Søndergaard K, Carstens J, Jørgensen J, Zachariae H.
DOI: 10.2340/0001555575150152

Glucocorticoids and immunosuppressive agents can induce remission in most patients with pemphigus, but mortality remains at 5 to 15% due to complications from these drugs. We reviewed the adjunctive effect of long-term plasmapheresis in 8 patients with pemphigus. Four cases had been resistant to conventional therapy. One or two large-volume plasmapheresis treatments were given monthly for 5 to 73 months. All patients were in clinical remission within 2 months after the addition of plasmapheresis. Relapses of pemphigus seldom occurred, and the patients stayed in remission 90% (40-100%) (median and ranges) of the plasmapheresis period. In all cases the daily dose of glucocorticoid was reduced. The prednisone level could be decreased significantly from 38 (15-80) mg/day to 10 (5-35) mg/day (p = 0.008). The overall level of other immunosuppressive agents remained unchanged, except in one patient where cyclosporine was introduced. This first report of long-term plasmapheresis demonstrates clinical efficacy in pemphigus and a considerable steroid-sparing effect.