An unusual case of scleroderma lung disease

Abstract

ABSTRACTSystemic sclerosis is a disease characterized by autoimmunity, vasculopathy and fibrosis. Interstitial lung disease and pulmonary hypertension are the most common manifestations of lung disease in systemic sclerosis and, together, are the leading cause of mortality in systemic sclerosis. This study presents a case of a young man with limited systemic sclerosis and no smoking history who underwent chest imaging for suspicion of interstitial lung disease and was found to have extensive emphysema. This case outlines the importance of recognizing the presence of combined fibrosis and emphysema in those with systemic sclerosis.