Abstract

Eosinophilic granulomatous with polyangiitis (EGPA) is systemic vasculitis characterized by concomitant symptoms of asthma, allergic rhinitis, and marked increase in peripheral eosinophilia. It was previously known as Churg-Strauss syndrome. EGPA incidence in Japan is very low, with only approximately 1,800 cases reported. Renal involvement occurs in approximately 20-25% of EGPA patients, and the most typical expression is pauciimmune crescentic glomerulonephritis. We herein report a case of 69-year-old Japanese woman with fever and high titer of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and eosinophilia. Her renal biopsy showed massive interstitial nephritis with granulomatous vasculitis like lesion without apparent active crescent formation in glomeruli. Immediately after steroid treatment (prednisolone [PSL] 30 mg/day), she had symptomatic relief and was discharged with a reduction in MPO-ANCA.

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