Plasma exchange therapy in progressive systemic sclerosis

Abstract

Eight patients with severe progressive systemic sclerosis were treated with plasma exchange accompanied by cyclophosphamide and/or captopril, a converting enzyme inhibitor. All patients presented with visceral involvement of heart, lung, or kidney, and/or extensive skin lesions with tissue necrosis. Raynaud's syndrome improved in all patients after plasma exchange, and all patients showed an objective improvement in their most prominent clinical features, resulting in reduction of heart failure, improvement in lung function tests, decrease in creatinemia, and healing of ulcers. These results suggest the use of plasma exchange either with short courses of intensive exchanges for vasculitis or acute kidney or heart involvement, or with longer courses of regular exchanges for pulmonary fibrosis. Four deaths were observed, but follow-up (up to 62 months) showed the possibility of a long term stability under captopril therapy in patients with poor prognosis.