Treatment of progressive systemic sclerosis with plasma exchange. Seven cases

Seven patients, 4 women and 3 men afflicted with severe progressive systemic sclerosis (PSS) were treated with Plasma Exchange after failure of different other treatment. All patients presented Raynaud phenomenon and arthritis, 6 patients presented extensive skin lesions, 5 of them digestive manifestations, 3 pulmonary fibrosis. In one case PSS was associated with polymyositis, one patient presented bilateral recurrent cornea ulcerations, (Sjögren Syndrome++) and one patient numerous skin ulcerations. In 5 patients adjuvant corticosteroid therapy was given during the course of PE. In 3 patients PE must be stopped after one or two sessions because of insufficient venous access. Among the 4 other patients 8 to 20 PE were performed: the patient with cornea ulcerations became blind during the treatment, skin ulcerations and severe Raynaud phenomenon did not improved in two other patients. Benefit of PE was noted in only one patient with regressive myositis, and improvement of articular and cutaneous symptoms. Therefore, PE are not useful in most patients afflicted with PSS, they are difficult to realize in numerous patients and did not improve clinical symptoms in most cases.