Treating Henoch-Schönlein Purpura with Plasma Exchange

In a recent article published in the American Journal of Kidney Diseases, Augusto et al of Angers, France, presented their 22 year experience with Henoch-Schönlein purpura (HSP). During that time, they cared for 14 patients with HSP, 11 of whom were treated with plasmapheresis and corticosteroids. Three patients were enrolled in the CESAR trial, a multi-center trial that compared steroids to steroids plus cyclophosphamide for renal complications of HSP. There was no identifiable therapeutic effect of cyclophosphamide.

Outside of the 3 patients enrolled in CESAR, Augusto’s group employed a fairly uniform treatment protocol consisting of:

  • 3 days of pulse methylprednisolone (10-15 mg/kg/day), followed by
  • prednisone 1 mg/kg tapering to 0.5 mg/kg over 8 weeks
  • plasma exchange (PE) with albumin replacement continued for 10-15 sessions over 2 months

The patients were all adults between ages 18 and 85. Only one patient progressed to end-stage renal disease (this patient ultimately died of sepsis), and 2 reached a Birmingham Vasculitis Activity Score (BVAS) of zero. Two of 11 (18%) is about twice the rate of remission found in the CESAR trial where 6 of 54 (11%) patients reach a BVAS of zero.

What was the role for PE in this protocol? What toxic substance was being removed? It is postulated that pathologic immunoglobulin A1 immune complexes are cleared by PE, while  steroids stop their production. Alternatively, could PE be removing pro-inflammatory or pro-coagulatory substances? PE treatment generally has been looked at with skepticism in the renal literature. PE offers benefit in severe antineutrophil cytoplasmic antibody vasculitis and glomerular basement membrane disease. Augusto et al postulate that similar to vasculitis, the PE will prevent aggressive crescentic formation and fibrosis. We have to keep in mind that PE is temporary, and should always accompany immunosuppressive therapy to stop antibody production. We urge all to look at an entire series of papers published on use of plasma exchange and apheresis.

Joel Topf, MD
eAJKD Advisory Board member

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