Glomerular Center, Division of Kidney Diseases and Hypertension, Northwell Health, Great Neck.
University College London (UCL) Department of Renal Medicine, Royal Free Hospital, London, UK.
Adv Kidney Dis Health. 2024 May;31(3):206-215. doi: 10.1053/j.akdh.2024.04.007.
Anti-glomerular basement membrane disease is a small-vessel vasculitis involving the kidneys (∼90%) and the lungs (∼60%). Antibodies against the glomerular basement membrane are directly pathogenic in anti-glomerular basement membrane disease; however, recent research has highlighted the critical role of T cells. Novel autoantigens within the glomerular basement membrane are also now recognized. Atypical forms of the disease are reported along with preceding triggers, such as immune checkpoint inhibitors, immunomodulatory drugs, and vaccines. Kidney outcomes in anti-glomerular basement membrane disease remain poor despite significant improvement in patient survival in the last 2 to 3 decades. Treatment typically relies on combined plasmapheresis with intensive immunosuppression. Dialysis dependency at presentation is a dominant predictor of kidney outcome. Histologically, a low (<10%) percentage of normal glomeruli, 100% crescents, together with dialysis dependency at presentation, is associated with poor kidney outcomes. In such cases, an individualized approach weighing the risks and benefits of treatment is recommended. There is a need for better ways to stop the toxic inflammatory activity associated with this disease. In this narrative review, we discuss recent updates on the pathogenesis and management of anti-glomerular basement membrane disease relevant to patients of all ages.
抗肾小球基底膜病是一种累及肾脏（约 90%）和肺部（约 60%）的小血管血管炎。抗肾小球基底膜抗体在抗肾小球基底膜病中具有直接致病性；然而，最近的研究强调了 T 细胞的关键作用。现在也认识到了肾小球基底膜内的新自身抗原。该疾病的非典型形式以及先前的触发因素（如免疫检查点抑制剂、免疫调节药物和疫苗）都有报道。尽管在过去 2-3 十年中患者的生存率显著提高，但抗肾小球基底膜病的肾脏预后仍然较差。治疗通常依赖于联合血浆置换和强化免疫抑制。就诊时需要透析依赖是肾脏预后的主要预测因素。组织学上，肾小球正常比例低（<10%），新月体形成率 100%，同时就诊时需要透析，与肾脏预后不良相关。在这种情况下，建议采取个体化方法权衡治疗的风险和获益。需要寻找更好的方法来阻止与这种疾病相关的毒性炎症活动。在这篇叙述性综述中，我们讨论了与所有年龄段患者相关的抗肾小球基底膜病发病机制和治疗的最新进展。
