Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio, TX, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Semin Diagn Pathol. 2024 Mar;41(2):88-94. doi: 10.1053/j.semdp.2023.12.001. Epub 2023 Dec 20.
IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.
IgG4 相关肾脏疾病（IgG4-RKD）包括所有形式的肾脏疾病，这些疾病都是 IgG4 相关疾病（IgG4-RD）的一部分。首先被认为是 IgG4 相关肾小管间质性肾炎（IgG4-TIN），然后是 IgG4 相关膜性肾小球肾炎（IgG4-MGN），我们现在认识到其他形式的间质性肾炎、肾小球疾病和血管疾病，这些都可以被视为 IgG4-RKD 的一部分。临床表现多种多样，可包括急性或慢性肾损伤、蛋白尿或肾病综合征、肿块病变和梗阻。虽然通常与 IgG4-RD 的其他器官受累有关，但 IgG4-RKD 中约有 20%的患者仅肾脏受累。与 IgG4-RD 整体相比，IgG4-RKD 患者更有可能出现血清 IgG4 或 IgG 升高，更有可能出现低补体血症。在这篇综述中，我们广泛涵盖了其他类型的自身免疫性和浆细胞丰富性间质性肾炎、形成肿块的肾脏炎症性疾病以及 IgG4-TIN 的其他类似物，特别是 ANCA 相关性疾病。
