Interstitial lung disease

About: Interstitial lung disease is a research topic. Over the lifetime, 8951 publications have been published within this topic receiving 208937 citations. The topic is also known as: ILD & interstitial pneumonitis.

Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.

Abstract: Background: This document provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). It represents a collaborative effort between the American Thoracic Society, Eur...

Cyclophosphamide versus placebo in scleroderma lung disease.

Abstract: BACKGROUND We conducted a double-blind, randomized, placebo-controlled trial to determine the effects of oral cyclophosphamide on lung function and health-related symptoms in patients with evidence of active alveolitis and scleroderma-related interstitial lung disease. METHODS At 13 clinical centers throughout the United States, we enrolled 158 patients with scleroderma, restrictive lung physiology, dyspnea, and evidence of inflammatory interstitial lung disease on examination of bronchoalveolar-lavage fluid, thoracic highresolution computed tomography, or both. Patients received oral cyclophosphamide (≤2 mg per kilogram of body weight per day) or matching placebo for one year and were followed for an additional year. Pulmonary function was assessed every three months during the first year, and the primary end point was the forced vital capacity (FVC, expressed as a percentage of the predicted value) at 12 months, after adjustment for the baseline FVC. RESULTS Of 158 patients, 145 completed at least six months of treatment and were included in the analysis. The mean absolute difference in adjusted 12-month FVC percent predicted between the cyclophosphamide and placebo groups was 2.53 percent (95 percent confidence interval, 0.28 to 4.79 percent), favoring cyclophosphamide (P<0.03). There were also treatment-related differences in physiological and symptom outcomes, and the difference in FVC was maintained at 24 months. There was a greater frequency of adverse events in the cyclophosphamide group, but the difference between the two groups in the number of serious adverse events was not significant. CONCLUSIONS One year of oral cyclophosphamide in patients with symptomatic sclerodermarelated interstitial lung disease had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and the health-related quality of life. The effects on lung function were maintained through the 24 months of the study.

Changes in causes of death in systemic sclerosis, 1972-2002.

Abstract: Background: Survival of scleroderma has changed since the renal crisis treatment has become possible. Aims: To document the changes in survival and organ system causes of mortality in systemic sclerosis (SSc) over the past 25 years in patients from a single medical centre. Methods: Consecutive patients evaluated at the University of Pittsburgh, Pittsburgh, Pennsylvania, USA between 1 January 1972 and 31 December 1996 were studied. Survival was determined in five 5-year time periods between 1972 and 1997. Causes of death included scleroderma-related (scleroderma renal crisis, pulmonary arterial hypertension, pulmonary fibrosis (PF), gastrointestinal (GI), heart and multiorgan failure) and non-scleroderma-related (cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown) causes. Results: The 10-year survival improved steadily from 54% to 66% during each of the time intervals. There was a significant improvement in survival for patients during 1982–91 compared with those during 1972–81 (p Conclusion: The change in the pattern of scleroderma-related deaths over the past 30 years demonstrates that the lung (both pulmonary hypertension and PF) is the primary cause of scleroderma-related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of SSc.

Clinical course and prediction of survival in idiopathic pulmonary fibrosis.

Abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some patients live much longer. Respiratory failure resulting from disease progression is the most frequent cause of death. To date we have limited information as to predictors of mortality in patients with IPF, and research in this area has failed to yield prediction models that can be reliably used in clinical practice to predict individual risk of mortality. The goal of this concise clinical review is to examine and summarize the current data on the clinical course, individual predictors of survival, and proposed clinical prediction models in IPF. Finally, we will discuss challenges and future directions related to predicting survival in IPF.

Management of spontaneous pneumothorax: British Thoracic Society pleural disease guideline 2010

Abstract: The term ‘pneumothorax’ was first coined by Itard and then Laennec in 1803 and 1819 respectively,1 and refers to air in the pleural cavity (ie, interspersed between the lung and the chest wall). At that time, most cases of pneumothorax were secondary to tuberculosis, although some were recognised as occurring in otherwise healthy patients (‘pneumothorax simple’). This classification has endured subsequently, with the first modern description of pneumothorax occurring in healthy people (primary spontaneous pneumothorax, PSP) being that of Kjaergaard2 in 1932. It is a significant global health problem, with a reported incidence of 18–28/100 000 cases per annum for men and 1.2–6/100 000 for women.3 Secondary pneumothorax (SSP) is associated with underlying lung disease, in distinction to PSP, although tuberculosis is no longer the commonest underlying lung disease in the developed world. The consequences of a pneumothorax in patients with pre-existing lung disease are significantly greater, and the management is potentially more difficult. Combined hospital admission rates for PSP and SSP in the UK have been reported as 16.7/100 000 for men and 5.8/100 000 for women, with corresponding mortality rates of 1.26/million and 0.62/million per annum between 1991 and 1995.4 With regard to the aetiology of pneumothorax, anatomical abnormalities have been demonstrated, even in the absence of overt underlying lung disease. Subpleural blebs and bullae are found at the lung apices at thoracoscopy and on CT scanning in up to 90% of cases of PSP,5 6 and are thought to play a role. More recent autofluorescence studies7 have revealed pleural porosities in adjacent areas that were invisible with white light. Small airways obstruction, mediated by an influx of inflammatory cells, often characterises pneumothorax and may become manifest in the smaller airways at an earlier stage with ‘emphysema-like changes’ (ELCs).8 Smoking has been implicated in this …