Cyclosporin and plasmapheresis in treatment of progressive systemic lupus erythematosus (pSLE)

The prognosis of patients suffering from progressive systemic lupus erythematosus (pSLE) is poor, despite treatment with intensive drug regimens with combinations of corticosteroids, azathioprine and cyclophosphamide. Side-effects such as infections and malignomas often occur. In the present trial, 21 patients (four male, 17 female, aged 37.9 +/- 12.8) suffering from pSLE for 9.4 +/- 2.6 years, were treated for 2.3 +/- 1.8 years with drug regimens as mentioned above. Then, over a period of 6.4 +/- 2.6 (range 1-8) years, in addition to conventional therapies, cyclosporin (2.5 +/- 0.6 mg/kg body wt/d) and, in active stages of the disease with extremely high concentrations of anti-ds-DNA-, anti-nuclear antibodies and circulating immunocomplexes, plasmapheresis (therapeutic plasma exchange (TPE)) have been applied. Compared with previous treatment modalities, significantly (P < 0.05) more effective and rapid reduction of antibodies was achieved. Clinical symptoms improved within 2 to 4 weeks. Under the new therapeutic regimen all patients reported increased performance and a better quality of life. After 5 to 48 (17.5 +/- 13.8) months, cyclosporin was established as mono-therapy for 8/21 patients. In] the other cases, corticosteroids, azathioprine and cyclophosphamide were reduced by 40 to 100%. No severe side-effects were seen. In acute stages of pSLE and in forms with persistently high antibody levels, the addition of TPE to conventional therapy was very effective, with regard to improving both clinical and laboratory parameters.