Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
Chin Med J (Engl). 2019 Jul 20;132(14):1723-1732. doi: 10.1097/CM9.0000000000000325.
Cryoglobulinemia often causes systemic vasculitis, thereby damaging to skin and internal organs including kidneys, even life-threatening. This review aimed to introduce the advances in understanding, detection, and treatment of this disease in recent years, with a particular concern to clinical practice.
All the data in this review were from the English or Chinese literature in the PubMed and China National Knowledge Infrastructure databases as of March 2019.
This review selected important original articles, meaningful reviews, and some reports on cryoglobulinemia published in recent years and in history, as well as the guidelines for treatment of underlying diseases which lead to cryoglobulinemia.
Diagnosis of cryoglobulinemia relies on serum cryoglobulin test, in which to ensure that the blood sample temperature is not less than 37°C in the entire pre-analysis phase is the key to avoid false negative results. Cryoglobulinemic vasculitis (Cryo Vas), including cryoglobulinemic glomerulonephritis (Cryo GN), usually occurs in types II and III mixed cryoglobulinemia, and can also be seen in type I cryoglobulinemia caused by monoclonal IgG3 or IgG1. Skin purpura, positive serum rheumatoid factor, and decreased serum levels of C4 and C3 are important clues for prompting types II and III Cryo Vas. Renal biopsy is an important means for diagnosis of Cryo GN, while membranous proliferative GN is the most common pathological type of Cryo GN. In recent years, great advances have been made in the treatment of Cryo Vas and its underlying diseases, and this review has briefly introduced these advances.
Laboratory examinations of serum cryoglobulins urgently need standardization. The recent advances in the diagnosis and treatment of Cryo Vas and GN need to be popularized among the clinicians in related disciplines.
冷球蛋白血症常导致系统性血管炎，从而损害皮肤和包括肾脏在内的内脏器官，甚至危及生命。本综述旨在介绍近年来对该病的认识、检测和治疗进展，特别关注临床实践。
本综述中的所有数据均来自 2019 年 3 月之前PubMed 和中国知网数据库中发表的英文或中文文献。
本综述选择了近年来及历史上发表的有关冷球蛋白血症的重要原始文章、有意义的综述以及一些报告，以及导致冷球蛋白血症的基础疾病治疗指南。
冷球蛋白血症的诊断依赖于血清冷球蛋白检测，在整个分析前阶段确保血液样本温度不低于 37°C 是避免假阴性结果的关键。冷球蛋白血管炎（Cryo Vas），包括冷球蛋白性肾小球肾炎（Cryo GN），通常发生在 II 型和 III 型混合性冷球蛋白血症中，也可见于由单克隆 IgG3 或 IgG1 引起的 I 型冷球蛋白血症。皮肤紫癜、血清类风湿因子阳性和血清 C4、C3 水平降低是提示 II 型和 III 型 Cryo Vas 的重要线索。肾活检是诊断 Cryo GN 的重要手段，而膜性增生性 GN 是 Cryo GN 最常见的病理类型。近年来，冷球蛋白血管炎及其基础疾病的治疗取得了重大进展，本综述对此进行了简要介绍。
血清冷球蛋白的实验室检查急需标准化。近年来冷球蛋白血管炎和 GN 的诊断和治疗进展需要在相关学科的临床医生中推广。
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