Anca Associated With Vasculitis In A 9-Year-Old

Antineutrophil cytoplasmic autoantibody (ANCA) associated small vessel vasculitis constitutes a group of life-threatening diseases and renal involvement is its most severe and common manifestation. Microscopic polyangiitis (MPA) is a rare form of such vasculitis in children characterized by pulmonary-renal syndrome with pauci-immune rapidly progressive glomerulonephritis. A 9-yearold boy was admitted to our hospital because of rash, arthralgia and dark urine. Urine analyses showed hematuria and proteinuria. Blood examination revealed anemia, renal failure and positive p-ANCA. A chest CT revealed diffuse infitration. The renal biopsy demonstrated crescentic glomerulonephritis, immunofluorescent examination was negative. He was diagnosed MPA. High-dose methylprednisolone and high-dose cyclophosphamide therapy improved all of the lung infltrates and renal function.