is one of the most common human zoonosis, and is generally benign in most of the individuals. Pulmonary involvement is common in immunocompromised subjects, but very rare in immunocompetents and there are scarce reports of tomographic findings in the literature. The aim of the study is to describe three immunocompetent patients diagnosed with acute pulmonary toxoplasmosis and their respective thoracic tomographic findings. Acute toxoplasmosis was diagnosed according to the results of serological tests suggestive of recent primary infection and the absence of an alternative etiology. From 2009 to 2013, three patients were diagnosed with acute respiratory failure secondary to acute toxoplasmosis. The patients were two female and one male, and were 38, 56 and 36 years old. Similarly they presented a two-week febrile illness and progressive dyspnea before admission. Laboratory tests demonstrated lymphocytosis, slight changes in liver enzymes and high inflammatory markers. Tomographic findings were bilateral smooth septal and peribronchovascular thickening (100%), ground-glass opacities (100%), atelectasis (33%), random nodules (33%), lymph node enlargement (33%) and pleural effusion (66%). All the patients improved their symptoms after treatment, and complete resolution of tomographic findings were found in the followup. These cases provide a unique description of the presentation and evolution of pulmonary tomographic manifestations of toxoplasmosis in immunocompetent patients. Toxoplasma pneumonia manifests with fever, dyspnea and a non-productive cough that may result in respiratory failure. In animal models, changes were described as interstitial pneumonitis with focal infiltrates of neutrophils that can finally evolve into a pattern of diffuse alveolar damage with focal necrosis. The tomographic findings are characterized as ground glass opacities, smooth septal and marked peribronchovascular thickening; and may mimic pulmonary congestion

Pulmonary phenotypes associated with genetic variation in telomere-related genes.

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Hoffman, Thijs W; van Moorsel, Coline H M; Borie, Raphael; Crestani, Bruno

2018-05-01

Genomic mutations in telomere-related genes have been recognized as a cause of familial forms of idiopathic pulmonary fibrosis (IPF). However, it has become increasingly clear that telomere syndromes and telomere shortening are associated with various types of pulmonary disease. Additionally, it was found that also single nucleotide polymorphisms (SNPs) in telomere-related genes are risk factors for the development of pulmonary disease. This review focuses on recent updates on pulmonary phenotypes associated with genetic variation in telomere-related genes. Genomic mutations in seven telomere-related genes cause pulmonary disease. Pulmonary phenotypes associated with these mutations range from many forms of pulmonary fibrosis to emphysema and pulmonary vascular disease. Telomere-related mutations account for up to 10% of sporadic IPF, 25% of familial IPF, 10% of connective-tissue disease-associated interstitial lung disease, and 1% of COPD. Mixed disease forms have also been found. Furthermore, SNPs in TERT, TERC, OBFC1, and RTEL1, as well as short telomere length, have been associated with several pulmonary diseases. Treatment of pulmonary disease caused by telomere-related gene variation is currently based on disease diagnosis and not on the underlying cause. Pulmonary phenotypes found in carriers of telomere-related gene mutations and SNPs are primarily pulmonary fibrosis, sometimes emphysema and rarely pulmonary vascular disease. Genotype-phenotype relations are weak, suggesting that environmental factors and genetic background of patients determine disease phenotypes to a large degree. A disease model is presented wherever genomic variation in telomere-related genes cause specific pulmonary disease phenotypes whenever triggered by environmental exposure, comorbidity, or unknown factors.

Pulmonary langerhans cell histiocytosis

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Suri Harpreet S

2012-03-01

Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

Pathological and radiological correlation in an autopsy case of combined pulmonary fibrosis and emphysema

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Karata H

2015-07-01

Full Text Available Hiroki Karata,1 Tomonori Tanaka,1 Ryoko Egashira,2 Kazuhiro Tabata,1 Kyoko Otani,3 Ryuji Hayashi,4 Takashi Hori,5 Junya Fukuoka1 1Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan; 2Department of Radiology, Faculty of Medicine, Saga University, Saga, Japan; 3Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan; 4Department of Internal Medicine, University of Toyama, Faculty of Medicine, Toyama, Japan; 5Laboratory of Pathology, Toyama University Hospital, Toyama, Japan Abstract: We report an educational autopsy case of combined pulmonary fibrosis and emphysema. Radiological patterns of the upper lung were considered as mostly emphysema, but pathological observation revealed significant interstitial fibrosis of usual interstitial pneumonia as a major pathology. The patient eventually developed acute exacerbation of background interstitial pneumonia. Careful radiological and pathological correlation of the current case indicates that regions with distal acinar emphysema on computed tomography image may possess histologically marked dense fibrosis of lethal interstitial pneumonia. Keywords: interstitial pneumonia, CPFE, AEF, smoking, CT

Study onthe prevalence of pulmonary lesions of slaughtered sheep at Urmia abattoir

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abdollah araghisoureh

2013-11-01

Full Text Available In regard to importance of various pathologic factors affecting respiratory system and its role in the selection of specific therapies in the sheep, 626 slaughtered sheep were examined to describe the pathological lesions of lower respiratory tract at Urmia abattoir. A total of 114/626 lungs (18.12% with macroscopic lesions were condemned and macro-microscopically examined. The specimens were processed through routine method for paraffin embedded sectioning (5-7micron and stained by Hematoxillin& Eosin method. Incidence of lesions in order of frequency were interstitial pneumonia (51; 8.14%,verminous pneumonia(23;3.67%, atelectasis (22; 3.51%, granulomatous pneumonia (22;3.51%, chronic bronchitis (21; 3.35%, fibrosis (13;2.07%, adenocarcinomas  (11;1.75%,chronic bronchiolitis  (10;1.59%, hydatic cyst  (8;1.27 %, pulmonary abscesses (7;1.11%, emphysema (6;0.95%, pleuritis (2;0.31% and pulmonary edema (2;0.319%. The result of this study showed that interstitial pneumonia was interestingly predominant among other lesions of condemned lungs which may reflect a seasonal difference.

A pilot study: a combined therapy using polymyxin-B hemoperfusion and extracorporeal membrane oxygenation for acute exacerbation of interstitial pneumonia.

Science.gov (United States)

Itai, Junji; Ohshimo, Shinichiro; Kida, Yoshiko; Ota, Kohei; Iwasaki, Yasumasa; Hirohashi, Nobuyuki; Bonella, Francesco; Guzman, Josune; Costabel, Ulrich; Kohno, Nobuoki; Tanigawa, Koichi

2015-01-05

Direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) might be beneficial for treating acute exacerbation (AE) of interstitial pneumonia (IP). Venovenous extracorporeal membranous oxygenation (VV-ECMO) is an emerging tool to avoid ventilator-induced lung injury. This is a report presenting the first three patients with AE of IP treated with a combined therapy of PMX-DHP and VV-ECMO. Patient 1 was a 68-year-old male with acute interstitial pneumonia, patient 2 a 67-year-old male with AE of idiopathic pulmonary fibrosis, and patient 3 a 61-year-old female with AE of collagen vascular disease-associated interstitial pneumonia. All patients were severely hypoxemic and required mechanical ventilation. A combined therapy using PMX-DHP and VV-ECMO was initiated with support of intravenous corticosteroids and antibiotics. Radiological findings, oxygenation and laboratory findings markedly improved and all patients survived without severe complications. A combined therapy of PMX-DHP and VV-ECMO might be a therapeutic option for AE of IP.

Cigarette smoking and pulmonary diffusion defects in rheumatoid arthritis.

Science.gov (United States)

Westedt, M L; Hazes, J M; Breedveld, F C; Sterk, P J; Dijkman, J H

1998-01-01

The pathogenesis of lung disease in rheumatoid arthritis (RA) has still to be defined. Risk factors associated with lung involvement in RA were investigated by means of pulmonary function studies in 40 RA patients without apparent lung disease. A decreased carbon monoxide (CO) diffusion capacity indicative of interstitial lung disease (ILD) was the main pulmonary function defect found in the first 20 patients. The occurrence was associated with current cigarette smoking. This association was confirmed in a case control study performed subsequently. These data suggest that ILD in RA is stimulated by smoking and provide an additional argument that modification of smoking behaviour in RA patients might lead to less severe complications.

Radiodiagnosis of pulmonary alterations in systemic lupus erythematosus patients

International Nuclear Information System (INIS)

Kamenetskij, M.S.; Lezova, T.F.; Kajzerman, I.A.; Sinyachenko, O.V.; Dyadyk, A.I.; Nikolenko, Yu.I.

1982-01-01

X-ray examination was carried out in 170 patients with systemic lupus erythematosus. Certain parameters of specific immunity were studied in 60 of them, while X-ray data were compared with morphological findings on autopsy in 20 cases. A tendency toward escalation of specific cell and humoral parameters was discovered in pulmonary lesion, predetermined by vasculitis and perivasculitis, as well as inflammatory and fibrotic alterations in the interstitial tissue

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings

International Nuclear Information System (INIS)

Kirchner, J.; Stein, A.; Thalhammer, A.; Jacobi, V.

1999-01-01

Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease. There are obvious discrepancies in the literature concerning the appearance of LAM on CT scans of the lung. This study adds the imaging findings of 11 patients and demonstrates how the imaging findings changed over time in four patients. Twenty-two CT examinations, and radiographs that had been obtained close to the CT examinations, of 11 patients with LAM confirmed by open lung biopsy were retrospectively evaluated with particular attention to the size of cystic lesions and wall thickness. Furthermore the CT scans were analysed for the type of pulmonary infiltration process and its distribution, presence or absence of pleural effusion, pneumothorax and lymph node enlargement. Clinical and CT follow-up studies were available in four patients. The CT scans revealed an increase in the interstitial pattern in all patients. Architectural distortion was seen in two patients and cystic lesions were present in all. The size of the cysts varied from small lesions to bullous emphysema. The cystic lesions revealed a wall thickness up to 2 mm but a wall was not perceptible in all. Pneumothorax was seen in only two patients; pleural effusion was seen in two patients. CT examination of patients with LAM reveals neither a uniform nor a pathognomonic appearance. In the early stages of LAM or in cases with interstitial changes the differential diagnosis of centrilobular emphysema or idiopathic pulmonary fibrosis seems to be more difficult than most authors believe. (orig.) (orig.)

Formation and annealing of metastable (interstitial oxygen)-(interstitial carbon) complexes in n- and p-type silicon

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Makarenko, L F; Lastovskii, S B; Murin, L I; Moll, M; Pintilie, I

2014-01-01

It is shown experimentally that, in contrast to the stable configuration of (interstitial carbon)-(interstitial oxygen) complexes (CiOi), the corresponding metastable configuration (CiOi{*}) cannot be found in n-Si based structures by the method of capacitance spectroscopy. The rates of transformation CiOi{*} -> CiOi are practically the same for both n- and p-Si with a concentration of charge carriers of no higher than 10(13) cm(-3). It is established that the probabilities of the simultaneous formation of stable and metastable configurations of the complex under study in the case of the addition of an atom of interstitial carbon to an atom of interstitial oxygen is close to 50\\%. This is caused by the orientation dependence of the interaction potential of an atom of interstitial oxygen with an interstitial carbon atom, which diffuses to this oxygen atom.

Immune complexes, gallium lung scans, and bronchoalveolar lavage in idiopathic interstitial pneumonitis-fibrosis

International Nuclear Information System (INIS)

Gelb, A.F.; Dreisen, R.B.; Epstein, J.D.; Silverthorne, J.D.; Bickel, Y.; Fields, M.; Border, W.A.; Taylor, C.R.

1983-01-01

We obtained results of lung immune complexes (LIC), circulating immune complexes (CIC), 48-hour gallium lung scans (scans), bronchoalveolar lavage (BAL), and pulmonary function tests in 20 patients with idiopathic interstitial pneumonitis-fibrosis. Sixteen patients had predominantly interstitial (13 cases UIP) and/or intraalveolar (3 cases DIP) cellular disease (group 1). Prior to corticosteroid therapy in group 1, scans were positive in 75 percent, CIC were elevated in 86 percent, LIC were present in 64 percent, and BAL was abnormal in 90 percent. Duration of follow-up after treatment was 3.5 +/- 1.0 year. In group 1 after treatment with corticosteroids in 13 patients and corticosteroids and penicillamine (three patients) and plasmapheresis (one patient), only four patients remain stable or improved. After corticosteroid therapy, elevated CIC returned to normal values despite progressive patient deterioration. In three patients, lung immune complexes were still detected after circulating immune complexes had returned to normal after corticosteroid therapy. In group 2 were four patients with fibrotic disease; scans and CIC were uniformly negative, LIC were weakly present in only one patient, and BAL was abnormal in all. Despite corticosteroid therapy, all have died or deteriorated. These results suggest that positive gallium lung scans, BAL, circulating immune complexes, and to a lesser extent, lung immune complexes are associated with the cellular phase of interstitial pneumonia, but do not reliably identify a corticosteroid-responsive group

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

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Karen L Heathcote

2011-01-01

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

Pulmonary health effects of agriculture.

Science.gov (United States)

Nordgren, Tara M; Bailey, Kristina L

2016-03-01

Occupational exposures in the agricultural industry are associated with numerous lung diseases, including chronic obstructive pulmonary disease, asthma, hypersensitivity pneumonitis, lung cancer, and interstitial lung diseases. Efforts are ongoing to ascertain contributing factors to these negative respiratory outcomes and improve monitoring of environmental factors leading to disease. In this review, recently published studies investigating the deleterious effects of occupational exposures in the agricultural industry are discussed. Occupational exposures to numerous agricultural environment aerosols, including pesticides, fungi, and bacteria are associated with impaired respiratory function and disease. Increases in certain farming practices, including mushroom and greenhouse farming, present new occupational exposure concerns. Improved detection methods may provide opportunities to better monitor safe exposure levels to known lung irritants. In the agricultural industry, occupational exposures to organic and inorganic aerosols lead to increased risk for lung disease among workers. Increased awareness of respiratory risks and improved monitoring of agricultural environments are necessary to limit pulmonary health risks to exposed populations.

Idiopathic pulmonary fibrosis vs. pulmonary involvement of collagen vascular disease: HRCT findings

International Nuclear Information System (INIS)

Lim, Myung Kwan; Im, Jung Gi; Ahn, Joong Mo; Kim, Ji Hye; Lee, Seon Kyu

1993-01-01

Both idiopathic pulmonary fibrosis (IPF) and pulmonary involvement of collagen vascular disease (CVD) are well known cause of diffuse interstitial lung disease which lead to fibrosis and honeycombing. We analyzed HRCT findings of 33 patients with IPF and 14 patients with CVD in terms of predominant pattern, site of involvement, mediastinal lymph node enlargement, pleural change and pulmonary volume loss. Criteria of mediastinal lymph node enlargement and pleural thickening were 15 mm in long diameter and 3 mm, respectively. Volume loss of the lung was measured by using hilar height ratio (apex to hilum/hilum to diaphragmatic dome). Mean age was 61 years for IPF and 46 years for CVD and male: female ratio was 27:6, 4:10, respectively. Predominant HRCT pattern was honeycombing for IPF (63%), and ground-glass opacity for CVD (66%) (p=0.001). Predominantly, subpleural involvement was seen in 90% for IPF and 74% for CVD. Mediastinal lymph node enlargement was seen in 47% of the patient with IPF and 14% with CVD (p=0.004). Pleural thickening was seen in 97% of the patients with IPF and 42% with CVD (p=0.002). Pleural effusion was seen in 10% of the patients with IPF and 36% with CVD (p=0.009). Hilar height ratio of more than 1.5 was seen in 84% of the patients with IPF and 29% with CVD. In conclusion, our study shows that patients with IPF are prone to have more progressed stage of pulmonary fibrosis than the patients with CVD on HRCT

Idiopathic pulmonary fibrosis vs. pulmonary involvement of collagen vascular disease: HRCT findings

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Lim, Myung Kwan; Im, Jung Gi; Ahn, Joong Mo; Kim, Ji Hye; Lee, Seon Kyu [Seoul National University College of Medicine, Seoul (Korea, Republic of)

1993-11-15

Both idiopathic pulmonary fibrosis (IPF) and pulmonary involvement of collagen vascular disease (CVD) are well known cause of diffuse interstitial lung disease which lead to fibrosis and honeycombing. We analyzed HRCT findings of 33 patients with IPF and 14 patients with CVD in terms of predominant pattern, site of involvement, mediastinal lymph node enlargement, pleural change and pulmonary volume loss. Criteria of mediastinal lymph node enlargement and pleural thickening were 15 mm in long diameter and 3 mm, respectively. Volume loss of the lung was measured by using hilar height ratio (apex to hilum/hilum to diaphragmatic dome). Mean age was 61 years for IPF and 46 years for CVD and male: female ratio was 27:6, 4:10, respectively. Predominant HRCT pattern was honeycombing for IPF (63%), and ground-glass opacity for CVD (66%) (p=0.001). Predominantly, subpleural involvement was seen in 90% for IPF and 74% for CVD. Mediastinal lymph node enlargement was seen in 47% of the patient with IPF and 14% with CVD (p=0.004). Pleural thickening was seen in 97% of the patients with IPF and 42% with CVD (p=0.002). Pleural effusion was seen in 10% of the patients with IPF and 36% with CVD (p=0.009). Hilar height ratio of more than 1.5 was seen in 84% of the patients with IPF and 29% with CVD. In conclusion, our study shows that patients with IPF are prone to have more progressed stage of pulmonary fibrosis than the patients with CVD on HRCT.

Clinical application of subtraction CT imaging for evaluation of pulmonary vascular permeability

International Nuclear Information System (INIS)

Kato, Shiro; Asai, Toshihiko; Yatagai, Shigeo; Oonuma, Noboru; Ohno, Kunihiko; Nakamoto, Takaaki; Iizuka, Masahiko

1991-01-01

In this clinical study, one normal subject, one patient with primary interstitial pneumonia, one patient with segmental pneumonia due to Staphylococcus aureus, one patient with post-operative esophageal carcinoma, and two patients with mitral stenosis were studied. Dynamic CT scan images under continuous injection of low osmotic contrast medium were analyzed in series, in an attempt to evaluate vascular permeability quantitatively. The following results were obtained. Subtraction CT scan image 10 minutes after the start of contrast medium injection in two patients with pneumonia, showed a reduction of pulmonary vascular permeability following therapy. Subtraction CT scan image of the patient with post-operative esophageal carcinoma treated with 25 Gy radiation showed a discrepancy between pulmonary vascular permeability and other findings. In hemodynamically stable patients with mitral stenosis, subtraction CT images demonstrated that pulmonary vascular permeability was not affected by pulmonary congestion, irrespective of its severity. (author)

Patient considerations and drug selection in the treatment of idiopathic pulmonary fibrosis

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Trawinska MA

2016-04-01

Full Text Available Maria A Trawinska,1 Ruwani D Rupesinghe,1 Simon P Hart1,2 1Hull and East Yorkshire Hospitals NHS Trust, 2Hull York Medical School, Academic Respiratory Medicine, Castle Hill Hospital, Cottingham, East Yorkshire, UK Abstract: Idiopathic pulmonary fibrosis (IPF is a progressive interstitial lung disease of unknown cause. Approximately 5,000 people are diagnosed with IPF in the UK every year. People with IPF suffer significant morbidity and, without any curative treatment at present, survival rates remain poor with a median survival of 3 years. While treatment remains largely supportive, many drug therapies have been trialed in IPF over the years. Pirfenidone and nintedanib are newly licensed treatments for IPF and the first drugs to have shown convincing evidence of slowing disease progression. In addition to evaluating clinical evidence, we also discuss elements affecting drug choice from the viewpoint of patients and health care professionals. We discuss pharmacological and nonpharmacological aspects of providing best supportive care for patients with IPF. However, few good quality studies exist focusing on controlling symptoms specifically in patients with IPF, and recommendations are often extrapolated from evidence in other chronic diseases. In covering these topics, we hope to provide readers with a comprehensive review of the available evidence pertaining to all aspects of care for patients suffering with IPF. Keywords: interstitial lung disease, high-resolution computed tomography, forced vital capacity, usual interstitial pneumonia, clinical trials, decision making, idiopathic pulmonary fibrosis

The zinc transporter ZIP12 regulates the pulmonary vascular response to chronic hypoxia.

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Zhao, Lan; Oliver, Eduardo; Maratou, Klio; Atanur, Santosh S; Dubois, Olivier D; Cotroneo, Emanuele; Chen, Chien-Nien; Wang, Lei; Arce, Cristina; Chabosseau, Pauline L; Ponsa-Cobas, Joan; Frid, Maria G; Moyon, Benjamin; Webster, Zoe; Aldashev, Almaz; Ferrer, Jorge; Rutter, Guy A; Stenmark, Kurt R; Aitman, Timothy J; Wilkins, Martin R

2015-08-20

The typical response of the adult mammalian pulmonary circulation to a low oxygen environment is vasoconstriction and structural remodelling of pulmonary arterioles, leading to chronic elevation of pulmonary artery pressure (pulmonary hypertension) and right ventricular hypertrophy. Some mammals, however, exhibit genetic resistance to hypoxia-induced pulmonary hypertension. We used a congenic breeding program and comparative genomics to exploit this variation in the rat and identified the gene Slc39a12 as a major regulator of hypoxia-induced pulmonary vascular remodelling. Slc39a12 encodes the zinc transporter ZIP12. Here we report that ZIP12 expression is increased in many cell types, including endothelial, smooth muscle and interstitial cells, in the remodelled pulmonary arterioles of rats, cows and humans susceptible to hypoxia-induced pulmonary hypertension. We show that ZIP12 expression in pulmonary vascular smooth muscle cells is hypoxia dependent and that targeted inhibition of ZIP12 inhibits the rise in intracellular labile zinc in hypoxia-exposed pulmonary vascular smooth muscle cells and their proliferation in culture. We demonstrate that genetic disruption of ZIP12 expression attenuates the development of pulmonary hypertension in rats housed in a hypoxic atmosphere. This new and unexpected insight into the fundamental role of a zinc transporter in mammalian pulmonary vascular homeostasis suggests a new drug target for the pharmacological management of pulmonary hypertension.

Atypical bovine interstitial pneumonia in a semi-intensive beef cattle system

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Ana Carolina Barreto Coelho

2017-11-01

Full Text Available ABSTRACT: The aim of the present study was to describe an outbreak of atypical interstitial pneumonia affecting 15 Angus cattle aged 7-30 months in the south of Rio Grande do Sul. The clinical course of the disease was approximately 1-3 days. The cattle were kept in the native field and had received feed containing the remains of wafers from a food factory before the outbreak. Clinical signs included dyspnea and abdominal breathing, with a clinical evolution period of up to 3 days. Nine animals presented apathy and respiratory difficulty and died within 24h, while three animals exhibited a clinical evolution period of 36-48h. Three other animals died without any clinical signs. The main macroscopic lesions were observed in the lungs, which did not collapse at the opening of the thoracic cavity and showed a rubbery consistency. On the cut surface, the organs were firm and dark red, with marked distension of the interlobular septa due to edema. Histopathological analysis revealed thickening of the interlobular pulmonary septa; interstitial edema and emphysema; mild mononuclear cell, eosinophil, and neutrophil infiltration; areas of type II pneumocyte proliferation, and thickening of the alveolar walls. Fibrin was observed in the alveolar lumen with the formation of hyaline membranes. On the basis of macroscopic and histological examinations and epidemiological data, a final diagnosis of atypical interstitial pneumonia was made. Although, the cause of the disease has not been identified, it is believed that the bran wafer meal contained ingredients that were not suitable for animals, consequently triggering the disease.

Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient

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J.J. Egan

2011-06-01

Full Text Available Idiopathic pulmonary fibrosis (IPF is a chronic, progressive, fatal form of diffuse interstitial lung disease. Management of IPF requires an orderly approach, with regular evaluations and implementation of both pharmacological and nonpharmacological treatments. Pulmonary rehabilitation can relieve patients from the distressing symptoms of IPF and improve quality of life. Oxygen therapy is central to treatment of all patients. Lung transplantation enhances survival in selected patients. Mechanical ventilation may be used in patients with acute exacerbations, but the prognosis is poor in these cases. Palliative care focuses on symptom management, advance directives and end-of-life planning. Patient support groups may also play an important role.

Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non-smoker female patient.

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Gupta, Pawan; Dash, Devijyoti; Mittal, Richa; Chhabra, Sunil K

2017-05-01

The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under-recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non-smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre-existent interstitial fibrosis, female gender and absence of a history of smoking. © 2015 John Wiley & Sons Ltd.

A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy.

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Jungmann, Sven; Ludwig, Wolf-Dieter; Schönfeld, Nicolas; Blum, Torsten-Gerriet; Großwendt, Claudia; Boch, Christian; Rehbock, Beate; Griff, Sergej; Schmittel, Alexander; Bauer, Torsten T

2017-01-01

We present a 74-year-old male with nonspecific interstitial pneumonia (NSIP) during treatment with ibrutinib for mantle cell lymphoma. Previously, the patient had received six cycles of bendamustine and rituximab and six cycles of R-CHOP, followed by rituximab maintenance therapy. Respiratory tract complications of ibrutinib other than infectious pneumonia have not been mentioned in larger trials, but individual case reports hinted to a possible association with the development of pneumonitis. In our patient, the onset of alveolitis that progressed towards NSIP together with the onset of ibrutinib treatment suggests causality. One week after ibrutinib was discontinued, nasal symptoms resolved first. A follow-up CT showed a reduction in the reticular hyperdensities and ground-glass opacities, suggestive of restitution of the lung disease. To our knowledge, this is the first case showing a strong link between ibrutinib and interstitial lung disease, strengthening a previous report on subacute pneumonitis. Our findings have clinical implications because pulmonary side effects were reversible at this early stage. We, therefore, suggest close monitoring for respiratory side effects in patients receiving ibrutinib.

A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy

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Sven Jungmann

2017-01-01

Full Text Available We present a 74-year-old male with nonspecific interstitial pneumonia (NSIP during treatment with ibrutinib for mantle cell lymphoma. Previously, the patient had received six cycles of bendamustine and rituximab and six cycles of R-CHOP, followed by rituximab maintenance therapy. Respiratory tract complications of ibrutinib other than infectious pneumonia have not been mentioned in larger trials, but individual case reports hinted to a possible association with the development of pneumonitis. In our patient, the onset of alveolitis that progressed towards NSIP together with the onset of ibrutinib treatment suggests causality. One week after ibrutinib was discontinued, nasal symptoms resolved first. A follow-up CT showed a reduction in the reticular hyperdensities and ground-glass opacities, suggestive of restitution of the lung disease. To our knowledge, this is the first case showing a strong link between ibrutinib and interstitial lung disease, strengthening a previous report on subacute pneumonitis. Our findings have clinical implications because pulmonary side effects were reversible at this early stage. We, therefore, suggest close monitoring for respiratory side effects in patients receiving ibrutinib.

Digital Clubbing Is Associated with Higher Serum KL-6 Levels and Lower Pulmonary Function in Patients with Interstitial Lung Disease

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Kazushige Shiraishi

2018-01-01

Full Text Available Background. Although digital clubbing is a common presentation in patients with interstitial lung disease (ILD, little has been reported regarding its role in assessing patients with ILD. This study evaluated patients with ILD for the presence of clubbing and investigated its association with clinical data. Methods. We evaluated patients with ILD who visited the teaching hospital at which the study was conducted, between October 2014 and January 2015. Clubbing, evaluated using a Vernier caliper for individual patients, was defined as a phalangeal depth ratio > 1. We examined the association of clubbing with clinical data. Results. Of 102 patients with ILD, we identified 17 (16.7% with clubbing. The partial pressure of oxygen in arterial blood was lower (65.2 ± 5.9 mmHg versus 80.2 ± 3.1 mmHg; p=0.03, serum Krebs von den Lugen-6 (KL-6 levels were higher (1495.0 ± 277.4 U/mL versus 839.1 ± 70.2 U/mL; p=0.001, and the percent predicted diffusing capacity of carbon monoxide was lower (50.0 ± 6.0 versus 73.5 ± 3.1; p=0.002 in these patients with clubbing. Conclusions. Patients with clubbing had lower oxygen levels, higher serum KL-6 levels, and lower pulmonary function than those without clubbing.

[Pulmonary involvement in connective tissue disease].

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Bartosiewicz, Małgorzata

2016-01-01

The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible. Thus, the identification of reversible disease , and separately progressive disease, are important clinical issues. The frequency, clinical presentation, prognosis and responce to therapy are different, depending on the pattern of involvement as well as on specyfic diagnostic method used to identify it. High- resolution computed tompography plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease. In this article, pulmonary lung involvement accompanying most frequently apperaing connective tissue diseases - rheumatoid arthritis, systemic sclerosis, lupus erythematosus, polymyositis/dermatomyositis, Sjögrens syndrome and mixed connective tissue disaese are reviewed.

An Exome Sequencing Study to Assess the Role of Rare Genetic Variation in Pulmonary Fibrosis.

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Petrovski, Slavé; Todd, Jamie L; Durheim, Michael T; Wang, Quanli; Chien, Jason W; Kelly, Fran L; Frankel, Courtney; Mebane, Caroline M; Ren, Zhong; Bridgers, Joshua; Urban, Thomas J; Malone, Colin D; Finlen Copeland, Ashley; Brinkley, Christie; Allen, Andrew S; O'Riordan, Thomas; McHutchison, John G; Palmer, Scott M; Goldstein, David B

2017-07-01

Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung disease of unknown etiology. The aim of this study was to use whole-exome sequencing to improve understanding of the genetic architecture of pulmonary fibrosis. We performed a case-control exome-wide collapsing analysis including 262 unrelated individuals with pulmonary fibrosis clinically classified as IPF according to American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guidelines (81.3%), usual interstitial pneumonia secondary to autoimmune conditions (11.5%), or fibrosing nonspecific interstitial pneumonia (7.2%). The majority (87%) of case subjects reported no family history of pulmonary fibrosis. We searched 18,668 protein-coding genes for an excess of rare deleterious genetic variation using whole-exome sequence data from 262 case subjects with pulmonary fibrosis and 4,141 control subjects drawn from among a set of individuals of European ancestry. Comparing genetic variation across 18,668 protein-coding genes, we found a study-wide significant (P RTEL1, and PARN. A model qualifying ultrarare, deleterious, nonsynonymous variants implicated TERT and RTEL1, and a model specifically qualifying loss-of-function variants implicated RTEL1 and PARN. A subanalysis of 186 case subjects with sporadic IPF confirmed TERT, RTEL1, and PARN as study-wide significant contributors to sporadic IPF. Collectively, 11.3% of case subjects with sporadic IPF carried a qualifying variant in one of these three genes compared with the 0.3% carrier rate observed among control subjects (odds ratio, 47.7; 95% confidence interval, 21.5-111.6; P = 5.5 × 10 -22 ). We identified TERT, RTEL1, and PARN-three telomere-related genes previously implicated in familial pulmonary fibrosis-as significant contributors to sporadic IPF. These results support the idea that telomere dysfunction is involved in IPF pathogenesis.

Connective tissue growth factor stimulates the proliferation, migration and differentiation of lung fibroblasts during paraquat-induced pulmonary fibrosis.

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Yang, Zhizhou; Sun, Zhaorui; Liu, Hongmei; Ren, Yi; Shao, Danbing; Zhang, Wei; Lin, Jinfeng; Wolfram, Joy; Wang, Feng; Nie, Shinan

2015-07-01

It is well established that paraquat (PQ) poisoning can cause severe lung injury during the early stages of exposure, finally leading to irreversible pulmonary fibrosis. Connective tissue growth factor (CTGF) is an essential growth factor that is involved in tissue repair and pulmonary fibrogenesis. In the present study, the role of CTGF was examined in a rat model of pulmonary fibrosis induced by PQ poisoning. Histological examination revealed interstitial edema and extensive cellular thickening of interalveolar septa at the early stages of poisoning. At 2 weeks after PQ administration, lung tissue sections exhibited a marked thickening of the alveolar walls with an accumulation of interstitial cells with a fibroblastic appearance. Masson's trichrome staining revealed a patchy distribution of collagen deposition, indicating pulmonary fibrogenesis. Western blot analysis and immunohistochemical staining of tissue samples demonstrated that CTGF expression was significantly upregulated in the PQ-treated group. Similarly, PQ treatment of MRC-5 human lung fibroblast cells caused an increase in CTGF in a dose-dependent manner. Furthermore, the addition of CTGF to MRC-5 cells triggered cellular proliferation and migration. In addition, CTGF induced the differentiation of fibroblasts to myofibroblasts, as was evident from increased expression of α-smooth muscle actin (α-SMA) and collagen. These findings demonstrate that PQ causes increased CTGF expression, which triggers proliferation, migration and differentiation of lung fibroblasts. Therefore, CTGF may be important in PQ-induced pulmonary fibrogenesis, rendering this growth factor a potential pharmacological target for reducing lung injury.

Lung clearance of 99mTc-DTPA aerosols in chronic obstructive pulmonary disease and interstitial pneumopathy diffuse

International Nuclear Information System (INIS)

Maliska, Carmelindo; Mello, Rossana Corbo de; Fonseca, Lea M.B.; Maliska, Celsa M.; Wolosker, Sara; Albernaz, Marta S.; Marinho, J.C.A.; Lima, Jose E.T.; Jansen, Jose M.

1996-01-01

An increasement in pulmonary epithelial permeability occurs when the alveolar-capillary units are disrupted. This permeability increasement can be measured by the 99m Tc-DTPA aerosol washout from lungs. Twenty three patients (10 healthy normal controls, 6 with COPD, 5 with diffuse infiltrative lung disease (DILD) and 2 asyntomatic patients that underwent radioiodine therapy dose) in order to verify the lung clearance rate of 99m Tc-DTPA in our population. The mean and standard deviation of pulmonary clearance rate in the normal group was 0,722±0,098%min -1 . The COPD patients rate resembled to the normal group rate. Four in five DILD patients had increased pulmonary clearance rate. The 2 patients that underwent radioiodine therapeutic showed normal clearance values of 99m Tc-DTPA from lungs. Our preliminary observations suggest that 99 'm Tc-DTPA clearance can be used as valuable tool in assessing early stages of pulmonary disorders in systemic diseases that may affect the lung. We also suggest that COPD does not seem to raise the pulmonary epithelial permeability. (author)

Comparative study of two models of combined pulmonary fibrosis and emphysema in mice.

Science.gov (United States)

Zhang, Wan-Guang; Wu, Si-Si; He, Li; Yang, Qun; Feng, Yi-Kuan; Chen, Yue-Tao; Zhen, Guo-Hua; Xu, Yong-Jian; Zhang, Zhen-Xiang; Zhao, Jian-Ping; Zhang, Hui-Lan

2017-04-01

Combined pulmonary fibrosis and emphysema (CPFE) is an "umbrella term" encompassing emphysema and pulmonary fibrosis, but its pathogenesis is not known. We established two models of CPFE in mice using tracheal instillation with bleomycin (BLM) or murine gammaherpesvirus 68 (MHV-68). Experimental mice were divided randomly into four groups: A (normal control, n=6), B (emphysema, n=6), C (emphysema+MHV-68, n=24), D (emphysema+BLM, n=6). Group C was subdivided into four groups: C1 (sacrificed on day 367, 7 days after tracheal instillation of MHV-68); C2 (day 374; 14days); C3 (day 381; 21days); C4 (day 388; 28days). Conspicuous emphysema and interstitial fibrosis were observed in BLM and MHV-68 CPFE mouse models. However, BLM induced diffuse pulmonary interstitial fibrosis with severely diffuse pulmonary inflammation; MHV-68 induced relatively modest inflammation and fibrosis, and the inflammation and fibrosis were not diffuse, but instead around bronchioles. Inflammation and fibrosis were detectable in the day-7 subgroup and reached a peak in the day-28 subgroup in the emphysema + MHV-68 group. Levels of macrophage chemoattractant protein-1, macrophage inflammatory protein-1α, interleukin-13, and transforming growth factor-β1 in bronchoalveolar lavage fluid were increased significantly in both models. Percentage of apoptotic type-2 lung epithelial cells was significantly higher; however, all four types of cytokine and number of macrophages were significantly lower in the emphysema+MHV-68 group compared with the emphysema +BLM group. The different changes in pathology between BLM and MHV-68 mice models demonstrated different pathology subtypes of CPFE: macrophage infiltration and apoptosis of type-II lung epithelial cells increased with increasing pathology score for pulmonary fibrosis. Copyright © 2017 Elsevier GmbH. All rights reserved.

Intracranial interstitial radiation

International Nuclear Information System (INIS)

Willis, D.; Rittenmeyer, H.; Hitchon, P.

1986-01-01

Primary malignant brain tumors are fatal, with 90% of patients having these tumors dying within two years following diagnosis. Cranial interstitial radiation therapy, a technique under investigation to control these tumors, involves implantation of radioactive iodine 125 seeds into the tumor bed by stereotaxic technique. The interstitial radiation technique, monitoring of radiation, and nursing care of patients are discussed. Case histories are presented, along with discussion of results attained using this therapy, and its future

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings

Energy Technology Data Exchange (ETDEWEB)

Kirchner, J.; Stein, A.; Thalhammer, A.; Jacobi, V. [Mainz Univ. (Germany). Inst. fuer Allgemeine Roentgendiagnostik; Viel, K.; Dietrich, C.F. [Frankfurt Univ. (Germany). Medizinische Klinik II; Schneider, M. [Zentrum fuer Pathologie, Frankfurt Univ. (Germany)

1999-02-01

Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease. There are obvious discrepancies in the literature concerning the appearance of LAM on CT scans of the lung. This study adds the imaging findings of 11 patients and demonstrates how the imaging findings changed over time in four patients. Twenty-two CT examinations, and radiographs that had been obtained close to the CT examinations, of 11 patients with LAM confirmed by open lung biopsy were retrospectively evaluated with particular attention to the size of cystic lesions and wall thickness. Furthermore the CT scans were analysed for the type of pulmonary infiltration process and its distribution, presence or absence of pleural effusion, pneumothorax and lymph node enlargement. Clinical and CT follow-up studies were available in four patients. The CT scans revealed an increase in the interstitial pattern in all patients. Architectural distortion was seen in two patients and cystic lesions were present in all. The size of the cysts varied from small lesions to bullous emphysema. The cystic lesions revealed a wall thickness up to 2 mm but a wall was not perceptible in all. Pneumothorax was seen in only two patients; pleural effusion was seen in two patients. CT examination of patients with LAM reveals neither a uniform nor a pathognomonic appearance. In the early stages of LAM or in cases with interstitial changes the differential diagnosis of centrilobular emphysema or idiopathic pulmonary fibrosis seems to be more difficult than most authors believe. (orig.) (orig.) With 5 figs., 2 tabs., 21 refs.

Further characterization of computed tomographic and clinical features for staging and prognosis of idiopathic pulmonary fibrosis in West Highland white terriers.

Science.gov (United States)

Thierry, Florence; Handel, Ian; Hammond, Gawain; King, Lesley G; Corcoran, Brendan M; Schwarz, Tobias

2017-07-01

Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology resulting in progressive interstitial fibrosis, with a known predilection in West Highland white terriers. In humans, computed tomography (CT) is a standard method for providing diagnostic and prognostic information, and plays a major role in the idiopathic pulmonary fibrosis staging process. Objectives of this retrospective, analytical, cross-sectional study were to establish descriptive criteria for reporting CT findings and test correlations among CT, clinical findings and survival time in West Highland white terriers with idiopathic pulmonary fibrosis. Inclusion criteria for affected West Highland white terriers were a diagnosis of idiopathic pulmonary fibrosis and available CT, bronchoscopy, bronchoalveolar lavage, echocardiography, and routine blood analysis findings. Clinically normal West Highland white terriers were recruited for the control group. Survival times were recorded for affected dogs. The main CT lung pattern and clinical data were blindly and separately graded as mild, moderate, or severe. Twenty-one West Highland white terriers with idiopathic pulmonary fibrosis and 11 control West Highland white terriers were included. The severity of pulmonary CT findings was positively correlated with severity of clinical signs (ρ = 0.48, P = 0.029) and negatively associated with survival time after diagnosis (ρ = -0.56, P = 0.025). Affected dogs had higher lung attenuation (median: -563 Hounsfield Units (HU)) than control dogs (median: -761 HU), (P idiopathic pulmonary fibrosis in West Highland white terriers and providing prognostic information for owners. © 2017 The Authors. Veterinary Radiology & Ultrasound published by Wiley Periodicals, Inc. on behalf of American College of Veterinary Radiology.

[Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema].

Science.gov (United States)

Casas, Juan Pablo; Abbona, Horacio; Robles, Adriana; López, Ana María

2008-01-01

Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of 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. Previous reports suggest that when both diseases coexist, pulmonary volumes are compensated and a smaller than expected reduction or even normal lung volumes can be found. We report 4 male patients of 64, 60, 73 and 70 years, all with heavy cigarette smoking history and progressive breathlessness. Three of them had severe limitation in their quality of life. All four showed advanced lung interstitial involvement, at high resolution CT scan, fibrotic changes predominantly in the subpleural areas of lower lung fields and concomitant emphysema in the upper lobes. Emphysema and pulmonary fibrosis was confirmed by open lung biopsy in one patient. The four patients showed normal spirometry and lung volumes with severe compromise of gas exchange and poor exercise tolerance evaluated by 6 minute walk test. Severe pulmonary arterial hypertension was also confirmed in three patients. Normal lung volumes does 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.

Pulmonary hypertension in chronic obstructive and interstitial lung diseases

DEFF Research Database (Denmark)

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

2013-01-01

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

Adenoviral transfer of HSP-70 into pulmonary epithelium ameliorates experimental acute respiratory distress syndrome.

Science.gov (United States)

Weiss, Yoram G; Maloyan, Alina; Tazelaar, John; Raj, Nichelle; Deutschman, Clifford S

2002-09-01

The acute respiratory distress syndrome (ARDS) provokes three pathologic processes: unchecked inflammation, interstitial/alveolar protein accumulation, and destruction of pulmonary epithelial cells. The highly conserved heat shock protein HSP-70 can limit all three responses but is not appropriately expressed in the lungs after cecal ligation and double puncture (2CLP), a clinically relevant model of ARDS. We hypothesize that restoring expression of HSP-70 using adenovirus-mediated gene therapy will limit pulmonary pathology following 2CLP. We administered a vector containing the porcine HSP-70 cDNA driven by a CMV promoter (AdHSP) into the lungs of rats subjected to 2CLP or sham operation. Administration of AdHSP after either sham operation or 2CLP increased HSP-70 protein expression in lung tissue, as determined by immunohistochemistry and Western blot hybridization. Administration of AdHSP significantly attenuated interstitial and alveolar edema and protein exudation and dramatically decreased neutrophil accumulation, relative to a control adenovirus. CLP-associated mortality at 48 hours was reduced by half. Modulation of HSP-70 production reduces pathologic changes and may improve outcome in experimental ARDS.

Migration of di- and tri-interstitials in silicon

International Nuclear Information System (INIS)

Posselt, M.; Gao, F.; Zwicker, D.

2005-01-01

A comprehensive study on the migration of di- and tri-interstitials in silicon is performed using classical molecular dynamics simulations with the Stillinger-Weber potential. The initial di- and tri-interstitial configurations with the lowest formation energies are determined, and then, the defect migration is investigated for temperatures between 800 and 1600 K. The defect diffusivity and the self-diffusion coefficient per defect are calculated. Compared to the mono-interstitial, the di-interstitial migrates faster, whereas the tri-interstitial diffuses slower. The migration mechanism of the di-interstitial shows a pronounced dependence on the temperature. Like in the case of the mono-interstitial, the mobility of the di-interstitial is higher than the mobility of the lattice atoms during the defect diffusion. On the other hand, the tri-interstitial mobility is lower than the corresponding atomic mobility. The implications of the present results for the analysis of experimental data on defect evolution and migration are discussed

Pulmonary infection in patients with cyclosporine, azathioprine, and corticosteroids after cardiac transplantation

International Nuclear Information System (INIS)

Murayama, Sadayuki; Ikezoe, Junpei; Godwin, J.D.; Marglin, S.I.; Allen, M.D.

1991-01-01

Between November 1985 and November 1989, 54 patients have undergone 55 cardiac transplants, 5 of whom died during operation or one week after transplantation. The remaining 49 patients with a minimum follow-up of 5 months were studied to examine pulmonary infection clinically and radiologically while receiving triple drug immunosuppression consisting of cyclosporine, azathioprine, and prednisolone. Pulmonary infection occurred in 14 patients (29%) with a total of 21 occasions. Causative organisms were identified in 9 occasions, with the most common organism being Cytomegalovirus (CMV). One patient died of pulmonary infection with Aspergillus. Causative organisms occurring in the remaining 12 occasions of pulmonary infection were unknown, which did not lead to death. Because pulmonary infection of unknown organisms rapidly responded to convensional antibiotics, it seemed to have been caused by bacteria. Pulmonary infection of unknown organism occurred 13.2±3.2 months after transplantation, as compared with 3.3±1.0 months in pulmonary infection of known organisms. Chest plain radiographic features fell into four types: (1) interstitial shadow seen in pulmonary infection of CMV, Pneumocystis carinii, or Hemophilia influenza, (2) patchy, and basilar and lobular consolidation shadows in bacterial pneumonia, (3) localized nodular shadow in aspergillosis, and (4) multiple patchy and confluent opacity patterns occurring in herpes simplex viral infection. Pulmonary infection of influenza bacteria for one patient and pulmonary infection of unknown organisms for 4 patients were difficult to identify from pulmonary infection of CMV. (N.K.)

18F-Fluorodeoxyglucose positron emission tomography pulmonary imaging in idiopathic pulmonary fibrosis is reproducible: implications for future clinical trials

International Nuclear Information System (INIS)

Win, Thida; Lambrou, Tryphon; Hutton, Brian F.; Kayani, Irfan; Endozo, Raymondo; Shortman, Robert I.; Groves, Ashley M.; Screaton, Nicholas J.; Porter, Joanna C.; Maher, Toby M.; Lukey, Pauline

2012-01-01

Noninvasive markers of disease activity in patients with idiopathic pulmonary fibrosis (IPF) are lacking. We performed this study to investigate the reproducibility of pulmonary 18 F-FDG PET/CT in patients with IPF. The study group comprised 13 patients (11 men, 2 women; mean age 71.1 ± 9.9 years) with IPF recruited for two thoracic 18 F-FDG PET/CT studies performed within 2 weeks of each other. All patients were diagnosed with IPF in consensus at multidisciplinary meetings as a result of typical clinical, high-resolution CT and pulmonary function test features. Three methods for evaluating pulmonary 18 F-FDG uptake were used. The maximal 18 F-FDG pulmonary uptake (SUVmax) in the lungs was determined using manual region-of-interest placement. An 18 F-FDG uptake intensity histogram was automatically constructed from segmented lungs to evaluate the distribution of SUVs. Finally, mean SUV was determined for volumes-of-interest in pulmonary regions with interstitial lung changes identified on CT scans. Processing included correction for tissue fraction effects. Bland-Altman analysis was performed and interclass correlation coefficients (ICC) were determined to assess the reproducibility between the first and second PET scans, as well as the level of intraobserver and interobserver agreement. The mean time between the two scans was 6.3 ± 4.3 days. The interscan ICCs for pulmonary SUVmax analysis and mean SUV corrected for tissue fraction effects were 0.90 and 0.91, respectively. Intensity histograms were different in only 1 of the 13 paired studies. Intraobserver agreement was also excellent (0.80 and 0.85, respectively). Some bias was observed between observers, suggesting that serial studies would benefit from analysis by the same observer. This study demonstrated that there is excellent short-term reproducibility in pulmonary 18 F-FDG uptake in patients with IPF. (orig.)

First-principles studies of di-arsenic interstitial and its implications for arsenic-interstitial diffusion in crystalline silicon

International Nuclear Information System (INIS)

Kim, Yonghyun; Kirichenko, Taras A.; Kong, Ning; Larson, Larry; Banerjee, Sanjay K.

2007-01-01

We propose new structural configurations and novel diffusion mechanisms for neutral di-arsenic interstitial (As 2 I 2 ) in silicon with a first-principle density functional theory simulation within the generalized gradient approximation. With an assumption of excess silicon interstitials and high arsenic concentrations, neutral As 2 I 2 is expected to be favorable and mobile with low-migration barrier. Moreover, because the diffusion barrier of arsenic interstitial pairs (AsI) is very low ( 2 I 2 can be easily formed and likely intermediate stage of larger arsenic interstitial clusters

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

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

2014-12-15

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

Pulmonary imaging using dual-energy CT, a role of the assessment of iodine and air distribution

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Ferda, Jiri, E-mail: e-mail@fnplzen.cz [Radiodiagnostic Clinic, Charles University Teaching Hospital Plzen, Alej Svobody 80, 30640 Plzen (Czech Republic); Ferdova, Eva; Mirka, Hynek; Baxa, Jan; Bednarova, Alena [Radiodiagnostic Clinic, Charles University Teaching Hospital Plzen, Alej Svobody 80, 30640 Plzen (Czech Republic); Flohr, Thomas; Schmidt, Bernhard [Siemens Healthcare, Computed Tomography, 91301 Siemensstr. 1, Forchheim (Germany); Matejovic, Martin [1st Internal Department, Charles University Teaching Hospital Plzen, Alej Svobody 80, 30640 Plzen (Czech Republic); Kreuzberg, Boris [Radiodiagnostic Clinic, Charles University Teaching Hospital Plzen, Alej Svobody 80, 30640 Plzen (Czech Republic)

2011-02-15

Aim: The aim of the study is to present the feasibility of using dual-energy CT and the evaluation of iodine and air distribution in differentiation of pathological conditions. Material and method: We used the data of 50 CT examinations performed due to suspected pulmonary embolism with any pathological finding except consolidation of the parenchyma. The patients underwent CT angiography of the pulmonary arteries on a dual-source CT (DSCT), with the two tubes independently operated at 140 and 80 kV. By exploiting the dual-energy information, iodine distribution maps were obtained in addition to the conventional CT images which served as a marker of pulmonary perfusion. Minimum intensity projections (MinIP) were used as a marker of air content. Results: By comparing the iodine distribution maps and MinIP images, it was possible to differentiate between the following templates of lung parenchyma: A - normal iodine and air distribution; B - iodine content deficit with minimal or with no redistribution of air; C - reduced iodine content and increased content of air; D - deficit of iodine content and increased content of air; E - increased iodine content and normal content of air; F - increased iodine content and reduced content of air; G - reduced perfusion and reduced content of air. The type A (five cases) was typical for the pulmonary embolism with preserved normal conditions of perfusion and ventilation. Type B (18 cases) occurred in pulmonary embolism; type C was found in case of inflammation of small respiratory airways (five cases); emphysema was typical for type D (nine cases); increased perfusion was observed in the parenchyma preserved from emphysema or preserved from embolism in cases of emphysema or pulmonary embolism; type F occurred in pulmonary interstitial edema (four cases) both with pulmonary infection; finally type G was found in interstitial lung diseases (five cases). Conclusion: Imaging of the pulmonary circulation by means of dual-energy CT opens

Interstitial irradiation for craniopharyngioma

International Nuclear Information System (INIS)

Barlas, O.; Bayindir, C.; Can, M.

2000-01-01

The results of interstitial irradiation treatment for craniopharyngioma in two patients with six year follow-ups are presented. Stereotactic interstitial irradiation with iodine-125 sources as sole therapy was employed in two adult patients who refused surgical resection. The diagnoses were confirmed by stereotactic biopsy. The first tumour which underwent interstitial irradiation was solid and 4 cm in diameter, and the second, 2.7 cm in diameter, had both cystic and solid components. The implanted iodine-125 seeds delivered 67 Gy and 60 Gy to tumour periphery at the rate of 12 and 14 cGy/h, respectively, were removed at the end of designated radiation periods. Tumour shrinkage and central hypo density, first observed 3 months after irradiation, continued until one tumour shrank to less than 1 cm at 12 months, and the other disappeared completely at 24 months. In both cases functional integrity was restored, and neither radiation induced toxicity nor recurrence has occurred six years after treatment. The results in these two cases suggest that solid craniopharyngiomas are sensitive to interstitial irradiation. (author)

Protein S is protective in pulmonary fibrosis.

Science.gov (United States)

Urawa, M; Kobayashi, T; D'Alessandro-Gabazza, C N; Fujimoto, H; Toda, M; Roeen, Z; Hinneh, J A; Yasuma, T; Takei, Y; Taguchi, O; Gabazza, E C

2016-08-01

Essentials Epithelial cell apoptosis is critical in the pathogenesis of idiopathic pulmonary fibrosis. Protein S, a circulating anticoagulant, inhibited apoptosis of lung epithelial cells. Overexpression of protein S in lung cells reduced bleomycin-induced pulmonary fibrosis. Intranasal therapy with exogenous protein S ameliorated bleomycin-induced pulmonary fibrosis. Background Pulmonary fibrosis is the terminal stage of interstitial lung diseases, some of them being incurable and of unknown etiology. Apoptosis plays a critical role in lung fibrogenesis. Protein S is a plasma anticoagulant with potent antiapoptotic activity. The role of protein S in pulmonary fibrosis is unknown. Objectives To evaluate the clinical relevance of protein S and its protective role in pulmonary fibrosis. Methods and Results The circulating level of protein S was measured in patients with pulmonary fibrosis and controls by the use of enzyme immunoassays. Pulmonary fibrosis was induced with bleomycin in transgenic mice overexpressing human protein S and wild-type mice, and exogenous protein S or vehicle was administered to wild-type mice; fibrosis was then compared in both models. Patients with pulmonary fibrosis had reduced circulating levels of protein S as compared with controls. Inflammatory changes, the levels of profibrotic cytokines, fibrosis score, hydroxyproline content in the lungs and oxygen desaturation were significantly reduced in protein S-transgenic mice as compared with wild-type mice. Wild-type mice treated with exogenous protein S showed significant decreases in the levels of inflammatory and profibrotic markers and fibrosis in the lungs as compared with untreated control mice. After bleomycin infusion, mice overexpressing human protein S showed significantly low caspase-3 activity, enhanced expression of antiapoptotic molecules and enhanced Akt and Axl kinase phosphorylation as compared with wild-type counterparts. Protein S also inhibited apoptosis of alveolar

[Adult-onset Still's disease with pulmonary and cardiac involvement and response to intravenous immunoglobulin].

Science.gov (United States)

Neto, Nilton Salles Rosa; Waldrich, Leandro; de Carvalho, Jozélio Freire; Pereira, Rosa Maria Rodrigues

2009-01-01

Cardiopulmonary manifestations of adult-onset Still's disease (AOSD) include pericarditis, pleural effusion, transient pulmonary infiltrates, pulmonary interstitial disease and myocarditis. Serositis are common but pneumonitis and myocarditis are not and bring elevated risk of mortality. They may manifest on disease onset or flares. Previously reported cases were treated with high-dose glucocorticoids and immunosupressants and, when refractory, intravenous immunoglobulin (IVIG). We report an AOSD patient whose flare presented with severe pleupneumonitis and myopericarditis and, following nonresponse to a methylprednisolone pulse, high dose of prednisone and cyclosporine A, recovered after a 2-day 1g/kg/day IVIG infusion.

Automated Quantitative Computed Tomography Versus Visual Computed Tomography Scoring in Idiopathic Pulmonary Fibrosis: Validation Against Pulmonary Function.

Science.gov (United States)

Jacob, Joseph; Bartholmai, Brian J; Rajagopalan, Srinivasan; Kokosi, Maria; Nair, Arjun; Karwoski, Ronald; Raghunath, Sushravya M; Walsh, Simon L F; Wells, Athol U; Hansell, David M

2016-09-01

The aim of the study was to determine whether a novel computed tomography (CT) postprocessing software technique (CALIPER) is superior to visual CT scoring as judged by functional correlations in idiopathic pulmonary fibrosis (IPF). A total of 283 consecutive patients with IPF had CT parenchymal patterns evaluated quantitatively with CALIPER and by visual scoring. These 2 techniques were evaluated against: forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), diffusing capacity for carbon monoxide (DLco), carbon monoxide transfer coefficient (Kco), and a composite physiological index (CPI), with regard to extent of interstitial lung disease (ILD), extent of emphysema, and pulmonary vascular abnormalities. CALIPER-derived estimates of ILD extent demonstrated stronger univariate correlations than visual scores for most pulmonary function tests (PFTs): (FEV1: CALIPER R=0.29, visual R=0.18; FVC: CALIPER R=0.41, visual R=0.27; DLco: CALIPER R=0.31, visual R=0.35; CPI: CALIPER R=0.48, visual R=0.44). Correlations between CT measures of emphysema extent and PFTs were weak and did not differ significantly between CALIPER and visual scoring. Intriguingly, the pulmonary vessel volume provided similar correlations to total ILD extent scored by CALIPER for FVC, DLco, and CPI (FVC: R=0.45; DLco: R=0.34; CPI: R=0.53). CALIPER was superior to visual scoring as validated by functional correlations with PFTs. The pulmonary vessel volume, a novel CALIPER CT parameter with no visual scoring equivalent, has the potential to be a CT feature in the assessment of patients with IPF and requires further exploration.

The behavior of interstitials in irradiated graphite

International Nuclear Information System (INIS)

Pedraza, D.F.

1991-01-01

A computer model is developed to simulate the behavior of self-interstitials with particular attention to clustering. Owing to the layer structure of graphite, atomistic simulations can be performed using a large parallelepipedic supercell containing a few layers. In particular, interstitial clustering is studied here using a supercell that contains two basal planes only. Frenkel pairs are randomly produced. Interstitials are placed at sites between the crystal planes while vacancies are distributed in the two crystal planes. The size of the computational cell is 20000 atoms and periodic boundary conditions are used in two dimensions. Vacancies are assumed immobile whereas interstitials are given a certain mobility. Two point defect sinks are considered, direct recombination of Frenkel pairs and interstitial clusters. The clusters are assumed to be mobile up to a certain size where they are presumed to become loop nuclei. Clusters can shrink by emission of singly bonded interstitials or by recombination of a peripheral interstitial with a neighboring vacancy. The conditions under which interstitial clustering occurs are reported. It is shown that when clustering occurs the cluster size population gradually shifts towards the largest size cluster. The implications of the present results for irradiation growth and irradiation-induced amorphization are discussed

Interstitial Cells of Blood Vessels

Directory of Open Access Journals (Sweden)

Vladimír Pucovský

2010-01-01

Full Text Available Blood vessels are made up of several distinct cell types. Although it was originally thought that the tunica media of blood vessels was composed of a homogeneous population of fully differentiated smooth muscle cells, more recent data suggest the existence of multiple smooth muscle cell subpopulations in the vascular wall. One of the cell types contributing to this heterogeneity is the novel, irregularly shaped, noncontractile cell with thin processes, termed interstitial cell, found in the tunica media of both veins and arteries. While the principal role of interstitial cells in veins seems to be pacemaking, the role of arterial interstitial cells is less clear. This review summarises the knowledge of the functional and structural properties of vascular interstitial cells accumulated so far, offers hypotheses on their physiological role, and proposes directions for future research.

Immersion pulmonary oedema in a healthy diver not exposed to cold or strenuous exercise.

Science.gov (United States)

Castagna, Olivier; de Maistre, Sébastien; Schmid, Bruno; Caudal, Delphine; Regnard, Jacques

2018-03-31

In healthy divers, the occurrence of immersion pulmonary oedema (IPE) is commonly caused by contributory factors including strenuous exercise, cold water and negative-pressure breathing. Contrary to this established paradigm, this case reports on a 26-year-old, well-trained combat swimmer who succumbed to acute IPE during static immersion in temperate (21°C) water, while using a front-mounted counterlung rebreather. The incident occurred during repeated depth-controlled ascent practice at the French military diving school. It was discovered that the diver had attempted to stop any gas leakage into the system by over-tightening the automatic diluent valve (ADV) (25th notch of 27) during the dive, thus causing a high resistance to inspiratory flow. The ventilatory constraints imposed by this ADV setting were assessed as a 3.2 Joules·L⁻¹ inspiratory work of breathing and -5 kPa (-50 mbar) transpulmonary pressure. This report confirms the key role of negative pressure breathing in the development of interstitial pulmonary oedema. Such a breathing pattern can cause a lowering of thoracic, airway and interstitial lung pressure, leading to high capillary pressure during each inspiration. Repetition of the diving drills resulted in an accumulation of interstitial lung water extravasation, causing pathological decompensation and proven symptoms. Copyright: This article is the copyright of the authors who grant Diving and Hyperbaric Medicine a non-exclusive licence to publish the article in electronic and other forms.

Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia.

Science.gov (United States)

Sellarés, Jacobo; Hernández-González, Fernanda; Lucena, Carmen M; Paradela, Marina; Brito-Zerón, Pilar; Prieto-González, Sergio; Benegas, Mariana; Cuerpo, Sandra; Espinosa, Gerard; Ramírez, José; Sánchez, Marcelo; Xaubet, Antoni

2016-02-01

Auscultation of Velcro crackles has been proposed as a key finding in physical lung examination in patients with interstitial lung diseases (ILDs), especially in idiopathic pulmonary fibrosis (IPF). However, no studies have been carried out to assess the association of Velcro crackles with other clinical variables.We evaluated a cohort of 132 patients, prospectively and consecutively included in our ILD diagnostic program at a tertiary referral center. All patients were auscultated during the physical examination. The patients were divided into 2 groups: "presence" or "nonpresence" of bilateral Velcro crackles.Of all patients assessed, 83 (63%) presented Velcro crackles in the respiratory auscultation. Patients with Velcro crackles usually had more frequently cough and dyspnea at the moment of diagnosis. Forced vital capacity (P = 0.002) and lung diffusion capacity for carbon monoxide (P = 0.04) was lower in these patients. The ILD-GAP index was higher in the group with Velcro crackles (P = 0.01). All patients with usual interstitial pneumonia (UIP) in high-resolution computed tomography and all patients with final IPF diagnosis presented Velcro crackles. In multivariate analysis, the presence of Velcro crackles was independently associated with an UIP pattern.In patients suspected of having ILD, the auscultation of Velcro crackles was associated with UIP, a possibility which must be taken into consideration in early ILD detection in primary care.

Non-specific interstitial pneumonia in cigarette smokers: a CT study

International Nuclear Information System (INIS)

Marten, Katharina; Milne, David; Antoniou, Katerina M.; Nicholson, Andrew G.; Tennant, Rachel C.; Wells, Athol U.; Hansel, Trevor T.; Hansell, David M.

2009-01-01

The goal of this study was to seek indirect evidence that smoking is an aetiological factor in some patients with non-specific interstitial pneumonia (NSIP). Ten current and eight ex-smokers with NSIP were compared to controls including 137 current smokers with no known interstitial lung disease and 11 non-smokers with NSIP. Prevalence and extent of emphysema in 18 smokers with NSIP were compared with subjects meeting GOLD criteria for chronic obstructive pulmonary disease (COPD; group A; n = 34) and healthy smokers (normal FEV 1 ; group B; n = 103), respectively. Emphysema was present in 14/18 (77.8%) smokers with NSIP. Emphysema did not differ in prevalence between NSIP patients and group A controls (25/34, 73.5%), but was strikingly more prevalent in NSIP patients than in group B controls (18/103, 17.5%, P < 0.0005). On multiple logistic regression, the likelihood of emphysema increased when NSIP was present (OR = 18.8; 95% CI = 5.3-66.3; P < 0.0005) and with increasing age (OR = 1.04; 95% CI = 0.99-1.11; P = 0.08). Emphysema is as prevalent in smokers with NSIP as in smokers with COPD, and is strikingly more prevalent in these two groups than in healthy smoking controls. The association between NSIP and emphysema provides indirect support for a smoking pathogenesis hypothesis in some NSIP patients. (orig.)

Non-specific interstitial pneumonia in cigarette smokers: a CT study

Energy Technology Data Exchange (ETDEWEB)

Marten, Katharina [Georg August University of Goettingen, Department of Radiology, Goettingen (Germany); Milne, David [Green Lane Hospital, Department of Radiology, Auckland (New Zealand); Antoniou, Katerina M. [University of Crete, Department of Thoracic Medicine, Heraklion, Crete (Greece); Nicholson, Andrew G. [Royal Brompton Hospital, Department of Histopathology, London (United Kingdom); Tennant, Rachel C.; Wells, Athol U. [Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom); Hansel, Trevor T. [Royal Brompton Hospital, Clinical Trials Unit, London (United Kingdom); Hansell, David M. [Royal Brompton Hospital, Department of Radiology, London (United Kingdom)

2009-07-15

The goal of this study was to seek indirect evidence that smoking is an aetiological factor in some patients with non-specific interstitial pneumonia (NSIP). Ten current and eight ex-smokers with NSIP were compared to controls including 137 current smokers with no known interstitial lung disease and 11 non-smokers with NSIP. Prevalence and extent of emphysema in 18 smokers with NSIP were compared with subjects meeting GOLD criteria for chronic obstructive pulmonary disease (COPD; group A; n = 34) and healthy smokers (normal FEV{sub 1}; group B; n = 103), respectively. Emphysema was present in 14/18 (77.8%) smokers with NSIP. Emphysema did not differ in prevalence between NSIP patients and group A controls (25/34, 73.5%), but was strikingly more prevalent in NSIP patients than in group B controls (18/103, 17.5%, P < 0.0005). On multiple logistic regression, the likelihood of emphysema increased when NSIP was present (OR = 18.8; 95% CI = 5.3-66.3; P < 0.0005) and with increasing age (OR = 1.04; 95% CI = 0.99-1.11; P = 0.08). Emphysema is as prevalent in smokers with NSIP as in smokers with COPD, and is strikingly more prevalent in these two groups than in healthy smoking controls. The association between NSIP and emphysema provides indirect support for a smoking pathogenesis hypothesis in some NSIP patients. (orig.)

Genetics and Early Detection in Idiopathic Pulmonary Fibrosis

Science.gov (United States)

Putman, Rachel K.; Rosas, Ivan O.

2014-01-01

Genetic studies hold promise in helping to identify patients with early idiopathic pulmonary fibrosis (IPF). Recent studies using chest computed tomograms (CTs) in smokers and in the general population have demonstrated that imaging abnormalities suggestive of an early stage of pulmonary fibrosis are not uncommon and are associated with respiratory symptoms, physical examination abnormalities, and physiologic decrements expected, but less severe than those noted in patients with IPF. Similarly, recent genetic studies have demonstrated strong and replicable associations between a common promoter polymorphism in the mucin 5B gene (MUC5B) and both IPF and the presence of abnormal imaging findings in the general population. Despite these findings, it is important to note that the definition of early-stage IPF remains unclear, limited data exist to definitively connect abnormal imaging findings to IPF, and genetic studies assessing early-stage pulmonary fibrosis remain in their infancy. In this perspective we provide updated information on interstitial lung abnormalities and their connection to IPF. We summarize information on the genetics of pulmonary fibrosis by focusing on the recent genetic findings of MUC5B. Finally, we discuss the implications of these findings and suggest a roadmap for the use of genetics in the detection of early IPF. PMID:24547893

{sup 18}F-Fluorodeoxyglucose positron emission tomography pulmonary imaging in idiopathic pulmonary fibrosis is reproducible: implications for future clinical trials

Energy Technology Data Exchange (ETDEWEB)

Win, Thida [Lister Hospital, Respiratory Medicine, Stevenage (United Kingdom); Lambrou, Tryphon; Hutton, Brian F.; Kayani, Irfan; Endozo, Raymondo; Shortman, Robert I.; Groves, Ashley M. [UCL/UCH, Institute of Nuclear Medicine, London (United Kingdom); Screaton, Nicholas J. [Papworth Hospital, Radiology Department, Cambridge (United Kingdom); Porter, Joanna C. [UCL/UCH, Centre for Respiratory Diseases, London (United Kingdom); Maher, Toby M. [Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom); Lukey, Pauline [GSK, Fibrosis DPU, Research and Development, Stevenage (United Kingdom)

2012-03-15

Noninvasive markers of disease activity in patients with idiopathic pulmonary fibrosis (IPF) are lacking. We performed this study to investigate the reproducibility of pulmonary {sup 18}F-FDG PET/CT in patients with IPF. The study group comprised 13 patients (11 men, 2 women; mean age 71.1 {+-} 9.9 years) with IPF recruited for two thoracic {sup 18}F-FDG PET/CT studies performed within 2 weeks of each other. All patients were diagnosed with IPF in consensus at multidisciplinary meetings as a result of typical clinical, high-resolution CT and pulmonary function test features. Three methods for evaluating pulmonary {sup 18}F-FDG uptake were used. The maximal {sup 18}F-FDG pulmonary uptake (SUVmax) in the lungs was determined using manual region-of-interest placement. An {sup 18}F-FDG uptake intensity histogram was automatically constructed from segmented lungs to evaluate the distribution of SUVs. Finally, mean SUV was determined for volumes-of-interest in pulmonary regions with interstitial lung changes identified on CT scans. Processing included correction for tissue fraction effects. Bland-Altman analysis was performed and interclass correlation coefficients (ICC) were determined to assess the reproducibility between the first and second PET scans, as well as the level of intraobserver and interobserver agreement. The mean time between the two scans was 6.3 {+-} 4.3 days. The interscan ICCs for pulmonary SUVmax analysis and mean SUV corrected for tissue fraction effects were 0.90 and 0.91, respectively. Intensity histograms were different in only 1 of the 13 paired studies. Intraobserver agreement was also excellent (0.80 and 0.85, respectively). Some bias was observed between observers, suggesting that serial studies would benefit from analysis by the same observer. This study demonstrated that there is excellent short-term reproducibility in pulmonary {sup 18}F-FDG uptake in patients with IPF. (orig.)

The role of inducible nitric oxide synthase for interstitial remodeling of alveolar septa in surfactant protein D-deficient mice

Science.gov (United States)

Atochina-Vasserman, Elena N.; Massa, Christopher B.; Birkelbach, Bastian; Guo, Chang-Jiang; Scott, Pamela; Haenni, Beat; Beers, Michael F.; Ochs, Matthias; Gow, Andrew J.

2015-01-01

Surfactant protein D (SP-D) modulates the lung's immune system. Its absence leads to NOS2-independent alveolar lipoproteinosis and NOS2-dependent chronic inflammation, which is critical for early emphysematous remodeling. With aging, SP-D knockout mice develop an additional interstitial fibrotic component. We hypothesize that this age-related interstitial septal wall remodeling is mediated by NOS2. Using invasive pulmonary function testing such as the forced oscillation technique and quasistatic pressure-volume perturbation and design-based stereology, we compared 29-wk-old SP-D knockout (Sftpd−/−) mice, SP-D/NOS2 double-knockout (DiNOS) mice, and wild-type mice (WT). Structural changes, including alveolar epithelial surface area, distribution of septal wall thickness, and volumes of septal wall components (alveolar epithelium, interstitial tissue, and endothelium) were quantified. Twenty-nine-week-old Sftpd−/− mice had preserved lung mechanics at the organ level, whereas elastance was increased in DiNOS. Airspace enlargement and loss of surface area of alveolar epithelium coexist with increased septal wall thickness in Sftpd−/− mice. These changes were reduced in DiNOS, and compared with Sftpd−/− mice a decrease in volumes of interstitial tissue and alveolar epithelium was found. To understand the effects of lung pathology on measured lung mechanics, structural data were used to inform a computational model, simulating lung mechanics as a function of airspace derecruitment, septal wall destruction (loss of surface area), and septal wall thickening. In conclusion, NOS2 mediates remodeling of septal walls, resulting in deposition of interstitial tissue in Sftpd−/−. Forward modeling linking structure and lung mechanics describes the complex mechanical properties by parenchymatous destruction (emphysema), interstitial remodeling (septal wall thickening), and altered recruitability of acinar airspaces. PMID:26320150

Use of High-Flow Nasal Cannula Oxygen Therapy in a Pregnant Woman with Dermatomyositis-Related Interstitial Pneumonia

Directory of Open Access Journals (Sweden)

Tomohiro Shoji

2017-01-01

Full Text Available A 33-year-old pregnant woman was referred to our hospital with respiratory distress at 30 weeks of gestation. Chest computed tomography (CT scans revealed pulmonary infiltrates along the bronchovascular bundles and ground-glass opacities in both lungs. Despite immediate treatment with steroid pulse therapy for suspected interstitial pneumonia, the patient’s condition worsened. Respiratory distress was slightly alleviated after the initiation of high-flow nasal cannula (HFNC oxygen therapy (40 L/min, FiO2 40%. We suspected clinically amyopathic dermatomyositis (CADM complicating rapidly progressive refractory interstitial pneumonia. In order to save the life of the patient, the use of combination therapy with immunosuppressants was necessary. The patient underwent emergency cesarean section and was immediately treated with immunosuppressants while continuing HFNC oxygen therapy. The neonate was treated in the neonatal intensive care unit. The patient’s condition improved after 7 days of hospitalization; by this time, she was positive for myositis-specific autoantibodies and was diagnosed with interstitial pneumonia preceding dermatomyositis. This condition can be potentially fatal within a few months of onset and therefore requires early combination immunosuppressive therapy. This case demonstrates the usefulness of HFNC oxygen therapy for respiratory management as it negates the need for intubation and allows for various treatments to be quickly performed.

Granulomatous interstitial pneumonia in a miniature swine associated with repeated intravenous injections of Tc-99m human serum albumin: concise communication

International Nuclear Information System (INIS)

Whinnery, J.E.; Young, J.T.

1980-01-01

Albumin lung-scanning agents have a proven high degree of safety, with the only contraindication to their use being allergic hypersensitivity. We have used these agents to investigate the physiologic effects of high G/sub z/ acceleratory forces on pulmonary perfusion using the miniature swine. Multiple doses of human macroaggregated albumin and human-albumin microspheres were given to a miniature swine at various levels of centrifugal acceleration over a 6-wk period. The dosages given were the same per kilogram as those used for routine clinical human studies. The animal subsequently died from a severe granulomatous interstitial pneumonia. The granulomatous lesions suggest that the pathogenesis may have involved a cell-mediated delayed hypersensitivity. This interstitial pneumonia may represent the end point in a chronic hypersensitivity response to the human-albumin lung-scanning agents

Smoking and Pulmonary Fibrosis: Novel Insights

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Katerina D. Samara

2011-01-01

Full Text Available The relationship between smoking and pulmonary fibrosis is under debate and intense investigation. The aim of this paper is to review the existing literature and identify further areas of research interest. Recently the negative influence of cigarette smoking on IPF outcome was highlighted, as non-smokers exhibit a better survival than ex-smokers and combined current- and ex-smokers. In patients with non-specific interstitial pneumonia (NSIP, a high prevalence of emphysema was recently demonstrated, providing an indirect support for a smoking pathogenetic hypothesis in NSIP. The coexistence of pulmonary fibrosis and emphysema has been extensively described in a syndrome termed combined pulmonary fibrosis and emphysema (CPFE. Connective tissue disorders (CTDs are a group of autoimmune diseases which affect the lung, as one of the most common and severe manifestations. However, the relationship between smoking and autoimmune disorders is still conflicting. Rheumatoid arthritis results from the interaction between genetic and environmental factors, while the best established environmental factor is tobacco smoking. Smoking has also a negative impact on the response of the RA patients to treatment. The aforementioned smoking-related implications give rise to further research questions and certainly provide one more important reason for physicians to advocate smoking cessation and smoke-free environment.

Chylothorax in dermatomyositis complicated with interstitial pneumonia.

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Isoda, Kentaro; Kiboshi, Takao; Shoda, Takeshi

2017-04-01

Chylothorax is a disease in which chyle leaks and accumulates in the thoracic cavity. Interstitial pneumonia and pneumomediastinum are common thoracic manifestations of dermatomyositis, but chylothorax complicated with dermatomyositis is not reported. We report a case of dermatomyositis with interstitial pneumonia complicated by chylothorax. A 77-year-old woman was diagnosed as dermatomyositis with Gottron's papules, skin ulcers, anti-MDA5 antibody and rapid progressive interstitial pneumonia. Treatment with betamethasone, tacrolimus and intravenous high-dose cyclophosphamide was initiated, and her skin symptoms and interstitial pneumonia improved once. However, right-sided chylothorax began to accumulate and gradually increase, and at the same time, her interstitial pneumonia began to exacerbate, and skin ulcers began to reappear on her fingers and auricles. Although her chylothorax improved by fasting and parenteral nutrition, she died due to further exacerbations of dermatomyositis and interstitial pneumonia in spite of steroid pulse therapy, increase in the betamethasone dosage, additional intravenous high-dose cyclophosphamide and plasma pheresis. An autopsy showed no lesions such as malignant tumors in the thoracic cavity. This is the first report of chylothorax complicated by dermatomyositis with interstitial pneumonia.

Pituitary and pulmonary abnormalities in an adult patient with Langerhans cell histiocytosis

International Nuclear Information System (INIS)

Perez, Adrian; Castro, Ricardo; Pantuso, Silvina

2003-01-01

Pulmonary histiocytosis is an uncommon interstitial lung disease, which is infrequent in adults and is part of a spectrum of disorders characterized by monoclonal proliferation and infiltration of organs by Langerhans cells. Histiocytosis X affects only the lungs in more than 85% of the cases. We report a case of a 21-years-old male patient with Diabetes Insipidus (DI) and respiratory symptoms due to a spontaneous pneumothorax with favorable clinical evolution. High-resolution CT demonstrated bilateral nodular and cystic pulmonary lesions. MRI revealed a nodular widening of the pituitary stalk. An endoscopic trans bronchial biopsy was negative for Langerhans cells. After thoracoscopy with surgical biopsy of the lung the diagnosis of Langerhans' cell histiocytosis was confirmed. (author)

Radionuclide two-indicator method to determine the indices of extravascular pulmonary fluid

International Nuclear Information System (INIS)

Frenkel', V.Kh.; Morgunov, N.B.; Kamenker, S.M.; Filatova, N.P.

1982-01-01

A radionuclide two-indicator method with 131 I-human serum albumin and 169 Yb-DTRA was used for examination of 32 persons without any diseases of the circulatory and respiratory organs and 75 patients with myocardial ischemia, different stages of cardiac insufficiency. The results showed that the amount of extravascular pulmonary fluid (EPF) in patients with ischemia was much higher as compared to the controls. EPF also increased significantly with the growing of the severity of cardiac insufficiency. A conclusion has been made that the radionuclide method of the determination of EPF indices is simple and effective in studies on pulmonary water metabolisn and can be used for diagnosis of early forms of interstitial edema

The HRCT appearances of granulomatous pulmonary disease in common variable immune deficiency

International Nuclear Information System (INIS)

Park, J.E.S.; Beal, I.; Dilworth, J.P.; Tormey, V.; Haddock, J.

2005-01-01

Approximately 10% of patients with common variable immune deficiency have systemic granulomatous disease with associated interstitial lung disease. From a population of patients with CVID attending a large tertiary referral clinic for primary immunodeficiency diseases we selected a cohort who had a restrictive defect or impaired gas transfer on pulmonary function testing and/or histologically proven granulomatous disease. HRCT scans of the thorax were reviewed retrospectively in 18 patients by two radiologists. Thirteen patients had diffuse reticulation, which varied from fine to coarse with features of fibrosis. Nodules were found in eight patients. In seven, these were associated with reticulation and in one they were an isolated finding. Bronchiectasis was found as the only abnormality in three and in addition to diffuse reticulation or nodules in another three patients. Greater appreciation of the spectrum of the radiological abnormalities in CVID patients with interstitial lung disease is important. Deteriorating lung function in patients with granulomatous CVID may be secondary to interstitial lung disease rather than bronchiectasis, and treatment should be tailored accordingly

The HRCT appearances of granulomatous pulmonary disease in common variable immune deficiency

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Park, J.E.S. [Royal Free and Hampstead NHS Trust, Pond Street, London NW3 2QG (United Kingdom); Beal, I. [Royal Free and Hampstead NHS Trust, Pond Street, London NW3 2QG (United Kingdom); Dilworth, J.P. [Royal Free and Hampstead NHS Trust, Pond Street, London NW3 2QG (United Kingdom); Tormey, V. [Royal Free and Hampstead NHS Trust, Pond Street, London NW3 2QG (United Kingdom); Haddock, J. [Royal Free and Hampstead NHS Trust, Pond Street, London NW3 2QG (United Kingdom)]. E-mail: jamandahaddock@royalfree.nhs.uk

2005-06-01

Approximately 10% of patients with common variable immune deficiency have systemic granulomatous disease with associated interstitial lung disease. From a population of patients with CVID attending a large tertiary referral clinic for primary immunodeficiency diseases we selected a cohort who had a restrictive defect or impaired gas transfer on pulmonary function testing and/or histologically proven granulomatous disease. HRCT scans of the thorax were reviewed retrospectively in 18 patients by two radiologists. Thirteen patients had diffuse reticulation, which varied from fine to coarse with features of fibrosis. Nodules were found in eight patients. In seven, these were associated with reticulation and in one they were an isolated finding. Bronchiectasis was found as the only abnormality in three and in addition to diffuse reticulation or nodules in another three patients. Greater appreciation of the spectrum of the radiological abnormalities in CVID patients with interstitial lung disease is important. Deteriorating lung function in patients with granulomatous CVID may be secondary to interstitial lung disease rather than bronchiectasis, and treatment should be tailored accordingly.

Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

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Amirmasoud Zangiabadi

2014-01-01

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.

Bronchovesicular bundle abnormalities and pathologic basis in diffuse pulmonary diseases on high resolution CT

International Nuclear Information System (INIS)

Nie Yongkang; Ma Daqing; Li Tieyi

2000-01-01

Objective: To investigate CT appearances of the normal and abnormal bronchovesicular bundles (BVB) and their corresponding pathologic basis, and to evaluate the role of these findings in diagnosing diffuse lung diseases. Methods: 6 fresh lungs obtained at autopsy and 8 surgical lung lobe specimens were studied. All lung specimens were inflated and fixed by Heitzman's method. HRCT with 10 mm thickness slice soft X-ray radiography was performed and HRCT-pathologic correlation was done. HRCT images of 100 cases with diffuse pulmonary disease were analyzed. Results: Thickened BVB with coarse margin and distorted structure correlated pathologically with peri-bronchovesicular pulmonary parenchyma inflammation and fibrosis, as well as peri-bronchovesicular interstitial disorder. Abnormalities of centrilobular BVB were recognizable by an increase in prominence of centrilobular structure on HRCT. In pathology, there were centrilobular peri-bronchovesicular dust induced granuloma in coal-miner's pneumoconiosis and centrilobular lymphangitis dilatation and filling with tumor cell in lymphangitis carcinomatosis. Increased centrilobular branching structure correlated pathologically with the presence of dilate intra-lobular bronchioles, inflammatory bronchiolar wall thickening, intraluminal secretions, and peri-bronchiolar inflammation. In 80 patients with predominant pulmonary interstitial diseases, thickened BVB with coarse margin or distortion were common in patients with pulmonary fibrosis. Nodular bronchovesicular bundle thickening were seen in sarcoidosis, lymphangitis carcinomatosis, and lymphoma. These were demonstrated in 8 of 20 sarcoidosis, 6 of 8 lymphangitis carcinomatosis , 3 of 5 lymphoma, and 2 of 15 coal-miner's pneumoconiosis. Increased centrilobular branching structures were seen in 14 of 20 patients with predominant bronchial diseases. Conclusion: Authors can limit the differential diagnostic range of diffuse lung diseases according to the appearances of BVB

Experimental depletion of different renal interstitial cell populations

International Nuclear Information System (INIS)

Bohman, S.O.; Sundelin, B.; Forsum, U.; Tribukait, B.

1988-01-01

To define different populations of renal interstitial cells and investigate some aspects of their function, we studied the kidneys of normal rats and rats with hereditary diabetes insipidus (DI, Brattleboro) after experimental manipulations expected to alter the number of interstitial cells. DI rats showed an almost complete loss of interstitial cells in their renal papillae after treatment with a high dose of vasopressin. In spite of the lack of interstitial cells, the animals concentrated their urine to the same extent as vasopressin-treated normal rats, indicating that the renomedullary interstitial cells do not have an important function in concentrating the urine. The interstitial cells returned nearly to normal within 1 week off vasopressin treatment, suggesting a rapid turnover rate of these cells. To further distinguish different populations of interstitial cells, we studied the distribution of class II MHC antigen expression in the kidneys of normal and bone-marrow depleted Wistar rats. Normal rats had abundant class II antigen-positive interstitial cells in the renal cortex and outer medulla, but not in the inner medulla (papilla). Six days after 1000 rad whole body irradiation, the stainable cells were almost completely lost, but electron microscopic morphometry showed a virtually unchanged volume density of interstitial cells in the cortex and outer medulla, as well as the inner medulla. Thus, irradiation abolished the expression of the class II antigen but caused no significant depletion of interstitial cells

Exhaled ethane: an in vivo biomarker of lipid peroxidation in interstitial lung diseases.

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Kanoh, Soichiro; Kobayashi, Hideo; Motoyoshi, Kazuo

2005-10-01

Oxidative stress plays a role in the pathogenesis and progression of interstitial lung disease (ILD). Exhaled ethane is a product of lipid peroxidation that has been proposed as a biomarker of oxidative stress in vivo. To determine whether the exhaled ethane level is elevated in patients with ILD and to compare it with other clinical parameters. Breath samples were collected from 34 patients with ILD, including 13 with idiopathic pulmonary fibrosis (IPF), 9 patients with cryptogenic organizing pneumonia, 6 patients with collagen vascular disease-associated interstitial pneumonia, and 6 patients with pulmonary sarcoidosis. Gas samples were obtained at hospital admission and after 3 weeks. After each expired sample was concentrated using a trap-and-purge procedure, the ethane level was analyzed by gas chromatography. Exhaled ethane levels were elevated in ILD patients (n = 34, mean +/- SD, 8.5 +/- 8.0 pmol/dL) compared with healthy volunteers (n = 16, 2.9 +/- 1.0 pmol/dL; p ethane levels were largely consistent with the clinical course. Four patients with IPF who had persistently high ethane levels died or deteriorated, whereas those with ethane levels ethane concentrations were positively correlated with levels of lactate dehydrogenase (Spearman rank correlation coefficient [rs], 0.28, p = 0.026) and C-reactive protein (rs, 0.38, p = 0.025) and were inversely correlated with Pa(O2) (rs, - 0.40, p = 0.0026). Patients showing increased uptake on (67)Ga scintigraphy demonstrated higher ethane levels (n = 19, 7.5 +/- 5.7 pmol/dL) compared with those who did not show increased uptake on scintigraphy (n = 10, 3.0 +/- 2.4 pmol/dL; p ethane is elevated in patients with ILD and is correlated with the clinical outcome, suggesting that it provides useful information about ongoing oxidative stress, and thereby disease activity and severity in ILD.

CT manifestations of pulmonary Langerhans cell histiocytosis

International Nuclear Information System (INIS)

Qiang Jun; Yu Wei; Gao Wanqin; Song Haiqiao; Ma Yingjian

2010-01-01

Objective: To analyzes the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH). Methods: CT features of 11 patients with PLCH proved pathologically were analyzed retrospectively. Results: The main findings in 11 PLCHs were cysts and nodules. Two cases only had cysts, and 1 only had nodules, which most had cavitations. The other 8 cases showed cysts and nodules with 4 cases mainly manifested with cysts and nine mainly manifested with nodules. Two cases had pulmonary interstitial changes. One case only had cysts in the left upper lung field and 10 cases had lesions not only in the upper and middle but in the lower lung field, which 2 cases had more lesions in the lower and costo-phrenic angle field and 8 cases had less lesions in the costo-phrenic angle field. Three of these 8 cases had more lesions in the superior lobe and apical segment of lower lobe. Conclusion: CT manifestations of PLCH are helpful for the early diagnosis. (authors)

Immunohistochemical detection of virus through its nuclear cytopathic effect in idiopathic interstitial pneumonia other than acute exacerbation

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G.C. dos Santos

2013-11-01

Full Text Available Idiopathic interstitial pneumonias include complex diseases that have a strong interaction between genetic makeup and environmental factors. However, in many cases, no infectious agent can be demonstrated, and these clinical diseases rapidly progress to death. Theoretically, idiopathic interstitial pneumonias could be caused by the Epstein-Barr virus, cytomegalovirus, adenovirus, hepatitis C virus, respiratory syncytial virus, and herpesvirus, which may be present in such small amounts or such configuration that routine histopathological analysis or viral culture techniques cannot detect them. To test the hypothesis that immunohistochemistry provides more accurate results than the mere histological demonstration of viral inclusions, this method was applied to 37 open lung biopsies obtained from patients with idiopathic interstitial pneumonias. As a result, immunohistochemistry detected measles virus and cytomegalovirus in diffuse alveolar damage-related histological patterns of acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia in 38 and 10% of the cases, respectively. Alveolar epithelium infection by cytomegalovirus was observed in 25% of organizing pneumonia patterns. These findings were coincident with nuclear cytopathic effects but without demonstration of cytomegalovirus inclusions. These data indicate that diffuse alveolar damage-related cytomegalovirus or measles virus infections enhance lung injury, and a direct involvement of these viruses in diffuse alveolar damage-related histological patterns is likely. Immunohistochemistry was more sensitive than the histological demonstration of cytomegalovirus or measles virus inclusions. We concluded that all patients with diffuse alveolar damage-related histological patterns should be investigated for cytomegalovirus and measles virus using sensitive immunohistochemistry in conjunction with routine procedures.

Pulmonary lymphomatoid granulomatosis in seven dogs (1976-1987)

International Nuclear Information System (INIS)

Berry, C.R.; Moore, P.F.; Thomas, W.P.; Sisson, D.; Koblik, P.D.

1990-01-01

Seven dogs with pulmonary lymphomatoid granulomatosis were reviewed. The disease occurred in six large-breed and one small-breed dogs. The dogs were five to 14 years old (mean, 8.4; median, 7), and four of seven dogs were males. Three dogs had been previously treated with adulticide therapy for canine dirofilariasis. Clinical histories included a progressive respiratory disease characterized by varying degrees of cough, dyspnea, exercise intolerance, and weight loss. Thoracic radiographic features included hilar lymphadenopathy, pulmonary masses of varying sizes, and mixed pulmonary patterns of lobar consolidation with ill-defined interstitial and alveolar pulmonary infiltrates. Cardiovascular changes compatible with chronic dirofilariasis were present in three dogs. The clinical course was usually progressive and fatal. The survival time ranged from six days to four years (mean, 12.5 mos; median, 3 mos). Gross and histologic features included mass lesions with areas of necrosis that replaced normal pulmonary architecture. Cytologically, these lesions were characterized by infiltration with pleomorphic, angioinvasive mononuclear cells that often resulted in vascular obliteration. The infiltrating cells resembled large lymphoid cells that possessed large hyperchromatic nuclei and small amounts of cytoplasm. Systemic lymphoid neoplasia with peripheral lymphadenopathy was diagnosed in two dogs. In both cases, lymph-node cytology was similar to the cellular infiltrates found in the lungs and consistent with a diagnosis of lymphomatoid granulomatosis. These features are compared with previously reported cases of canine lymphomatoid granulomatosis and those features identified in a similar disease described in man

An ROC study detecting ability of idiopathic pulmonary fibrosis using digital radiography

International Nuclear Information System (INIS)

Chung, Eun Chul; Im, Jung Gi; Han, Man Chung; Kim, Jong Hyo

1991-01-01

One potential advantage of the digital radiography system is its ability to enhance image quality by various types of processing. Digital unsharp masking is one of the simplest and most useful forms of enhancing processes. The efficacy of unsharp masking in radiological diagnosis has not been investigated thoroughly. To evaluate the effects of unsharp masking in film-digital chest images, 3 observers were shown 150 test radiographs. These test radiographs consisted of 50 unprocessed images (25 normals and 25 patients with idiopathic pulmonary fibrosis with honey combing) and their 100 processed images by using 450 and 15-sized masks respectively. An ROC analysis of these data suggests that unsharp masking is more effective in detecting idiopathic pulmonary fibrosis than unprocessed image (ρ < 0.05), and so it may improve diagnostic accuracy for interstitial fibrosis. In addition, the smaller mask size (15) is more effective than the larger one (mask size 45) (ρ < 0.05). By using this analytic approach, an optimal parameter in digital chest radiography may be investigated in many other forms of pulmonary disease such as pulmonary nodule or mediastinal mass

An ROC study detecting ability of idiopathic pulmonary fibrosis using digital radiography

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Chung, Eun Chul; Im, Jung Gi; Han, Man Chung; Kim, Jong Hyo [College of Medicine, Ewha Womens University, Seoul (Korea, Republic of)

1991-03-15

One potential advantage of the digital radiography system is its ability to enhance image quality by various types of processing. Digital unsharp masking is one of the simplest and most useful forms of enhancing processes. The efficacy of unsharp masking in radiological diagnosis has not been investigated thoroughly. To evaluate the effects of unsharp masking in film-digital chest images, 3 observers were shown 150 test radiographs. These test radiographs consisted of 50 unprocessed images (25 normals and 25 patients with idiopathic pulmonary fibrosis with honey combing) and their 100 processed images by using 450 and 15-sized masks respectively. An ROC analysis of these data suggests that unsharp masking is more effective in detecting idiopathic pulmonary fibrosis than unprocessed image ({rho} < 0.05), and so it may improve diagnostic accuracy for interstitial fibrosis. In addition, the smaller mask size (15) is more effective than the larger one (mask size 45) ({rho} < 0.05). By using this analytic approach, an optimal parameter in digital chest radiography may be investigated in many other forms of pulmonary disease such as pulmonary nodule or mediastinal mass.

Biopsy in idiopathic pulmonary fibrosis: back to the future.

Science.gov (United States)

Rossi, Giulio; Spagnolo, Paolo

2017-09-01

Idiopathic Pulmonary Fibrosis (IPF) is a relentlessly progressive, fibrosing interstitial pneumonia characterized by a radiologic and/or histologic pattern of usual interstitial pneumonia (UIP). The availability of two effective anti-fibrotic drugs in IPF has encouraged the identification and treatment of patients in early stages in order to maximize clinical benefit. The ability of high-resolution computed tomography (HRCT) to identify a 'definite' UIP pattern is suboptimal, particularly in the absence of honeycombing. Therefore, radiologic criteria for UIP are currently being redefined. Histology represents the major source of information to define a UIP pattern. Novel and less invasive approaches (particularly cryobiopsy) to sample interstitial lung diseases have demonstrated high sensitivity and specificity. In parallel, researchers are focusing on molecular mechanisms underlying IPF with the aim to identify more specific druggable targets. Lung tissue is therefore essential for diagnostic, pathogenetic and therapeutic purposes. Areas covered: We identified and critically reviewed the most relevant recent literature related to the limitations of current radiologic criteria, new lung sampling procedures, and molecular pathways in support of the need of lung tissue to better understand IPF. Expert commentary: The development of truly effective treatments for IPF requires the identification of key pathogenetic molecules and pathways. To this end, the availability of lung tissue is vital.

Pulmonary and cardiac pathology in sudden unexpected death in epilepsy (SUDEP).

Science.gov (United States)

Nascimento, Fábio A; Tseng, Zian H; Palmiere, Cristian; Maleszewski, Joseph J; Shiomi, Takayuki; McCrillis, Aileen; Devinsky, Orrin

2017-08-01

To review studies on structural pulmonary and cardiac changes in SUDEP cases as well as studies showing pulmonary or cardiac structural changes in living epilepsy patients. We conducted electronic literature searches using the PubMed database for articles published in English, regardless of publication year, that included data on cardiac and/or pulmonary structural abnormalities in SUDEP cases or in living epilepsy patients during the postictal period. Fourteen postmortem studies reported pulmonary findings in SUDEP cases. Two focused mainly on assessing lung weights in SUDEP cases versus controls; no group difference was found. The other 12 reported descriptive autopsy findings. Among all SUDEP cases with available descriptive postmortem pulmonary examination, 72% had pulmonary changes, most often pulmonary edema/congestion, and, less frequently, intraalveolar hemorrhage. Eleven studies reported on cardiac pathology in SUDEP. Cardiac abnormalities were found in approximately one-fourth of cases. The most common findings were myocyte hypertrophy and myocardial fibrosis of various degrees. Among living epilepsy patients, postictal pulmonary pathology was the most commonly reported pulmonary abnormality and the most common postictal cardiac abnormality was transient left ventricular dysfunction - Takotsubo or neurogenic stunned myocardium. Cardiac and pulmonary pathological abnormalities are frequent among SUDEP cases, most commonly pulmonary edema/congestion and focal interstitial myocardial fibrosis. Most findings are not quantified, with subjective elements and undefined interobserver reliability, and lack of controls such as matched epilepsy patients who died from other causes. Further, studies have not systematically evaluated potential confounding factors, including postmortem interval to autopsy, paramedic resuscitation and IV fluids administration, underlying heart/lung disease, and risk factors for cardiac or pulmonary disease. Prospective studies with

Balance point characterization of interstitial fluid volume regulation.

Science.gov (United States)

Dongaonkar, R M; Laine, G A; Stewart, R H; Quick, C M

2009-07-01

The individual processes involved in interstitial fluid volume and protein regulation (microvascular filtration, lymphatic return, and interstitial storage) are relatively simple, yet their interaction is exceedingly complex. There is a notable lack of a first-order, algebraic formula that relates interstitial fluid pressure and protein to critical parameters commonly used to characterize the movement of interstitial fluid and protein. Therefore, the purpose of the present study is to develop a simple, transparent, and general algebraic approach that predicts interstitial fluid pressure (P(i)) and protein concentrations (C(i)) that takes into consideration all three processes. Eight standard equations characterizing fluid and protein flux were solved simultaneously to yield algebraic equations for P(i) and C(i) as functions of parameters characterizing microvascular, interstitial, and lymphatic function. Equilibrium values of P(i) and C(i) arise as balance points from the graphical intersection of transmicrovascular and lymph flows (analogous to Guyton's classical cardiac output-venous return curves). This approach goes beyond describing interstitial fluid balance in terms of conservation of mass by introducing the concept of inflow and outflow resistances. Algebraic solutions demonstrate that P(i) and C(i) result from a ratio of the microvascular filtration coefficient (1/inflow resistance) and effective lymphatic resistance (outflow resistance), and P(i) is unaffected by interstitial compliance. These simple algebraic solutions predict P(i) and C(i) that are consistent with reported measurements. The present work therefore presents a simple, transparent, and general balance point characterization of interstitial fluid balance resulting from the interaction of microvascular, interstitial, and lymphatic function.

Interstitial cystitis

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... symptoms get better. Reduce or stop consuming caffeine, chocolate, carbonated beverages, citrus drinks, and foods with a ... rarely done anymore Support Groups Some people may benefit from taking part in interstitial cystitis support groups . ...

Interstitial Cystitis

Science.gov (United States)

... relieve symptoms. Diet. Alcohol, tomatoes, spices, carbonated drinks, chocolate, caffeine, citrus fruits and drinks, pickled foods, artificial ... at scheduled times and using relaxation techniques. Physical therapy. People who have interstitial cystitis may have painful ...

Classical patterns of interstitial lung diseases

International Nuclear Information System (INIS)

Mueller-Mang, C.

2014-01-01

High resolution computed tomography (HRCT) is the most important non-invasive tool in the diagnostics and follow-up of patients with interstitial lung disease (ILD). A systematic review of the HRCT patterns of ILD was carried out and the most relevant differential diagnoses are discussed in order to provide a road map for the general radiologist to successfully navigate the complex field of ILD. Using HRCT four basic patterns of ILD can be identified: linear and reticular patterns, the nodular pattern, the high attenuation and low attenuation patterns. These patterns can be further differentiated according to their localization within the secondary pulmonary lobule (SPL), e.g. centrilobular or perilymphatic and their distribution within the lungs (e.g. upper or lower lobe predominance). Relevant clinical data, such as smoking history and course of the disease provide useful additional information in the diagnosis of ILD. On the basis of the pattern and anatomical distribution on HRCT, an accurate diagnosis can be achieved in some cases of ILD; however, due to morphological and clinical overlap the final diagnosis of many ILDs requires close cooperation between clinicians, radiologists and pathologists. (orig.) [de

A new model of progressive pulmonary fibrosis in rats

Energy Technology Data Exchange (ETDEWEB)

Last, J.A.; Gelzleichter, T.R.; Pinkerton, K.E.; Walker, R.M.; Witschi, H. (Univ. of California, Davis (United States))

1993-08-01

Sprague-Dawley rats were exposed for 6 h daily to 0.8 ppm of ozone and 14.4 ppm of nitrogen dioxide. Approximately 7 to 10 wk after the initiation of exposure, animals began to demonstrate respiratory insufficiency and severe weight loss. About half of the rats died between Days 55 and 78 of exposure; no overt ill effects were observed in animals exposed to filtered air, to ozone alone, or to nitrogen dioxide. Biochemical findings in animals exposed to ozone and nitrogen dioxide included increased lung content of DNA, protein, collagen, and elastin, which was about 300% higher than the control values. The collagen-specific crosslink hydroxy-pyridinium, a biomarker for mature collagen in the lung, was decreased by about 40%. These results are consistent with extensive breakdown and remodeling of the lung parenchyma and its associated vasculature. Histopathologic evaluation showed severe fibrosis, alveolar collapse, honeycombing, macrophage and mast cell accumulation, vascular smooth muscle hypertrophy, and other indications of severe progressive interstitial pulmonary fibrosis and end-stage lung disease. This unique animal model of progressive pulmonary fibrosis resembles the final stages of human idiopathic pulmonary fibrosis and should facilitate studying underlying mechanisms and potential therapy of progressive pulmonary fibrosis.

Relationship between signal intensity of blood flow in the pulmonary artery obtained by magnetic resonance imaging and results of right cardiac catheterization in patients with pulmonary disease

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Yuguchi, Yasutoshi; Nagao, Keiichi; Kouno, Norihiro; Tanabe, Nobuhiro; Okita, Shinya; Tojima, Hirokazu; Okada, Osamu; Kuriyama, Takayuki [Chiba Univ. (Japan). School of Medicine; Yamaguchi, Tetsuo

1992-08-01

Electrocardiogram-gated spin-echo magnetic resonance (MR) images of the chest were obtained in five normal controls and 35 patients with pulmonary disease (11 chronic obstructive pulmonary disease, 6 pulmonary thromboembolism, 5 primary pulmonary hypertension, 4 interstitial pulmonary disease, 4 pulmonary hypertension with disturbance of portal circulation, and 5 other diseases) who underwent right cardiac catheterization. In transverse images at the level of the right main pulmonary artery (rPA) and sagittal images at the level through the midsternal line and the spinal cord, the signal intensity of blood flow in the rPA was quantitatively evaluated, and the correlations with the MR signal intensity of intravascular flow and the parameters of hemodynamics were studied. In diastole MR images of both normal controls and patients mostly showed a significant signal and visible flow images. In systolic MR images, the mean values of hemodynamic parameters (mean pulmonary arterial pressure (mPAP), pulmonary arteriolar resistance (PAR), and cardiac index (CI)) were abnormal in patients with significant signal intensity of flow compared with those in patients without sufficient MR signal. The signal intensity was not correlated with mPAP; however, it significantly increased as PAR increased, and it increased as CI decreased both in diastole and in systole. Especially in systole, there was good correlation between the signal intensity in transverse MR images and CI and between signal intensity in sagittal MR images and PAR . These results suggest that the signal intensity of blood flow in the rPA on MR images can be used as an index of the severity of right heart failure associated with pulmonary disease. MR imaging is a useful modality to evaluate pulmonary circulation disturbance because of its ability to assess blood flow in the pulmonary artery noninvasively without interference from other structures such as bone and normal lung. (J.P.N.).

Interstitial cystitis: painful bladder syndrome

Directory of Open Access Journals (Sweden)

R F Sholan

2018-02-01

Full Text Available Interstitial cystitis, or painful bladder syndrome, is a chronic inflammatory disease of a bladder of unknown etiology. It negatively affects the quality of life, causes depressive disorders, anxiety, and sexual dysfunction. Despite numerous studies, the etiology of interstitial cystitis is still unclear and it’s considered as painful bladder syndrome with multifactorial origin. According to the US National Health and Nutrition Examination Survey, 470/100 000 people (60/100 000 men, 850/100 000 women are diagnosed with interstitial cystitis. Diagnosis of the disease is difficult and is substantially based on clinical symptoms. Pelvic pain, urinary urgency, frequency and nocturia are the basic complaints in this pathology. The diagnosis requires exclusion of diseases with similar manifestations. So interstitial cystitis is frequently misdiagnosed as urinary tract infection, overactive bladder, urethral obstruction or diverticulosis, chronic prostatitis, bladder cancer, vulvodynia, endometriosis, and chronic pelvic pain. Etiopathogenesis of the disease is uncertain, which makes etiologic treatment impossible. Currently scientific discussions on the causes of disease continue as well as different treatment regimens are offered, but are often ineffective, palliative and temporary. The treatment for intersticial cystitis should focus on restoring normal bladder function, prevention of relapse of symptoms and improvement of patients’ quality of life. The literature review presents current view on the terminology, epidemiology, diagnosis and treatment of interstitial cystitis.

Evaluation of Tl-201 lung uptake and impairment of pulmonary perfusion on scintigraphies in pulmonary tuberculosis

International Nuclear Information System (INIS)

Fujii, Tadashige; Tanaka, Masao; Koizumi, Tomonori; Kubo, Keishi

2000-01-01

Tl-201 lung uptake in 74 patients (85 lesions) and pulmonary perfusion in 105 patients were studied to evaluate clinical usefulness of Tl-201 lung uptake and perfusion lung scintigraphy in pulmonary tuberculosis, using a scintillation camera with a mini-computer system. As indices of Tl-201 lung uptake, lung (lesion) to upper mediastinum uptake ratio (L/M) and visual grading were used. L/M in pulmonary tuberculosis was 1.96±0.66, which was significantly larger than 1.04±0.24 in healthy controls and lower than that in heart diseases with left heart failure and idiopathic interstitial pneumonia, and showed no significant differences with that in acute pneumonia, pyothorax, primary lung cancer and malignant mediastinal tumor. L/M in pulmonary tuberculosis did not correlate with CRP, erythrocyte sedimentation rate, Gaffky number of sputum and body temperature. It correlated with the type of pulmonary tuberculosis according to the Gakken Classification reflecting the disease activity. It was larger in the exudative type, caseo-infiltrative one, disseminated one, one with cavity in infiltrative lesion than the fibro-caseous one. On perfusion lung scintigram, impairment of pulmonary perfusion larger than area of the entire unilateral lung was observed in 68 cases (64.8%). Area of hypoperfused lung field, which correlated with % vital capacity (r=0.60, p=0.0002) and PaO 2 (r=0.39,p=0.0024), was significantly larger in patients with silicosis and those with bilateral pleural involvements such as pleural callosity than in those with type III according to the Gakkai Classification. Most of the patients showed decreased pulmonary perfusion and Tl-201 accumulation of which grade reflects the disease activity in active tuberculous lesion. Patients with miliary tuberculosis and those with silicotuberculosis showed diffuse Tl-201 accumulation in the both lungs. Tl-201 lung scintigraphy seems to be useful for visualizing active tuberculous lesions, particularly the ones that

Rare variants in RTEL1 are associated with familial interstitial pneumonia.

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Cogan, Joy D; Kropski, Jonathan A; Zhao, Min; Mitchell, Daphne B; Rives, Lynette; Markin, Cheryl; Garnett, Errine T; Montgomery, Keri H; Mason, Wendi R; McKean, David F; Powers, Julia; Murphy, Elissa; Olson, Lana M; Choi, Leena; Cheng, Dong-Sheng; Blue, Elizabeth Marchani; Young, Lisa R; Lancaster, Lisa H; Steele, Mark P; Brown, Kevin K; Schwarz, Marvin I; Fingerlin, Tasha E; Schwartz, David A; Lawson, William E; Loyd, James E; Zhao, Zhongming; Phillips, John A; Blackwell, Timothy S

2015-03-15

Up to 20% of cases of idiopathic interstitial pneumonia cluster in families, comprising the syndrome of familial interstitial pneumonia (FIP); however, the genetic basis of FIP remains uncertain in most families. To determine if new disease-causing rare genetic variants could be identified using whole-exome sequencing of affected members from FIP families, providing additional insights into disease pathogenesis. Affected subjects from 25 kindreds were selected from an ongoing FIP registry for whole-exome sequencing from genomic DNA. Candidate rare variants were confirmed by Sanger sequencing, and cosegregation analysis was performed in families, followed by additional sequencing of affected individuals from another 163 kindreds. We identified a potentially damaging rare variant in the gene encoding for regulator of telomere elongation helicase 1 (RTEL1) that segregated with disease and was associated with very short telomeres in peripheral blood mononuclear cells in 1 of 25 families in our original whole-exome sequencing cohort. Evaluation of affected individuals in 163 additional kindreds revealed another eight families (4.7%) with heterozygous rare variants in RTEL1 that segregated with clinical FIP. Probands and unaffected carriers of these rare variants had short telomeres (RTEL1 function. Rare loss-of-function variants in RTEL1 represent a newly defined genetic predisposition for FIP, supporting the importance of telomere-related pathways in pulmonary fibrosis.

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

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Maria Aparecida S. S. Paiva

2007-06-01

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

Interstitial shadow on chest CT is associated with the onset of interstitial lung disease caused by chemotherapeutic drugs

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Niho, Seiji; Goto, Koichi; Yoh, Kiyotaka; Kim, Y.H.; Ohmatsu, Hironobu; Kubota, Kaoru; Saijo, Nagahiro; Nishiwaki, Yutaka

2006-01-01

Pretreatment computerized tomography (CT) films of the chest was studied to clarify the influence of interstitial shadow on developing interstitial lung disease (ILD). Eligible patients were those lung cancer patients who started to receive first-line chemotherapy between October 2001 and March 2004. Patients who received thoracic radiotherapy to the primary lesion, mediastinum, spinal or rib metastases were excluded. We reviewed pretreatment conventional CT and plain X-ray films of the chest. Ground-glass opacity, consolidation or reticular shadow without segmental distribution was defined as interstitial shadow, with this event being graded as mild, moderate or severe. If interstitial shadow was detected on CT films of the chest, but not via plain chest X-ray, it was graded as mild. Patients developing ILD were identified from medial records. A total of 502 patients were eligible. Mild, moderate and severe interstitial shadow was identified in 7, 8 and 5% of patients, respectively. A total of 188 patients (37%) received tyrosine kinase inhibitor (TKI) treatment, namely gefitinib or erlotinib. Twenty-six patients (5.2%) developed ILD either during or after chemotherapy. Multivariate analyses revealed that interstitial shadow on CT films of the chest and treatment history with TKI were associated with the onset of ILD. It is recommended that patients with interstitial shadow on chest CT are excluded from future clinical trials until this issue is further clarified, as it is anticipated that use of chemotherapeutic agents frequently mediate onset of ILD in this context. (author)

Usefulness of the Negative Multidetector CT Angiography in Patients with Suspected Pulmonary Embolism

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Holguin Holguin, Alfonso Jose; Lombana, Milton; Collazos, Giovanny; Bravo, Hector; Abella Calle, Jose

2012-01-01

Objective: The aim of the study is to evaluate alternative findings in pulmonary CT angiography using MDCT reported as negative in patients with suspected pulmonary embolism. Material and method: Cross sectional study with 178 patients. We recognized other findings reported in pulmonary CT angiography using MDCT whose result was negative. Subsequently these findings were classified into six different categories of clinical relevance and their association with clinical scenario was analyzed. Results: from November 2004 to June 2007 we requested 231 pulmonary CT angiography using MDCT in patients with suspected pulmonary embolism. 217 patients were included in the final analysis. Of these, 39 (185) were positive for pulmonary embolism. The 178 remaining negative studies, 8.43% were normal, 53.37% were classified as category A, 20.79% category B, 14.04% category C; category D 1.12% and 2.25% were not interpretable. The most frequent diagnoses were pneumonia, interstitial lung disease and cardiogenic pulmonary edema. It was less likely to find a normal report whether the study was requested in the hospital department in emergency patients (OR = 0.38 95% 0.16 to 0.88, p = 0.0007). No differences were found in other categories or between patients younger or older than 65 years (p = 0.436). Conclusion: Pulmonary CT angiography as first line imaging tool in patients with suspected pulmonary embolism is useful, offering alternative diagnoses in hospitalized and emergency room patients, regardless of their age and clinical findings.

Regional distribution of ventilation-perfusion ratio in patients with interstitial pneumonia using Kr-81m

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Sekine, Hiroshi; Kawakami, Kenji; Shimada, Takao

1983-01-01

In order to know the pathophysiological change of interstitial pneumonia, it is important to detect the morphological change of vascular and air way systems in the lung. The study group consisted of 12 patients of interstitial pneumonia including IIP, PSS, RA, sarcoidosis, and hypersensitive pneumonia. The Kr-81m bolus inhalation from 3 different lung volumes were analyzed to detect regional ventilation abnormalities. The regional distribution of ventilation-perfusion ratio (V/Q) in the lung was obtained from the continuous inhalation and infusion Kr-81 m. In 7 patients, regional distribution of V/Q was also measured at rest and during exercise. In advanced stage of interstitial pneumonia, radioactivity in lower lung fields was decreased in bolus inhalation from TLC-400 ml lung volume, and present in lower lung in bolus inhalation from RV level. These findings are representing air way rigidity especially in lower lung fields. However, in early stage or reversible cases, it is difficult to detect the abnormal distribution of radioactivity in lung, in spite of decreased PO 2 . So it seems impossible to screen early stage or reversible cases of interstitial pneumonia by bolus inhalation method alone. At rest for normal subjects in the upright position, the apical zone had a high V/Q, but in the lower parts two third of V/Q distribution was relatively homogeneous. During exercise at 50W, the distribution became more uniform. In most cases of the disease, weak exercise (less than 40W) produced the same uniform V/Q distribution. It should be noted that in the disease not only was perfusion in upper lung fields increased, but that perfusion of lower lung fields was reduced. So late in the cource of extension of the disease, pulmonary vascular bed in the lower lung fields is restricted.(J.P.N.)

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

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Bahtouee, Mehrzad; Saberifard, Jamshid; Javadi, Hamid; Nabipour, Iraj; Malakizadeh, Hasan; Monavvarsadegh, Gholamhossein; Ilkhani Pak, Hoda; Sadeghi, Azadeh; Assadi, Majid

2015-01-01

The discrimination of inactive inflammatory processes from the active form of the disease is of great importance in the management of interstitial lung disease (ILD). The aim of this study was to determine the efficacy of 99mTc-IgG scan for the detection of severity of disease compared to high-resolution computed tomography (HRCT) and pulmonary function test (PFT). Eight known cases of ILD including four cases of Mustard gas (MG) intoxication and four patients with ILD of unknown cause were included in this study. A population of six patients without lung disease was considered as the control group. The patients underwent PFT and high-resolution computed tomography, followed by 99mTc-IgG scan. They were followed up for one year. 99mTc-IgG scan assessment of IgG uptake was accomplished both qualitatively (subjectively) and semiquantitatively. All eight ILD patients demonstrated a strong increase in 99mTc-IgG uptake in the lungs, compared to the control patients. The 99mTc-IgG scan scores were higher in the patient group (0.64[95% confidence interval(CI)=0.61-0.69])) than the control group (0.35 (0.35[95% CI=0.28-0.40]), (P<0.05)). In patients, a statistically significant positive correlation was detected between 99mTc-IgG scan and HRCT scores (Spearman’s correlation coefficient = 0.92, P < 0.008). The 99mTc-Human Immunoglobulin (HIG) scores were not significantly correlated with PFT findings (including FVC, FEV1, FEV1/FVC), O 2 saturation and age (P values > 0.05). There were no significant correlations between 99mTc-IgG score and HRCT patterns including ground glass opacity, reticular fibrosis and honeycombing (P value > 0.05). The present results confirmed that 99mTc-IgG scan could be applied to detect the severity of pulmonary involvement, which was well correlated with HRCT findings. This data also showed that the 99mTc-IgG scan might be used as a complement to HRCT in the functional evaluation of the clinical status in ILD; however, further studies are

Computer-based quantitative computed tomography image analysis in idiopathic pulmonary fibrosis: A mini review.

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Ohkubo, Hirotsugu; Nakagawa, Hiroaki; Niimi, Akio

2018-01-01

Idiopathic pulmonary fibrosis (IPF) is the most common type of progressive idiopathic interstitial pneumonia in adults. Many computer-based image analysis methods of chest computed tomography (CT) used in patients with IPF include the mean CT value of the whole lungs, density histogram analysis, density mask technique, and texture classification methods. Most of these methods offer good assessment of pulmonary functions, disease progression, and mortality. Each method has merits that can be used in clinical practice. One of the texture classification methods is reported to be superior to visual CT scoring by radiologist for correlation with pulmonary function and prediction of mortality. In this mini review, we summarize the current literature on computer-based CT image analysis of IPF and discuss its limitations and several future directions. Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

Effects of water immersion to the neck on pulmonary circulation and tissue volume in man

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Begin, R.; Epstein, M.; Sackner, M. A.; Levinson, R.; Dougherty, R.; Duncan, D.

1976-01-01

A rapid noninvasive breathing method is used to obtain serial measurements of the pulmonary capillary blood flow, diffusing capacity per unit of alveolar volume, combined pulmonary tissue plus capillary volume, functional residual capacity, and oxygen consumption in five normal subjects undergoing 6 h of sitting, 4 h of sitting while immersed to the neck in thermoneutral water, and 4 h of lying in thermoneutral water to the neck. The rebreathing method employed a test gas mixture containing 0.5% C2H2, 0.3% C(18)O, 10% He, 21% O2, and balance N2. It is shown that immersion to the neck in the seated posture results in significant increases in sodium excretion cardiac output, and diffusing capacity per unit of alveolar volume. The pulmonary tissue plus capillary volume did not change, demonstrating that the central vascular engorgement induced by water immersion is not accompanied by significant extravasation of fluid into the pulmonary interstitial space.

Pulmonary complications of induction therapy for acute myeloid leukemia in adults. Findings of chest X-rays and computed tomography

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Kirchner, J.; Huettmann, C.; Jacobi, V.; Boehme, A.

1998-01-01

To exclude pulmonary complications, 359 chest radiographs and 50 computed tomographs of the lung were performed in 95 patients suffering from acute myeloid leukemia. The radiological findings were registered, described and correlated with clinical findings in the present study on 2395 days of observation. Results: In summary, 52 patients showed alterations of the lung. Pulmonary hyperhydration was seen in 21 cases, bacterial pneumonia was found in 18 cases, invasive pulmonary aspergillosis was documented in 14 cases, and 5 cases of severe haemorrhage were seen. An unexplained pulmonary edema in 13 patients with interstitial and alveolar infiltrates is considered to be a complication of treatment with cytosine-arabinoside. Conclusion: The results demonstrate that chest X-ray and computed tomography have a high impact in detection and treatment of pulmonary complications following intensive chemotherapy. We may expect the development of diffuse opacity following administration of cytosine-arabinoside in medium-sized doses. (orig.) [de

A Time- and Compartment-Specific Activation of Lung Macrophages in Hypoxic Pulmonary Hypertension.

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Pugliese, Steven C; Kumar, Sushil; Janssen, William J; Graham, Brian B; Frid, Maria G; Riddle, Suzette R; El Kasmi, Karim C; Stenmark, Kurt R

2017-06-15

Studies in various animal models suggest an important role for pulmonary macrophages in the pathogenesis of pulmonary hypertension (PH). Yet, the molecular mechanisms characterizing the functional macrophage phenotype relative to time and pulmonary localization and compartmentalization remain largely unknown. In this study, we used a hypoxic murine model of PH in combination with FACS to quantify and isolate lung macrophages from two compartments over time and characterize their programing via RNA sequencing approaches. In response to hypoxia, we found an early increase in macrophage number that was restricted to the interstitial/perivascular compartment, without recruitment of macrophages to the alveolar compartment or changes in the number of resident alveolar macrophages. Principal component analysis demonstrated significant differences in overall gene expression between alveolar and interstitial macrophages (IMs) at baseline and after 4 and 14 d hypoxic exposure. Alveolar macrophages at both day 4 and 14 and IMs at day 4 shared a conserved hypoxia program characterized by mitochondrial dysfunction, proinflammatory gene activation, and mTORC1 signaling, whereas IMs at day 14 demonstrated a unique anti-inflammatory/proreparative programming state. We conclude that the pathogenesis of vascular remodeling in hypoxic PH involves an early compartment-independent activation of lung macrophages toward a conserved hypoxia program, with the development of compartment-specific programs later in the course of the disease. Thus, harnessing time- and compartment-specific differences in lung macrophage polarization needs to be considered in the therapeutic targeting of macrophages in hypoxic PH and potentially other inflammatory lung diseases. Copyright © 2017 by The American Association of Immunologists, Inc.

Diagnostic accuracy of computed tomography and histopathology in the diagnosis of usual interstitial pneumonia

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Aaloekken, Trond Mogens; Mynarek, Georg; Kolbenstvedt, Alf; Naalsund, Anne; Elnaes Berstad, Audun; Solberg, Steinar; Stroem, Erik H.; Scott, Helge; Soeyseth, Vidar

2012-01-01

Background: The relative clinical benefit of histopathology and computed tomography (CT) in patients with idiopathic interstitial pneumonia (IIP) is under debate. Purpose: To analyze thin-section CT features and histopathologic findings in patients with usual interstitial pneumonia (UIP) in the clinical context of idiopathic pulmonary fibrosis (IPF), and to evaluate and compare diagnostic accuracy of the two methods among patients with an appropriate spectrum of IIP. Material and Methods: The study included 91 patients (49 men; mean age 53.2 years; median follow-up 7.2 years) with clinically suspected interstitial lung disease. All underwent surgical lung biopsy and thin-section CT. Two independent readers retrospectively assessed the CT images for the extent and pattern of abnormality and made a first-choice diagnosis. Two pathologists retrospectively assessed the histopathologic slides. In 64 patients with IIP, a retrospective composite reference standard identified 41 patients with UIP. CT characteristics of UIP and IIPs other than UIP were compared with univariate and multivariate analyses. Results: There was good agreement between the readers for the correct first-choice CT diagnosis of UIP (κ = 0.79). The sensitivity, specificity, and positive predictive value of the CT diagnosis of UIP were 63%, 96%, and 96%, respectively. The sensitivity, specificity, and positive predictive value of the histological diagnosis of UIP were 73%, 74%, and 83%, respectively. The CT feature that best differentiated UIP from IIPs other than UIP was the extent of reticular pattern (odds ratio, 5.1). Conclusion: Surgical lung biopsy may not be warranted in patients with thin-section CT diagnosis of UIP

Diagnostic accuracy of computed tomography and histopathology in the diagnosis of usual interstitial pneumonia

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Aaloekken, Trond Mogens; Mynarek, Georg; Kolbenstvedt, Alf (Dept. of Radiology, Oslo Univ. Hospital Rikshospitalet, Oslo (Norway)), Email: trond.mogens.aalokken@rikshospitalet.no; Naalsund, Anne (Dept. of Pulmonology, Oslo Univ. Hospital Rikshospitalet (Norway)); Elnaes Berstad, Audun (Dept. of Radiology, Oslo Univ. Hospital Rikshospitalet, Oslo (Norway); Univ. of Oslo (Norway)); Solberg, Steinar (Dept. of Thoracic Surgery, Oslo Univ. Hospital Rikshospitalet (Norway)); Stroem, Erik H.; Scott, Helge (Dept. of Pathology, Oslo Univ. Hospital Rikshospitalet (Norway)); Soeyseth, Vidar (Univ. of Oslo (Norway); Depts. of Pulmonology, Akershus Univ. Hospital, Loerenskog (Norway))

2012-04-15

Background: The relative clinical benefit of histopathology and computed tomography (CT) in patients with idiopathic interstitial pneumonia (IIP) is under debate. Purpose: To analyze thin-section CT features and histopathologic findings in patients with usual interstitial pneumonia (UIP) in the clinical context of idiopathic pulmonary fibrosis (IPF), and to evaluate and compare diagnostic accuracy of the two methods among patients with an appropriate spectrum of IIP. Material and Methods: The study included 91 patients (49 men; mean age 53.2 years; median follow-up 7.2 years) with clinically suspected interstitial lung disease. All underwent surgical lung biopsy and thin-section CT. Two independent readers retrospectively assessed the CT images for the extent and pattern of abnormality and made a first-choice diagnosis. Two pathologists retrospectively assessed the histopathologic slides. In 64 patients with IIP, a retrospective composite reference standard identified 41 patients with UIP. CT characteristics of UIP and IIPs other than UIP were compared with univariate and multivariate analyses. Results: There was good agreement between the readers for the correct first-choice CT diagnosis of UIP (kappa = 0.79). The sensitivity, specificity, and positive predictive value of the CT diagnosis of UIP were 63%, 96%, and 96%, respectively. The sensitivity, specificity, and positive predictive value of the histological diagnosis of UIP were 73%, 74%, and 83%, respectively. The CT feature that best differentiated UIP from IIPs other than UIP was the extent of reticular pattern (odds ratio, 5.1). Conclusion: Surgical lung biopsy may not be warranted in patients with thin-section CT diagnosis of UIP

Doença pulmonar intersticial associada a bronquiolite respiratória Respiratory bronchilitis-associated interstitial lung disease

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Sílvia CS. Rodrigues

2004-12-01

Full Text Available A doença pulmonar intersticial associada a bronquiolite respiratória faz parte do espectro anatomopatológico das lesões pulmonares infiltrativas difusas induzidas pela fumaça de cigarro. Raramente tem apresentação clínico-funcional exuberante. Descrevemos dois casos diagnosticados por biópsia pulmonar aberta, caracterizados por dispnéia de evolução insidiosa, baqueteamento digital, lesões císticas à tomografia computadorizada e hipoxemia ao exercício. Enfatizamos considerar, em indivíduos tabagistas, a doença pulmonar intersticial associada a bronquiolite respiratória no contexto das pneumopatias intersticiais císticas, juntamente com a linfangioleiomiomatose, o granuloma eosinofílico e a fibrose pulmonar idiopática.Respiratory bronchiolitis-associated interstitial lung disease is one of many within the spectrum of smoking-related diffuse infiltrative lung diseases. The clinical and functional characteristics are typically subtle. Herein, we describe two cases of diagnosed through open-lung biopsy, and characterized by insidious evolution of dyspnea, digital clubbing, cystic lesions on computed tomography scans, and hipoxemia upon exertion. We emphasize that, when smokers are evaluated, it is imprtant to consider a diagnosis of respiratory bronchiolitis-associated interstitial lung disease in the context of interstitial cystitis, as well as in that of lymphangioleiomyomatosis, eosinophilic granuloma and idiophatic pulmonary fibrosis.

Gallium interstitial contributions to diffusion in gallium arsenide

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Schick, Joseph T.; Morgan, Caroline G.

2011-09-01

A new diffusion path is identified for gallium interstitials, which involves lower barriers than the barriers for previously identified diffusion paths [K. Levasseur-Smith and N. Mousseau, J. Appl. Phys. 103, 113502 (2008), P. A. Schultz and O. A. von Lilienfeld, Modelling and Simulation in Materials Science and Engineering 17, 084007 (2009)] for the charge states which dominate diffusion over most of the available range of Fermi energies. This path passes through the ⟨110⟩ gallium-gallium split interstitial configuration, and has a particularly low diffusion barrier of 0.35 eV for diffusion in the neutral charge state. As a part of this work, the character of the charge states for the gallium interstitials which are most important for diffusion is investigated, and it is shown that the last electron bound to the neutral interstitial occupies a shallow hydrogenic bound state composed of conduction band states for the hexagonal interstitial and both tetrahedral interstitials. How to properly account for the contributions of such interstitials is discussed for density-functional calculations with a k-point mesh not including the conduction band edge point. Diffusion barriers for gallium interstitials are calculated in all the charge states which can be important for a Fermi level anywhere in the gap, q = 0, +1, +2, and +3, for diffusion via the ⟨110⟩ gallium-gallium split interstitial configuration and via the hexagonal interstitial configuration. The lowest activation enthalpies over most of the available range of Fermi energies are found to correspond to diffusion in the neutral or singly positive state via the ⟨110⟩ gallium-gallium split interstitial configuration. It is shown that several different charge states and diffusion paths contribute significantly for Fermi levels within 0.2 eV above the valence band edge, which may help to explain some of the difficulties [H. Bracht and S. Brotzmann, Phys. Rev. B 71, 115216 (2005)] which have been

An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features

Science.gov (United States)

2014-01-01

Background Clinical evaluation to differentiate the characteristic features of pulmonary fibrosis and emphysema is often difficult in patients with combined pulmonary fibrosis and emphysema (CPFE), but diagnosis of pulmonary fibrosis is important for evaluating treatment options and the risk of acute exacerbation of interstitial pneumonia of such patients. As far as we know, it is the first report describing a correlation among clinical, radiological, and whole-lung pathological features in an autopsy cases of CPFE patients. Methods Experts retrospectively reviewed the clinical charts and examined chest computed tomography (CT) images and pathological findings of an autopsy series of 22 CPFE patients, and compared these with findings from 8 idiopathic pulmonary fibrosis (IPF) patients and 17 emphysema-alone patients. Results All patients had a history of heavy smoking. Forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC%) was significantly lower in the emphysema-alone group than the CPFE and IPF-alone groups. The percent predicted diffusing capacity of the lung for carbon monoxide (DLCO%) was significantly lower in the CPFE group than the IPF- and emphysema-alone groups. Usual interstitial pneumonia (UIP) pattern was observed radiologically in 15 (68.2%) CPFE and 8 (100%) IPF-alone patients and was pathologically observed in all patients from both groups. Pathologically thick-cystic lesions involving one or more acini with dense wall fibrosis and occasional fibroblastic foci surrounded by honeycombing and normal alveoli were confirmed by post-mortem observation as thick-walled cystic lesions (TWCLs). Emphysematous destruction and enlargement of membranous and respiratory bronchioles with fibrosis were observed in the TWCLs. The cystic lesions were always larger than the cysts of honeycombing. The prevalence of both radiological and pathological TWCLs was 72.7% among CPFE patients, but no such lesions were observed in patients with IPF or emphysema

Interstitial Lung Disease

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... of Medicine Division of Pulmonary, Critical Care and Sleep Medicine (Adult) Familial Pulmonary Fibrosis (FPF) Genetic Counseling Program Pulmonary Rehabilitation Section of Sleep Medicine Search Directory Our Specialists Kevin K. Brown, MD + × ...

[Pulmonary involvement in systemic sclerosis. Alveolitis, fibrosis and pulmonar arterial hypertension].

Science.gov (United States)

Navarro, Carmen

2006-11-01

Pulmonary involvement in systemic sclerosis. Alveolitis, fibrosis and pulmonar arterial hypertension Lung disease is present in most of the patients with systemic sclerosis and is now the most important cause of mortality. Interstitial lung disease and pulmonary hypertension are, so far, the main disorders found and both are difficult to detect at the earliest stages. However, diagnostic tools such as immunological test, lung function test, high resolution CT, bronchoalveolar lavage, echocardiography, right-side cardiac catheterization, or lung biopsy are necessary to accurately evaluate the clinical status and allow to improve the management organ-specific ad hoc. Progress in immunological and vascular therapies as well as other emergence drugs offer new expectations to scleroderma patients. Copyright © 2006 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved.

How to adapt the pulmonary rehabilitation programme to patients with chronic respiratory disease other than COPD

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Anne E. Holland

2013-12-01

Full Text Available Dyspnoea, fatigue, reduced exercise tolerance, peripheral muscle dysfunction and mood disorders are common features of many chronic respiratory disorders. Pulmonary rehabilitation successfully treats these manifestations in chronic obstructive pulmonary disease (COPD and emerging evidence suggests that these benefits could be extended to other chronic respiratory conditions, although adaptations to the standard programme format may be required. Whilst the benefits of exercise training are well established in asthma, pulmonary rehabilitation can also provide evidence-based interventions including breathing techniques and self-management training. In interstitial lung disease, a small number of trials show improved exercise capacity, symptoms and quality of life following pulmonary rehabilitation, which is a positive development for patients who may have few treatment options. In pulmonary arterial hypertension, exercise training is safe and effective if patients are stable on medical therapy and close supervision is provided. Pulmonary rehabilitation for bronchiectasis, including exercise training and airway clearance techniques, improves exercise capacity and quality of life. In nonsmall cell lung cancer, a comprehensive interdisciplinary approach is required to ensure the success of pulmonary rehabilitation following surgery. Pulmonary rehabilitation programmes provide important and underutilised opportunities to improve the integrated care of people with chronic respiratory disorders other than COPD.

Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

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Nakamura, Yutaro; Suda, Takafumi

2015-01-01

Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

Modeling of interstitial diffusion of ion-implanted boron

International Nuclear Information System (INIS)

Velichko, O.I.; Knyazheva, N.V.

2009-01-01

A model of the interstitial diffusion of ion-implanted boron during rapid thermal annealing of silicon layers previously amorphized by implantation of germanium has been proposed. It is supposed that the boron interstitials are created continuously during annealing due to generation, dissolution, or rearrangement of the clusters of impurity atoms which are formed in the ion-implanted layers with impurity concentration above the solubility limit. The local elastic stresses arising due to the difference of boron atomic radius and atomic radius of silicon also contribute to the generation of boron interstitials. A simulation of boron redistribution during thermal annealing for 60 s at a temperature of 850 C has been carried out. The calculated profile agrees well with the experimental data. A number of the parameters of interstitial diffusion have been derived. In particular, the average migration length of nonequilibrium boron interstitials is equal to 12 nm. It was also obtained that approximately 1.94% of boron atoms were converted to the interstitial sites, participated in the fast interstitial migration, and then became immobile again transferring into a substitutional position or forming the electrically inactive complexes with crystal lattice defects. (authors)

Regulation of tumor invasion by interstitial fluid flow

International Nuclear Information System (INIS)

Shieh, Adrian C; Swartz, Melody A

2011-01-01

The importance of the tumor microenvironment in cancer progression is undisputed, yet the significance of biophysical forces in the microenvironment remains poorly understood. Interstitial fluid flow is a nearly ubiquitous and physiologically relevant biophysical force that is elevated in tumors because of tumor-associated angiogenesis and lymphangiogenesis, as well as changes in the tumor stroma. Not only does it apply physical forces to cells directly, but interstitial flow also creates gradients of soluble signals in the tumor microenvironment, thus influencing cell behavior and modulating cell–cell interactions. In this paper, we highlight our current understanding of interstitial fluid flow in the context of the tumor, focusing on the physical changes that lead to elevated interstitial flow, how cells sense flow and how they respond to changes in interstitial flow. In particular, we emphasize that interstitial flow can directly promote tumor cell invasion through a mechanism known as autologous chemotaxis, and indirectly support tumor invasion via both biophysical and biochemical cues generated by stromal cells. Thus, interstitial fluid flow demonstrates how important biophysical factors are in cancer, both by modulating cell behavior and coupling biophysical and biochemical signals

Acute interstitial pneumonia (AIP): relationship to Hamman-Rich syndrome, diffuse alveolar damage (DAD), and acute respiratory distress syndrome (ARDS).

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Mukhopadhyay, Sanjay; Parambil, Joseph G

2012-10-01

Acute interstitial pneumonia (AIP) is a term used for an idiopathic form of acute lung injury characterized clinically by acute respiratory failure with bilateral lung infiltrates and histologically by diffuse alveolar damage (DAD), a combination of findings previously known as the Hamman-Rich syndrome. This review aims to clarify the diagnostic criteria of AIP, its relationship with DAD and acute respiratory distress syndrome (ARDS), key etiologies that need to be excluded before making the diagnosis, and the salient clinical features. Cases that meet clinical and pathologic criteria for AIP overlap substantially with those that fulfill clinical criteria for ARDS. The main differences between AIP and ARDS are that AIP requires a histologic diagnosis of DAD and exclusion of known etiologies. AIP should also be distinguished from "acute exacerbation of IPF," a condition in which acute lung injury (usually DAD) supervenes on underlying usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF). Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary arterial hypertension

Science.gov (United States)

2013-01-01

Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should systematically been screened regarding to underlying mutations of BMPR2 gene (bone morphogenetic protein receptor type 2) or more rarely of ACVRL1 (activine receptor-like kinase type 1), ENG (endogline) or Smad8 genes. Pulmonary veno occusive disease and pulmonary capillary hemagiomatosis are individualized and designated as clinical group 1'. Group 2 'Pulmonary hypertension due to left heart diseases' is divided into three sub-groups: systolic dysfonction, diastolic dysfonction and valvular dysfonction. Group 3 'Pulmonary hypertension due to respiratory diseases' includes a heterogenous subgroup of respiratory diseases like PH due to pulmonary fibrosis, COPD, lung emphysema or interstitial lung disease for exemple. Group 4 includes chronic thromboembolic pulmonary hypertension without any distinction of proximal or distal forms. Group 5 regroup PH patients with unclear multifactorial mechanisms. Invasive hemodynamic assessment with right heart catheterization is requested to confirm the definite diagnosis of PH showing a resting mean pulmonary artery pressure (mPAP) of ≥ 25 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg. The assessment of PCWP may allow the distinction between pre-capillary and post-capillary PH (PCWP > 15 mmHg). Echocardiography is an important tool in the management of patients with underlying suspicion of PH. The European Society of Cardiology and the European Respiratory Society (ESC-ERS) guidelines specify its role

Acute exacerbation of subclinical idiopathic pulmonary fibrosis triggered by hypofractionated stereotactic body radiotherapy in a patient with primary lung cancer and slightly focal honeycombing

International Nuclear Information System (INIS)

Takeda, Atsuya; Sanuki, Naoko; Enomoto, Tatsuji; Takeda, Toshiaki; Kunieda, Etsuo; Nakajima, Takeshi; Sayama, Koichi

2008-01-01

Hypofractionated stereotactic body radiotherapy (SBRT) for pulmonary lesions provides a high local control rate, allows completely painless ambulatory treatment, and is not associated with adverse reactions in most cases. Here we report a 70-year-old lung cancer patient with slight focal pulmonary honeycombing in whom subclinical idiopathic pulmonary fibrosis was exacerbated by SBRT. This experience has important implications for the development of selection criteria prior to SBRT for pulmonary lesions. For SBRT candidates with lung tumors, attention must be paid to the presence of co-morbid interstitial pneumonia even if findings are minimal. Such patients must be informed of potential risks, and careful decision-making must take place when SBRT is being considered. (author)

Heterogeneity in Fibroblast Proliferation and Survival in Idiopathic Pulmonary Fibrosis

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David Michael Habiel

2014-01-01

Full Text Available Idiopathic pulmonary fibrosis (IPF is the most common form of interstitial lung disease characterized by the persistence of activated myofibroblasts resulting in excessive deposition of extracellular matrix proteins and profound tissue remodeling. Myofibroblasts have been shown to arise from interstitial fibroblasts, epithelial to mesenchymal transition of type II alveolar epithelial cells, and the differentiation of recruited fibrocytes. There are many mechanisms that are utilized by these cells for survival, proliferation and persistent activation including up-regulation of cytokines (i.e. Interlukin 6 (IL-6, cytokine receptors (i.e. Interlukin 6 Receptor 1 (IL-6R1, Glycoprotein 130 (gp130 and C-C Chemokine Receptor type 7 (CCR7 and innate pattern recognition receptors (PRRs; i.e. Toll Like Receptor 9 (TLR9. In this review, we will discuss the role of the cytokines IL-6 and CCL21, their receptors and the pattern recognition receptor (PRR, TLR9, in fibroblast recruitment, activation, survival and differentiation into myofibroblasts in IPF.

Silencing of Carbohydrate Sulfotransferase 15 Hinders Murine Pulmonary Fibrosis Development

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Yoshiro Kai

2017-03-01

Full Text Available Pulmonary fibrosis is a progressive lung disorder characterized by interstitial fibrosis, for which no effective treatments are available. Chondroitin sulfate proteoglycan (CSPG has been shown to be a mediator, but the specific component of glycosaminoglycan chains of CSPG has not been explored. We show that chondroitin sulfate E-type (CS-E is involved in fibrogenesis. Small interfering RNA (siRNA targeting carbohydrate sulfotransferase 15 (CHST15 was designed to inhibit CHST15 mRNA and its product, CS-E. CS-E augments cell contraction and CHST15 siRNA inhibits collagen production. We found that bleomycin treatment increased CHST15 expression in interstitial fibroblasts at day 14. CHST15 siRNA was injected intranasally on days 1, 4, 8, and 11, and CHST15 mRNA was significantly suppressed by day 14. CHST15 siRNA reduced lung CSPG and the grade of fibrosis. CHST15 siRNA repressed the activation of fibroblasts, as evidenced by suppressed expression of α smooth muscle actin (αSMA, connective tissue growth factor (CTGF, lysyl oxidase like 2 (LOXL2, and CC-chemokine ligand 2 (CCL2/monocyte chemoattractant protein-1 (MCP-1. Inflammatory infiltrates in the bronchoalveolar lavage fluid (BALF and interstitium were diminished by CHST15 siRNA. These results indicate a pivotal role for CHST15 in fibroblast-mediated lung fibrosis and suggest a possible new therapeutic role for CHST15 siRNA in pulmonary fibrosis.

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

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Shahrzad M Lari

2014-05-01

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

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

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Yuki Nakajima

2016-01-01

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

Pulmonary infection in patients with cyclosporine, azathioprine, and corticosteroids after cardiac transplantation; Clinical and radiographic assessment

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Murayama, Sadayuki; Ikezoe, Junpei; Godwin, J.D.; Marglin, S.I.; Allen, M.D. (University of Washington Medical Center, Seattle, WA (United States))

1991-07-01

Between November 1985 and November 1989, 54 patients have undergone 55 cardiac transplants, 5 of whom died during operation or one week after transplantation. The remaining 49 patients with a minimum follow-up of 5 months were studied to examine pulmonary infection clinically and radiologically while receiving triple drug immunosuppression consisting of cyclosporine, azathioprine, and prednisolone. Pulmonary infection occurred in 14 patients (29%) with a total of 21 occasions. Causative organisms were identified in 9 occasions, with the most common organism being Cytomegalovirus (CMV). One patient died of pulmonary infection with Aspergillus. Causative organisms occurring in the remaining 12 occasions of pulmonary infection were unknown, which did not lead to death. Because pulmonary infection of unknown organisms rapidly responded to convensional antibiotics, it seemed to have been caused by bacteria. Pulmonary infection of unknown organism occurred 13.2{+-}3.2 months after transplantation, as compared with 3.3{+-}1.0 months in pulmonary infection of known organisms. Chest plain radiographic features fell into four types: (1) interstitial shadow seen in pulmonary infection of CMV, Pneumocystis carinii, or Hemophilia influenza, (2) patchy, and basilar and lobular consolidation shadows in bacterial pneumonia, (3) localized nodular shadow in aspergillosis, and (4) multiple patchy and confluent opacity patterns occurring in herpes simplex viral infection. Pulmonary infection of influenza bacteria for one patient and pulmonary infection of unknown organisms for 4 patients were difficult to identify from pulmonary infection of CMV. (N.K.).

Bleomycin induced pulmonary to cytotoxicity in patients with germ cell tumours

International Nuclear Information System (INIS)

Usman, M.; Faruqui, Z.S.; Din, N.U.

2010-01-01

Background: Bleomycin is a cytotoxic drug used in treatment of Germ Cell Tumours (GCTs) and is associated with pulmonary toxicity. Bleomycin pulmonary toxicity (BPT) manifests predominantly as pulmonary fibrosis, organising pneumonia (OP) or Nonspecific Interstitial Pneumonitis (NSIP). Our objectives were to determine the incidence of BPT, describe the common HRCT patterns of pulmonary toxicity and to find out the correlation of variables (cumulative dose of bleomycin, age and glomerular filtration rate) with pulmonary toxicity. Methods: The study included the data of 96 patients from March 2006 to September 2008. All patients had histologically proven GCT and received bleomycin containing regimes. Variables age, GFR at the time of initial presentation along with cumulative dose of bleomycin at completion of chemotherapy or at the time of BPT were recorded. The High resolution CT chest (HRCT) of these patients was independently reviewed by two radiologists. Bleomycin toxicity was reported on the radiologic features of pulmonary fibrosis, OP or NSIP. Results : Fourteen patients (14.6%) developed BPT. Common patterns of BPT were, pulmonary fibrosis (5.2%), OP (5.2%) and NSIP (4.2%). Using the Univariate regression analysis there was significant relationship between BPT and age, cumulative bleomycin dose an d initial GFR at the beginning of treatment. Conclusions: Because BPT can be progressive and fatal, early recognition is important. The diagnosis of pulmonary toxicity should be considered in any patient with new or progressive respiratory complaints. BPT can be difficult to diagnose; therefore, knowledge and understanding of radiologic manifestations of toxicity caused by Bleomycin are necessary for institution of appropriate treatment. There is increasing incidence of BPT with increasing age, cumulative dose and decreasing GFR. (author)

Clinical application of radioaerosol studies - pulmonary embolism, inhalation burns and glue-sniffers and COPD

International Nuclear Information System (INIS)

Sundram, Felix

1994-01-01

The alveolar epithelium and the capillary endothelium together form the alveolar capillary membrane. Fluid exchange occurs across this membrane, and is dependent on intravascular and interstitial hydrostatic and oncotic pressures, and on permeability of this membrane. Damage to either the alveolar or capillary component can result in a high permeability pulmonary oedema, even though the alveolar epithelium forms an extremely tight membrane which is ten times less permeable than the capillary endothelium. Nuclear medicine methods can be used to observe changes in integrity of pulmonary capillary endothelium (with first pass dual-indicator dilution technique using successive injections of radiotracer), and of alveolar epithelium, and it is important that the damage should be detected before patients develop clinical pulmonary oedema so that intensive therapy can be instituted early. We have used 99m Tc DTPA radioaerosol to measure alteration in pulmonary epithelial permeability and to image the distribution of ventilation in normal and some pathological states. In some clinical studies Tc-99m (tin) colloid radioaerosol has been used to obtain the ventilation images

Interstitial hydraulic conductivity and interstitial fluid pressure for avascular or poorly vascularized tumors.

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Liu, L J; Schlesinger, M

2015-09-07

A correct description of the hydraulic conductivity is essential for determining the actual tumor interstitial fluid pressure (TIFP) distribution. Traditionally, it has been assumed that the hydraulic conductivities both in a tumor and normal tissue are constant, and that a tumor has a much larger interstitial hydraulic conductivity than normal tissue. The abrupt transition of the hydraulic conductivity at the tumor surface leads to non-physical results (the hydraulic conductivity and the slope of the TIFP are not continuous at tumor surface). For the sake of simplicity and the need to represent reality, we focus our analysis on avascular or poorly vascularized tumors, which have a necrosis that is mostly in the center and vascularization that is mostly on the periphery. We suggest that there is an intermediary region between the tumor surface and normal tissue. Through this region, the interstitium (including the structure and composition of solid components and interstitial fluid) transitions from tumor to normal tissue. This process also causes the hydraulic conductivity to do the same. We introduce a continuous variation of the hydraulic conductivity, and show that the interstitial hydraulic conductivity in the intermediary region should be monotonically increasing up to the value of hydraulic conductivity in the normal tissue in order for the model to correspond to the actual TIFP distribution. The value of the hydraulic conductivity at the tumor surface should be the lowest in value. Copyright © 2015 Elsevier Ltd. All rights reserved.

Computed chest tomography in rats with pulmonary damage due to microembolism

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Wegener, T.; Wegenius, G.; Hemmingsson, A.; Jung, B.; Saldeen, T.

Computed chest tomography was performed in 13 rats with pulmonary damage due to microembolism, caused by injection of thrombin (500 NIH/kg body weight) and tranexamic acid, a fibrinolytic inhibitor (200 mg/kg body weight), and in 9 control rats. The purpose of the investigation was to perform attenuation measurements at two levels of the right lung, each with three regions of interest (anterior, mid and posterior). Alterations in attenuation, compared with controls, were correlated with lung weight. Compared with controls, the attenuation was significantly increased in the anterior and posterior regions at both levels in animals with pulmonary damage, but not in the mid regions. There was a statistically significant correlation between increasing attenuation and increasing lung weight. A significant difference was found between damaged and control lungs regarding the microscopic grade of interstitial oedema, alveolar oedema and fibrin. Histograms of attenuation values in computed tomograms might be of value in detecting alveolar oedema. It is concluded that computed chest tomography is a good method for detection pulmonary oedema at an early stage of experimental microembolism in the rat.

Computed chest tomography in rats with pulmonary damage due to microembolism

International Nuclear Information System (INIS)

Wegener, T.; Wegenius, G.; Hemmingsson, A.; Jung, B.; Saldeen, T.; Uppsala Univ.; Uppsala Univ.; Uppsala Univ.

1986-01-01

Computed chest tomography was performed in 13 rats with pulmonary damage due to microembolism, caused by injection of thrombin (500 NIH/kg body weight) and tranexamic acid, a fibrinolytic inhibitor (200 mg/kg body weight), and in 9 control rats. The purpose of the investigation was to perform attenuation measurements at two levels of the right lung, each with three regions of interest (anterior, mid and posterior). Alterations in attenuation, compared with controls, were correlated with lung weight. Compared with controls, the attenuation was significantly increased in the anterior and posterior regions at both levels in animals with pulmonary damage, but not in the mid regions. There was a statistically significant correlation between increasing attenuation and increasing lung weight. A significant difference was found between damaged and control lungs regarding the microscopic grade of interstitial oedema, alveolar oedema and fibrin. Histograms of attenuation values in computed tomograms might be of value in detecting alveolar oedema. It is concluded that computed chest tomography is a good method for detection pulmonary oedema at an early stage of experimental microembolism in the rat. (orig.)

Modeling of long-range migration of boron interstitials

International Nuclear Information System (INIS)

Velichko, O.I.; Burunova, O.N.

2009-01-01

A model of the interstitial migration of ion-implanted dopant in silicon during low-temperature thermal treatment has been formulated. It is supposed that the boron interstitials are created during ion implantation or at the initial stage of annealing. During thermal treatment a migration of these impurity interstitials to the surface and in the bulk of a semiconductor occurs. On this basis, a simulation of boron redistribution during thermal annealing for 35 minutes at a temperature of 800 0 C has been carried out. The calculated boron profile agrees well with the experimental data. A number of the parameters describing the interstitial diffusion have been derived. In particular, the average migration length of nonequilibrium boron interstitials is equal to 0.092 μm at a temperature of 800 0 C. To carry out modeling of ion-implanted boron redistribution, the analytical solutions of nonstationary diffusion equation for impurity interstitials have been obtained. The case of Dirichlet boundary conditions and the case of reflecting boundary on the surface of a semiconductor have been considered. (authors)

Grape seed extract ameliorates bleomycin-induced mouse pulmonary fibrosis.

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Liu, Qi; Jiang, Jun-Xia; Liu, Ya-Nan; Ge, Ling-Tian; Guan, Yan; Zhao, Wei; Jia, Yong-Liang; Dong, Xin-Wei; Sun, Yun; Xie, Qiang-Min

2017-05-05

Pulmonary fibrosis is common in a variety of inflammatory lung diseases, such as interstitial pneumonia, chronic obstructive pulmonary disease, and silicosis. There is currently no effective clinical drug treatment. It has been reported that grape seed extracts (GSE) has extensive pharmacological effects with minimal toxicity. Although it has been found that GSE can improve the lung collagen deposition and fibrosis pathology induced by bleomycin in rat, its effects on pulmonary function, inflammation, growth factors, matrix metalloproteinases and epithelial-mesenchymal transition remain to be researched. In the present study, we studied whether GSE provided protection against bleomycin (BLM)-induced mouse pulmonary fibrosis. ICR strain mice were treated with BLM in order to establish pulmonary fibrosis models. GSE was given daily via intragastric administration for three weeks starting at one day after intratracheal instillation. GSE at 50 or 100mg/kg significantly reduced BLM-induced inflammatory cells infiltration, proinflammatory factor protein expression, and hydroxyproline in lung tissues, and improved pulmonary function in mice. Additionally, treatment with GSE also significantly impaired BLM-induced increases in lung fibrotic marker expression (collagen type I alpha 1 and fibronectin 1) and decreases in an anti-fibrotic marker (E-cadherin). Further investigation indicated that the possible molecular targets of GSE are matrix metalloproteinases-9 (MMP-9) and TGF-β1, given that treatment with GSE significantly prevented BLM-induced increases in MMP-9 and TGF-β1 expression in the lungs. Together, these results suggest that supplementation with GSE may improve the quality of life of lung fibrosis patients by inhibiting MMP-9 and TGF-β1 expression in the lungs. Copyright © 2017 Elsevier B.V. All rights reserved.

Inflammatory myofibroblastic tumors of the lung carrying a chimeric A2M-ALK gene: report of 2 infantile cases and review of the differential diagnosis of infantile pulmonary lesions.

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Tanaka, Mio; Kohashi, Kenichi; Kushitani, Kei; Yoshida, Misa; Kurihara, Sho; Kawashima, Masumi; Ueda, Yuka; Souzaki, Ryota; Kinoshita, Yoshiaki; Oda, Yoshinao; Takeshima, Yukio; Hiyama, Eiso; Taguchi, Tomoaki; Tanaka, Yukichi

2017-08-01

We report 2 infantile cases of pulmonary tumor carrying a chimeric A2M-ALK gene. A2M-ALK is a newly identified anaplastic lymphoma kinase (ALK)-related chimeric gene from a tumor diagnosed as fetal lung interstitial tumor (FLIT). FLIT is a recently recognized infantile pulmonary lesion defined as a mass-like lesion that morphologically resembles the fetal lung. Grossly, FLIT characteristically appears as a well-circumscribed spongy mass, whereas the tumors in these patients were solid and firm. Histologically, the tumors showed intrapulmonary lesions composed of densely proliferating polygonal or spindle-shaped mesenchymal cells with diffuse and dense infiltrations of inflammatory cells forming microcystic or micropapillary structures lined by thyroid transcription factor 1-positive pneumocytes, favoring inflammatory myofibroblastic tumor rather than FLIT. The proliferating cells were immunoreactive for ALK, and A2M-ALK was identified in both tumors with reverse-transcription polymerase chain reaction. The dense infiltration of inflammatory cells, immunoreactivity for ALK, and identification of an ALK-related chimeric gene suggested a diagnosis of inflammatory myofibroblastic tumor. Histologically, most reported FLITs show sparse inflammatory infiltrates and a relatively low density of interstitial cells in the septa, although prominent infiltration of inflammatory cells and high cellularity of interstitial cells are seen in some FLITs. The present cases suggest that ALK rearrangements, including the chimeric A2M-ALK gene, may be present in these infantile pulmonary lesions, especially those with inflammatory cell infiltration. We propose that these infantile pulmonary lesions containing a chimeric A2M-ALK gene be categorized as a specific type of inflammatory myofibroblastic tumor that develops exclusively in neonates and infants. Copyright © 2017 Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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

2015-01-01

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, P<0.028). Fibrosis 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.

Stability and mobility of self-interstitials and small interstitial clusters in α-iron: ab initio and empirical potential calculations

International Nuclear Information System (INIS)

Willaime, F.; Fu, C.C.; Marinica, M.C.; Dalla Torre, J.

2005-01-01

The stability and mobility of self-interstitials and small interstitial clusters, I n , in α-Fe is investigated by means of calculations performed in the framework of the density functional theory using the SIESTA code. The mono-, di- and tri-interstitials are shown to be made of (parallel) dumbbells and to migrate by nearest-neighbor translation-rotation jumps, according to Johnson's mechanism. The orientation of the dumbbells becomes energetically more favourable for I 5 and larger clusters. The performance of a semi-empirical potential recently developed for Fe, including ab initio self-interstitial data in the fitted properties, is evaluated over the present results. The superiority over previous semi-empirical potentials is confirmed. Finally the impact of the present results on the formation mechanism of loops, observed experimentally in α-Fe is discussed

Idiopathic interstitial pneumonias: radiologic-pathologic correlation

International Nuclear Information System (INIS)

Yoon, Young Cheol; Suh, Gee Young; Han, Joung Ho; Lee, Kyung Soo

2002-01-01

Idiopathic interstitial pneumonias are at present classified as one of four types: usual, nonspecific, acute, or desquamative. The acute form has the worst prognosis, followed by the usual and the nonspecific form; it is in desquamative cases that prognosis is best. At high-resolution CT, usual interstitial pneumonia, the most frequent type, manifests as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing, which the nonspecific type, the second most frequent, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity. Acute interstitial pneumonia demonstrates extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation in middle and lower lung zones

Avaliação de parâmetros histológicos na pneumonia intersticial usual (fibrose pulmonar idiopática Evaluation of the histological parameters in usual interstitial pneumonia (idiopathic pulmonary fibrosis

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RIMARCS GOMES FERREIRA

2000-12-01

Full Text Available A fibrose pulmonar idiopática (alveolite fibrosante criptogênica é uma doença pulmonar intersticial progressiva de etiologia desconhecida, morfologicamente reconhecida como pneumonia intersticial usual. Desde a publicação de Hamman e Rich (1944 até os dias atuais, uma das grandes preocupações foi a tentativa de encontrar um marcador histológico para correlacionar com prognóstico e resposta terapêutica. A busca desta situação não tem sido muito alentadora, pois existem vários pontos duvidosos na patogênese desta doença. Admite-se que a resposta terapêutica desta entidade se relaciona com a celularidade e fibrose presentes no tecido. A proposta deste estudo é descrever os resultados de método semiquantitativo segundo a avaliação independente de dois patologistas, das alterações exsudativo-inflamatórias, reparativo-fibróticas e de vias aéreas, em 24 pacientes com diagnóstico de fibrose pulmonar idiopática, submetidos à biópsia a céu aberto. Foram analisados 14 parâmetros histológicos segundo escala de 0 a 5 para as alterações intersticiais e de 0 a 2 para o comprometimento de vias aéreas e de espaços aéreos. Da análise independente realizada pelos dois observadores constatou-se concordância significante em todas as variáveis histológicas com Kw (teste de Kappa indo de 0,47 a 0,92. Apenas na análise da intensidade da inflamação septal as discordâncias também foram significantes, sugerindo que para este parâmetro a percentagem de comprometimento tecidual deve ser previamente discutida entre os observadores. O método utilizado demonstrou ser rápido e eficiente.Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis is a progressive interstitial pulmonary disease of unknown etiology. Since Hamman's and Rich's (1944 reports, many studies have tried to find a histological marker for the correlation between prognosis and response to therapy. However, there are many doubts regarding pathogenesis

Autopsy-proven causes of death in lungs of patients immunocompromised by secondary interstitial pneumonia Causas de óbito por pneumonia intersticial secundária em autópsias pulmonares de pacientes imunocomprometidos

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Alberto Antonio Terrabuio Junior

2007-02-01

Full Text Available PURPOSE: To present the more frequent associations found in autopsies of immunocompromised patients who developed secondary interstitial pneumonia as well as the risk of death (odds ratio in having specific secondary interstitial pneumonia according to the cause of immunocompromise. METHOD: From January 1994 to March 2004, 17,000 autopsies were performed at Hospital das Clínicas, São Paulo University Medical School. After examining the pathology report review, we selected 558 of these autopsies (3.28% from patients aged 15 years or more with primary underlying diseases who developed radiologically diffuse infiltrates of the lung during their hospital course and died after secondary interstitial pneumonia (bronchopneumonia, lobar pneumonia, interstitial pneumonia, diffuse alveolar damage, pulmonary recurrence of underlying disease, drug-induced lung disease, cardiogenic pulmonary edema, or pulmonary embolism. Histology slides were reviewed by experienced pathologists to confirm or not the presence of secondary interstitial pneumonia. Statistical analysis included the Fisher exact test to verify any association between histopathology and the cause of immunocompromise; a logistic regression was used to predict the risk of death for specific histological findings for each of the independent variables in the model. RESULTS: Secondary interstitial pneumonia was histologically represented by diffuse interstitial pneumonitis ranging from mild nonspecific findings (n = 213 to a pattern of diffuse alveolar damage (n = 273. The principal causes of immunocompromise in patients with diffuse alveolar damage were sepsis (136 cases, neoplasia (113 cases, diabetes mellitus (37 cases, and transplantation (48 cases. A high risk of death by pulmonary edema was found for patients with carcinoma of colon. Similarly, in patients with lung cancer or cachexia, A high risk of death by bronchopneumonia (OR = 3.6; OR = 2.6, respectively was found. Pulmonary

Clinical Impact of Emphysema Evaluated by High-Resolution Computed Tomography on Idiopathic Pulmonary Fibrosis Diagnosed by Surgical Lung Biopsy.

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Kohashi, Yasuo; Arai, Toru; Sugimoto, Chikatoshi; Tachibana, Kazunobu; Akira, Masanori; Kitaichi, Masanori; Hayashi, Seiji; Inoue, Yoshikazu

2016-01-01

The prognosis of combined cases of pulmonary fibrosis and emphysema is unresolved partially because radiological differentiation between usual interstitial pneumonia and nonspecific interstitial pneumonia is difficult in coexisting emphysema cases. The purpose of this study was to clarify the clinical impact of emphysema on the survival of patients with idiopathic pulmonary fibrosis (IPF). One hundred and seven patients with interstitial lung diseases were diagnosed by surgical lung biopsies between 2006 and 2012, and 47 patients were diagnosed with IPF through multidisciplinary discussion. Emphysema on high-resolution computed tomography scans was evaluated semiquantitatively by visual scoring. Eight out of the 47 IPF patients showed a higher emphysema score (>3) and were diagnosed to have IPF-emphysema. The median survival time of patients with IPF-emphysema (1,734 days) from the initial diagnosis was significantly shorter than that of patients with IPF alone (2,229 days) by Kaplan-Meier analysis (p = 0.007, log-rank test). Univariate Cox proportional hazard regression analyses revealed that a higher total emphysema score (>3.0) was a significantly poor prognostic factor in addition to Krebs von den Lungen-6, surfactant protein-D, arterial oxygen tension, percent forced vital capacity, and percent diffusing capacity of carbon monoxide (%DLCO). Multivariate Cox proportional hazard regression analyses with the stepwise method showed that higher total emphysema score (>3) and %DLCO were significantly poor prognostic factors. The prognosis of IPF-emphysema was significantly worse than that of IPF alone. © 2016 S. Karger AG, Basel.

Ventilation-perfusion scans in neonatal regional pulmonary emphysema complicating ventilatory assistance

International Nuclear Information System (INIS)

Leonidas, J.C.; Moylan, F.M.B.; Kahn, P.C.; Ramenofsky, M.L.

1978-01-01

Two cases of ventilator-related neonatal lobar overexpansion with similar radiographic appearance, but probably different pathogenesis, are presented. In one infant, persistent interstitial lobar emphysema was confirmed by markedly decreased perfusion shown on scintigraphy; this information was of great value in predicting the beneficial effect of lobectomy. In the other case, ventilation and perfusion scans indicated functional value of the emphysematous lobe and correctly suggested conservative management. Radioisotope lung scans may provide valuable information regarding lung function in regional pulmonary emphysema associated with assisted ventilation in neonatal respiratory distress syndrome, and thus determine patient management

Microstructures and phase transformations in interstitial alloys of tantalum

International Nuclear Information System (INIS)

Dahmen, U.

1979-01-01

The analysis of microstructures, phases, and possible ordering of interstitial solute atoms is fundamental to an understanding of the properties of metal-interstitial alloys in general. As evidenced by the controversies on phase transformations in the particular system tantalum--carbon, our understanding of this class of alloys is inferior to our knowledge of substitutional metal alloys. An experimental clarification of these controversies in tantalum was made. Using advanced techniques of electron microscopy and ultrahigh vacuum techology, an understanding of the microstructures and phase transformations in dilute interstitial tantalum--carbon alloys is developed. Through a number of control experiments, the role and sources of interstitial contamination in the alloy preparation (and under operating conditions) are revealed. It is demonstrated that all previously published work on the dilute interstitially ordered phase Ta 64 C can be explained consistently in terms of ordering of the interstitial contaminants oxygen and hydrogen, leading to the formation of the phases Ta 12 O and Ta 2 H

Interstitial Granulomatous Dermatitis (IGD

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Tiberiu Tebeica

2017-07-01

Full Text Available We report the case of a 42 years old male patient suffering from skin changes , which appeared in the last 7-8 years.  Two biopsies were performed during the evolution of the lesion. Both showed similar findings that consisted in a busy dermis with interstitial, superficial and deep infiltrates of lymphocytes and histiocytes dispersed among collagen bundles, with variable numbers of neutrophils scattered throughout. Some histiocytes were clustered in poorly formed granuloma that included rare giant cells, with discrete Palisades and piecemeal collagen degeneration, but without mucin deposition or frank necrobiosis of collagen. The clinical and histologic findings were supportive for interstitial granulomatous dermatitis. Interstitial granulomatous dermatitis (IGD is a poorly understood entity that was regarded by many as belonging to the same spectrum of disease or even synonym with palisaded and neutrophilic granulomatous dermatitis (PNGD. Although IGD and PNGD were usually related to connective tissue disease, mostly rheumatoid arthritis, some patients with typical histologic findings of IGD never develop autoimmune disorders, but they have different underlying conditions, such as metabolic diseases, lymphoproliferative disorders or other malignant tumours. These observations indicate that IGD and PNGD are different disorders with similar manifestations.

Understanding the Determinants of Health-Related Quality of Life in Rheumatoid Arthritis-Associated Interstitial Lung Disease

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Natalini, Jake G.; Swigris, Jeff J.; Morisset, Julie; Elicker, Brett M.; Jones, Kirk D.; Fischer, Aryeh; Collard, Harold R.; Lee, Joyce S.

2017-01-01

Rationale Health-related quality of life (HRQL) is impaired among patients with interstitial lung disease (ILD). Little is understood about HRQL in specific subtypes of ILD. Objectives The aim of this study was to characterize and identify clinical determinants of HRQL among patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and compare them to patients with idiopathic pulmonary fibrosis (IPF). Methods We identified patients with a diagnosis of RA-ILD and IPF from an ongoing longitudinal cohort of ILD patients. HRQL was measured at their baseline visit using the Short Form Health Survey (SF-36), versions 1 and 2. Regression models were used to characterize and understand the relationship between selected baseline clinical covariates, the physical component score (PCS) and mental component score (MCS) of the SF-36. Measurements and Main Results RA-ILD patients (n=50) were more likely to be younger and female compared to IPF patients (n=50). After controlling for age and pulmonary function, RA-ILD patients had a lower HRQL compared to IPF patients, as measured by the PCS (P=0.03), with significant differences in two of four PCS domains – bodily pain (P<0.01) and general health (P=0.01). Clinical covariates most strongly associated with a lower PCS in RA-ILD patients compared to IPF patients were the presence of joint pain or stiffness and dyspnea severity (P<0.01). Mental and emotional health, as measured by the MCS, was similar between RA-ILD and IPF patients. Conclusion The physical components of HRQL appear worse in RA-ILD patients compared to IPF patients as measured by the PCS of the SF-36. Differences in the PCS of the SF-36 can be explained in part by dyspnea severity and joint symptoms among patients with RA-ILD. PMID:28502413

Análise de 39 casos de pneumonia intersticial crônica idiopática Analysis of 39 cases of idiopathic chronic interstitial pneumonia

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Rogério Rufino

2006-12-01

performed between 1977 and 1999, were reviewed, and 39 cases of idiopathic interstitial lung disease were selected and re-evaluated by two pathologists in accordance with the American Thoracic Society/European Respiratory Society classification system. RESULTS: Among those 39 cases, the diagnoses were maintained in 28 (71.8%. A new pathologic entity, nonspecific interstitial pneumonia, was included in the reclassification, and overlapping patterns were observed in 6 cases. Of the 28 cases in which the diagnosis of chronic idiopathic interstitial pneumonia remained unchanged, idiopathic pulmonary fibrosis was accompanied by cryptogenic organizing pneumonia in 4, cryptogenic organizing pneumonia was accompanied by nonspecific interstitial pneumonia in 1, and desquamative interstitial pneumonia was accompanied by nonspecific interstitial pneumonia in 1. All cases of idiopathic pulmonary fibrosis were confirmed, although 3 of those were found to be accompanied by cryptogenic organizing pneumonia. Virtually all prior diagnoses were maintained in the review of the biopsy samples (p > 0,05. CONCLUSION: The American Thoracic Society/European Respiratory Society system of classifying interstitial lung disease is a useful tool for pathologists who deal with lung biopsies.

Pulmonary function-morphologic relationships assessed by SPECT-CT fusion images

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Suga, Kazuyoshi

2012-01-01

Pulmonary single photon emission computed tomography-computed tomography (SPECT-CT) fusion images provide objective and comprehensive assessment of pulmonary function and morphology relationships at cross-sectional lungs. This article reviewed the noteworthy findings of lung pathophysiology in wide-spectral lung disorders, which have been revealed on SPECT-CT fusion images in 8 years of experience. The fusion images confirmed the fundamental pathophysiologic appearance of lung low CT attenuation caused by airway obstruction-induced hypoxic vasoconstriction and that caused by direct pulmonary arterial obstruction as in acute pulmonary thromboembolism (PTE). The fusion images showed better correlation of lung perfusion distribution with lung CT attenuation changes at lung mosaic CT attenuation (MCA) compared with regional ventilation in the wide-spectral lung disorders, indicating that lung heterogeneous perfusion distribution may be a dominant mechanism of MCA on CT. SPECT-CT angiography fusion images revealed occasional dissociation between lung perfusion defects and intravascular clots in acute PTE, indicating the importance of assessment of actual effect of intravascular colts on peripheral lung perfusion. Perfusion SPECT-CT fusion images revealed the characteristic and preferential location of pulmonary infarction in acute PTE. The fusion images showed occasional unexpected perfusion defects in normal lung areas on CT in chronic obstructive pulmonary diseases and interstitial lung diseases, indicating the ability of perfusion SPECT superior to CT for detection of mild lesions in these disorders. The fusion images showed frequent ''steal phenomenon''-induced perfusion defects extending to the surrounding normal lung of arteriovenous fistulas and those at normal lungs on CT in hepatopulmonary syndrome. Comprehensive assessment of lung function-CT morphology on fusion images will lead to more profound understanding of lung pathophysiology in wide-spectral lung

Acute interstitial pneumonia

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Cuervo M, Francisco; Carrillo Bayona, Jorge; Ojeda, Paulina

2004-01-01

The paper refers to a 71 year-old patient, to who is diagnosed acute interstitial pneumonia; with square of 20 days of evolution of cough dry emetizant, fever, general uneasiness, migraine, progressive dyspnoea and lost of weight

The interstitial pneumonitis induced by cytostatics

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Dubrava, M.; Markova, I.; Mistina, L.

1998-01-01

The author presents a cause of 9-year old boy with ALL-F2B in the stage of the prevention treatment where in the its course the induced interstitial pneumonitis by cytostatics was developed. The bacterial, virus, mycological and parasitic causes of the interstitial pneumonitis on the basis of the bronchoscopy, BAL, CT, scintigraphy, laboratory and by cultivation were excluded. (authors)

Lung volumes and emphysema in smokers with interstitial lung abnormalities.

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Washko, George R; Hunninghake, Gary M; Fernandez, Isis E; Nishino, Mizuki; Okajima, Yuka; Yamashiro, Tsuneo; Ross, James C; Estépar, Raúl San José; Lynch, David A; Brehm, John M; Andriole, Katherine P; Diaz, Alejandro A; Khorasani, Ramin; D'Aco, Katherine; Sciurba, Frank C; Silverman, Edwin K; Hatabu, Hiroto; Rosas, Ivan O

2011-03-10

Cigarette smoking is associated with emphysema and radiographic interstitial lung abnormalities. The degree to which interstitial lung abnormalities are associated with reduced total lung capacity and the extent of emphysema is not known. We looked for interstitial lung abnormalities in 2416 (96%) of 2508 high-resolution computed tomographic (HRCT) scans of the lung obtained from a cohort of smokers. We used linear and logistic regression to evaluate the associations between interstitial lung abnormalities and HRCT measurements of total lung capacity and emphysema. Interstitial lung abnormalities were present in 194 (8%) of the 2416 HRCT scans evaluated. In statistical models adjusting for relevant covariates, interstitial lung abnormalities were associated with reduced total lung capacity (-0.444 liters; 95% confidence interval [CI], -0.596 to -0.292; Ppulmonary disease (COPD) (odds ratio, 0.53; 95% CI, 0.37 to 0.76; P<0.001). The effect of interstitial lung abnormalities on total lung capacity and emphysema was dependent on COPD status (P<0.02 for the interactions). Interstitial lung abnormalities were positively associated with both greater exposure to tobacco smoke and current smoking. In smokers, interstitial lung abnormalities--which were present on about 1 of every 12 HRCT scans--were associated with reduced total lung capacity and a lesser amount of emphysema. (Funded by the National Institutes of Health and the Parker B. Francis Foundation; ClinicalTrials.gov number, NCT00608764.).

Extracorporeal Membrane Oxygenation for End-Stage Interstitial Lung Disease With Secondary Pulmonary Hypertension at Rest and Exercise: Insights From Simulation Modeling.

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Chicotka, Scott; Burkhoff, Daniel; Dickstein, Marc L; Bacchetta, Matthew

Interstitial lung disease (ILD) represents a collection of lung disorders with a lethal trajectory with few therapeutic options with the exception of lung transplantation. Various extracorporeal membrane oxygenation (ECMO) configurations have been used for bridge to transplant (BTT), yet no optimal configuration has been clearly demonstrated. Using a cardiopulmonary simulation, we assessed different ECMO configurations for patients with end-stage ILD to assess the physiologic deficits and help guide the development of new long-term pulmonary support devices. A cardiopulmonary ECMO simulation was created, and changes in hemodynamics and blood gases were compared for different inflow and outflow anatomic locations and for different sweep gas and blood pump flow rates. The system simulated the physiologic response of patients with severe ILD at rest and during exercise with central ECMO, peripheral ECMO, and with no ECMO. The output parameters were total cardiac output (CO), mixed venous oxygen (O2) saturation, arterial pH, and O2 delivery (DO2)/O2 utilization (VO2) at different levels of exercise. The model described the physiologic state of progressive ILD and showed the relative effects of using various ECMO configurations to support them. It elucidated the optimal device configurations and required physiologic pump performance and provided insight into the physiologic demands of exercise in ILD patients. The simulation program was able to model the pathophysiologic state of progressive ILD with PH and demonstrate how mechanical support devices can be implemented to improve cardiopulmonary function at rest and during exercise. The information generated from simulation can be used to optimize ECMO configuration selection for BTT patients and provide design guidance for new devices to better meet the physiologic demands of exercise associated with normal activities of daily living.

Quantitatively assessed CT imaging measures of pulmonary interstitial pneumonia: Effects of reconstruction algorithms on histogram parameters

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Koyama, Hisanobu; Ohno, Yoshiharu; Yamazaki, Youichi; Nogami, Munenobu; Kusaka, Akiko; Murase, Kenya; Sugimura, Kazuro

2010-01-01

This study aimed the influences of reconstruction algorithm for quantitative assessments in interstitial pneumonia patients. A total of 25 collagen vascular disease patients (nine male patients and 16 female patients; mean age, 57.2 years; age range 32-77 years) underwent thin-section MDCT examinations, and MDCT data were reconstructed with three kinds of reconstruction algorithm (two high-frequencies [A and B] and one standard [C]). In reconstruction algorithm B, the effect of low- and middle-frequency space was suppressed compared with reconstruction algorithm A. As quantitative CT parameters, kurtosis, skewness, and mean lung density (MLD) were acquired from a frequency histogram of the whole lung parenchyma in each reconstruction algorithm. To determine the difference of quantitative CT parameters affected by reconstruction algorithms, these parameters were compared statistically. To determine the relationships with the disease severity, these parameters were correlated with PFTs. In the results, all the histogram parameters values had significant differences each other (p < 0.0001) and those of reconstruction algorithm C were the highest. All MLDs had fair or moderate correlation with all parameters of PFT (-0.64 < r < -0.45, p < 0.05). Though kurtosis and skewness in high-frequency reconstruction algorithm A had significant correlations with all parameters of PFT (-0.61 < r < -0.45, p < 0.05), there were significant correlations only with diffusing capacity of carbon monoxide (DLco) and total lung capacity (TLC) in reconstruction algorithm C and with forced expiratory volume in 1 s (FEV1), DLco and TLC in reconstruction algorithm B. In conclusion, reconstruction algorithm has influence to quantitative assessments on chest thin-section MDCT examination in interstitial pneumonia patients.

Quantitatively assessed CT imaging measures of pulmonary interstitial pneumonia: Effects of reconstruction algorithms on histogram parameters

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Koyama, Hisanobu [Department of Radiology, Hyogo Kaibara Hospital, 5208-1 Kaibara, Kaibara-cho, Tanba 669-3395 (Japan)], E-mail: hisanobu19760104@yahoo.co.jp; Ohno, Yoshiharu [Department of Radiology, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017 (Japan)], E-mail: yosirad@kobe-u.ac.jp; Yamazaki, Youichi [Department of Medical Physics and Engineering, Faculty of Health Sciences, Graduate School of Medicine, Osaka University, 1-7 Yamadaoka, Suita 565-0871 (Japan)], E-mail: y.yamazk@sahs.med.osaka-u.ac.jp; Nogami, Munenobu [Division of PET, Institute of Biomedical Research and Innovation, 2-2 MInamimachi, Minatojima, Chu0-ku, Kobe 650-0047 (Japan)], E-mail: aznogami@fbri.org; Kusaka, Akiko [Division of Radiology, Kobe University Hospital, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017 (Japan)], E-mail: a.kusaka@hosp.kobe-u.ac.jp; Murase, Kenya [Department of Medical Physics and Engineering, Faculty of Health Sciences, Graduate School of Medicine, Osaka University, 1-7 Yamadaoka, Suita 565-0871 (Japan)], E-mail: murase@sahs.med.osaka-u.ac.jp; Sugimura, Kazuro [Department of Radiology, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017 (Japan)], E-mail: sugimura@med.kobe-u.ac.jp

2010-04-15

This study aimed the influences of reconstruction algorithm for quantitative assessments in interstitial pneumonia patients. A total of 25 collagen vascular disease patients (nine male patients and 16 female patients; mean age, 57.2 years; age range 32-77 years) underwent thin-section MDCT examinations, and MDCT data were reconstructed with three kinds of reconstruction algorithm (two high-frequencies [A and B] and one standard [C]). In reconstruction algorithm B, the effect of low- and middle-frequency space was suppressed compared with reconstruction algorithm A. As quantitative CT parameters, kurtosis, skewness, and mean lung density (MLD) were acquired from a frequency histogram of the whole lung parenchyma in each reconstruction algorithm. To determine the difference of quantitative CT parameters affected by reconstruction algorithms, these parameters were compared statistically. To determine the relationships with the disease severity, these parameters were correlated with PFTs. In the results, all the histogram parameters values had significant differences each other (p < 0.0001) and those of reconstruction algorithm C were the highest. All MLDs had fair or moderate correlation with all parameters of PFT (-0.64 < r < -0.45, p < 0.05). Though kurtosis and skewness in high-frequency reconstruction algorithm A had significant correlations with all parameters of PFT (-0.61 < r < -0.45, p < 0.05), there were significant correlations only with diffusing capacity of carbon monoxide (DLco) and total lung capacity (TLC) in reconstruction algorithm C and with forced expiratory volume in 1 s (FEV1), DLco and TLC in reconstruction algorithm B. In conclusion, reconstruction algorithm has influence to quantitative assessments on chest thin-section MDCT examination in interstitial pneumonia patients.

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

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Karina Portillo

2012-01-01

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

Lung radiology and pulmonary function of children chronically exposed to air pollution.

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Calderón-Garcidueñas, Lilian; Mora-Tiscareño, Antonieta; Fordham, Lynn A; Chung, Charles J; Valencia-Salazar, Gildardo; Flores-Gómez, Silvia; Solt, Anna C; Gomez-del Campo, Alberto; Jardón-Torres, Ricardo; Henríquez-Roldán, Carlos; Hazucha, Milan J; Reed, William

2006-09-01

We analyzed the chest radiographs (CXRs) of 249 clinically healthy children, 230 from southwest Mexico City and 19 from Tlaxcala. In contrast to children from Tlaxcala, children from southwest Mexico City were chronically exposed to ozone levels exceeding the U.S. National Ambient Air Quality Standards for an average of 4.7 hr/day and to concentrations of particulate matter (PM) with aerodynamic diameters interstitial markings were significantly more common in Mexico City children (p < 0.0002 and 0.00006 respectively) . Mexico City boys had a higher probability of developing interstitial markings with age (p = 0.004) . Computed tomography (CT) scans were obtained in 25 selected Mexico City children with abnormal CXRs. Mild bronchial wall thickening was seen in 10 of 25, prominent central airways in 4 of 25, air trapping in 8 of 21, and pulmonary nodules in 2 of 21. Only 7.8% of Mexico City children had abnormal lung function tests based on predicted values. These findings are consistent with bronchiolar, peribronchiolar, and/or alveolar duct inflammation, possibly caused by ozone, PM, and lipopolysaccharide exposure. The epidemiologic implications of these findings are important for children residing in polluted environments, because bronchiolar disease could lead to chronic pulmonary disease later in life.

New insights into canted spiro carbon interstitial in graphite

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EL-Barbary, A. A.

2017-12-01

The self-interstitial carbon is the key to radiation damage in graphite moderator nuclear reactor, so an understanding of its behavior is essential for plant safety and maximized reactor lifetime. The density functional theory is applied on four different graphite unit cells, starting from of 64 carbon atoms up to 256 carbon atoms, using AIMPRO code to obtain the energetic, athermal and mechanical properties of carbon interstitial in graphite. This study presents first principles calculations of the energy of formation that prove its high barrier to athermal diffusion (1.1 eV) and the consequent large critical shear stress (39 eV-50 eV) necessary to shear graphite planes in its presence. Also, for the first time, the gamma surface of graphite in two dimensions is calculated and found to yield the critical shear stress for perfect graphite. Finally, in contrast to the extensive literature describing the interstitial of carbon in graphite as spiro interstitial, in this work the ground state of interstitial carbon is found to be canted spiro interstitial.

Exchangeable pulmonary water space evaluation using giant liposomes

International Nuclear Information System (INIS)

Santos, A.C.; Ribeiro, M.J.; Ferreira, N.; De Lima, J.J.P.

1998-01-01

The present work aims to study the potential use of liposomes for the evaluation of pulmonary exchangeable water space, important parameter in some pulmonary oedema situations. This study is based upon the delivery of a diffusible water radiotracer into pulmonary capillary network, which equilibrates with interstitial water space of the lung and returns to the blood circulation. The time constant of this phenomena depends on the magnitude of the water space under study. The release of the diffusible radiotracer in lung capillaries is performed using liposomes with specific formulation. The giant liposomes (15-30μm diameter) used in this study are instable at 37 deg. C. They are biocompatible, biodegradable, with low toxicity and showed no immunogenicity. A water tracer labelled with 99m Tc, encapsulated in the aqueous phase of giant liposomes, has been used. Liposomes were prepared in sterile conditions and with apyrogenic materials. The lipid films composition is L-α-diestearoylphosphatidylcholine (DSPC), L-α-phosphatidyl-DL-glycerol (EPG) and cholesterol (CHOL) (60%/10%/30% mass ratio). After iv injection at +-20 deg. C in the femoral vein of Wistar rats (300g-600g) or albine rabbits (4.5-5Kg), the thermolabile liposomes will be entrapped in lung capillaries and release the radiotracer locally. When the radiodrug is diffusible we will evaluate the volume of the exchangeable pulmonary water analyzing the activity/time curves. These curves are slower for greater water spaces. When the radiotracer is non-diffusible, the disappearance curves are not influenced by the extravascular water space. (author)

Indoxyl Sulfate as a Mediator Involved in Dysregulation of Pulmonary Aquaporin-5 in Acute Lung Injury Caused by Acute Kidney Injury

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Nozomi Yabuuchi

2016-12-01

Full Text Available High mortality of acute kidney injury (AKI is associated with acute lung injury (ALI, which is a typical complication of AKI. Although it is suggested that dysregulation of lung salt and water channels following AKI plays a pivotal role in ALI, the mechanism of its dysregulation has not been elucidated. Here, we examined the involvement of a typical oxidative stress-inducing uremic toxin, indoxyl sulfate (IS, in the dysregulation of the pulmonary predominant water channel, aquaporin 5 (AQP-5, in bilateral nephrectomy (BNx-induced AKI model rats. BNx evoked AKI with the increases in serum creatinine (SCr, blood urea nitrogen (BUN and serum IS levels and exhibited thickening of interstitial tissue in the lung. Administration of AST-120, clinically-used oral spherical adsorptive carbon beads, resulted in a significant decrease in serum IS level and thickening of interstitial tissue, which was accompanied with the decreases in IS accumulation in various tissues, especially lung. Interestingly, a significant decrease in AQP-5 expression of lung was observed in BNx rats. Moreover, the BNx-induced decrease in pulmonary AQP-5 protein expression was markedly restored by oral administration of AST-120. These results suggest that BNx-induced AKI causes dysregulation of pulmonary AQP-5 expression, in which IS could play a toxico-physiological role as a mediator involved in renopulmonary crosstalk.

Erythroid-specific transcriptional changes in PBMCs from pulmonary hypertension patients.

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Chris Cheadle

Full Text Available Gene expression profiling of peripheral blood mononuclear cells (PBMCs is a powerful tool for the identification of surrogate markers involved in disease processes. The hypothesis tested in this study was that chronic exposure of PBMCs to a hypertensive environment in remodeled pulmonary vessels would be reflected by specific transcriptional changes in these cells.The transcript profiles of PBMCs from 30 idiopathic pulmonary arterial hypertension patients (IPAH, 19 patients with systemic sclerosis without pulmonary hypertension (SSc, 42 scleroderma-associated pulmonary arterial hypertensio patients (SSc-PAH, and 8 patients with SSc complicated by interstitial lung disease and pulmonary hypertension (SSc-PH-ILD were compared to the gene expression profiles of PBMCs from 41 healthy individuals. Multiple gene expression signatures were identified which could distinguish various disease groups from controls. One of these signatures, specific for erythrocyte maturation, is enriched specifically in patients with PH. This association was validated in multiple published datasets. The erythropoiesis signature was strongly correlated with hemodynamic measures of increasing disease severity in IPAH patients. No significant correlation of the same type was noted for SSc-PAH patients, this despite a clear signature enrichment within this group overall. These findings suggest an association of the erythropoiesis signature in PBMCs from patients with PH with a variable presentation among different subtypes of disease.In PH, the expansion of immature red blood cell precursors may constitute a response to the increasingly hypoxic conditions prevalent in this syndrome. A correlation of this erythrocyte signature with more severe hypertension cases may provide an important biomarker of disease progression.

PULMONARY AND LIVER DAMAGE DURING TREATMENT WITH ACETAMINOPHEN (PARACETAMOL

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L. I. Dvoretski

2016-01-01

Full Text Available This is a case report of pulmonary damage in the form of intestinal pneumonitis with severe respiratory failure during administration of acetaminophen (paracetamol. In addition, significant increase of ALT and AST levels without clinical signs of liver damage was observed in this patient. After glucocorticoids administration regression of radiological abnormal findings in the lungs along with normalization of liver enzymes values were registered. The rarity of interstitial pneumonitis induced by acetaminophen (paracetamol, especially in combination with liver damage, is emphasized. The presented patient history is the first case report of drug-induced hepatopulmonary syndrome during acetaminophen (paracetamol administration.

Muc1 deficiency exacerbates pulmonary fibrosis in a mouse model of silicosis.

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Kato, Kosuke; Zemskova, Marina A; Hanss, Alec D; Kim, Marianne M; Summer, Ross; Kim, Kwang Chul

2017-11-25

MUC1 (MUC in human and Muc in animals) is a membrane-tethered mucin expressed on the apical surface of lung epithelial cells. However, in the lungs of patients with interstitial lung disease, MUC1 is aberrantly expressed in hyperplastic alveolar type II epithelial (ATII) cells and alveolar macrophages (AM), and elevated levels of extracellular MUC1 are found in bronchoalveolar lavage (BAL) fluid and the serum of these patients. While pro-fibrotic effects of extracellular MUC1 have recently been described in cultured fibroblasts, the contribution of MUC1 to the pathobiology of pulmonary fibrosis is unknown. In this study, we hypothesized that MUC1 deficiency would reduce susceptibility to pulmonary fibrosis in a mouse model of silicosis. We employed human MUC1 transgenic mice, Muc1 deficient mice and wild-type mice on C57BL/6 background in these studies. Some mice received a one-time dose of crystalline silica instilled into their oropharynx in order to induce pulmonary fibrosis and assess the effects of Muc1 deficiency on fibrotic and inflammatory responses in the lung. As previously described in other mouse models of pulmonary fibrosis, we found that extracellular MUC1 levels were markedly increased in whole lung tissues, BALF and serum of human MUC1 transgenic mice after silica. We also detected an increase in total MUC1 levels in the lungs of these mice, indicating that production as well as release contributed to elevated levels after lung injury. Immunohistochemical staining revealed that increased MUC1 expression was mostly confined to ATII cells and AMs in areas of fibrotic remodeling, illustrating a pattern similar to the expression of MUC1 in human fibrotic lung tissues. However, contrary to our hypothesis, we found that Muc1 deficiency resulted in a worsening of fibrotic remodeling in the mouse lung as judged by an increase in number of silicotic nodules, an increase in lung collagen deposition and an increase in the severity of pulmonary inflammation

Pulmonary rehabilitation programs in lung transplant: a literature review

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Juliana Maria de Sousa Pinto

2015-09-01

Full Text Available Objective: To analyze, using a literature review, Pulmonary Rehabilitation (RP Programs in lung transplant. Methods: A literature review in July 2014 in Ebsco Host, Periódicos Capes, BVS and Science Direct data bases using descriptors in English (“lung transplantation”, “lung transplant” AND/OR “rehabilitation” and Portuguese (“reabilitação” AND/OR “transplante pulmonar”. The eligibility criterions were interventional studies of PR before and/or after lung transplant; participants who were candidates to lung transplant or lung transplant recipients; studies that applied any kind of PR program (hospital-based, homebased or outpatient and articles published in English, Spanish or Portuguese. Literature reviews, guidelines and case reports were excluded. The search process yielded 46 articles of which two were duplicated. After title and abstract screening 13 articles remained for full text reading. Six studies met the inclusion eligibility and were included in the review. Results: The studies involved patients with Chronic Obstructive Pulmonary Disease, Cystic Fibrosis, Pulmonary Hypertension, Interstitial Lung Disease and Pulmonary Fibrosis. Pulmonary function, exercise capacity, quality of life (QoL and quadriceps force were evaluated. Most interventions were outpatient programs with three months duration, three times a week and session with at least one hour. Protocols included physical training, educational approach and just one included nutritional, psychiatric and social assistant follow-up. The studies presented significant change in the six-minute walking distance, QoL and quadriceps force after PR programs. Conclusion: This review showed the benefits of the PR in the QoL and exercise capacity contributing to the Health Promotion of the patients.

Application of 2 mm thin-slice scanning with bone algorithm on conventional CT in diagnosis of the pulmonary diseases

International Nuclear Information System (INIS)

Zhang Xianheng; Li Xiuhua; Wang Fenghua

2004-01-01

Objective: To evaluate the value of 2 mm thin-slice conventional CT scan with bone algorithm in diagnosis and differential diagnosis in the pulmonary diseases. Methods: In total 135 cases of the pulmonary diseases were routinely scanned by conventional scan, 10 mm per slice, with standard algorithm, then the 2 mm thin-slice scan with bone algorithm was performed at the interested region of the lungs. Result: According to the comparative study of the CT signs between 10 mm slice scan with standard algorithm and 2 mm thin-slice scan with bone algorithm, the latter was better on displaying the pulmonary axial interstium, intralobular septum, subpleura lines, honeycombing, 2-5 mm nodulars and anomalies of bronchial wall. Conclusion: According to the study of 135 cases, 2 mm thin-slice scan with bone algorithm is superior to 10 mm slice scan with standard algorithm in demonstrating the pulmonary lesions. It has a similar value with high-resolution spiral CT in the diagnosis of the pulmonary solitary or diffuse nodules, pulmonary diffuse interstitial lesions and the lesions of the airway. It is practical and advisable in the community hospital

99mTc-DTPA Pulmonary Clearance in Normals

International Nuclear Information System (INIS)

Chung, Soo Kyo; Yang, Woo Jin; Sohn, Hyung Sun; Shinn, Kyung Sub; Bahk, Yong Whee

1994-01-01

Pulmonary clearance of 99m Tc-DTPA(PCD) has been used for the measurement of pulmonary epithelial permeability. It has been reported to be increased not only in variety of pulmonary diseases including ARDS, interstitial fibrosis, and smokers, but also in normal subjects on positive end expiratory pressure respirator, or after exercise. It was also noted that decrease of pulmonary blood flow due to pulmonary arterial obstruction results in delayed PCD. Normal range of PCD varies with institutes. We prospectively measured PCD in 17 normals (5 males and 12 females) consisted of staffs and trainees in the department of radiology of Kangnam St, Marys hospital using original Bark Nebulizer (India). Age ranged from 32 to 43 years. 370 MBq of 99m Tc-DTPA was inhaled in supine position and supine posterior images were subsequently obtained with 1 min/frame, 64 X 64 matrix and word mode for 30 min. Regions of interest. Were set on each lung, whole lungs, and upper, middle and lower thirds of right lung, respectively. Best fit regression curve was obtained by least square method from initial 7 min after peak activity on each curve and time for half clearance of maximum activity (tl/2) was calculated. Mean tl/2 was 51 ± 11.2 min for whole lung. There was no significant difference between tl/ 2 of right and left lungs. Initial uptake was higher in the lower third and tl/2 was shorter in the lower third than in the upper third(P<0.05). We reviewed several reports on PCD and compared our data with the others. In this study, faster clearance in the lower third may be due to the position imaged with or the environment the subjects belong to, and further investigation is under way.

Functional ability and fate of pulmonary alveolar macrophages after intratracheal instillation into rats

International Nuclear Information System (INIS)

Snipes, M.B.; Feddersen, D.; Mueller, H.L.; Guilmette, R.A.; Haley, P.J.

1988-01-01

Pulmonary alveolar macrophages (PAM) from donor rats were intratracheally instilled into recipient rats to determine if donor macrophages were functionally similar to the recipient's own macrophages. Recipient and donor (extrinsic) PAM were equivalent in their ability to phagocytize 1.7 μm and 3.9 μm latex microspheres in vivo and sensitized sheep red blood cells in vitro. Also, the extrinsic PAM appeared functionally equivalent to recipient PAM with respect to ability to translocate into interstitial tissue and migrate to the lung-associated lymph nodes (LALN). The recipient PAN appeared to phagocytize the extrinsic PAM, but the extrinsic PAM did not appear to phagocytize the recipient PAM. This could represent a different degree of physiological coordination of intrinsic and extrinsic PAM activities in the lung. Overall, results indicated that extrinsic PAM can live and function in the lungs of recipient rats, and perform most or all of the functions ascribed to recipient PAM. Results also support the hypothesis that PAM are able to move into the pulmonary interstitium and translocate to the LALM without the involvement of other pulmonary macrophages. (author)

The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema

International Nuclear Information System (INIS)

Fink, A. Michelle; Edis, Brian; Massie, John

2005-01-01

Congenital lobar emphysema (CLE) is a cause of severe neonatal respiratory distress. Overexpansion of the affected pulmonary lobe in the fetus is due to narrowing of the airway, with a resultant 'ball-valve' effect. At birth, there may be delayed clearance of fetal lung fluid. Early chest radiographs show opacification of the hyperexpanded lobe. The CT findings in the immediate neonatal period have not been previously reported. We describe the imaging in a neonate with tetralogy of Fallot and absent pulmonary valve with secondary CLE. CT demonstrates the hyperexpanded lobe with initial thickening of the interlobular septa and alveolar ground glass attenuation, with subsequent clearing. This resorption of fetal lung fluid via the pulmonary interstitium should not be confused with interstitial lung disease. (orig.)

The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema

Energy Technology Data Exchange (ETDEWEB)

Fink, A. Michelle [Royal Children' s Hospital, Department of Medical Imaging, Parkville, Victoria (Australia); University of Melbourne, Melbourne, Victoria (Australia); Edis, Brian [Royal Children' s Hospital, Department of Cardiology, Parkville, Victoria (Australia); Massie, John [University of Melbourne, Melbourne, Victoria (Australia); Royal Children' s Hospital, Department of Respiratory Medicine, Parkville, Victoria (Australia); Murdoch Children' s Research Institute, Melbourne, Victoria (Australia)

2005-09-01

Congenital lobar emphysema (CLE) is a cause of severe neonatal respiratory distress. Overexpansion of the affected pulmonary lobe in the fetus is due to narrowing of the airway, with a resultant 'ball-valve' effect. At birth, there may be delayed clearance of fetal lung fluid. Early chest radiographs show opacification of the hyperexpanded lobe. The CT findings in the immediate neonatal period have not been previously reported. We describe the imaging in a neonate with tetralogy of Fallot and absent pulmonary valve with secondary CLE. CT demonstrates the hyperexpanded lobe with initial thickening of the interlobular septa and alveolar ground glass attenuation, with subsequent clearing. This resorption of fetal lung fluid via the pulmonary interstitium should not be confused with interstitial lung disease. (orig.)

Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib

Directory of Open Access Journals (Sweden)

Bonella F

2015-12-01

Full Text Available Francesco Bonella,1 Susanne Stowasser,2 Lutz Wollin3 1Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, 2Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, 3Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany Abstract: Idiopathic pulmonary fibrosis (IPF is the most common type of idiopathic interstitial pneumonia and is characterized by a poor prognosis, with an estimated 5-year survival of approximately 20%. Progressive and irreversible lung functional impairment leads to chronic respiratory insufficiency with a severely impaired quality of life. In the last 2 decades, novel treatments for IPF have been developed as a consequence of an increasing understanding of disease pathogenesis and pathobiology. In IPF, injured dysfunctional alveolar epithelial cells promote fibroblast recruitment and proliferation, resulting in scarring of the lung tissue. Recently, pirfenidone and nintedanib have been approved for the treatment of IPF, having shown efficacy to slow functional decline and disease progression. This article focuses on the pharmacologic characteristics and clinical evidence supporting the use of nintedanib, a potent small-molecule tyrosine kinase inhibitor, as therapy for IPF. After introducing the mechanism of action and pharmacokinetics, an overview of the safety and efficacy results from the most recent clinical trials of nintedanib in IPF is presented. Keywords: tyrosine kinase, disease progression, treatment outcome, usual interstitial pneumonia, therapeutics

Tumor interstitial fluid

DEFF Research Database (Denmark)

Gromov, Pavel; Gromova, Irina; Olsen, Charlotta J.

2013-01-01

Tumor interstitial fluid (TIF) is a proximal fluid that, in addition to the set of blood soluble phase-borne proteins, holds a subset of aberrantly externalized components, mainly proteins, released by tumor cells and tumor microenvironment through various mechanisms, which include classical...

High-resolution CT of lymphoid interstitial pneumonia

International Nuclear Information System (INIS)

Vilgrain, V.; Frija, J.; Yana, C.; Couderc, L.J.; David, M.; Clauvel, J.P.; Laval-Jeantet, M.

1989-01-01

Three patients with lymphoid interstitial pneumonia (two HIV 1+ patients with chronic lymphadenopathic syndromes and one with a not-characterized autoimmune disease) have been studied with high-resolution computed tomography (HR-CT). This technique reveals septal lines, small reticulonodular opacities, polyhedral micronodular opacities, 'ground-glass' opacities and a dense, subpleural, curved broken line in one patient. The lesions dominate in the bases of the lungs. They are not characteristic for lymphoid interstitial pneumonia. If a patient presents with a chronic lymphadenopathic syndrome, the diagnosis of an opportunistic infection should not be automatically made, since the syndrome can be caused by lymphoid interstitial pneumonia [fr

Radiologic features of pyogenic pulmonary infection in AIDS patients and HIV-infected patients

International Nuclear Information System (INIS)

Wojtycha-Kwasnica, B.; Leszczynski, S.; Mian, M.; Mydlowska, A.

1994-01-01

400 HIV-infected patients were examined by chest radiographs and CT examinations. The radiological diagnosis of pyogenic bacterial pneumonia was confirmed by clinical examinations, laboratory tests and in cases by autopsy. Lobar consolidation often, bilateral, with abscesses and atelectasis, pleural effusions, diffuse interstitial infiltrates were noted mostly in the bacterial pneumonia group. In spite of immunodeficiency in these patients lobar consolidation and abscesses regressed after therapy. The relapse of pyogenic pulmonary infection, often in the same localization, which also regressed during treatment were observed. (author)

A comparison in cosmetic outcome between per-operative interstitial breast implants and delayed interstitial breast implants after external beam radiotherapy

NARCIS (Netherlands)

Pieters, Bradley R.; Hart, Augustinus A. M.; Russell, Nicola S.; Jansen, Edwin P. M.; Peterse, Johannes L.; Borger, Jacques; Rutgers, Emiel J. Th

2003-01-01

Background and purpose: Interstitial implants for brachytherapy boost in the breast conserving therapy of breast cancer can be performed in two ways; implants during the tumor excision (per-operative implants) or after the external beam therapy (delayed interstitial implants). Differences in

Lymphocytic interstitial pneumonia in children with AIDS: high-resolution CT findings

International Nuclear Information System (INIS)

Becciolini, V.; Gudinchet, F.; Schnyder, P.; Cheseaux, J.J.

2001-01-01

Pulmonary involvement in children with acquired immunodeficiency syndrome (AIDS) represents a wide spectrum of diseases. Among the non-infectious, non-neoplastic affections associated with AIDS, lymphocytic interstitial pneumonia (LIP) is now a well-recognized entity, but its radiological pattern studied with high-resolution computed tomography (HRCT) has rarely been described in children. The aim of this study was to illustrate the HRCT spectrum of pulmonary involvement in children with LIP and to evaluate its usefulness in the early diagnosis of this entity. Twelve children with AIDS, aged 3-9 years (mean age 5 years 7 months), underwent chest radiographs and HRCT. A control group of 7 healthy aged-matched children was also studied in the same conditions. Diagnosis of LIP was based on clinical data and HRCT findings. Eight children of 12 had a reticulonodular pattern on chest radiographs. Two children had normal chest films and two children showed peribronchiolar thickening. High-resolution CT displayed micronodules, 1-3 mm in diameter, with a perilymphatic distribution in all patients. High-resolution CT demonstrated also subpleural nodules in children without reticulonodular opacities on chest radiographs. High-resolution CT is able to define a more specific pattern of abnormalities than conventional chest radiographs in children with LIP, allows an earlier and more confident diagnosis and may be useful for the detection of other pathologies associated with AIDS, such as opportunistic infections or superimposed malignancies. (orig.)

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

LENUS (Irish Health Repository)

Haroon, Muhammad

2011-10-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

2005-07-01

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

Evolution of anisotropy in bcc Fe distorted by interstitial boron

Science.gov (United States)

Gölden, Dominik; Zhang, Hongbin; Radulov, Iliya; Dirba, Imants; Komissinskiy, Philipp; Hildebrandt, Erwin; Alff, Lambert

2018-01-01

The evolution of magnetic anisotropy in bcc Fe as a function of interstitial boron atoms was investigated in thin films grown by molecular beam epitaxy. The thermodynamic nonequilibrium conditions during film growth allowed one to stabilize an interstitial boron content of about 14 at .% accompanied by lattice tetragonalization. The c /a ratio scaled linearly with the boron content up to a maximum value of 1.05 at 300 °C substrate growth temperature, with a room-temperature magnetization of. In contrast to nitrogen interstitials, the magnetic easy axis remained in-plane with an anisotropy of approximately -5.1 ×106erg /cm3 . Density functional theory calculations using the measured lattice parameters confirm this value and show that boron local ordering indeed favors in-plane magnetization. Given the increased temperature stability of boron interstitials as compared to nitrogen interstitials, this study will help to find possible ways to manipulate boron interstitials into a more favorable local order.

Mucin 5B promoter polymorphism is associated with susceptibility to interstitial lung diseases in Chinese males.

Directory of Open Access Journals (Sweden)

Chunli Wang

Full Text Available The variation of G>T in the MUC5B promoter (rs35705950 has been associated with idiopathic pulmonary fibrosis (IPF and familial interstitial pneumonia (FIP in Caucasians, but no information is available regarding this variant in the Chinese population. We recruited 405 patients with interstitial lung diseases (ILD, including 165 IPF patients and 2043 healthy controls, for genotyping the MUC5B gene in the Chinese population. One hundred three patients with pneumonia and 360 patients with autoimmune diseases (ADs were recruited as disease controls. Our results indicated that the prevalence of the minor allele (T of the polymorphism rs35705950 in healthy Chinese subjects was approximately 0.66%, which was lower than that described in the Caucasian population. The frequencies of the T allele were 3.33% and 2.22% in IPF and ILD patients, respectively, and these values were significantly higher than those of healthy controls (P = 0.001, OR = 4.332 for IPF, and P = 0.002, OR = 2.855 for ILD. A stratified analysis showed that this variant in MUC5B associated with the risk for ILD mainly in older male Chinese subjects. No difference was observed between patients with pneumonia, AD patients, and healthy controls.

Leflunomide-Induced Interstitial Lung Disease: A Case Report

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Aygül Güzel

2015-04-01

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

Interstitial integrals in the multiple-scattering model

International Nuclear Information System (INIS)

Swanson, J.R.; Dill, D.

1982-01-01

We present an efficient method for the evaluation of integrals involving multiple-scattering wave functions over the interstitial region. Transformation of the multicenter interstitial wave functions to a single center representation followed by a geometric projection reduces the integrals to products of analytic angular integrals and numerical radial integrals. The projection function, which has the value 1 in the interstitial region and 0 elsewhere, has a closed-form partial-wave expansion. The method is tested by comparing its results with exact normalization and dipole integrals; the differences are 2% at worst and typically less than 1%. By providing an efficient means of calculating Coulomb integrals, the method allows treatment of electron correlations using a multiple scattering basis set

Carboxyhemoglobin formation secondary to nitric oxide therapy in the setting of interstitial lung disease and pulmonary hypertension.

Science.gov (United States)

Ruisi, Phillip; Ruisi, Michael

2011-01-01

Carbon monoxide (CO) has been widely recognized as an exogenous poison, although endogenous mechanisms for its formation involve heme-oxygenase (HO) isoforms, more specifically HO-1, in the setting of oxidative stress such as acute respiratory distress syndrome, sepsis, trauma, and nitric oxide use have been studied. In patients with refractory hypoxemia, inhaled nitric oxide (iNO) therapy is used to selectively vasodilate the pulmonary vasculature and improve ventilation-perfusion match. Inhaled nitric oxide is rapidly inactivated on binding to hemoglobin in the formation of nitrosyl- and methemoglobin in the pulmonary vasculature. Hence, inhaled nitric oxide has minimal systemic dissemination. Several experimental design studies involving lab rats have demonstrated increased levels of carboxyhemoglobin and exhaled CO as a result of nitric oxide HO-1 induction.

Time scales of transient enhanced diffusion: Free and clustered interstitials

Science.gov (United States)

Cowern, N. E. B.; Huizing, H. G. A.; Stolk, P. A.; Visser, C. C. G.; de Kruif, R. C. M.; Kyllesbech Larsen, K.; Privitera, V.; Nanver, L. K.; Crans, W.

1996-12-01

Transient enhanced diffusion (TED) and electrical activation after nonamorphizing Si implantations into lightly B-doped Si multilayers shows two distinct timescales, each related to a different class of interstitial defect. At 700°C, ultrafast TED occurs within the first 15 s with a B diffusivity enhancement of > 2 × 10 5. Immobile clustered B is present at low concentration levels after the ultrafast transient and persists for an extended period (˜ 10 2-10 3 s). The later phase of TED exhibits a near-constant diffusivity enhancement of ≈ 1 × 10 4, consistent with interstitial injection controlled by dissolving {113} interstitial clusters. The relative contributions of the ultrafast and regular TED regimes to the final diffusive broadening of the B profile depends on the proportion of interstitials that escape capture by {113} clusters growing within the implant damage region upon annealing. Our results explain the ultrafast TED recently observed after medium-dose B implantation. In that case there are enough B atoms to trap a large proportion of interstitials in SiB clusters, and the remaining interstitials contribute to TED without passing through an intermediate {113} defect stage. The data on the ultrafast TED pulse allows us to extract lower limits for the diffusivities of the Si interstitial ( DI > 2 × 10 -10 cm 2s -1) and the B interstitial(cy) defect ( DBi > 2 × 10 -13 cm 2s -1) at 700°C.

Analysis of Dissolved Organic Nutrients in the Interstitial Water of Natural Biofilms.

Science.gov (United States)

Tsuchiya, Yuki; Eda, Shima; Kiriyama, Chiho; Asada, Tomoya; Morisaki, Hisao

2016-07-01

In biofilms, the matrix of extracellular polymeric substances (EPSs) retains water in the interstitial region of the EPS. This interstitial water is the ambient environment for microorganisms in the biofilms. The nutrient condition in the interstitial water may affect microbial activity in the biofilms. In the present study, we measured the concentrations of dissolved organic nutrients, i.e., saccharides and proteins, contained in the interstitial water of biofilms formed on the stones. We also analyzed the molecular weight distribution, chemical species, and availability to bacteria of some saccharides in the interstitial water. Colorimetric assays showed that the concentrations of saccharides and proteins in the biofilm interstitial water were significantly higher (ca. 750 times) than those in the surrounding lake waters (p Chromatographic analyses demonstrated that the saccharides in the interstitial waters were mainly of low molecular-weight saccharides such as glucose and maltose, while proteins in the interstitial water were high molecular-weight proteins (over 7000 Da). Bacterial growth and production of EPS occurred simultaneously with the decrease in the low molecular-weight saccharide concentrations when a small portion of biofilm suspension was inoculated to the collected interstitial water, suggesting that the dissolved saccharides in the interstitial water support bacterial growth and formation of biofilms.

Interstitial Fluid Flow: The Mechanical Environment of Cells and Foundation of Meridians

Directory of Open Access Journals (Sweden)

Wei Yao

2012-01-01

Full Text Available Using information from the deep dissection, microobservation, and measurement of acupoints in the upper and lower limbs of the human body, we developed a three-dimensional porous medium model to simulate the flow field using FLUENT software and to study the shear stress on the surface of interstitial cells (mast cells caused by interstitial fluid flow. The numerical simulation results show the following: (i the parallel nature of capillaries will lead to directional interstitial fluid flow, which may explain the long interstitial tissue channels or meridians observed in some experiments; (ii when the distribution of capillaries is staggered, increases in the velocity alternate, and the velocity tends to be uniform, which is beneficial for substance exchange; (iii interstitial fluid flow induces a shear stress, with magnitude of several Pa, on interstitial cell membranes, which will activate cells and lead to a biological response; (iv capillary and interstitial parameters, such as capillary density, blood pressure, capillary permeability, interstitial pressure, and interstitial porosity, affect the shear stress on cell surfaces. The numerical simulation results suggest that in vivo interstitial fluid flow constitutes the mechanical environment of cells and plays a key role in guiding cell activities, which may explain the meridian phenomena and the acupuncture effects observed in experiments.

Four Cases of Interstitial Lung Disease Induced by Erlotinib 
and A Review of the Literatures

Directory of Open Access Journals (Sweden)

Xiaoling WU

2012-08-01

Full Text Available Erlotinib is an agent of oral epidermal growth factor receptor (EGFR tyrosine kinase inhibitors which are used for non-small cell lung cancer. Although this class of agents is considered to be relatively safe, the most serious, but rare, adverse reaction is drug-associated interstitial lung disease (ILD. ILD induced by gefitinib been often described, but the ILD induced by erlotinib is relatively less well known. We here describle four cases of ILD related to erlotinib and review recent literatures to help physicians earlier alert erlotinib-induced ILD. It is important to carefully monitor pulmonary symptoms in all patients who are receiving erlotinib. Early diagnosis and timely intervention is critical in the treatment of drug-induced ILD.

Di-interstitial defect in silicon revisited

International Nuclear Information System (INIS)

Londos, C. A.; Antonaras, G.; Chroneos, A.

2013-01-01

Infrared spectroscopy was used to study the defect spectrum of Cz-Si samples following fast neutron irradiation. We mainly focus on the band at 533 cm −1 , which disappears from the spectra at ∼170 °C, exhibiting similar thermal stability with the Si-P6 electron paramagnetic resonance (EPR) spectrum previously correlated with the di-interstitial defect. The suggested structural model of this defect comprises of two self-interstitial atoms located symmetrically around a lattice site Si atom. The band anneals out following a first-order kinetics with an activation energy of 0.88 ± 0.3 eV. This value does not deviate considerably from previously quoted experimental and theoretical values for the di-interstitial defect. The present results indicate that the 533 cm −1 IR band originates from the same structure as that of the Si-P6 EPR spectrum

Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis. Sociedad Española de Neumología y Cirugía Torácica (SEPAR) Research Group on Diffuse Pulmonary Diseases.

Science.gov (United States)

Xaubet, Antoni; Ancochea, Julio; Bollo, Elena; Fernández-Fabrellas, Estrella; Franquet, Tomás; Molina-Molina, Maria; Montero, Maria Angeles; Serrano-Mollar, Anna

2013-08-01

Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options. Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.

[Nonspecific interstitial pneumonitis: a clinicopathologic entity, histologic pattern or unclassified group of heterogeneous interstitial pneumonitis?].

Science.gov (United States)

Morais, António; Moura, M Conceição Souto; Cruz, M Rosa; Gomes, Isabel

2004-01-01

Nonspecific interstitial pneumonitis (NSIP) initially described by Katzenstein and Fiorelli in 1994, seems to be a distinct clinicopathologic entity among idiopathic interstitial pneumonitis (IIP). Besides different histologic features from other IIP, NSIP is characterized by a better long-term outcome, associated with a better steroids responsiveness than idiopathic pulmonar fibrosis (IPF), where usually were included. Thus, differentiating NSIP from other IIP, namely IPF is very significant, since it has important therapeutic and prognostic implications. NSIP encloses different pathologies, namely those with inflammatory predominance (cellular subtype) or fibrous predominance (fibrosing subtype). NSIP is reviewed and discussed by the authors, after two clinical cases description.

Combined Pulmonary Fibrosis and Emphysema in Scleroderma-Related Lung Disease Has a Major Confounding Effect on Lung Physiology and Screening for Pulmonary Hypertension.

Science.gov (United States)

Antoniou, K M; Margaritopoulos, G A; Goh, N S; Karagiannis, K; Desai, S R; Nicholson, A G; Siafakas, N M; Coghlan, J G; Denton, C P; Hansell, D M; Wells, A U

2016-04-01

To assess the prevalence of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) patients with interstitial lung disease (ILD) and the effect of CPFE on the pulmonary function tests used to evaluate the severity of SSc-related ILD and the likelihood of pulmonary hypertension (PH). High-resolution computed tomography (HRCT) scans were obtained in 333 patients with SSc-related ILD and were evaluated for the presence of emphysema and the extent of ILD. The effects of emphysema on the associations between pulmonary function variables and the extent of SSc-related ILD as visualized on HRCT and echocardiographic evidence of PH were quantified. Emphysema was present in 41 (12.3%) of the 333 patients with SSc-related ILD, in 26 (19.7%) of 132 smokers, and in 15 (7.5%) of 201 lifelong nonsmokers. When the extent of fibrosis was taken into account, emphysema was associated with significant additional differences from the expected values for diffusing capacity for carbon monoxide (DLco) (average reduction of 24.1%; P emphysema had a greater effect than echocardiographically determined PH on the FVC/DLco ratio, regardless of whether it was analyzed as a continuous variable or using a threshold value of 1.6 or 2.0. Among patients with SSc-related ILD, emphysema is sporadically present in nonsmokers and is associated with a low pack-year history in smokers. The confounding effect of CPFE on measures of gas exchange has major implications for the construction of screening algorithms for PH in patients with SSc-related ILD. © 2016, American College of Rheumatology.

Connective matrix organization in human pulmonary fibrosis. Collagen polymorphism analysis in fibrotic deposits by immunohistological methods.

Science.gov (United States)

Takiya, C; Peyrol, S; Cordier, J F; Grimaud, J A

1983-01-01

In the interstitium of the alveolar septa in the peripheral parts of the lung, four molecular types of collagen (I, III, IV and V) each with different morphological appearances, can be identified. The structural integrity of collagens accounts for the physiological efficiency of the lung. Fibrous thickening of alveolar septa is an invariable result of various diseases affecting the interstitium of the lung. The light and electron microscopic findings, and the immunological typing of collagens in six cases of fibrotic alveolar disease, are described. In the alveolar septa, two different compartments (the alveolo-capillary junction and the supportive axis) were affected by fibrosis: the alveolo-capillary junction was widened by the addition of interstitial collagens to basement membranes. In the axis, the increase of interstitial (types I and III) collagen gave rise to different patterns of connective matrix organization, graded as Loose or Dense depending on quantitative alterations of the type I/III ratio. The mode of organization of the fibrotic lung connective matrix, which depends on the quality of deposits in the matrix, may be correlated with the evolution of interstitial pulmonary fibrosis, in terms of its stability, remodelling ability and reversibility.

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

Directory of Open Access Journals (Sweden)

Olivia Meira Dias

2014-01-01

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

Genetic basis for childhood interstitial lung disease among Japanese infants and children.

Science.gov (United States)

Hayasaka, Itaru; Cho, Kazutoshi; Akimoto, Takuma; Ikeda, Masahiko; Uzuki, Yutaka; Yamada, Masafumi; Nakata, Koh; Furuta, Itsuko; Ariga, Tadashi; Minakami, Hisanori

2018-02-01

BackgroundGenetic variants responsible for childhood interstitial lung disease (chILD) have not been studied extensively in Japanese patients.MethodsThe study population consisted of 62 Japanese chILD patients. Twenty-one and four patients had pulmonary hypertension resistant to treatment (PH) and hypothyroidism, respectively. Analyses of genetic variants were performed in all 62 patients for SFTPC and ABCA3, in all 21 PH patients for FOXF1, and in a limited number of patients for NKX2.1.ResultsCausative genetic variants for chILD were identified in 11 (18%) patients: SFTPC variants in six, NKX2.1 variants in three, and FOXF1 variants in two patients. No patients had ABCA3 variants. All three and two patients with NKX2.1 variants had hypothyroidism and developmental delay, respectively. We found six novel variants in this study.ConclusionMutations in SFTPC, NKX2.1, and FOXF1 were identified among Japanese infants and children with chILD, whereas ABCA3 mutations were rare.

Burden, resilience and coping in caregivers of patients with interstitial lung disease.

Science.gov (United States)

Shah, R J; Collard, H R; Morisset, J

Prior work has described the experience of caregiving in idiopathic pulmonary fibrosis, but the effect on caregivers in interstitial lung disease (ILD) has not been explored. Describe the burden, resilience, and health related quality of life (HRQoL) of caregivers of people with ILD. In a mixed methods study, ILD caregivers completed questionnaires and participated in focus groups. A qualitative thematic analysis of the focus group transcripts was conducted. Thirty seven caregivers completed the survey, and 15 participated in the focus groups. 65% were female; the average age was 66 (SD = 13). The mean Short Form-36 role emotional and mental health scores were 18 (SD = 4) and 46 (SD = 7). The focus groups identified 4 major themes: emotional burden, changes in relationship, coping strategies, and unmet needs of caregivers. Caregiving for patients with ILD significantly impairs HRQoL, particularly, emotional health. Increasing resources could improve the caregiving experience in ILD. Copyright © 2018 Elsevier Inc. All rights reserved.

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

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

2016-05-15

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

Interstitial impurity interactions and dislocation microdynamics in Mo crystals

International Nuclear Information System (INIS)

Kwok, D.N.

1975-05-01

The effects of interstitial impurities on the mechanical properties of molybdenum are explored by comparing results obtained for crystals of various interstitial contents controlled by ultra-high vacuum outgassing. Results show a modulus reduction for as-grown samples and for outgassed specimens at low applied stresses. As a function of plastic microstrain, the values of modulus defect for both as-grown and outgassed specimens saturate at the same value. Interstitial impurities act as pinning agents to dislocation bowing, but when all the easy dislocation loops have broken away from local interstitial pins, the modulus defect reaches a constant saturation value. Etch pitting techniques were used to correlate microstrain observations with dislocation generation and motion. It has been found that edge dislocation generation and movement are active in the microstrain region while screw dislocations are relatively inactive until the macrostrain region is reached. Dislocation velocities range from 10 -6 to 10 -3 cm/s and the average distance between interstitial impurity pinning points is found to be approximately 8 x 10 -4 cm. (U.S.)

Effects of γ irradiation of hydra: elimination of interstitial cells from viable hydra

International Nuclear Information System (INIS)

Fradkin, M.; Kakis, H.; Campbell, R.D.

1978-01-01

Hydra attenuata and H. magnipapillata were γ-irradiated from a cesium source. All doses which had any observable effect (3000 rad and above) resulted in a reduction in the number of interstitial cells and of their differentiated product cells, or in the complete elimination of these cells. Interstitial cells were essentially completely eliminated within 5 days after irradiation doses above 5500 rad, and these hydra died. Irradiation doses of 4200 to 5500 rad resulted in a mixture of effects: some hydra recovered completely, some lost all interstitial cells and died, and some lost interstitial cells but could be propagated, as asexually reproducing clones, by hand feeding them. Hydra of some of these hand-fed clones entirely lacked interstitial cells and did not recover interstitial cells during subsequent culturing. Yet when these hydra were repopulated by interstitial cells from a normal hydra, they were restored to normal. Nerve cells became depleted more slowly than interstitial cells following irradiation, so animals can be obtained which possess nerve but no stem (interstitial) cells. The nerve cells and other derivatives of interstitial cells eventually disappear upon prolonged culture of the hydra. Thus γ irradiation can be used to eliminate interstitial cells from hydra, leaving viable polyps composed only of epithelial cells

Selective Uterine Artery Embolization for Management of Interstitial Ectopic Pregnancy

International Nuclear Information System (INIS)

Yang, Seung Boo; Lee, Sang Jin; Joe, Hwan Sung; Goo, Dong Erk; Chang, Yun Woo; Kim, Dong Hun

2007-01-01

Interstitial pregnancy is defined as any gestation that develops in the uterine portion of the fallopian tubes lateral to the round ligament. Interstitial pregnancies account for 2-4% of all ectopic pregnancies and have been reported to have an associated 2% to 2.5% maternal mortality rate. The traditional treatment for interstitial pregnancy using surgical cornual resection may cause infertility or uterine rupture in subsequent pregnancies. Recently, the early identification of intact interstitial pregnancy has been made possible in many cases with high resolution transvaginal ultrasound as well as more sensitive assays for betahuman chorionic gonadotropin (β-hCG). The treatment includes: hysteroscopic transcervical currettage, local and systemic methotrexate (MTX) therapy and prostaglandin or potassium chloride injection of the ectopic mass under sonographic guidance. We describe a case of successful treatment of interstitial pregnancy using uterine artery embolization, after failure of methotrexate treatment

Selective Uterine Artery Embolization for Management of Interstitial Ectopic Pregnancy

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Yang, Seung Boo; Lee, Sang Jin; Joe, Hwan Sung; Goo, Dong Erk; Chang, Yun Woo [Soonchunhyang University Gumi Hospital, Gumi (Korea, Republic of); Kim, Dong Hun [Chosun University Hospital, Gwangju (Korea, Republic of)

2007-04-15

Interstitial pregnancy is defined as any gestation that develops in the uterine portion of the fallopian tubes lateral to the round ligament. Interstitial pregnancies account for 2-4% of all ectopic pregnancies and have been reported to have an associated 2% to 2.5% maternal mortality rate. The traditional treatment for interstitial pregnancy using surgical cornual resection may cause infertility or uterine rupture in subsequent pregnancies. Recently, the early identification of intact interstitial pregnancy has been made possible in many cases with high resolution transvaginal ultrasound as well as more sensitive assays for betahuman chorionic gonadotropin ({beta}-hCG). The treatment includes: hysteroscopic transcervical currettage, local and systemic methotrexate (MTX) therapy and prostaglandin or potassium chloride injection of the ectopic mass under sonographic guidance. We describe a case of successful treatment of interstitial pregnancy using uterine artery embolization, after failure of methotrexate treatment.

Anomalies of Pulmonary Circulation as a Cause of Hemoptysis: A Series of Unusual Cases and Review of the Literature

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Kamini Gupta

2015-05-01

Full Text Available The expectoration of blood originating from the lower respiratory tract, called hemoptysis, is a common clinical condition with many potential etiologies. Massive hemoptysis is life threatening and needs urgent intervention. Multidetector computed tomography (MDCT is a useful non-invasive imaging modality for the initial assessment of hemoptysis. Using MDCT with multiplanar reformatted images has improved the diagnosis and management of hemoptysis by providing a more precise depiction of bronchial and non-bronchial systemic arteries than conventional computed tomography (CT. In 95% of hemoptysis cases, the systemic arterial system is the origin of bleeding and pulmonary vascular anomalies are a rare cause. Among these, pulmonary arteriovenous malformation, hereditary hemorrhagic telangiectasia, and Osler-Weber-Rendu disease are well known entities. However, primary anomalies affecting pulmonary vessels in the mediastinum or diseases secondarily affecting the pulmonary vessels are unusual causes. Here we present three cases where patients had pulmonary vascular anomalies causing hemoptysis. These patients had decreased pulmonary arterial pressures leading to bronchial and systemic arterial hypertrophy and development of bronchopulmonary collaterals. Secondary CT signs in the parenchyma and mediastinum (mosaic attenuation, ground glass haze, subpleural interstitial thickening, and hypertrophied bronchial arteries were similar in all patients. Hence, evaluation of the MDCT images for primary abnormality led to the diagnosis.

Evaluation of imaging of the ventilatory lung motion in pulmonary diseases

International Nuclear Information System (INIS)

Fujii, Tadashige; Kanai, Hisakata; Tanaka, Masao; Hirayama, Jiro; Handa, Kenjiro

1988-01-01

Using perfusion lung scintigram with 99m Tc-macroaggregated albumin at maximal expiration (E) and inspiration (I), images of the motion of the regional pulmonary areas and lung margins during ventilation ((E-I)/I) was obtained in patients with various respiratory diseases. The image of (E-I)/I consisted of positive and negative components. The former component visualized the motion of the regional pulmonary areas that corresponded with the ventilatory amplitude of the videodensigram. The sum of the positive component of (E-I)/I in both lungs correlated with the vital capacity (n = 50, r = 0.62). It was 163.5 ± 52.5 in cases with a vital capacity of more than 3.01, 94.1 ± 61.5 in primary lung cancer, 89.2 ± 44.7 in chronic obstructive lung diseases and 69.0 ± 27.5 in diffuse interstitial pneumonia. The distribution pattern of pulmonary perfusion and the positive component of (E-I)/I matched fairly in many cases, but did not match in some cases. The negative component of (E-I)/I demonstrated the ventilatory motion of the lung margin and its decreased activity was shown in cases with hypoventilation of various causes including pleural diseases. The sum of the negative component of (E-I)/I in the both lungs correlated with the vital capacity (n = 50, r = 0.44). These results suggest that this technique is useful to estimate the regional pulmonary ventilatioin and motion of the lung margins. (author)

Dual-time-point 18F-FDG PET imaging for diagnosis of disease type and disease activity in patients with idiopathic interstitial pneumonia

International Nuclear Information System (INIS)

Umeda, Yukihiro; Demura, Yoshiki; Ishizaki, Takeshi; Ameshima, Shingo; Miyamori, Isamu; Saito, Yuji; Tsuchida, Tatsuro; Fujibayashi, Yasuhisa; Okazawa, Hidehiko

2009-01-01

Individual clinical courses of idiopathic interstitial pneumonia (IIP) are variable and difficult to predict because the pathology and disease activity are contingent, and chest computed tomography (CT) provides little information about disease activity. In this study, we applied dual-time-point [ 18 F]-fluoro-2-deoxy-D-glucose ( 18 F-FDG) positron emission tomography (PET), commonly used for diagnosis of malignant tumours, to the differential diagnosis and prediction of disease progression in IIP patients. Fifty patients with IIP, including idiopathic pulmonary fibrosis (IPF, n = 21), non-specific interstitial pneumonia (NSIP, n = 18) and cryptogenic organizing pneumonia (COP, n = 11), underwent 18 F-FDG PET examinations at two time points: scan 1 at 60 min (early imaging) and scan 2 at 180 min (delayed imaging) after 18 F-FDG injection. The standardized uptake values (SUV) at the two points and the retention index (RI-SUV) calculated from them were evaluated and compared with chest CT findings, disease progression and disease types. To evaluate short-term disease progression, all patients were examined by pulmonary function test every 3 months for 1 year after 18 F-FDG PET scanning. The early SUV for COP (2.47 ± 0.74) was significantly higher than that for IPF (0.99 ± 0.29, p = 0.0002) or NSIP (1.22 ± 0.44, p= 0.0025). When an early SUV cut-off value of 1.5 and greater was used to distinguish COP from IPF and NSIP, the sensitivity, specificity and accuracy were 90.9, 94.3 and 93.5%, respectively. The RI-SUV for IPF and NSIP lesions was significantly greater in patients with deteriorated pulmonary function after 1 year of follow-up (progressive group, 13.0 ± 8.9%) than in cases without deterioration during the 1-year observation period (stable group, -16.8 ± 5.9%, p 18 F-FDG PET are useful parameters for the differential diagnosis and prediction of disease progression in patients with IIP. (orig.)

The diagnosis efficacy and safety of video-assisted thoracoscopy surgery (VATS) in undefined interstitial lung diseases: a retrospective study.

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Luo, Qun; Han, Qian; Chen, Xiaobo; Xie, Jiaxing; Wu, Lulu; Chen, Rongchang

2013-06-01

To evaluate the efficacy and safety of lung biopsies by video-assisted thoracoscopy surgery (VATS) in the diagnosis of undefined interstitial lung disease (ILD). The retrospective analysis was performed in 32 who patients underwent VATS for the diagnosed with ILD from Jan 2007 to Dec 2011. The main reason for VATS for all the patients was due to no specific diagnosis could be obtained after non-invasive methods, transbronchial lung biopsy (TBLB) examination and the consultation with pulmonologist, radiologist and pathologist. The clinical profiles, chest high resolution computerized tomography (HRCT), laboratory profile, TBLB as well as the diagnosis of before and after the VATS were analyzed. The surgery site, biopsy number, duration of the thoracic drain, post-operative complications were also recorded. The 30- and 90-day post-operative mortality rates were calculated. The risk factors associated with the incidence of post-operative complications were assessed. The specific diagnosis could be established in all patients after VATS lung biopsies, with change from previous ones in 27 (84.4%). Among 20 cases (62.5%) diagnosed as unclassified ILD before the surgery, 14 (70.0%) were diagnosed as nonspecific interstitial pneumonia (NSIP), 3 (15.0%) as idiopathic pulmonary fibrosis (IPF) and 3 (15.0%) as connective tissue disease-related ILD (CTD-ILD). Among the 7 cases with complete change of diagnosis after VATS, 4 (57.1%) were cryptogenic organizing pneumonia (COP). The number of site of biopsy had no significant impact on the diagnostic efficacy. There were no significant change of vital sign and lung function after the VATS. 21 (65.6%) patients had post-operative complications, including pulmonary infection (56.3%), pulmonary atelectasis (28.1%) and pneumothorax (25.0%). The 30- and 90-day mortality rates were 0 and 5.2% respectively. Patients were divided into 2 groups based on the incidence of post-operative complications, and no significant difference was found

Pulmonary Vascular Congestion: A Mechanism for Distal Lung Unit Dysfunction in Obesity.

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Oppenheimer, Beno W; Berger, Kenneth I; Ali, Saleem; Segal, Leopoldo N; Donnino, Robert; Katz, Stuart; Parikh, Manish; Goldring, Roberta M

2016-01-01

Obesity is characterized by increased systemic and pulmonary blood volumes (pulmonary vascular congestion). Concomitant abnormal alveolar membrane diffusion suggests subclinical interstitial edema. In this setting, functional abnormalities should encompass the entire distal lung including the airways. We hypothesize that in obesity: 1) pulmonary vascular congestion will affect the distal lung unit with concordant alveolar membrane and distal airway abnormalities; and 2) the degree of pulmonary congestion and membrane dysfunction will relate to the cardiac response. 54 non-smoking obese subjects underwent spirometry, impulse oscillometry (IOS), diffusion capacity (DLCO) with partition into membrane diffusion (DM) and capillary blood volume (VC), and cardiac MRI (n = 24). Alveolar-capillary membrane efficiency was assessed by calculation of DM/VC. Mean age was 45±12 years; mean BMI was 44.8±7 kg/m2. Vital capacity was 88±13% predicted with reduction in functional residual capacity (58±12% predicted). Despite normal DLCO (98±18% predicted), VC was elevated (135±31% predicted) while DM averaged 94±22% predicted. DM/VC varied from 0.4 to 1.4 with high values reflecting recruitment of alveolar membrane and low values indicating alveolar membrane dysfunction. The most abnormal IOS (R5 and X5) occurred in subjects with lowest DM/VC (r2 = 0.31, ppulmonary vascular congestion and failure to achieve the high output state of obesity. Pulmonary vascular congestion and consequent fluid transudation and/or alterations in the structure of the alveolar capillary membrane may be considered often unrecognized causes of airway dysfunction in obesity.

Evidence of interstitial microsegregation in iron obtained by ion microscopy

International Nuclear Information System (INIS)

Price, C.W.

1984-01-01

Segregation of impurity atoms to the strain fields of dislocations and the effective locking of the dislocations by the impurity atmospheres have been suggested earlier by others. The formation of interstitial atmospheres and their effect in iron was first treated mathematically by Cottrell and Bilby (Proc. Phys. Soc.; A62: 49(1949). Hirth and Lothe (Theory of Discolations, McGraw-Hill, New York (1968) reviewed more recent evidence of interstitial effects and theoretical treatments of interstitial dislocation interactions. This paper describes additional evidence of microsegregation of several interstitial elements in iron that were detected using secondary-ion mass spectroscopy (SIMS). 10 references, 2 figures

Ultrastructural characterization of the pulmonary cellular defences in the lung of a bird, the rock dove, Columba livia

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Maina, J. N.; Cowley, H. M.

1998-01-01

Free (surface) avian respiratory macrophages (FARMs) were harvested by lavage of the lung/air-sac system of the rock dove, Columba livia. The presence of FARMs in the atria and infundibula was confirmed by scanning electron microscopy. The respiratory system has developed several cellular defence lines that include surface macrophages, epithelial, subepithelial and interstitial phagocytes, and pulmonary intravascular macrophages (PIMs). Hence, C. livia appears to have a multiple pulmonary cellular protective armoury. Ultrastructurally, the FARMs and the PIMs were similar to the corresponding cells of mammals. The purported high susceptibility of birds to respiratory diseases, a state that has largely been deduced from morbidities and mortalities of commercial birds, and which has chiefly been attributed to paucity of the FARMs, is not supported by the present observations.

Pulmonary MR imaging with ultra-short TEs: Utility for disease severity assessment of connective tissue disease patients

International Nuclear Information System (INIS)

Ohno, Yoshiharu; Nishio, Mizuho; Koyama, Hisanobu; Takenaka, Daisuke; Takahashi, Masaya; Yoshikawa, Takeshi; Matsumoto, Sumiaki; Obara, Makoto; Cauteren, Marc van; Sugimura, Kazuro

2013-01-01

Purpose: To evaluate the utility of pulmonary magnetic resonance (MR) imaging with ultra-short echo times (UTEs) at a 3.0 T MR system for pulmonary functional loss and disease severity assessments of connective tissue disease (CTD) patients with interstitial lung disease (ILD). Materials and methods: This prospective study was approved by the institutional review board, and written informed consent was obtained from 18 CTD patients (eight men and ten women) and eight normal subjects with suspected chest disease (three men and five women). All subjects underwent thin-section MDCT, pulmonary MR imaging with UTEs, pulmonary function test and serum KL-6. Regional T2* maps were generated from each MR data set, and mean T2* values were determined from ROI measurements. From each thin-section MDCT data set, CT-based disease severity was evaluated with a visual scoring system. Mean T2* values for normal and CTD subjects were statistically compared by using Student's t-test. To assess capability for pulmonary functional loss and disease severity assessments, mean T2* values were statistically correlated with pulmonary functional parameters, serum KL-6 and CT-based disease severity. Results: Mean T2* values for normal and CTD subjects were significantly different (p = 0.0019) and showed significant correlations with %VC, %DL CO , serum KL-6 and CT-based disease severity of CTD patients (p < 0.05). Conclusion: Pulmonary MR imaging with UTEs is useful for pulmonary functional loss and disease severity assessments of CTD patients with ILD

Pulmonary MR imaging with ultra-short TEs: Utility for disease severity assessment of connective tissue disease patients

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Ohno, Yoshiharu, E-mail: yosirad@kobe-u.ac.jp [Advanced Biomedical Imaging Research Center, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Division of Functional and Diagnostic Imaging Research, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Nishio, Mizuho [Division of Functional and Diagnostic Imaging Research, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Koyama, Hisanobu [Division of Radiology, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Takenaka, Daisuke [Division of Radiology, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Department of Radiology, Hyogo Cancer Center, Akashi, Hyogo (Japan); Takahashi, Masaya [Advanced Imaging Research Center, Department of Radiology, University of Texas Southwestern Medical Center, Houston, TX (United States); Yoshikawa, Takeshi; Matsumoto, Sumiaki [Advanced Biomedical Imaging Research Center, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Division of Functional and Diagnostic Imaging Research, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Obara, Makoto; Cauteren, Marc van [Philips Electronics Japan, Tokyo (Japan); Sugimura, Kazuro [Division of Radiology, Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan)

2013-08-15

Purpose: To evaluate the utility of pulmonary magnetic resonance (MR) imaging with ultra-short echo times (UTEs) at a 3.0 T MR system for pulmonary functional loss and disease severity assessments of connective tissue disease (CTD) patients with interstitial lung disease (ILD). Materials and methods: This prospective study was approved by the institutional review board, and written informed consent was obtained from 18 CTD patients (eight men and ten women) and eight normal subjects with suspected chest disease (three men and five women). All subjects underwent thin-section MDCT, pulmonary MR imaging with UTEs, pulmonary function test and serum KL-6. Regional T2* maps were generated from each MR data set, and mean T2* values were determined from ROI measurements. From each thin-section MDCT data set, CT-based disease severity was evaluated with a visual scoring system. Mean T2* values for normal and CTD subjects were statistically compared by using Student's t-test. To assess capability for pulmonary functional loss and disease severity assessments, mean T2* values were statistically correlated with pulmonary functional parameters, serum KL-6 and CT-based disease severity. Results: Mean T2* values for normal and CTD subjects were significantly different (p = 0.0019) and showed significant correlations with %VC, %DL{sub CO}, serum KL-6 and CT-based disease severity of CTD patients (p < 0.05). Conclusion: Pulmonary MR imaging with UTEs is useful for pulmonary functional loss and disease severity assessments of CTD patients with ILD.

Determination of the population of octahedral and tetrahedral interstitials in zirconium hydrides

International Nuclear Information System (INIS)

Fedorov, V.M.; Gogava, V.V.; Shilo, S.I.; Biryukova, E.A.

1983-01-01

Results of neutron investigations of ZrHsub(1.66), ZrHsub(1.75) and ZrHsub(1.98) zirconium hydrides are presented. Investigations were conducted using plane polycrystal samples by multidetector system of scattered neutron detection. Neutron diffraction method was used to determine the number of interstitial hydrogen atoms in interstitials of the lattice cell in the case of statistic atom distribution. The numbers of interstitial atoms in octahedral interstitials for zirconium hydrides were determined experimentally; the difference of potential energies of hydrogen atoms in octa- and tetrahedral interstitials was determined as well. It is shown that experimentally determined difference of potential energies of hydrogen atoms, occupying octa- and tetrahedral positions in investigated zirconium hydrides results at room temperature in the pretailing occupation of tetrahedral interstitials by hydrogen atoms (85-90%); the occupation number grows with temperature decrease and the ordering of interstitial vacancies with formation of hydrogen superstructure takes place at low temperatures

Positional shifting of HRCT findings in patients with pulmonary edema

International Nuclear Information System (INIS)

Kim, Young Sun; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Seo, Heung Suk; Lee, Seung Rho; Hahm, Chang Kok

2001-01-01

To assess the value of positional shifting to a gravity-dependent area, as revealed by HRCT, in differentiating pulmonary edema (PE) from other conditions. Sixteen consecutive patients in whom plain radiographs suggested the presence of pulmonary edema but the clinical findings were indefinite underwent HRCT of the lung. For initial scanning they were in the supine position, and then in the prone position. Findings of ground-glass opacity, interlobular septal thickening and peribronchovascular interistitial thickening were analyzed in terms of the presence and degree of shifting to a gravity-dependent area, a grade of high, intermediate or low being assigned. PE was diagnosed in 8 of 16 cases, the remainder being designated as non-pulmonary edema (NPE). Ground-glass opacity was observed in all 16, while the degree of positional shifting was found to be high in ten (PE:NPE=6:4), intermediate in four (PE:NPE=2:2), and low in two (PE:NPE=0:2). There was no significant difference between the two groups (ρ > 0.05). Interlobular septal thickening was observed in all but two NPE cases; the degree of shifting was high in six (PE:NPE=6:0), intermediate in one (PE), and low in seven (PE:NPE=1:6). Shifting was significantly more prominent in PE than in NPE case (ρ <0.05). Peribronchovascular interstitial thickening was positive in all PE cases and one NPE case, with no positional shifting. Positional shifting of interlobular septal thickening to a gravity-dependent area, as demonstrated by HRCT, is the most specific indicator of pulmonary edema

Evaluation of pulmonary function in beta-thalassemia major patients

International Nuclear Information System (INIS)

Eidani, I.; Keikhaei, B.; Rahim, F.; Bagheri, A.

2010-01-01

Objective: To describe and quantify the functional change of the lung in patients with beta-thalassemia major (TM) and determine the correlation between pulmonary function test (PFT) results with hemoglobin, ferritin and age changes. Methodology: Pulmonary function tests were performed on 60 transfusion-dependent patients with TM, ranging in age from 10 to 45 years. Percent-predicted values for forced expiratory volume in one second (FEV1), and forced expiratory flows (FEF) 25-75% were significantly reduced, whereas forced expiratory vital capacity (FVC) and FEV1/FVC were closed to normal limits, indicating a restrictive disease. All factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were negatively correlated with age and ferritin levels. In contrast, all factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were positively correlated with hemoglobin (Hb). We performed linear regression analysis to study the simultaneous influence of the presence of age, ferritin, and Hb on obstructive PFT indexes. Results: Pulmonary function test results were normal in only 32 (53.3%) of 60 patients and the rest 28 cases (46.7%) showed abnormal pulmonary function. FEV1 and FEF 25% - 75% have significant negative correlation with age (r = - 0.64 p(r) = 0.003 and r = - 0.58 p(r) = 0.02 respectively), also have significant positive correlation with Hb (r = 0.31 p(r) = 0.015 and r = 0.33 p(r) = 0.01 respectively), and only FEF 25% - 75% has significant negative correlation with ferritin (r -0.26 p(r) = 0.04). Conclusion: The present study has shown that restrictive disease and reduced lung diffusing capacity are the predominant abnormalities of pulmonary function patients with TM. The low hemoglobin concentration and a fall in the diffusing capacity of the alveola - capillary membrane, together with the dependence of the reduced pulmonary diffusing capacity on age and serum ferritin levels, as well as of the entity of restrictive disease on age, suggests that pulmonary

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

Directory of Open Access Journals (Sweden)

Olga Aleksandrova Koneva

2010-01-01

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

[New toxicity of fotemustine: diffuse interstitial lung disease].

Science.gov (United States)

Bertrand, M; Wémeau-Stervinou, L; Gauthier, S; Auffret, M; Mortier, L

2012-04-01

Fotemustine is an alkylating cytostatic drug belonging to the nitrosourea family and is used in particular in the treatment of disseminated malignant melanoma. Herein, we report a case of interstitial lung disease associated with fotemustine. An 81-year-old man treated with fotemustine for metastatic melanoma presented acute interstitial lung disease 20 days after a fourth course of fotemustine monotherapy. The condition regressed spontaneously, with the patient returning to the clinical, radiological and blood gas status that had preceded fotemustine treatment. After other potential aetiologies had been ruled out, acute fotemustine-induced lung toxicity was considered and this treatment was definitively withdrawn. Other cytostatic agents belonging to the nitrosourea family can cause similar pictures, with a number of cases of interstitial lung disease thus being ascribed to fotemustine and dacarbazine. To our knowledge, this is the first case of interstitial lung disease induced by fotemustine monotherapy. This diagnosis should be considered where respiratory signs appear in melanoma patients undergoing fotemustine treatment. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Spectrum of interstitial lung diseases at a tertiary center in a developing country: A study of 803 subjects.