角膜実質炎で初発し全身性の炎症性病変を併発したCogan症候群の1例

Interstitial keratitis as the initial manifestation in a case of Cogan syndrome with inflammatory systemic lesions

要約　目的：角膜実質炎で初発し，全身性の炎症性病変を併発したCogan症候群の報告。症例：36歳女性。2か月前より左眼充血を繰返していた。所見：発症から2年間で両眼周辺部角膜実質炎，右眼虹彩炎，大動脈炎，壊疽性膿皮症，無菌性多発肝膿瘍，内耳障害，上顎洞炎，右眼中間部ぶどう膜炎，右眼無菌性結膜下膿瘍が生じた。角膜実質炎，感音性難聴，内耳障害に加え各種炎症性疾患の合併からCogan症候群と診断した。いくつかの炎症性病変は再燃する傾向にあるが，初診から2年後の現在まで，ステロイドと免疫抑制剤の全身投与，ステロイド点眼で症状は軽快しており，外来にて診療中である。結論：Cogan症候群を発症した場合は全身の炎症性病変の発症にも注意が必要である。

Abstract.　Purpose：To report a case of Cogan syndrome with interstitial keratitis as the initial manifestation followed by multiple inflammatory systemic lesions. Case：A 36-year-old female presented with recurring conjunctival hyperemia in the left eye in the past two months. Findings：Corrected visual acuity was 1.0 right and 0.5 left. Both eyes showed opaque corneal stroma, leading to the diagnosis of interstitial keratitis in both eyes. During the following two years, she developed aortitis, pyoderma gangrenosum, multiple sterile liver abscess, sterile subconjunctival abscess in the right eye, and intermediate uveitis in the right eye. She was eventually diagnosed with Cogan syndrome. Recurrence of these inflammatory lesions appear to be well-controlled after initiation of treatment with immunosuppressive agents and prednisolone applied topically and systemically. Conclusion：The present case illustrates that Cogan syndrome may be associated with multiple systemic inflammatory symptoms.