Cryoglobulinemia vasculitis: an update
Cryoglobulinemia vasculitis: an update
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冷球蛋白血症性血管炎：更新
DOI：
10.1097/bor.0b013e32835b15f7
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发表时间：
2013
影响因子：
5.1
通讯作者：
P. Cacoub
中科院分区：
文献类型：
--
作者：
B. Terrier;P. Cacoub
综述目的冷球蛋白血症血管炎（CryoVas）是一种与慢性感染[特别是丙型肝炎病毒（HCV）]、自身免疫性疾病和B细胞淋巴组织增生性疾病相关的小血管炎。本文就其诊断、预后及治疗的最新研究进展作一综述。最近的发现大量HCV阳性和阴性混合CryoVas患者和单克隆I型CryoVas患者描述了HCV筛查时代CryoVas患者的表现和预后。CryoVas领域的欧洲专家为其诊断制定了新的分类标准。最后，法国、意大利和北美的临床研究表明，与单独使用皮质类固醇或其他免疫抑制剂的治疗相比，以利妥昔单抗为基础的治疗方案非常有效。然而，利妥昔单抗似乎与一个子集的患者严重感染的风险增加。最近的研究确定了生存的预后因素，并证明利妥昔单抗是非常有效的，但仍然与一个子集的患者严重感染。这些结果可以支持根据临床模式和相关合并症进行个体治疗分层。
Purpose of reviewCryoglobulinemia vasculitis (CryoVas) is a small-vessel vasculitis associated with chronic infections [in particular hepatitis C virus, (HCV)], autoimmune disorders and B-cell lymphoproliferative disorders. The most recent studies on its diagnosis, prognosis and therapeutic management are reviewed here. Recent findingsLarge series of patients with HCV-positive and negative mixed CryoVas and patients with monoclonal type I CryoVas have described the presentation and the prognosis of patients with CryoVas in the era of HCV screening. European experts in the field of CryoVas developed new classification criteria for its diagnosis. Finally, French, Italian and North American clinical studies demonstrated that rituximab-based regimens were highly effective in comparison with corticosteroids alone or other immunosuppressive agents-based therapy. However, rituximab seems to be associated with an increased risk of severe infections in a subset of patients. SummaryRecent studies identified prognostic factors of survival and demonstrated that rituximab is highly effective but remains associated with severe infections in a subset of patients. These results could support individual therapeutic stratification according to the clinical pattern and associated comorbidities.