好酸球性多発血管炎性肉芽腫症に前部虚血性視神経症と網膜動脈系循環障害を合併した1例

A case of eosinophilic granulomatosis with polyangiitis associated with anterior ischemic optic neuropathy and disturbance in the retinal artery circulation

要約　目的：右眼の前部虚血性視神経症と左眼の網膜動脈循環障害を生じた好酸球性多発血管炎性肉芽腫症（EGPA）の1例を報告する。

症例：47歳，女性。主訴は両眼視力低下で，3年前に気管支喘息と診断されていた。視力は右（0.3），左（0.5）で，右眼に前部虚血性視神経症，左眼に網膜毛細血管前細動脈と毛細血管の循環障害を認めた。喘息の既往，好酸球増加，末梢血管炎の3症状および皮膚生検でフィブリノイド壊死を伴う血管炎を認めたのでEGPAと診断した。抗好中球細胞質抗体陽性であった。ステロイドパルス療法とシクロホスファミド大量療法により全身状態は改善し，視野障害は残存するも矯正視力は両眼1.2に回復した。

結論：早期診断および治療により全身的改善と両眼の視機能の改善を得た。

Abstract　Purpose：We report a case of eosinophilic granulomatosis with polyangiitis associated with anterior ischemic optic neuropathy in the right eye and disturbance in the circulation of the retinal artery in the left eye.

Case and Findings：A 47-year-old female who had a past history of bronchial asthma three years before complained of bilateral vision loss. Her best corrected visual acuity was 0.3 in the right eye and 0.5 in the left eye. Anterior ischemic optic neuropathy was noted in the right eye and severe blood flow disturbance of the retinal precapillary arteriole and capillary vessels was noted in the left eye. She was diagnosed with eosinophilic granulomatosis with polyangiitis based on her history of asthma, eosinophilia, peripheral vasculitis and pathological finding of vasculitis with fibrinoid necrosis. Anti-neutrophil cytoplasmic antibody was positive. Her general condition improved by systemic corticosteroid and cyclophosphamide administration. The visual acuity improved to 1.2 in both eyes, although some disturbance of the visual field in both eyes remained.

Conclusions：The general condition and visual function of both eyes recovered because of the early diagnosis and treatment.