UPMC Univ Paris 06, UMR 7211, F-75005, Paris, France.
Curr Opin Rheumatol. 2013 Jan;25(1):10-8. doi: 10.1097/BOR.0b013e32835b15f7.
Cryoglobulinemia 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.
Large 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.
Recent 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.
冷球蛋白血症性血管炎（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 细胞淋巴增生性疾病相关的小血管血管炎。本文回顾了其诊断、预后和治疗管理的最新研究。
大量丙型肝炎病毒阳性和阴性混合 CryoVas 患者以及单克隆 I 型 CryoVas 患者的系列研究描述了在 HCV 筛查时代 CryoVas 患者的表现和预后。CryoVas 领域的欧洲专家制定了新的诊断分类标准。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相比，基于利妥昔单抗的方案具有更高的疗效。然而，利妥昔单抗似乎与某些患者严重感染的风险增加有关。
最近的研究确定了生存的预后因素，并表明利妥昔单抗具有高度疗效，但在某些患者中仍与严重感染有关。这些结果可以支持根据临床模式和相关合并症进行个体化治疗分层。
