ISSN: 2161-1149 (Printed)
Scleroderma or systemic sclerosis (SSc) is a clinically heterogeneous, multi-system autoimmune disorder characterized by endothelial dysfunction, dysregulation of fibroblasts resulting in excessive production of collagen and profound abnormalities of the immune system. Pulmonary involvement is common and occurs in all SSc subsets, including limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and SSC sine scleroderma. Aside from the common complications of pulmonary vasculopathy and interstitial lung disease (ILD], other less frequent pulmonary complications have been reported in SSc. The emphasis of this review will be on other lung complications in systemic sclerosis.