Jachiet M, Flageul B, Bouaziz J-D, Bagot M, Terrier B
Service de dermatologie, faculté de médecine, hôpital Saint-Louis, université Paris-Diderot, Assistance publique-Hôpitaux de Paris, 75010 Paris, France.
Service de médecine interne, faculté de médecine, centre de référence national pour les maladies auto-immunes et systémiques rares, en particulier les vascularites systémiques, hôpital Cochin, université Paris-Descartes, Assistance publique-Hôpitaux de Paris, 75014 Paris, France.
Rev Med Interne. 2018 Feb;39(2):90-98. doi: 10.1016/j.revmed.2017.03.005. Epub 2017 Apr 28.
Hypocomplementemic urticarial vasculitis (HUV), called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, is a rare systemic vasculitis of unknown etiology, affecting small vessels. HUV is characterized by urticarial lesions, hypocomplementemia and systemic manifestations, mostly musculoskeletal and ocular, but also gastrointestinal, pulmonary and kidney involvement. Anti-C1q antibodies are detected in only half of the patients, and low C1q seems to represent a more sensitive marker. Published data on the therapeutic management are scarce in the literature. Hydroxychloroquine and colchicine seem to represent effective first-line therapies. In patient with relapsing or refractory disease, response rates appeared slightly higher for corticosteroids together with conventional immunosuppressive agents, in particular azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. The best strategy for treating HUV has yet to be defined.
低补体血症性荨麻疹性血管炎（HUV），在2012年修订的国际 Chapel Hill 共识会议血管炎命名法中被称为抗C1q血管炎，是一种病因不明的罕见系统性血管炎，累及小血管。HUV的特征是荨麻疹样皮疹、低补体血症和全身表现，主要是肌肉骨骼和眼部表现，但也可累及胃肠道、肺部和肾脏。仅半数患者可检测到抗C1q抗体，而低C1q似乎是一个更敏感的标志物。文献中关于治疗管理的已发表数据很少。羟氯喹啉和秋水仙碱似乎是有效的一线治疗药物。对于复发或难治性疾病患者，糖皮质激素联合传统免疫抑制剂，特别是硫唑嘌呤、霉酚酸酯或环磷酰胺，有效率似乎略高，而基于利妥昔单抗的方案往往疗效更高。治疗HUV的最佳策略尚未明确。
