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与白塞病相关的肌炎和巩膜炎:一种非典型表现。

Myositis and scleritis associated with Behcet's disease: an atypical presentation.

作者信息

Dursun Dilek, Akova Yonca, Yücel Eftal

机构信息

Department of Ophthalmology, Başkent University Medical School, Ankara, Turkey.

出版信息

Ocul Immunol Inflamm. 2004 Dec;12(4):329-32. doi: 10.1080/092739490500264.

Abstract

We report a case of an 18-year-old female, who was diagnosed as having systemic Behcet's disease with chief complaints of vision loss in the right eye and redness and pain in both eyes. In addition to an anterior necrotizing scleritis, she had several attacks of retrobulbar neuritis and lateral rectus myositis. She responded well to i.v. and topical prednisolone and her subsequent attacks were treated with i.v. cyclophosphamide (500 mg on presentation and on Days 7, 10, 12, 15, and 20; the cycle was repeated every 20 days). She benefited on this treatment and her vision improved dramatically. Her final acuity of 20/50 was thought to be due to presumed previous attacks of optic neuritis and anterior segment inflammation. During the five-month follow-up period, she had no other attacks. Ischemia and inflammation are common in Behcet's disease and sometimes thrombosis and vasculitic changes may cause a decrease in vision. In this case, the immunosuppressive therapy helped attenuate the inflammatory vasculitic episodes during the disease course.

摘要

我们报告一例18岁女性患者,她被诊断为系统性贝赫切特病,主要症状为右眼视力丧失以及双眼发红和疼痛。除了前部坏死性巩膜炎外,她还多次发作球后视神经炎和外直肌肌炎。她对静脉注射和局部使用泼尼松龙反应良好,随后的发作采用静脉注射环磷酰胺治疗(就诊时及第7、10、12、15和20天各注射500毫克;每20天重复一个周期)。她通过这种治疗获益,视力显著改善。她最终的视力为20/50,被认为是由于之前推测的视神经炎发作和前段炎症所致。在五个月的随访期内,她没有其他发作。缺血和炎症在贝赫切特病中很常见,有时血栓形成和血管炎改变可能导致视力下降。在该病例中,免疫抑制治疗有助于减轻病程中的炎症性血管炎发作。

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