ANCA関連副鼻腔粘膜病変により眼窩先端症候群を呈した1例

Orbital apex syndrome in a case of ANCA-related mucosal lesion of the paranasal sinus

要約　目的：眼窩先端症候群がANCA関連蝶形骨洞粘膜病変がある症例に生じた報告。症例：73歳女性が1週間前からの頭痛，複視，右眼の眼球突出と視力低下で受診した。既往として糖尿病，高血圧，脂質異常があった。所見：矯正視力は右0.01，左0.9で，右眼の眼球突出と全方向への運動障害があった。MRIで右蝶形骨洞に粘膜病変があり，視神経管と海綿静脈洞に接していた。MPO-ANCAが陽性で，多発血管炎性肉芽腫症が疑われた。プレドニゾロン20mg/日の全身投与で粘膜病変は消失し，6週後に視力と眼球運動が正常化した。13か月後の現在まで経過は良好である。結論：多発血管炎性肉芽腫症による眼窩病変は予後不良とされるが，本症例では蝶形骨洞の粘膜病変からの炎症性神経障害があったと推定され，経過は良好であった。

Abstract.　Purpose：To report orbital apex syndrome in a case of ANCA-related mucosal lesion of the sphenoidal sinus. Case：A 73-year-old female presented with headache, diplopia, proptosis and impaired vision in her right eye since one week before. She had had diabetes mellitus, hypertension and elevated lipids. Findings：Corrected visual acuity was 0.01 right and 0.9 left. The right eye showed proptosis and impaired motility in all directions. Magnetic resonance imaging（MRI）showed mucosal lesion in the right sphenoidal sinus adjacent to the optic canal and cavernous sinus. She was positive for MPO-ANCA, suggesting granulomatosis related to polyangiitis. Peroral prednisolone at the daily dosis of 20 mg was followed by normalization of mucosal lesion in the paranasal sinus and by normalization of visual acuity and motility 6 weeks later. She has been doing well for 13 months until present. Conclusion：Orbital lesion secondary to granulomatosis with polyangiitis shows poor prognosis in general. The present case resulted in favorable outcome, as inflammatory neuropathy may have been present secondary to paranasal sinusitis.