Recurrent Hematuria Syndrome in a Child: An Unusual Presentation of a Common Disease

Abstract

Immunoglobulin A vasculitis (IgAV) is a small vessels immunoglobulin A (IgA)-mediated vasculitis with multiorgan involvement. Kidney involvement (IgAV nephropathy [IgAVN]) occurs in 30%–50% of patients and usually appears at or within 6-month of initial presentation. The converse, with nephritis preceding other manifestations, is rare. We describe a 5.5-year-old boy, who presented with recurrent hematuria syndrome during upper respiratory tract infections. This preceded, by several months, the appearance of a palpable purpuric rash. Skin biopsy confirmed leukocytoclastic vasculitis with predominant IgA deposition. Serum level of complement was normal. Serum creatinine was elevated mildly and transiently during each gross hematuria event. Nephrotic range proteinuria resolved after macrohematuria resolution without residual proteinuria or decreased kidney function 12 months after initial presentation. IgAVN may manifest with isolated recurrent glomerulonephritis preceding purpura. This case emphasizes that children with normocomplementemic recurrent hematuria syndrome should be regularly examined for the possible later appearance of other signs of IgAV.