PLA2R-positive membranous nephropathy in IgG4-related disease
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...
| Main Authors: | , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2024-02-01
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| Series: | BMC Nephrology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12882-024-03511-3 |
| _version_ | 1797275526200557568 |
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| author | Yusuke Ushio Taro Akihisa Kazunori Karasawa Momoko Seki Shizuka Kobayashi Yoei Miyabe Hiroshi Kataoka Naoko Ito Sekiko Taneda Shin’ichi Akiyama Akira Hebisawa Mitsuhiro Kawano Kazuho Honda Junichi Hoshino |
| author_facet | Yusuke Ushio Taro Akihisa Kazunori Karasawa Momoko Seki Shizuka Kobayashi Yoei Miyabe Hiroshi Kataoka Naoko Ito Sekiko Taneda Shin’ichi Akiyama Akira Hebisawa Mitsuhiro Kawano Kazuho Honda Junichi Hoshino |
| author_sort | Yusuke Ushio |
| collection | DOAJ |
| description | 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. 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. |
| first_indexed | 2024-03-07T15:14:39Z |
| format | Article |
| id | doaj.art-0a68cc2b8696482493b2c3637a67b912 |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-03-07T15:14:39Z |
| publishDate | 2024-02-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-0a68cc2b8696482493b2c3637a67b9122024-03-05T17:57:24ZengBMCBMC Nephrology1471-23692024-02-012511510.1186/s12882-024-03511-3PLA2R-positive membranous nephropathy in IgG4-related diseaseYusuke Ushio0Taro Akihisa1Kazunori Karasawa2Momoko Seki3Shizuka Kobayashi4Yoei Miyabe5Hiroshi Kataoka6Naoko Ito7Sekiko Taneda8Shin’ichi Akiyama9Akira Hebisawa10Mitsuhiro Kawano11Kazuho Honda12Junichi Hoshino13Department of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Tokyo Women’s Medical UniversityDepartment of Surgical Pathology, Tokyo Women’s Medical UniversityDepartment of Surgical Pathology, Tokyo Women’s Medical UniversityDepartment of Nephrology, Nagoya University Graduate School of MedicineClinical Research Center, National Hospital Organization Tokyo National HospitalDepartment of Rheumatology, Graduate School of Medical Science, Kanazawa UniversityDepartment of Anatomy, Showa University School of MedicineDepartment of Nephrology, Tokyo Women’s Medical UniversityAbstract 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.https://doi.org/10.1186/s12882-024-03511-3IgG4-related DiseasePLA2R-positive membranous nephropathy |
| spellingShingle | Yusuke Ushio Taro Akihisa Kazunori Karasawa Momoko Seki Shizuka Kobayashi Yoei Miyabe Hiroshi Kataoka Naoko Ito Sekiko Taneda Shin’ichi Akiyama Akira Hebisawa Mitsuhiro Kawano Kazuho Honda Junichi Hoshino PLA2R-positive membranous nephropathy in IgG4-related disease BMC Nephrology IgG4-related Disease PLA2R-positive membranous nephropathy |
| title | PLA2R-positive membranous nephropathy in IgG4-related disease |
| title_full | PLA2R-positive membranous nephropathy in IgG4-related disease |
| title_fullStr | PLA2R-positive membranous nephropathy in IgG4-related disease |
| title_full_unstemmed | PLA2R-positive membranous nephropathy in IgG4-related disease |
| title_short | PLA2R-positive membranous nephropathy in IgG4-related disease |
| title_sort | pla2r positive membranous nephropathy in igg4 related disease |
| topic | IgG4-related Disease PLA2R-positive membranous nephropathy |
| url | https://doi.org/10.1186/s12882-024-03511-3 |
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