Division of Nephrology, University Health Network, ON, Canada; University of Toronto, ON, Canada; Bhumirajanagarindra Kidney Institute, Bangkok, Thailand.
University of Toronto, ON, Canada; Department of Laboratory Medicine and Pathobiology, University Health Network, Toronto General Hospital, ON, Canada.
Am J Kidney Dis. 2021 Sep;78(3):429-441. doi: 10.1053/j.ajkd.2021.01.024. Epub 2021 Jul 9.
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerular disease worldwide. The diagnostic histologic hallmark is dominant or codominant IgA staining on kidney biopsy; however, patients may present with various clinical syndromes ranging from asymptomatic abnormalities noted on urinalysis to rapidly progressive glomerulonephritis. Given substantial heterogeneity in the clinical course of disease, online risk calculators are available that may assist in prognostication and inform discussions with patients. Comprehensive supportive treatment is central in the initial therapy of IgAN; the additive benefit of currently available immunosuppressive agents remains an area of controversy. Although proteinuria is attenuated by the use of corticosteroids, the long-term benefits have been questioned, and the use of corticosteroids is associated with severe adverse effects, notably infection. Recent advances in our understanding of mucosal immunity and the role of the complement system in IgAN pathogenesis are leading to development of novel therapeutic options, which are being evaluated in ongoing clinical trials. In this installment of the AJKD Core Curriculum in Nephrology, IgAN pathogenesis, clinical manifestations, histology, prediction tools, and treatment are reviewed, and case examples are presented to illustrate the approach to the management of patients with IgAN.
免疫球蛋白 A 肾病 (IgAN) 是全球最常见的原发性肾小球疾病。诊断组织学的主要特征是肾活检时主要或共显性 IgA 染色；然而，患者可能表现出各种临床综合征，从尿液分析中发现的无症状异常到快速进行性肾小球肾炎不等。鉴于疾病的临床过程存在很大的异质性，现在有在线风险计算器可用于帮助预测，并为与患者的讨论提供信息。综合支持性治疗是 IgAN 初始治疗的核心；目前可用的免疫抑制剂的附加益处仍然存在争议。尽管皮质类固醇的使用可以减轻蛋白尿，但长期益处受到质疑，而且皮质类固醇的使用与严重的不良反应有关，特别是感染。我们对黏膜免疫和补体系统在 IgAN 发病机制中的作用的理解的最新进展正在导致新的治疗选择的发展，这些选择正在正在进行的临床试验中进行评估。在这一期 AJKD 肾脏病学核心课程中，我们回顾了 IgAN 的发病机制、临床表现、组织学、预测工具和治疗方法，并提供了病例示例，以说明 IgAN 患者管理的方法。
