Interstitial Lung Disease in Collagen Vascular Disease

Abstract

Collagen vascular diseases (CVDs) showing features of interstitial lung disease (ILD) pattern include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjögren’s syndrome, and mixed connective tissue disease (MCTD). Histopathologically, interstitial lung diseases associated with CVD are diverse and include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), cryptogenic organizing pneumonia (COP), apical fibrosis, diffuse alveolar damage (DAD), and lymphoid interstitial pneumonia (LIP) patterns (Tables 28.1 and 28.2). NSIP accounts for large proportion, especially in PSS, DM and PM, and MCTD. More favorable prognosis in interstitial pneumonia associated with CVDs than in idiopathic interstitial pneumonias (IIPs) may be explained by the larger proportion of NSIP pattern than UIP pattern. Thin-section CT (TSCT) seems to help characterize and determine the extent of ILD in CVDs [1].