Canberra Health Services Library, ACT Government, Garren, Australian Capital Territory, Australia
Ophthalmology Unit, Canberra Health Services, Garran, Australian Capital Territory, Australia.
BMJ Case Rep. 2021 May 10;14(5):e240041. doi: 10.1136/bcr-2020-240041.
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterised by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. The authors present the case of a 70-year-old man who presented to the ophthalmology clinic with bilateral scleritis and ocular hypertension. He was diagnosed with HUVS after a 6-month period of bilateral scleritis, vestibulitis, significant weight loss, mononeuritis multiplex and recurrent urticarial vasculitis with pronounced persistent hypocomplementemia and the presence of anti-C1q antibodies. Disease control was eventually obtained with mycophenolate and prednisolone.
低补体血症性荨麻疹性血管炎综合征（HUVS）是一种罕见的自身免疫性疾病，其特征为反复出现荨麻疹性皮损和获得性低补体血症伴全身表现。作者报告了一例 70 岁男性患者，因双侧巩膜炎和眼内压升高而就诊于眼科诊所。在经历了 6 个月的双侧巩膜炎、前庭神经炎、显著体重减轻、多发性单神经炎和复发性荨麻疹性血管炎后，他被诊断为 HUVS，表现为明显持续性低补体血症和抗 C1q 抗体。最终，霉酚酸酯和泼尼松龙控制了疾病。
