Division of Nephrology, Department of Medicine IV, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.
Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, VIC, Australia.
Nat Rev Immunol. 2023 Jul;23(7):453-471. doi: 10.1038/s41577-022-00816-y. Epub 2023 Jan 12.
'Glomerulonephritis' (GN) is a term used to describe a group of heterogeneous immune-mediated disorders characterized by inflammation of the filtration units of the kidney (the glomeruli). These disorders are currently classified largely on the basis of histopathological lesion patterns, but these patterns do not align well with their diverse pathological mechanisms and hence do not inform optimal therapy. Instead, we propose grouping GN disorders into five categories according to their immunopathogenesis: infection-related GN, autoimmune GN, alloimmune GN, autoinflammatory GN and monoclonal gammopathy-related GN. This categorization can inform the appropriate treatment; for example, infection control for infection-related GN, suppression of adaptive immunity for autoimmune GN and alloimmune GN, inhibition of single cytokines or complement factors for autoinflammatory GN arising from inborn errors in innate immunity, and plasma cell clone-directed or B cell clone-directed therapy for monoclonal gammopathies. Here we present the immunopathogenesis of GN and immunotherapies in use and in development and discuss how an immunopathogenesis-based GN classification can focus research, and improve patient management and teaching.
肾小球肾炎（GN）是一组描述由肾脏过滤单位（肾小球）炎症引起的异质性免疫介导疾病的术语。这些疾病目前主要基于组织病理学损伤模式进行分类，但这些模式与它们不同的病理机制并不吻合，因此无法提供最佳的治疗方法。相反，我们建议根据其免疫发病机制将 GN 疾病分为五类：感染相关 GN、自身免疫性 GN、同种免疫性 GN、自身炎症性 GN 和单克隆丙种球蛋白病相关 GN。这种分类可以为适当的治疗提供信息；例如，感染相关 GN 需要控制感染，自身免疫性 GN 和同种免疫性 GN 需要抑制适应性免疫，由先天免疫中固有免疫缺陷引起的自身炎症性 GN 需要抑制单一细胞因子或补体因子，单克隆丙种球蛋白病需要浆细胞克隆定向或 B 细胞克隆定向治疗。在这里，我们介绍 GN 的免疫发病机制和已使用及正在开发中的免疫疗法，并讨论基于免疫发病机制的 GN 分类如何能够聚焦研究，改善患者管理和教学。
