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[复发性慢性葡萄膜大脑炎综合征还是结节病?一个令人沮丧的诊断]

[Recurrent chronic Vogt-Koyanagi-Harada syndrome or sarcoidosis? A Gloomy diagnosis].

作者信息

Gómez-Piña Juan José, Vázquez-Hernández Adriana Elizabeth, Flores-Flores Nayeli, Cortés-Luis Marco Antonio, Olvera-Acevedo Arturo

机构信息

Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Servicio de Medicina Interna. Ciudad de México, México.

Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital General "Dr. Gaudencio González Garza", Servicio de Oftalmología. Ciudad de México, México.

出版信息

Rev Med Inst Mex Seguro Soc. 2023 Sep 4;61(5):702-706. doi: 10.5281/zenodo.8316491.

DOI:10.5281/zenodo.8316491
PMID:37773199
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10599787/
Abstract

BACKGROUND

Vogt-Koyanagi-Harada syndrome (VKH) is a systemic disease that affects organs profuse in melanocytes, presenting with a chronic and diffuse bilateral granulomatous panuveitis, as well as neurological, auditory, and cutaneous manifestations. In this article, a systematic approach is presented for the diagnostic management of VKH syndrome, considering relevant diagnostic possibilities to rule out other entities that manifest similar symptoms.

CLINICAL CASE

71-year-old man with a long-standing history of vitiligo, who experienced visual loss in his right eye 6 months before his admission, along with bilateral hearing loss predominantly in the right ear. During his hospitalization, he presented with chronic headache, fever, and significant involuntary weight loss. Ophthalmological examination revealed that his right eye only perceived light and had hyperemic bulbar conjunctiva, while the left eye had a visual acuity of 20/200. The right fundus had scattered pigmentation, while the left had an edematous optic disc and right optic atrophy.

CONCLUSIONS

The diagnosis of VKH syndrome is established by the presence of the 5 diagnostic criteria for complete disease, including retinal depigmentation, neurological alterations, and dermatological manifestations. Since patients can present with a wide variety of symptoms, initial differential diagnosis should be considered, which represents a diagnostic challenge.

摘要

背景

伏格特-小柳-原田综合征(VKH)是一种累及富含黑素细胞器官的全身性疾病,表现为慢性、双侧弥漫性肉芽肿性全葡萄膜炎,以及神经、听觉和皮肤表现。本文介绍了一种VKH综合征诊断管理的系统方法,考虑了相关诊断可能性以排除表现出类似症状的其他疾病。

临床病例

一名71岁男性,有长期白癜风病史,入院前6个月右眼视力丧失,同时伴有以右耳为主的双侧听力丧失。住院期间,他出现慢性头痛、发热和明显的非自愿体重减轻。眼科检查发现,他的右眼仅能感知光线,球结膜充血,而左眼视力为20/200。右眼眼底有散在色素沉着,而左眼视盘水肿,右眼视神经萎缩。

结论

VKH综合征的诊断依据完整疾病的5项诊断标准,包括视网膜色素脱失、神经改变和皮肤表现。由于患者可能出现各种各样的症状,应考虑进行初步鉴别诊断,这是一项诊断挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/08785dacc276/04435117-61-5-702-c001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/8cb910710340/04435117-61-5-702-f001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/bdacd2d873f5/04435117-61-5-702-f002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/08785dacc276/04435117-61-5-702-c001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/8cb910710340/04435117-61-5-702-f001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/bdacd2d873f5/04435117-61-5-702-f002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af53/10599787/08785dacc276/04435117-61-5-702-c001.jpg

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本文引用的文献

1
Long-term Outcomes of Uveitis Associated with Vogt-Koyanagi-Harada Disease in the Pediatric Age Group.小儿年龄组中与Vogt-小柳-原田病相关的葡萄膜炎的长期预后
Ocul Immunol Inflamm. 2022 Oct-Nov;30(7-8):1669-1677. doi: 10.1080/09273948.2021.1933074. Epub 2021 Jun 29.
2
Precise, simplified diagnostic criteria and optimised management of initial-onset Vogt-Koyanagi-Harada disease: an updated review.初发性 Vogt-小柳-原田病的精确简化诊断标准和优化管理:最新综述。
Eye (Lond). 2022 Jan;36(1):29-43. doi: 10.1038/s41433-021-01573-3. Epub 2021 Jun 18.
3
Melanin change of retinal pigment epithelium and choroid in the convalescent stage of Vogt-Koyanagi-Harada disease.
伏格特-小柳-原田病恢复期视网膜色素上皮和脉络膜的黑色素变化
Int J Ophthalmol. 2020 Dec 18;13(12):1928-1932. doi: 10.18240/ijo.2020.12.13. eCollection 2020.
4
Unique clinical spectrum with distinguishing diagnostic features in Vogt-Koyanagi-Harada syndrome.Vogt-小柳-原田综合征具有独特的临床谱及鉴别诊断特征。
BMJ Case Rep. 2019 Dec 29;12(12):e231397. doi: 10.1136/bcr-2019-231397.
5
Development and Evaluation of Diagnostic Criteria for Vogt-Koyanagi-Harada Disease.Vogt-Koyanagi-Harada 病诊断标准的制定与评估。
JAMA Ophthalmol. 2018 Sep 1;136(9):1025-1031. doi: 10.1001/jamaophthalmol.2018.2664.
6
High rate of clinical recurrence in patients with Vogt-Koyanagi-Harada disease treated with early high-dose corticosteroids.早期大剂量皮质类固醇治疗 Vogt-Koyanagi-Harada 病患者的临床复发率高。
Graefes Arch Clin Exp Ophthalmol. 2015 May;253(5):785-90. doi: 10.1007/s00417-014-2904-z. Epub 2015 Jan 16.
7
Indocyanine green angiography-guided management of Vogt-Koyanagi-Harada disease: differentiation between choroidal scars and active lesions.吲哚菁绿血管造影引导下Vogt-小柳-原田病的治疗:脉络膜瘢痕与活动性病变的鉴别
Int Ophthalmol. 2013 Oct;33(5):571-7. doi: 10.1007/s10792-012-9692-4. Epub 2013 Jan 1.
8
Indocyanine green angiography findings in patients with long-standing Vogt-Koyanagi-Harada disease: a cross-sectional study.久存性 Vogt-小柳原田病患者的吲哚菁绿血管造影检查结果:一项横断面研究。
BMC Ophthalmol. 2012 Aug 13;12:40. doi: 10.1186/1471-2415-12-40.
9
Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease.次优治疗可控制伏格特-小柳-原田病的临床显性疾病,但无法控制其亚临床进展。
Int Ophthalmol. 2010 Feb;30(1):41-50. doi: 10.1007/s10792-008-9288-1. Epub 2009 Jan 17.
10
Prognostic factors of Vogt-Koyanagi-Harada disease in Singapore.新加坡Vogt-小柳-原田病的预后因素
Am J Ophthalmol. 2009 Jan;147(1):154-161.e1. doi: 10.1016/j.ajo.2008.07.044. Epub 2008 Oct 2.