A case of ANCA positive refractory otitis media started with otitis media with effusion

多発血管炎性肉芽腫症は,細小動静脈や毛細血管などの小血管を主病変とし,全身に症状を呈する炎症性疾患である.最近では,この疾患群の中で,MPO-ANCA陽性で急激に進行する感音難聴を伴った難治性中耳炎の報告が増加している.これらは共通した臨床像を呈し,ANCA関連血管炎性中耳炎と呼ばれている.本疾患を治療する上では,全身に症状を呈する可能性があること,脳神経症状を続発し,死亡に至る例も存在することから,耳鼻咽喉科だけではなく,内科を含めた多くの診療科の関与が必要である.今回,我々は,難治性中耳炎の治療経過中に進行性感音難聴をきたし,最終的にANCA関連血管炎性中耳炎と診断された1例を経験したので,文献的考察を加えて報告する.

Antineutrophil cytoplasmic antibody(ANCA)associated vasculitis is characterized by systemic necrotizing vasculitis. We reported a case of ANCA positive refractory otitis media started with otitis media with effusion. The patient complained of progressive hearing loss, which was not improved by treatments with antibiotics. Therefore, the patient entered our hospital. The patient was diagnosed as otitis media with ANCA associated vasculitis(OMAAV),because the serum MPO-ANCA was detected in the first examination in our hospital. After the administration of steroid, her symptoms were sufficiently improved. It is known that otitis media with ANCA associated vasculitis can be secondary associated with polyneuritis and brain hemorrhage. Therefore, the treatment for OMAAV should be performed in cooperation with the internal departments.

難治性中耳炎

MPO-ANCA

ANCA関連血管炎