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[IgA肾病]

[IgA nephropathy].

作者信息

Floege Jürgen

机构信息

Medizinische Klinik II, Uniklinik der RWTH Aachen, Pauwelsstr. 30, 52074, Aachen, Deutschland.

出版信息

Inn Med (Heidelb). 2023 Oct;64(10):961-969. doi: 10.1007/s00108-023-01588-w. Epub 2023 Sep 6.

Abstract

IgA nephropathy (IgAN) is the most frequent primary form of glomerulonephritis. The origin of IgAN is only partially understood and appears to involve the occurrence of IgA, which is normally secreted by mucous membranes, in the circulation followed by its glomerular deposition and inflammatory changes. Clinically, IgAN mostly follows an inapparent course and the disease is often only first diagnosed by kidney biopsy when kidney function disorders are already manifested. Key prognostic indicators include the extent of proteinuria and the already manifested evidence of irreversible kidney damage. Treatment includes supportive measures. The effectiveness of high-dose systemic corticosteroid treatment in European patients is uncertain and controversial due to the adverse side effects. Nefecon (encapsulated budesonide) is the first specific drug licensed for treatment of high risk IgAN patients. A number of further approaches are currently in clinical trials.

摘要

IgA肾病(IgAN)是肾小球肾炎最常见的原发性形式。IgA肾病的发病原因仅部分为人所知,似乎涉及通常由黏膜分泌的IgA在循环中出现,随后在肾小球沉积并引发炎症变化。临床上,IgA肾病大多呈隐匿性病程,该病往往在肾功能障碍已经显现时才首次通过肾活检确诊。关键的预后指标包括蛋白尿的程度以及已出现的不可逆肾损伤证据。治疗措施包括支持性治疗。由于存在副作用,高剂量全身皮质类固醇治疗对欧洲患者的有效性尚不确定且存在争议。Nefecon(肠溶布地奈德)是首个获批用于治疗高危IgA肾病患者的特效药物。目前还有一些其他治疗方法正在进行临床试验。

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