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Abstract：
BACKGROUND: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease that affects multiple organs, including the pancreas, lacrimal glands, salivary glands, periaortic/retroperitoneum, and kidney. Interstitial nephritis is a typical renal disorder associated with IgG4-RD, but membranous nephropathy is also seen in some cases.
METHODS: Herein we report on the case of a 77-year-old male patient with nephrotic syndrome and IgG4-related lung disease. His serum phospholipase A2 receptor (PLA2R) antibody was positive. His renal biopsy specimen was also positive for PLA2R. The renal biopsy specimen showed membranous nephropathy with equal IgG3 and IgG4 immunofluorescence staining and no interstitial nephritis, suggesting IgG4-RD manifesting as membranous nephropathy.
CONCLUSIONS: Nephrotic syndrome caused by membranous nephropathy is sometimes associated with IgG4-RD. In such cases, even if serum PLA2R antibody is positive, it should be considered that the membranous nephropathy may be secondary to IgG4-RD.
METHODS: Herein we report on the case of a 77-year-old male patient with nephrotic syndrome and IgG4-related lung disease. His serum phospholipase A2 receptor (PLA2R) antibody was positive. His renal biopsy specimen was also positive for PLA2R. The renal biopsy specimen showed membranous nephropathy with equal IgG3 and IgG4 immunofluorescence staining and no interstitial nephritis, suggesting IgG4-RD manifesting as membranous nephropathy.
CONCLUSIONS: Nephrotic syndrome caused by membranous nephropathy is sometimes associated with IgG4-RD. In such cases, even if serum PLA2R antibody is positive, it should be considered that the membranous nephropathy may be secondary to IgG4-RD.
摘要：
背景：IgG4相关疾病（IgG4-RD）是一种影响多个器官的纤维炎症性疾病，包括胰腺,泪腺,唾液腺,主动脉周围/腹膜后,还有肾.间质性肾炎是一种典型的与IgG4-RD相关的肾脏疾病，但是在某些情况下也可以看到膜性肾病。
方法：本文报道一例77岁男性肾病综合征和IgG4相关性肺病患者。His血清磷脂酶A2受体(PLA2R)抗体阳性。他的肾活检标本PLA2R也呈阳性。肾活检标本显示膜性肾病，IgG3和IgG4免疫荧光染色相等，无间质性肾炎，提示IgG4-RD表现为膜性肾病。
结论：膜性肾病引起的肾病综合征有时与IgG4-RD相关。在这种情况下,即使血清PLA2R抗体阳性，应考虑膜性肾病可能继发于IgG4-RD。
方法：本文报道一例77岁男性肾病综合征和IgG4相关性肺病患者。His血清磷脂酶A2受体(PLA2R)抗体阳性。他的肾活检标本PLA2R也呈阳性。肾活检标本显示膜性肾病，IgG3和IgG4免疫荧光染色相等，无间质性肾炎，提示IgG4-RD表现为膜性肾病。
结论：膜性肾病引起的肾病综合征有时与IgG4-RD相关。在这种情况下,即使血清PLA2R抗体阳性，应考虑膜性肾病可能继发于IgG4-RD。
