Addition of Plasma Exchange to Glucocorticosteroids for the Treatment of Severe Henoch-Schonlein Purpura in Adults: A Case Series

Background: Adult Henoch-Schonlein purpura (HSP) has been associated with poor outcome and end-stage renal disease in >20% of cases. Although the benefit of adding another immunosuppressant to steroids in severe adult HSP has not been shown, the benefit of plasma exchange (PE) therapy has been poorly evaluated. Study Design: Case series. Setting & Participants: 11 consecutive patients with severe and newly diagnosed HSP since 1988 who were treated with steroids and PE. Outcome & Measurement: Patients' characteristics and outcome were analyzed. Birmingham Vasculitis Activity Score (BVAS), estimated glomerular filtration rate (eGFR), and proteinuria were measured at baseline, at the end of PE treatment, at months 6 and 12, and at the last visit. Side effects of corticoid treatment and PE were recorded. Results: 11 patients were identified in 1988-2010. Patients received intravenous corticoid pulses in 64% of cases, followed by oral prednisone for a median of 6.6 months. They received a median of 12 PE sessions. BVAS, eGFR, and proteinuria improved significantly between baseline and the last PE at a median of 2 months. PE sessions were well tolerated, except in one patient who developed central catheter-associated septicemia. One patient required dialysis therapy 15 days after HSP diagnosis and did not recover kidney function. At the last medical evaluation at a mean follow-up of 6 years, median eGFR and proteinuria were 83 +/- 22 mL/min/1.73 m(2) and protein excretion of 140 +/- 10 mg/d, respectively. 3 women had pregnancy without complications. Limitations: This case series did not have a control group. Conclusions: The combination of PE and corticoid therapy in severe forms of HSP was associated with fast improvement and good long-term outcome. Am J Kidney Dis. 59(5): 663-669. (C) 2012 by the National Kidney Foundation, Inc.