白塞氏综合征的眼部表现

Dumitrache Marieta, Lascu Rodica
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摘要

白塞氏综合征是一种炎症性、慢性、复发性、多系统疾病,常见于25-40岁的男性,在感染性(细菌)触发因子和HLA-B51存在的遗传参与下具有免疫决定论(包括自身免疫)。白塞氏综合征的特点是症状三联性:复发性非肉芽肿性葡萄膜炎伴垂体、口腔和生殖器溃疡以及皮肤改变。虹膜睫状体炎(前葡萄膜炎)伴急性、复发性垂体功能低下是白塞综合征最常见的眼部表现,并发展为不可预测的爆发,伴有后遗症和严重并发症,可导致视力丧失。白塞综合征的眼部表现还伴有后极受累,包括后葡萄膜炎、静脉和动脉闭塞性血管炎、坏死性视网膜炎、黄斑水肿、囊样黄斑水肿,这些都加重了视力的下降/丧失。白塞综合征的治疗应适应疾病的慢性进程,从一开始就应通过全身药物组合进行积极治疗:皮质类固醇/免疫抑制/免疫调节治疗和适当的眼科治疗:睫状体麻痹、抗青光眼滴眼液、滤光手术、白内障手术、可的松保护下的白内障手术、腔内手术、激光光凝。眼科医生和内科医生需要跨学科合作。
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Eye Manifestations in Behcet’s Syndrome
Behcet’s syndrome is an inflammatory, chronic, recurrent, multisystemic disease, common in men 25-40 years of age, with immunological determinism (including autoimmune) in the presence of infectious (bacterial) triggering agents and genetic involvement through the presence of HLA-B51. Behcet’s syndrome is characterized by a symptomatic triad of: recurrent nongranulomatous uveitis with hypopyon, oral and genital ulcers, and skin changes. Iridocyclitis (anterior uveitis) with acute, recurrent hypopyon is the most common ocular manifestation in Behcet’s syndrome and progresses to unpredictable outbreaks with sequelae and severe complications that cause loss / loss of vision. Ocular manifestations in Behcet’s syndrome are also accompanied by posterior pole involvement with posterior uveitis, venous and arterial occlusive vasculitis, necrotizing retinitis, macular edema, cystoid macular edema, which accentuates the decrease / loss of vision.Treatment of Behcet’s syndrome should be adapted to the chronic course of the disease and should be aggressive from the outset through systemic drug combinations: corticosteroid / immunosuppressive / immunomodulatory therapy and appropriate ophthalmic treatment: cycloplegics, antiglaucoma eye drops, filter surgery, cataract surgery, cataract surgery under cortisone protection, endoocular surgery, laser photocoagulation. Ophthalmologist / internist interdisciplinary collaboration is required.
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