腎臓学と治療学ジャーナル

Eosinophilic Granulomatous Polyangitis with Renal Granulomatous Angitis and Interstitial Eosinophilic Infiltration without Lung Granuloma

Abstract

Shinya Kawamoto*,Hideo Misawa,Katsuhiro Nagahori,Shigeyuki Ota,Atsushi Kitazawa,Atsunori Yoshino,Tetsuro Takeda

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.