Velegraki Magdalini, Stylianou Kostas G, Xydakis Dimitrios, Gakiopoulou Hariklia, Voudoukis Evangelos, Lygerou Dimitra, Dermitzaki Eleftheria-Kleio, Mpitouli Aphrodite, Paspatis Gregorios, Theodoropoulou Angeliki
Department of Gastroenterology, Venizeleion Hospital, Heraklion, Greece.
Department of Nephrology, University Hospital of Heraklion, Heraklion, Greece.
Case Rep Nephrol Dial. 2019 Jun 26;9(2):85-91. doi: 10.1159/000501260. eCollection 2019 May-Aug.
Membranous nephropathy (MN) with tubulointerstitial nephritis (TIN) is an established manifestation of immunoglobulin G4 (IgG4)-related disease (IgG4-RD). A pathological feature aiming to distinguish between primary and secondary MN is the presence or absence of glomerular staining for phospholipase A2 receptor (PLA2R), respectively. Isolated MN without TIN has been rarely reported in the context of IgG4-RD. This case report describes a patient with a history of MN successfully treated with steroids and cyclophosphamide, who, 3 years later, presented with unexplained exacerbation of diabetes mellitus due to IgG4-related autoimmune pancreatitis. Pancreatitis was treated, and diabetes improved after treatment with steroids. Based on the presence of isolated IgG4 glomerular capillary deposits along with negative staining for PLA2R and the metachronous appearance of autoimmune pancreatitis, MN was retrospectively classified as secondary to IgG4-RD. Isolated IgG4-positive/PLA2R-negative MN without TIN can be a prodrome of IgG4-RD, reminiscent of MN secondary to neoplasms.
膜性肾病（MN）合并肾小管间质性肾炎（TIN）是免疫球蛋白G4（IgG4）相关疾病（IgG4-RD）的一种既定表现。区分原发性和继发性MN的一个病理特征分别是是否存在磷脂酶A2受体（PLA2R）的肾小球染色。在IgG4-RD背景下，孤立性MN且无TIN的情况鲜有报道。本病例报告描述了一名有MN病史的患者，曾成功接受类固醇和环磷酰胺治疗，3年后因IgG4相关自身免疫性胰腺炎出现不明原因的糖尿病加重。胰腺炎得到治疗，类固醇治疗后糖尿病有所改善。基于孤立的IgG4肾小球毛细血管沉积物的存在、PLA2R染色阴性以及自身免疫性胰腺炎的异时出现，MN被回顾性分类为继发于IgG4-RD。孤立性IgG4阳性/PLA2R阴性且无TIN的MN可能是IgG4-RD的前驱症状，类似于肿瘤继发的MN。
