模仿科根综合征多发性肝脓肿的肝血管炎。

Acta reumatologica portuguesa Pub Date : 2019-07-08
João Dinis de Freitas, Flávio Costa, João Rovisco
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引用次数: 0

摘要

科根综合征是一种罕见的炎症性疾病,主要表现为间质性角膜炎和感音神经性听力损失。内耳疾病可能导致耳聋。有些患者还可能伴有全身性血管炎。该综合征非常罕见,自 1945 年 Cogan 首次描述其特征以来,鲜有病例报道。我们报告了一例典型科根综合征患者的罕见病例,该患者发展为肝血管炎,并伴有肝梗死,模仿多发性肝脓肿。由于其罕见性、发病时的非特异性临床症状以及缺乏确诊检查,CS 常常被漏诊或延误诊断。风湿病学家应该了解这种疾病及其可能的全身表现。本病例的独特之处在于血管炎的严重程度和受累部位。
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Hepatic vasculitis mimicking multiple liver abscesses in Cogan's Syndrome.

Cogan's syndrome is a rare inflammatory disorder, classically characterized by interstitial keratitis and sensorineural hearing loss. Inner ear disease may result in deafness. In some patients, it may also be accompanied by systemic vasculitis. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We report a rare case of a patient with typical Cogan syndrome who developed hepatic vasculitis with hepatic infarction mimicking multiple liver abscesses. Diagnosis of CS is often missed or delayed due to its rarity, the nonspecific clinical signs at onset, and the lack of a confirmatory diagnostic test. Rheumatologists should be aware of this entity and its possible systemic manifestations. The present case is unique because of the severity and involved area of the vasculitis.

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