PDF(Pubmed)
Abstract：
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 Tripterygium wilfordii. 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 T. wilfordii. 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 T. wilfordii, 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 T. wilfordii could be a promising option for elderly people with IgG4-related MN, as it has been found to have fewer adverse effects.
摘要：
IgG4相关性肾小管间质性肾炎(IgG4相关TIN)是IgG4相关疾病(IgG4-RD)的常见临床表现。然而,在不存在磷脂酶A2受体(PLA2R)的情况下,膜性肾病(MN)发生IgG4-RD的病例有限.没有使用雷公藤的适应症。这项研究报道了一例罕见的IgG4-RD与PLA2R相关的MN没有任何明显的IgG4相关的TIN。该患者用雷公藤有效治疗。一名71岁的患者在出现1个月的水肿病史和8个月的白蛋白尿后入院。肾活检组织检查证实在没有IgG4相关TIN的病理表现的情况下存在MN(II期)。免疫组织化学鉴定PLA2R++(颗粒状毛细血管)。血清PLA2R抗体效价为1：180(1：20)。患者符合IgG4-RD诊断。经过8年的随访，患者得到了低剂量激素和雷公藤的有效治疗，没有任何不良影响。这种MN被认为是IgG4-RD的独特形式，无论是否存在PLA2R抗体。研究表明，对于患有与IgG4相关的MN的老年人来说，雷公藤可能是一个有希望的选择，因为它被发现有较少的不利影响。
