Lung Disease in Systemic Sclerosis (Scleroderma)

Abstract

The lungs are often affected in patients with systemic sclerosis (SSc) and lung disease is now the leading cause of mortality. Two types of pulmonary disease predominate: interstitial lung disease and pulmonary hypertension. Both are difficult to detect at the earliest stages, thus management of patients with these manifestations can be difficult. Several recent studies using cyclophosphamide to treat SSc alveolitis, the inflammatory state associated with interstitial lung disease, have yielded promising results in terms of symptomatic improvement, as well as stabilization or improvement of lung volumes. Vasodilator therapy with prostacyclin analogues is being evaluated in SSc patients with pulmonary hypertension, based on results in primary pulmonary hypertension patients. Lung transplantation is often not an option for most SSc patients, thus optimal medical therapy is greatly needed for the SSc patient with severe lung disease.

Key Words:

scleroderma - pulmonary fibrosis - pulmonary hypertension