Visceral Improvement Following Combined Plasmapheresis and Immunosuppressive Drug Therapy in Progressive Systemic Sclerosis

Abstract

In a two-year prospective therapeutic trial, 15 patients with progressive systemic sclerosis (PSS) were treated with immunosuppressive drug therapy with or without long-term plasmapheresis. Before the trial all patients had severe involvement of either the esophagus, lungs or kidneys. One patient died of renal failure and another 2 patients withdrew unimproved. In the remaining 12 patients, objective improvement occurred in all but one. The degree and extent of skin involvement decreased significantly (p<0.01). Cineradiography revealed increased esophageal motility in 4 patients. Pulmonary function measured as total lung capacity and static lung compliance improved (p<0.01). In 4 patients the number of premature atrial or ventricular contractions at 24 h ECG monitoring decreased, as did the concentrations of immunoglobulins and ANA titres in serum. Although it could not be ascertained whether the clinical improvement was associated with combined therapy or immunosuppressive drug treatment alone, our results suggest that immunosuppressive therapy is beneficial in advanced PSS.

Key Words:

• progressive systemic sclerosis
• scleroderma
• plasmapheresis
• cyclophosphamide
• azathioprine