Lung disease in systemic sclerosis (scleroderma)

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 pat ients with these manifestations can be difficult. Several recent studies us ing cyclophosphamide to treat SSc alveolitis, the inflammatory state associ ated with interstitial lung disease, have yielded promising results in term s of symptomatic improvement, as well as stabilization or improvement of lu ng volumes, Vasodilator therapy with prostacyclin analogues is being evalua ted in SSc patients with pulmonary hypertension, based on results in primar y pulmonary hypertension patients. Lung transplantation is often not an opt ion for most SSc patients, thus optimal medical therapy is greatly needed f or the SSc patient with severe lung disease.