Plasmapheresis therapy in an elderly patient with rapidly progressive Henoch-Schönlein purpura with disseminated organ involvement

Abstract

Henoch-Schönlein purpura (HSP) frequently occurs in children below the age of 15 years and is rare with increasing age. Prognosis and therapy largely depend on the clinical presentation. The disease may be preceded by an upper respiratory tract infection, and drugs have also been implicated in the pathogenesis of the disease. Most children recover from the illness, whereas 40% of adults have persistent hematuria and 10% develop chronic renal failure. Recent studies strongly suggest that adults with HSP should be monitored for prolonged periods and treated aggressively. Here, we present a case of a patient with HSP who developed multiorgan failure requiring assistance in breathing and dialysis, and also sustained gastrointestinal bleeding despite aggressive immunosuppressive therapy. In analogy to published data in children with severe HSP, the patient was treated by plasma exchange in combination with low dose oral cyclophosphamide, while high dose steroids were reduced over time. The patient could be discharged 70 days after admission. One year after discharge, the patient is doing well without any signs of activity of HSP and completely unremarkable renal function. The maintenance daily dose of steroids is 7.5 mg.