Spectrum of Fibrosing Diffuse Parenchymal Lung Disease

Abstract

The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky‐Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end‐stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed. Mt Sinai J Med 76:2–23, © 2009 Mount Sinai School of Medicine

Inhibitory Effects of the Dual Endothelin Receptor Blocker Bosentan on Thrombin-Activated Lung Fibroblasts Isolated from Scleroderma Patients

“…Studies from our laboratory suggest that the serine protease thrombin contributes to the development of SSc-ILD by activation of microvascular endothelial cells and by inducing the differentiation of lung fibroblasts to myofibroblasts [6,[19][20][21]. In the early stages of SSc-ILD we observed that the formation of new vessels occurs and coincides with the appearance of myofibroblasts [22]. These results suggest an interaction of endothelial cells with myofibroblasts, which may be crucial in the development of pulmonary fibrosis as seen in SSc-ILD and other ILDs.…”

A case of pulmonary fibrosis associated with rheumatoid arthritis, scleroderma sine scleroderma and ANCA associated vasculitis.

“…PF is a well known complication in a number of autoimmune diseases (Morgenthau and Padilla 2009). The prognosis in these patients depends on several factors like histology, baseline lung function, presence of autoantibodies and its titer level (Morgenthau and Padilla 2009).…”

“…The prognosis in these patients depends on several factors like histology, baseline lung function, presence of autoantibodies and its titer level (Morgenthau and Padilla 2009). This is worse in overlap syndromes (Cambridge et al 1994;Derrett-Smith et al 2013;Szücs et al 2007).…”

“…Progression of disease results in impaired oxygen transfer and scarring within the lungs (Morgenthau and Padilla 2009). It can be idiopathic or associated with other diseases.…”

“…The major HRCT feature in NSIP is ground glass appearance. However ground glass opacities may be present as the first manifestation of UIP or during acute exacerbations (Morgenthau and Padilla 2009).…”