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葡萄膜炎与普通变异性免疫缺陷:来自 DEF-I 研究和文献复习的数据。

Uveitis and common variable immunodeficiency: data from the DEF-I study and literature review.

机构信息

Department of Internal Medicine, Hôpital Croix-Rousse, Lyon, France.

出版信息

Ocul Immunol Inflamm. 2012 Jun;20(3):163-70. doi: 10.3109/09273948.2012.674612. Epub 2012 Apr 17.

DOI:10.3109/09273948.2012.674612
PMID:22506485
Abstract

PURPOSE

To examine the relation between uveitis and common variable immunodeficiency (CVID).

METHODS

Retrospective analysis of patients included in the French DEFI cohort of adults with CVID and of patients identified by reviewing the literature.

RESULTS

Four patients were identified in the DEFI study (frequency of uveitis: 1.6%). The course of uveitis was not changed in the patients who started intravenous immunoglobulins replacement therapy after CVID diagnosis. Ten cases of CVID-associated uveitis were listed in the literature. Overall, among the 14 patients, uveitis was always chronic, usually bilateral (n = 11) and granulomatous (n = 9). Seven patients presented with a "sarcoid-likeâ syndrome. Nine patients had granulomatous uveitis suggestive of ocular sarcoidosis. Five patients were treated with local corticosteroids, and 9 required systemic treatment (corticosteroids alone n = 5 and/or immunosuppressive agents n = 4).

CONCLUSIONS

CVID can be associated with uveitis. Since uveitis can reveal the immunodeficiency, CVID diagnosis should be considered at the time of uveitis diagnosis.

摘要

目的

研究葡萄膜炎与常见可变免疫缺陷(CVID)之间的关系。

方法

对法国 CVID 成人 DEFI 队列中的患者以及通过文献复习确定的患者进行回顾性分析。

结果

在 DEFI 研究中发现了 4 例患者(葡萄膜炎发生率:1.6%)。在 CVID 诊断后开始静脉注射免疫球蛋白替代治疗的患者中,葡萄膜炎的病程没有改变。文献中列出了 10 例 CVID 相关葡萄膜炎病例。总的来说,在这 14 名患者中,葡萄膜炎始终是慢性的,通常是双侧(n = 11)和肉芽肿性(n = 9)。7 例患者表现出“类肉瘤样”综合征。9 例患者有肉芽肿性葡萄膜炎,提示眼类肉瘤病。5 例患者接受局部皮质类固醇治疗,9 例需要全身治疗(单独皮质类固醇治疗 n = 5 和/或免疫抑制剂治疗 n = 4)。

结论

CVID 可伴有葡萄膜炎。由于葡萄膜炎可提示免疫缺陷,因此在诊断葡萄膜炎时应考虑 CVID 诊断。

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