ホシノ　ジユンイチ   HOSHINO Jiyun'ichi   星野　純一    所属   医学部 医学科（東京女子医科大学病院）    職種   教授・基幹分野長
論文種別	原著
言語種別	英語
査読の有無	査読あり
表題	PLA2R-positive membranous nephropathy in IgG4-related disease
掲載誌名	正式名：BMC Nephrology ISSNコード：14712369
巻・号・頁	25(1),pp.66
著者・共著者	Ushio Yusuke, Akihisa Taro, Karasawa Kazunori, Seki Momoko, Kobayashi Shizuka, Miyabe Yoei, Kataoka Hiroshi, Ito Naoko , Taneda Sekiko , Akiyama Shinichi, Hebisawa Akira, Kawano Mitsuhiro, Honda Kazuho, Hoshino Junichi
担当区分	最終著者,責任著者
発行年月	2024/02/23
概要	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. Case presentation 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.
DOI	10.1186/s12882-024-03511-3
PMID	38395839