Cyclosporine and therapeutic plasma exchange in treatment of progressive autoimmune diseases

Despite treatment with intensive immunosuppressive drug regimens, the prognosis of patients suffering from severe progressive autoimmune diseases like systemic lupus erythematosus (SLE), nephrotic syndrome (NS), and Behcet's disease is poor. Side effects (infections and malignant tumors) often occur. In the present trial, 35 patients suffering from autoimmune diseases (SLE, n = 21; NS, n = 10; and Behcet's disease, n = 4) were treated for 3.7 +/- 2.0 years with 2.5 +/- 0.6 mg cyclosporine/kg body weight/day in addition to corticosteroids alone or in combination with azathioprine and/or cyclophosphamide. In active stages of the diseases with extremely high concentrations of anti-ds-DNA-antibodies, antinuclear antibodies, circulating immunocomplexes, and reduced complement concentrations, therapeutic plasma exchange (TPE) has been applied. Compared with previous treatment modalities, significantly (p < 0.05) more effective and rapid reductions of the antibodies were reached. Clinical disorders improved within 1-6 weeks. All patients reported increased performance and a better quality of life. After 1-12 months, the previously required doses of immunosuppressive drugs and the frequency of TPE could be reduced by 40-100%. After 13.4 +/- 11.8 months in 17 of 35 patients (8 with SLE, 5 with NS, 4 with Behcet's disease), cyclosporine was established as the monotherapy. No severe side effects were registered. In treating active stages of severe progressive autoimmune diseases and forms with persistent high antibody levels, the addition of TPE to conventional therapy was very effective, as observed in both clinical and laboratory parameters.