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[冷球蛋白与冷球蛋白血症性血管炎]

[Cryoglobulins and cryoglobulinemic vasculitis].

作者信息

Suszek Dorota, Majdan Maria

机构信息

Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin, Polska.

出版信息

Wiad Lek. 2018;71(1 pt 1):59-63.

Abstract

Cryoglobulinemia is defined as the presence of cryoglobulins in the blood. Cryoglobulinemia is often observed in the course of many diseases (infection, hematological disorders, autoimmune disorders) or has an idiopathic character. The classification of cryoglobulinemia is based on the immunological analysis of cryoglobulins and the activity of the rheumatoid factor (RF). The presence of cryoglobulins may induce cryoglobulinemic vasculitis (CV) which manifests with skin changes, arthritis and the dysfunction of internal organs. Diagnosis of cryoglobulinemia refers to the detection of cryoprecipitate in the blood serum sample. In the treatment of cryoglobulinemia various groups of medications may be used: corticosteroids, cyclophosphamide, azathioprine and rituximab. The purpose of the treatment is to reduce the production of cryoglobulins and the inflammation caused by their presence. Additionally, the treatment of organs complication is also crucial. If the viral infection is the causative agent of vasculitis, combination therapy will be recommended: antiviral and immunosuppressive therapy.

摘要

冷球蛋白血症的定义为血液中存在冷球蛋白。冷球蛋白血症常在许多疾病(感染、血液系统疾病、自身免疫性疾病)过程中出现,或具有特发性。冷球蛋白血症的分类基于冷球蛋白的免疫学分析和类风湿因子(RF)的活性。冷球蛋白的存在可能诱发冷球蛋白血症性血管炎(CV),其表现为皮肤改变、关节炎和内脏功能障碍。冷球蛋白血症的诊断是指在血清样本中检测到冷沉淀。在冷球蛋白血症的治疗中,可使用各类药物:皮质类固醇、环磷酰胺、硫唑嘌呤和利妥昔单抗。治疗的目的是减少冷球蛋白的产生及其所致的炎症。此外,器官并发症的治疗也至关重要。如果病毒感染是血管炎的致病因素,则推荐联合治疗:抗病毒和免疫抑制治疗。

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