Our search revealed an RCT of plasmapheresis, a case-controlled study and several case series. Guillaume et al31 enrolled 40 previously untreated patients in an RCT to determine the clinical efficacy of plasmapheresis in the treatment of pemphigus. Eighteen patients were treated with prednisolone alone (at least 0.5 mg/kg/day) and 22 with prednisolone plus 10 plasma exchanges over 4 weeks. There were no differences between the two groups with respect to clinical improvement or serum autoantibody titres. Eight patients, four in each group, did not achieve disease control with the highest steroid doses used in the study (2 mg/kg/day prednisolone). Four patients, all in the plasmapheresis group, died of sepsis or thromboembolism. The authors concluded that plasmapheresis, in association with "low" steroid doses, was not effective for treatment of pemphigus.
Although well designed, the study of Guillaume et al31 is difficult to interpret when one considers evidence that plasmapheresis without concurrent use of immunosuppressive agents results in antibody "rebound", or rapid synthesis of new autoantibody.32-34 Therefore, it is possible that there was no evidence of clinical improvement due to "rebounding" antibody titres following plasmapheresis. In a case-controlled study, Tan-Lim and Bystryn35 reported a more favourable conclusion regarding the effect of plasmapheresis on antibody titres when concurrently administered with corticosteroids and immunosuppressive agents. In 11 patients in the plasmapheresis group, three weeks of treatment resulted in a decrease in the average titres of autoantibodies of 83%. In contrast, titres decreased by only 18% among patients receiving the control regimen (P = 0-037). Although no clinical data were presented and long-term follow up was not reported, the drop in antibody titre is significant and represents a
Does immunomodulatory therapy (i.e. plasmapheresis, or intravenous immunoglobulin (IVIG)) induce remission and reduce mortality more effectively than prednisone alone or in combination with immunosuppressive agents in patients with severe widespread pemphigus vulgaris that is unresponsive to oral prednisone?
dramatic improvement over the results described by Guillaume et al.31
In a recent case series, Turner et al36 described their experience with plasmapheresis in seven patients with severe pemphigus vulgaris. All patients had been treated unsuccessfully with a variety of therapeutic agents for a period of 2 months to 10 years. Each patient underwent an average of nine plasma exchanges, during and immediately after which they received immunosuppressive therapy, pulse cyclophosphamide or pulse steroid, to prevent rebound autoantibody synthesis. Four patients achieved partial remission, two patients improved and were able to taper steroid dosage, and one patient showed initial improvement but relapsed within a month. Data concerning rates of remission and relapse are based on a follow up period of only 2 months. Because all patients received either pulse cyclophosphamide or pulse steroids, both believed to be effective for inducing remission in pemphigus, it is difficult to determine whether the observed benefits were the result of plasmapheresis or intensive immunosuppression.
In order to determine whether plasmapheresis is effective in controlling severe pemphigus, it is important to compare the clinical outcome in patients receiving immunosuppressive therapy with that in patients receiving immunosuppressive therapy plus plasmapheresis.
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