Josep Font Laboratory of Autoimmune Diseases, Institut Clínic de Medicina I Dermatologia, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain.
Lancet. 2012 Jan 28;379(9813):348-60. doi: 10.1016/S0140-6736(11)60242-0. Epub 2011 Aug 23.
Cryoglobulins are immunoglobulins that precipitate in vitro at temperatures less than 37°C and produce organ damage through two main pathways: vascular sludging (hyperviscosity syndrome, mainly in type I cryoglobulinaemia) and immune-mediated mechanisms (principally vasculitis, in mixed cryoglobulinaemia). Cryoglobulinaemia is associated with many illnesses, which can be broadly grouped into infections, autoimmune disorders, and malignancies; the most common cause is infection with hepatitis C virus. Mixed cryoglobulinaemic syndrome is diagnosed when a patient has typical organ involvement (mainly skin, kidney, or peripheral nerve) and circulating cryoglobulins. Cutaneous purpura is the most common manifestation of cryoglobulinaemic vasculitis. The most frequently affected internal organs are the peripheral nerves, kidneys, and joints. The course varies widely and prognosis is influenced by both cryoglobulinaemic damage to vital organs and by comorbidities associated with underlying diseases. More than 90% of cases of cryoglobulinaemia have a known underlying cause; therefore treatment is focused on the cause of the disorder rather than merely symptomatic relief. Studies suggest that both combined or sequential antiviral therapies and targeted biological treatments might be more effective than monotherapy.
冷球蛋白是指在低于 37°C 的温度下体外沉淀的免疫球蛋白，通过两种主要途径产生器官损伤：血管淤滞（主要见于 I 型冷球蛋白血症）和免疫介导的机制（主要是血管炎，见于混合性冷球蛋白血症）。冷球蛋白血症与许多疾病相关，可以大致分为感染、自身免疫性疾病和恶性肿瘤；最常见的原因是丙型肝炎病毒感染。当患者出现典型的器官受累（主要是皮肤、肾脏或周围神经）和循环冷球蛋白时，即可诊断为混合性冷球蛋白血症综合征。皮肤紫癜是冷球蛋白血管炎最常见的表现。最常受累的内脏器官是外周神经、肾脏和关节。病程差异很大，预后既受重要器官冷球蛋白损害的影响，也受潜在疾病相关合并症的影响。超过 90%的冷球蛋白血症病例有明确的潜在病因；因此，治疗的重点是针对疾病的病因，而不仅仅是对症治疗。研究表明，联合或序贯抗病毒治疗和靶向生物治疗可能比单一疗法更有效。
