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透析相关淀粉样变性

Authors
Jonathan Kay, MD
William L Henrich, MD, MACP
Wajeh Y Qunibi, MD
Section Editors
Jeffrey S Berns, MD
Steve J Schwab, MD
Deputy Editor
Alice M Sheridan, MD
Translators
王利华, 教授

引言

透析相关淀粉样变性(dialysis-related amyloidosis, DRA)是慢性肾脏病(chronic kidney disease, CKD)患者的一种致残性疾病，以β2-微球蛋白(beta2-microglobulin, β2-m)组成的淀粉样纤维蓄积和在骨、关节周围结构和内脏中沉积为特征[1-8]。β2-m是表达于细胞表面的主要组织相容性复合物的一种成分，正常情况下的清除方式为通过肾小球滤过，之后在近端小管重吸收和分解代谢。肾功能减退患者的清除能力下降，从而可导致其在血浆中蓄积和在组织中缓慢沉积。

随着高通量生物相容性膜的使用，DRA的患病率有所下降，因为这种膜可提供更好的β2-m清除作用，且不容易引起反应性炎症。 (参见下文‘流行病学和危险因素’)

然而，即使是使用这些更新型的血液透析治疗也可伴有β2-m的潴留。本专题将对DRA进行概述。与肾脏病相关的其他骨病和其他并发症将在别处讨论。 (参见“慢性肾脏病矿物质和骨异常(CKD-MBD)概述”和“慢性肾病透析前成人患者继发性甲状旁腺功能亢进及矿物质代谢异常的处理”和“透析患者继发性甲状旁腺功能亢进症和矿物质代谢异常的治疗”和“年龄较大患者血液透析的并发症”)

淀粉样变性的其他类型也将在别处讨论。 (参见“淀粉样变性概述”)

概述

DRA的特征为淀粉样蛋白在组织中沉积，尤其是在骨骼、关节软骨、滑膜、肌肉、肌腱及韧带内[9-11]。与原发性淀粉样变性中的免疫球蛋白轻链片段和继发性淀粉样变性中的血清淀粉样蛋白A不同，DRA中的淀粉样蛋白主要是由β2-m组成[2,12,13]。

                  

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Literature review current through: 2017-06 . | This topic last updated: 2016-07-01.
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