Scleroderma, also known as progressive systemic sclerosis (SSc), is a multisystem autoimmune disorder characterized by inflammation and fibrosis involving the skin as well as internal organs such as the vasculature, esophagus, and the respiratory tract. Pulmonary involvement consists most often of interstitial fibrosis and pulmonary vascular disease leading to pulmonary arterial hypertension (PAH). Interstitial lung disease (ILD) is the leading cause of death in patients with SSc. The extent of ILD on HRCT in initial evaluation and decline in PFT during the preceding 12 months are helpful in identifying such patients. PFT should be repeated every 6 monthly for patients at risk. Key words: Pulmonary, Systemic Sclerosis