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Henoch-Schönlein purpura (HSP) is a vasculitis characterized by purpuric rash, arthritis, nephritis and gastrointestinal symptoms and it is generally seen in children. We report here a case of crescentic HSP nephritis with perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) positivity.
Our case was 78-year-old man. He had admitted to hospital with complaints of oliguria, dyspnea, abdominal pain, arthralgia and edema. Purpuric eruptions were observed on the gluteal region and lower extremities on physical examination. He had renal failure, proteinuria and hematuria. Hemodialysis treatment was begun after hospitalisation. Granular deposits of IgA were found in vessel walls of the skin and crescentic IgA nephritis was detected in biopsy. He was considered to be suffering from crescentic HSP nephritis. Complement levels were normal, but p-ANCA was positive. High doses of methylprednisolone and cyclophosphamide were administered in addition to plasmapheresis.
Although HSP is a small vessel vasculitis, serum complement levels are generally normal and ANCA is negative. Only a few cases of HSP with ANCA positivity have been reported in the literature. The reason of the ANCA positivity in patients with HSP is not exactly known.
High-dose corticosteroids in combination with immunosuppressive agents and plasmapheresis are generally recommended in cases of crescentic HSPN nephritis.