Anti-neutrophil cytoplasmic autoantibodies in a child with pauci-immune necrotizing and crescentic glomerulonephritis.

We report a case of pauci-immune, necrotizing and crescentic glomerulonephritis in a 10-year-old child initially thought to have Henoch-Schönlein purpura. The diagnosis of a Wegener's granulomatosis-microscopic polyarteritis disorder was made on the basis of clinical presentation and a positive anti-neutrophil cytoplasmic autoantibody (ANCA). This case illustrates the usefulness of the ANCA in the diagnosis and management of childhood vasculitides.