Collagen Vascular Disease Associated With Interstitial Lung

Interstitial lung disease (ILD) refers to different manifestations of pulmonary disorders due to inflammation and/or fibrosis of the pulmonary parenchyma. Collagen vascular disease is a common type of ILD etiology and encompasses a diverse group of immunologically mediated entities that share overlapping clinical and histopathologic features as well as manifest in characteristic patterns of ILD. The collagen vascular diseases which commonly affect the pulmonary system include rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis (PM), dermatomyositis (DM), mixed connective tissue disease (MCTD), and Sjogren’s syndrome (SS). The degree of lung involvement and spectrum of thoracic findings vary among the disorders, may be seen with clinically early or late disease, and portends certain patient outcomes. Identification of pulmonary involvement has important therapeutic and prognostic implications. Collagen vascular disease may be associated with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), diffuse alveolar damage (DAD), and lymphocytic interstitial pneumonia (LIP). This article will review the etiology, epidemiology, pathogenesis, history and examination, evaluation, management, and prognosis of ILD related to collagen vascular disease.