Pulmonary Fibrosis in Collagen Vascular Diseases

In dermatomyositis (DM)/polymyositis (PM), ILD occurs in 20% to 30% of patients. Death is attributable to respiratory failure in 30% to 60% of patients with DM/PM (93,94). ILD in DM/PM has protean manifestations ranging from acute lung injury with DAD to chronic fibrosing ILD (Fig. 11 A) (95). NSIP is reported to be the most common pattern of fibrosis, but UIP, LIP, and organizing pneumonia also occur (96). Skeletal muscle disease may contribute to the respiratory dysfunction in patients with DM/PM. 

Sjogren syndrome (SS) is characterized by infiltration of CD4-positive lymphocytes into different organs, most notably the lacrimal and salivary glands. In the lung, the disease manifests as a prominent lymphoreticular infiltration in the tracheobronchial tree with follicular bronchiolitis, atrophy of glands, and fibrosis 

Fishbein MC. Diagnosis to biopsy or not to biopsy. Chest 2005 128(suppl) 520S-525S. 

Gal AA. Use and abuse of lung biopsy. Adv Anat Pathol 2005 12 195-202. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002 165 277-304. 

Visscher DW, Myers XL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006 3 322-329. 

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