冷球蛋白性血管炎:文献回顾及临床病例

H. Palhuyeva, E. Makarenko, V.V. Lahutchau
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摘要

本工作的目的是分析文献来源的诊断和治疗的冷球蛋白性血管炎与我们自己的数据证明。冷球蛋白性血管炎(CV)是一个困难和罕见的诊断不仅对内科医生,而且对风湿病学家。该病临床表现多形性,可出现多脏器、多系统病变。模棱两可的实验室研究结果给该病的诊断带来了额外的困难。CV常与淋巴增生性疾病,如弥漫性大b细胞淋巴瘤或非霍奇金淋巴瘤、自身免疫性疾病和感染性疾病有关。病毒性丙型肝炎是CV最常见的潜在病因,约占所有病例的80%。在病因不明的情况下,使用术语“特发性”或“原发性”CV。对于CV的诊断,提出了分类标准,包括问卷调查结果、临床表现和实验室研究数据。CV患者的治疗原则取决于其发病机制和临床表现的严重程度。当CV发生的病因确定后,应给予致病因治疗。表现严重或危及生命的患者需要紧急干预以抑制免疫复合物的形成。这是通过免疫抑制治疗,包括糖皮质激素、细胞抑制剂、利妥昔单抗和血浆置换来实现的。
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CRYOGLOBULINEMIC VASCULITIS: LITERATURE REVIEW AND CLINICAL CASE
The purpose of this work was to analyze literature sources on the diagnosis and treatment of cryoglobulinemic vasculitis with the demonstration of our own data. Cryoglobulinemic vasculitis (CV) is a difficult and rare diagnosis not only for a physician, but also for a rheumatologist. The disease has polymorphic clinical manifestations and can debut with lesions of various organs and systems. Additional difficulties in the diagnosis of the disease are created by ambiguous laboratory research results. CV is often associated with lymphoproliferative diseases such as diffuse large B-cell lymphoma or non-Hodgkin’s lymphoma, autoimmune and infectious diseases. Viral hepatitis C is the most common underlying cause of CV and makes up approximately 80% of all cases. In case when the etiological factor is not established, the term «idiopathic» or «essential» CV is used. For the diagnosis of CV, classification criteria are proposed, including the results of the questionnaire, clinical manifestations and laboratory research data. The principles of treating patients with CV are determined by the etiopathogenetic mechanisms and the severity of the clinical manifestations of the disease. When the etiological factor of CV development is established, etiotropic therapy is administered. Patients with severe or life-threatening manifestations require urgent intervention to suppress the formation of immune complexes. This is achieved by means of immunosuppressive therapy including glucocorticosteroids, cytostatics, rituximab, and plasmapheresis.
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