2-year-old infant with Henoch-Schönlein purpura nephritis grade VI resistant for various immunosuppressive therapy and plasmapheresis

Abstract

Henoch-Schönlein purpura nephritis (HSPN) is the major complication of Henoch-Schönlein purpura. In the case of the intractable condition of the resistance for corticosteroid therapy, no second-line therapeutic strategy has not been established at present. We encountered a 2-year-old infant who is resistance for the cocktail therapy using corticosteroid including methylpredonisolone pulse therapy, immunosuppressants including cyslophosphamide, cyclosporine A, and mizoribine combined with plasmapheresis. Regarding such intractable condition, mycophenolate mofetil was given, resulting in the incomplete remission of nephrotic condition. The infant is the youngest reported patient showing severe HSPN of ISKDC IV. The infant also showed heavy nephrotic range of proteinuria and intractable hypertension involving posterior reversible encephalopathy syndrome. Since histologic findings are unusual involving the survival of extraordinary number of the premature glomeluruli, the subsequent failure of glomerular maturation leading to the glomerular sclerosis is deeply concerned. We are afraid of the renal function would be worsen in near future in this infant.