Plasma exchange in the treatment of progressive systemic sclerosis

Six patients with progressive systemic sclerosis (PSS) were treated with plasma exchange (PE) alone or in association with low corticosteroid dosages. Five patients had diffuse scleroderma and one had CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia). All patients had failed to respond to drug therapy; three had worsening cardiopulmonary involvement, three had skin ulcers, and one had myositis. The treatment protocol was for a 40% plasma exchange three times per week for the first three to four weeks, to be slowly tapered in a two-to ten-week period. The replacement solution was 4% albumin in polysaline. Corticosteroid dosage was continued unchanged or reduced during the period of exchange in three patients. In one patient, PE was discontinued because of insufficient venous access. In the five patients who completed the protocol, a transient improvement of general status, skin thickness, and (whenever present) of flexion contractures were observed after PE. Skin ulcerations healed or improved, but cardiopulmonary function was not improved. Despite a course of short-term PE, four of six patients died within two years. The remaining two patients remained stable or improved with a second period of treatment.