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虹膜表现与治疗不足的慢性复发性 Vogt-Koyanagi-Harada 病。

Iris Manifestations in Inadequately Treated Chronic Recurrent Vogt-Koyanagi-Harada Disease.

机构信息

Ocular Inflammation and Immunology Service, Singapore National Eye Centre, Singapore, Singapore.

Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.

出版信息

Ocul Immunol Inflamm. 2022 Jul;30(5):1116-1122. doi: 10.1080/09273948.2020.1870701. Epub 2021 Feb 9.

Abstract

PURPOSE

To describe the iris changes in chronic recurrent Vogt-Koyanagi-Harada Disease (VKH).

METHODS

Retrospective case series. Clinical features of 3 VKH patients who developed bilateral depigmentation of the iris are described.

RESULTS

Patients had delayed diagnosis and inadequately treated chronic recurrent VKH. Patient 1 presented with bilateral multiple contiguous small granulomatous iris stromal lesions, severe diffuse iris swelling more severe near the iris root, producing peripheral iris undulations. Diffuse iris atrophy and peripheral depigmentation subsequently developed. Patient 2 presented with peripheral iris depigmentation, atrophy of the ciliary zone iris stroma and sparing of the sphincter pupillae, giving a 'sunflower appearance.' Patient 3 had extensive iris depigmentation, atrophy, and transillumination defects involving the entire iris.

CONCLUSION

Uncontrolled bilateral diffuse granulomatous stromal iris inflammation leads to subsequent depigmentation, transillumination defects and atrophy, beginning in the peripheral iris. Sphincter pupillae is initially spared but complete iris atrophy may develop.

摘要

目的

描述慢性复发性 Vogt-小柳原田病(VKH)的虹膜变化。

方法

回顾性病例系列。描述了 3 名出现双侧虹膜色素脱失的 VKH 患者的临床特征。

结果

患者诊断延迟,慢性复发性 VKH 治疗不充分。患者 1 表现为双侧多个连续的小肉芽肿性虹膜基质病变,严重弥漫性虹膜肿胀,根部附近更严重,导致周边虹膜起伏。随后出现弥漫性虹膜萎缩和周边色素脱失。患者 2 表现为周边虹膜色素脱失、睫状区虹膜基质萎缩和瞳孔括约肌保留,呈现“向日葵外观”。患者 3 则出现广泛的虹膜色素脱失、萎缩和透照缺陷,累及整个虹膜。

结论

未经控制的双侧弥漫性肉芽肿性基质虹膜炎症会导致随后的色素脱失、透照缺陷和萎缩,从周边虹膜开始。瞳孔括约肌最初不受影响,但可能会发展为完全的虹膜萎缩。

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