Internal Medicine Department, Centro Hospitalar Universitário de São João, Porto, Portugal.
Internal Medicine Department, Centro Hospitalar Universitário de São João, Porto, Portugal.
J Autoimmun. 2019 Dec;105:102313. doi: 10.1016/j.jaut.2019.102313. Epub 2019 Aug 2.
Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis. The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative, autoimmune diseases and hepatitis C virus infection. The diagnosis of cryoglobulinemic syndrome is predominantly based on the presence of clinical features and laboratorial demonstration of serum cryoglobulins. The treatment strategy depends on the cause of cryoglobulinemia. For patients with chronic HCV infection, antiviral therapy is indicated. Immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening manifestations. In this review, we discuss the main clinical presentations, diagnostic approach and treatment options.
冷球蛋白是在低温下沉淀并在复温后溶解的抗体。冷球蛋白血症是指循环冷球蛋白的存在，通常导致以疲劳、关节痛、紫癜、溃疡、神经病和/或肾小球肾炎为特征的全身性炎症综合征。该疾病主要累及中小血管，并因含冷球蛋白的免疫复合物导致血管炎。根据免疫球蛋白成分，冷球蛋白血症分为三型（I、II 和 III 型）。易患情况包括淋巴增生性疾病、自身免疫性疾病和丙型肝炎病毒感染。冷球蛋白血症的诊断主要基于临床特征和血清冷球蛋白的实验室证据。治疗策略取决于冷球蛋白血症的病因。对于慢性 HCV 感染患者，建议进行抗病毒治疗。对于有器官威胁表现的患者，可保留免疫抑制或免疫调节治疗，包括类固醇、血浆置换和细胞毒性药物。在这篇综述中，我们讨论了主要的临床表现、诊断方法和治疗选择。
