Haynes B F, Kaiser-Kupfer M I, Mason P, Fauci A S
Medicine (Baltimore). 1980 Nov;59(6):426-41.
Typical Cogan syndrome (CS) is a disease of young adults consisting of flares of interstitial keratitis (IK) and sudden onset of Ménière-like attacks. The prognosis of typical CS is excellent with life-threatening aortic insufficiency (AI) developing in only 10% of reported cases. Serious systemic necrotizing vasculitis (SNV) rarely, if ever, complicates typical CS. Atypical S (vestibuloauditory dysfunction with types of inflammatory eye disease other than IK) frequently overlaps other defined rheumatologic syndromes, is associated with vasculitis in 21% of cases, and carries a less favorable prognosis than typical CS. Topical ocular corticosteroids can usually control IK, and a short trial of systemic corticosteroids is warranted as soon as possible after the onset of hearing loss. SNV should be treated initially with prednisone and may also require the administration of cytotoxic agents, while aortitis and AI can be controlled with the administration of prednisone and surgical replacement of the aortic valve.
典型科根综合征（CS）是一种好发于年轻人的疾病，表现为间质性角膜炎（IK）发作和类似梅尼埃病发作的突然起病。典型CS的预后良好，仅10%的报告病例会发展为危及生命的主动脉瓣关闭不全（AI）。严重的系统性坏死性血管炎（SNV）很少（如果有的话）使典型CS复杂化。非典型CS（伴有IK以外其他类型炎症性眼病的前庭听觉功能障碍）常与其他明确的风湿性综合征重叠，21%的病例与血管炎相关，且预后比典型CS差。局部眼部皮质类固醇通常可控制IK，听力丧失发作后应尽快进行短期全身性皮质类固醇试验。SNV最初应使用泼尼松治疗，可能还需要使用细胞毒性药物，而主动脉炎和AI可通过使用泼尼松和手术置换主动脉瓣来控制。
