IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review
Abstract Background IgG4-related disease (IgG4-RD) often affects multiple organs and tissues, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases glomerulopathy (including membranous nephropathy). Case presentation In this article, we re...
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| Format: | Article |
| Language: | English |
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BMC
2019-07-01
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| Series: | BMC Nephrology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12882-019-1419-6 |
| _version_ | 1818215867677147136 |
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| author | Nan-Nan Zhang Yan-Yun Wang Ling-Xin Kong Wan-Zhong Zou Bao Dong |
| author_facet | Nan-Nan Zhang Yan-Yun Wang Ling-Xin Kong Wan-Zhong Zou Bao Dong |
| author_sort | Nan-Nan Zhang |
| collection | DOAJ |
| description | Abstract Background IgG4-related disease (IgG4-RD) often affects multiple organs and tissues, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases glomerulopathy (including membranous nephropathy). Case presentation In this article, we report a patient with nephrotic syndrome as the only initial manifestation. Membranous nephropathy was confirmed by renal biopsy, but without any renal interstitial lesions. The nephrotic syndrome completely resolved after treatment with immunosuppressants but recurred after drug withdrawal, which was accompanied by acute kidney injury. Ultimately, IgG4-related interstitial nephritis with membranous nephropathy was confirmed by a second renal biopsy. After routine administration of steroids and cyclophosphamide, renal function returned to normal after 2 months, and nephrotic syndrome was ameliorated after 5 months. Conclusion Special attention should be paid to this rare condition in the clinical setting. In patients with membranous nephropathy (MN) that is accompanied by multi-system damage, impaired renal function, elevated IgG4 levels (absolute or relative value), negative PLA2R, and/or renal interstitial plasma cell infiltration, the possibility of IgG4-related kidney disease (IgG4-RKD) should be carefully assessed. |
| first_indexed | 2024-12-12T06:42:54Z |
| format | Article |
| id | doaj.art-165553b929704d1e9911e6b157db67bc |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-12-12T06:42:54Z |
| publishDate | 2019-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-165553b929704d1e9911e6b157db67bc2022-12-22T00:34:16ZengBMCBMC Nephrology1471-23692019-07-012011610.1186/s12882-019-1419-6IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature reviewNan-Nan Zhang0Yan-Yun Wang1Ling-Xin Kong2Wan-Zhong Zou3Bao Dong4Department of Nephrology, Fangshan Hospital of Beijing University of Chinese MedicineDepartment of Nephrology, Fangshan Hospital of Beijing University of Chinese MedicineDepartment of Nephrology, Fangshan Hospital of Beijing University of Chinese MedicineDepartment of Nephrology, People’s Hospital of Peking UniversityDepartment of Nephrology, People’s Hospital of Peking UniversityAbstract Background IgG4-related disease (IgG4-RD) often affects multiple organs and tissues, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases glomerulopathy (including membranous nephropathy). Case presentation In this article, we report a patient with nephrotic syndrome as the only initial manifestation. Membranous nephropathy was confirmed by renal biopsy, but without any renal interstitial lesions. The nephrotic syndrome completely resolved after treatment with immunosuppressants but recurred after drug withdrawal, which was accompanied by acute kidney injury. Ultimately, IgG4-related interstitial nephritis with membranous nephropathy was confirmed by a second renal biopsy. After routine administration of steroids and cyclophosphamide, renal function returned to normal after 2 months, and nephrotic syndrome was ameliorated after 5 months. Conclusion Special attention should be paid to this rare condition in the clinical setting. In patients with membranous nephropathy (MN) that is accompanied by multi-system damage, impaired renal function, elevated IgG4 levels (absolute or relative value), negative PLA2R, and/or renal interstitial plasma cell infiltration, the possibility of IgG4-related kidney disease (IgG4-RKD) should be carefully assessed.http://link.springer.com/article/10.1186/s12882-019-1419-6IgG4-related kidney diseaseInterstitial nephritisMembranous nephropathyRepeated renal biopsies |
| spellingShingle | Nan-Nan Zhang Yan-Yun Wang Ling-Xin Kong Wan-Zhong Zou Bao Dong IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review BMC Nephrology IgG4-related kidney disease Interstitial nephritis Membranous nephropathy Repeated renal biopsies |
| title | IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review |
| title_full | IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review |
| title_fullStr | IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review |
| title_full_unstemmed | IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review |
| title_short | IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review |
| title_sort | igg4 related kidney disease igg4 rkd with membranous nephropathy as its initial manifestation report of one case and literature review |
| topic | IgG4-related kidney disease Interstitial nephritis Membranous nephropathy Repeated renal biopsies |
| url | http://link.springer.com/article/10.1186/s12882-019-1419-6 |
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