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本月病例 2:一例贝切特病:70%有眼部受累。

Lessons of the month 2: A case of Behçet's disease: 70% have ophthalmic involvement.

机构信息

St Peter's Hospital, Chertsey, UK.

Royal Surrey County Hospital, Guildford, UK

出版信息

Clin Med (Lond). 2019 Nov;19(6):519-522. doi: 10.7861/clinmed.2019.0149. Epub 2019 Oct 22.

DOI:10.7861/clinmed.2019.0149
PMID:31732597
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6899251/
Abstract

A 34-year-old man presented to the emergency department with acute painless loss of vision of the left eye. Past medical history included painful lumps in the legs and frequent mouth ulcers, which were undiagnosed. The patient's visual acuity was 6/5 and counting fingers in the right and left eye, respectively. There were extensive intraretinal haemorrhages and venous sheathing in the superior quadrant of the left eye with associated disc oedema. The case was discussed in a multidisciplinary team meeting in the presence of ophthalmology, dermatology and immunology and a diagnosis of Behçet's disease was reached. The patient was commenced on intravenous methylprednisolone for 3 days followed by a switch to oral prednisolone. Due to recalcitrant uveitis, an intravitreal dexamethasone implant was administered. Eventually, systemic azathioprine and infliximab were commenced with frequent review by ophthalmology and immunology. The macular oedema improved but, unfortunately, the patient's visual acuity did not recover. Behçet's disease is a complex vasculitis involving multiple organ systems. Ocular manifestations can occur in 70% of patients, comprising retinal vasculitis, anterior uveitis, iridocyclitis, chorioretinitis, scleritis, keratitis, vitreous haemorrhage, optic neuritis, conjunctivitis, retinal vein occlusion and retinal neovascularisation. A tailored multidisciplinary approach is required, with corticosteroids being the mainstay of treatment.

摘要

一位 34 岁男性因左眼无痛性视力丧失而到急诊就诊。既往病史包括腿部疼痛性肿块和频繁的口腔溃疡,但未明确诊断。患者右眼视力为 6/5,左眼视力分别为数指。左眼上方象限有广泛的视网膜内出血和静脉鞘,伴有视盘水肿。在眼科、皮肤科和免疫学多学科团队会议上讨论了该病例,诊断为贝赫切特病。患者开始接受静脉注射甲基强的松龙 3 天,然后改为口服强的松龙。由于葡萄膜炎顽固,给予玻璃体内地塞米松植入物。最终,开始使用系统性硫唑嘌呤和英夫利昔单抗治疗,并由眼科和免疫学频繁复查。黄斑水肿改善,但不幸的是,患者的视力未恢复。贝赫切特病是一种涉及多个器官系统的复杂血管炎。70%的患者会出现眼部表现,包括视网膜血管炎、前葡萄膜炎、虹膜炎、脉络膜炎、巩膜炎、角膜炎、玻璃体积血、视神经炎、结膜炎、视网膜静脉阻塞和视网膜新生血管形成。需要采用量身定制的多学科方法,皮质类固醇是主要的治疗方法。

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本文引用的文献

1
An update on the use of biologic therapies in the management of uveitis in Behçet's disease: a comprehensive review.白塞病葡萄膜炎治疗中生物疗法应用的最新进展:一项综合综述
Orphanet J Rare Dis. 2017 Jul 17;12(1):130. doi: 10.1186/s13023-017-0681-6.
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Update on the therapy of Behçet disease.白塞病治疗的最新进展。
Ther Adv Chronic Dis. 2014 May;5(3):112-34. doi: 10.1177/2040622314523062.
3
Behcet's disease: systemic and ocular manifestations.贝赫切特病:全身表现和眼部表现。
Biomed Res Int. 2013;2013:247345. doi: 10.1155/2013/247345. Epub 2013 Oct 3.
4
Behçet's disease.贝赫切特病。
Orphanet J Rare Dis. 2012 Apr 12;7:20. doi: 10.1186/1750-1172-7-20.
5
Histopathological Evaluation of Behçet's Disease and Identification of New Skin Lesions.白塞病的组织病理学评估及新皮肤病变的鉴定
Patholog Res Int. 2012;2012:209316. doi: 10.1155/2012/209316. Epub 2011 Oct 18.
6
New Evidence-Based Treatment Approach in Behçet's Disease.白塞病新的循证治疗方法
Patholog Res Int. 2012;2012:871019. doi: 10.1155/2012/871019. Epub 2011 Oct 5.
7
Behçet disease: visual prognosis and factors influencing the development of visual loss.白塞病:视觉预后和影响视力丧失发展的因素。
Am J Ophthalmol. 2011 Dec;152(6):1059-66. doi: 10.1016/j.ajo.2011.05.032. Epub 2011 Aug 26.
8
Major vessel involvement in Behçet's disease: an update.大血管受累的白塞病:更新。
Curr Opin Rheumatol. 2011 Jan;23(1):24-31. doi: 10.1097/BOR.0b013e3283410088.
9
Behçet's disease: global epidemiology of an Old Silk Road disease.白塞病:古丝绸之路疾病的全球流行病学
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10
Mucocutaneous lesions of Behcet's disease.白塞病的皮肤黏膜病变
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