Iannuzzella F, Garini G
Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università di Parma, Parma.
Reumatismo. 2008 Jul-Sep;60(3):163-73. doi: 10.4081/reumatismo.2008.163.
Cryoglobulinemia refers to the presence in serum of immunoglobulins, that reversibly precipitate at low temperatures. Cryoglobulins are classified according to their immunochemical properties as type I, composed of a single monoclonal immunoglobulin, and types II and III, referred as mixed cryoglobulinemia (MC), composed by a mixture of monoclonal (type II) and polyclonal (type III) IgM that have rheumatoid factor activity and bind to polyclonal IgGs. MC is a systemic vasculitis with cutaneous and multiple organ involvement including chronic hepatitis, membrano-proliferative glomerulonephritis, and peripheral neuropathy. In more than 90% of patients, MC is associated with chronic hepatitis C virus (HCV) infection, which is considered the triggering factor of the disease. Patients with HCV-related MC may be managed by means of etiological, pathogenetic or symptomatic therapeutic modalities. The choice of the more appropriate treatment is strictly related to the assessment of disease activity, and to the extent and severity of organ involvement. This paper reviews the currently available therapeutic strategies for MC syndrome, emphasizing the importance of HCV eradication, and the safety/efficacy of new biologic therapies for selective control of cryoglobulin-producing B-cells.
冷球蛋白血症是指血清中存在的免疫球蛋白，其在低温下会可逆性沉淀。冷球蛋白根据其免疫化学性质分为I型，由单一单克隆免疫球蛋白组成；II型和III型，称为混合性冷球蛋白血症（MC），由具有类风湿因子活性并与多克隆IgG结合的单克隆（II型）和多克隆（III型）IgM混合物组成。MC是一种系统性血管炎，累及皮肤和多个器官，包括慢性肝炎、膜增生性肾小球肾炎和周围神经病变。在90%以上的患者中，MC与慢性丙型肝炎病毒（HCV）感染有关，HCV感染被认为是该疾病的触发因素。HCV相关MC患者可通过病因、发病机制或对症治疗方式进行管理。选择更合适的治疗方法与疾病活动度评估以及器官受累的程度和严重程度密切相关。本文综述了目前可用的MC综合征治疗策略，强调了根除HCV的重要性以及新型生物疗法对选择性控制产生冷球蛋白的B细胞的安全性/有效性。
