Jari Mohsen, Mohammadi Taiiebeh, Taheri Ensiyeh
Department of Pediatric Rheumatology, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
Department of Pediatric, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
Case Rep Rheumatol. 2020 Oct 6;2020:8834399. doi: 10.1155/2020/8834399. eCollection 2020.
Behçet disease (BD) is a polygenic and chronic autoinflammatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children. . We reported an 8-year-old girl with a sudden visual loss and color vision impairment. The patient had a history of recurrent oral aphthous ulcers, genital ulcers, and chronic abdominal pain. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal. The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands and auto-antibodies were also normal. Pathergy skin test and human leukocyte antigens (HLA) B5 and HLA-B51 were positive. The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroids, azathioprine, colchicine, and infliximab.
Retrobulbar optic neuritis may be the first manifestation of neuro-BD.
白塞病(BD)是一种多基因慢性自身炎症性多系统血管炎。急性视神经炎在BD患者中很少见,尤其是儿童。我们报告了一名8岁突发视力丧失和色觉障碍的女孩。该患者有复发性口腔溃疡、生殖器溃疡和慢性腹痛病史。眼科检查显示双眼前房、后房及眼底正常。感染性和风湿性疾病的实验室检查结果正常。脑磁共振成像及脑脊液寡克隆带和自身抗体分析结果也正常。针刺反应皮肤试验及人类白细胞抗原(HLA)B5和HLA - B51呈阳性。该患者被诊断为BD相关的双侧球后视神经炎,并接受了皮质类固醇、硫唑嘌呤、秋水仙碱和英夫利昔单抗治疗。
球后视神经炎可能是神经白塞病的首发表现。