Huang Meichun, Liu Jun, Li Xiuxiu
Renal Department, Tongde Hospital of Zhejiang Province, No. 234 Gucui Road, Hangzhou, 310012, China.
Renal Department, Tongde Hospital of Zhejiang Province, Hangzhou, 310012, China.
Open Life Sci. 2024 Jul 25;19(1):20220921. doi: 10.1515/biol-2022-0921. eCollection 2024.
IgG4-related tubulointerstitial nephritis (IgG4-related TIN) is the prevalent clinical manifestation of IgG4-related diseases (IgG4-RD). However, there are limited cases of IgG4-RD occurring with membranous nephropathy (MN) in the absence of phospholipase A2 receptor (PLA2R). There have been no indications of treatment using . This study reported a rare case of IgG4-RD with PLA2R-associated MN without any of the distinct IgG4-related TIN. The patient was treated effectively with . A 71-year-old patient was admitted to the medical facility after presenting with a 1 month history of edema and 8 months of albuminuria. The renal biopsy tissue examination confirmed the presence of MN (phase II) in the absence of pathological manifestations of IgG4-related TIN. Immunohistochemistry identified PLA2R++ (granular capillaries). The serum PLA2R antibody titer was 1:180 (1:20). The patient met the diagnosis with IgG4-RD. Over 8 years of follow-up, the patient was effectively treated with low-dose hormones and , without any adverse effects. This MN is considered a unique form of IgG4-RD, regardless of whether PLA2R antibodies are present or not. Research suggests that could be a promising option for elderly people with IgG4-related MN, as it has been found to have fewer adverse effects.
IgG4相关性肾小管间质性肾炎（IgG4相关性TIN）是IgG4相关性疾病（IgG4-RD）的常见临床表现。然而，在无磷脂酶A2受体（PLA2R）的情况下，IgG4-RD合并膜性肾病（MN）的病例有限。目前尚无使用……进行治疗的指征。本研究报告了一例罕见的IgG4-RD合并PLA2R相关性MN且无任何典型IgG4相关性TIN表现的病例。该患者接受……治疗后效果良好。一名71岁患者因出现1个月的水肿病史和8个月的蛋白尿入院。肾活检组织检查证实存在MN（II期），无IgG4相关性TIN的病理表现。免疫组化显示PLA2R++（颗粒状毛细血管）。血清PLA2R抗体滴度为1:180（1:20）。该患者符合IgG4-RD的诊断。经过8年多的随访，该患者接受低剂量激素和……治疗效果良好，无任何不良反应。这种MN被认为是IgG4-RD的一种独特形式，无论是否存在PLA2R抗体。研究表明，……对于患有IgG4相关性MN的老年人可能是一个有前景的选择，因为已发现其不良反应较少。
