University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley (North-West Italy), San Giovanni Bosco Hub Hospital, ASL Città di Torino and Department of Clinical and Biological Sciences of the University of Turin, Turin, Italy.
Department of Medicine & Therapeutics, and Li Ka Shing Institute of Health Sciences, The Chinese University of Hong Kong, Hong Kong, China; Guangdong-Hong Kong Joint Laboratory on Immunological and Genetic Kidney Diseases,Guangdong Academy of Medical Sciences, Guangdong Provincial People's Hospital, Guangzhou, China.
Autoimmun Rev. 2024 Apr;23(4):103466. doi: 10.1016/j.autrev.2023.103466. Epub 2023 Oct 15.
Renal fibrosis is now recognized as a main determinant of renal pathology to include chronic kidney disease. Deposition of pathological matrix in the walls of glomerular capillaries, the interstitial space, and around arterioles predicts and contributes to the functional demise of the nephron and its surrounding vasculature. The recent identification of the major cell populations of fibroblast precursors in the kidney interstitium such as pericytes and tissue-resident mesenchymal stem cells, or bone-marrow-derived macrophages, and in the glomerulus such as podocytes, parietal epithelial and mesangial cells, has enabled the study of the fibrogenic process thought the lens of involved immunological pathways. Besides, a growing body of evidence is supporting the role of the lymphatic system in modulating the immunological response potentially leading to inflammation and ultimately renal damage. These notions have moved our understanding of renal fibrosis to be recognized as a clinical entity and new main player in autoimmunity, impacting directly the management of patients.
肾纤维化现在被认为是肾脏病理的主要决定因素，包括慢性肾脏病。病理性基质在肾小球毛细血管壁、间质和小动脉周围的沉积，预测并导致了肾单位及其周围血管的功能丧失。最近发现了肾间质中如周细胞和组织驻留间充质干细胞或骨髓来源的巨噬细胞以及肾小球中如足细胞、壁层上皮细胞和系膜细胞等成纤维细胞前体细胞的主要细胞群体，使我们能够通过涉及免疫途径的研究来了解纤维化过程。此外，越来越多的证据支持淋巴系统在调节免疫反应中的作用，这可能导致炎症并最终导致肾脏损伤。这些观点使我们对肾纤维化的认识已被视为一种临床实体和自身免疫中的新主要参与者，直接影响患者的治疗。
