Systemic Sclerosis

Systemic sclerosis (SSc), or scleroderma is a chronic autoimmune disorder of small vessels and connective tissue characterized by organ fibrosis, small vessel vasculopathy and disease-specific antibodies. It results in multi system organ fibrosis. The skin, lungs, heart, gastrointestinal tract and kidneys are frequently affected. Skin involvement is typical. Clinically evident pulmonary disease is seen in 25% of affected patients, but autopsy reports demonstrate some degree of pulmonary involvement in all patients with the systemic form of the disease. Pulmonary disease is second only to esophageal fibrosis and has surpassed renal disease as the leading cause of death. Systemic sclerosis associated-interstitial lung disease (SSc-ILD) and pulmonary vascular disease are the two major lung manifestations of SSc. Pulmonary hypertension is the leading cause of death. A normal chest radiograph does not exclude SSc-ILD. High resolution CT (HRCT) should be part of the initial evaluation of patients with SSc, even when pulmonary symptoms are not present. The radiologist should be able to recognize signs of early pulmonary fibrosis and pulmonary hypertension on radiography and HRCT due to the proven benefit of adequate therapy in overall outcome and mortality.