Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys
Fibronectin glomerulopathy (FNG) is a rare inherited kidney disease characterized by extensive deposition of fibronectin in the glomeruli, especially in the mesangial and subendothelial regions. The disease progresses slowly and eventually leads to kidney failure in 15–20 years. Here, we report an i...
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Frontiers Media S.A.
2022-06-01
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| Series: | Frontiers in Genetics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fgene.2022.839703/full |
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| author | Xiaona Wei Xiangdong Wang Rui Zhang Peifen Liang Bo Liu Lin Wang Shuling Yue Xiaojuan Li Wenfang Chen Qiongqiong Yang |
| author_facet | Xiaona Wei Xiangdong Wang Rui Zhang Peifen Liang Bo Liu Lin Wang Shuling Yue Xiaojuan Li Wenfang Chen Qiongqiong Yang |
| author_sort | Xiaona Wei |
| collection | DOAJ |
| description | Fibronectin glomerulopathy (FNG) is a rare inherited kidney disease characterized by extensive deposition of fibronectin in the glomeruli, especially in the mesangial and subendothelial regions. The disease progresses slowly and eventually leads to kidney failure in 15–20 years. Here, we report an interesting case. The patient presented with proteinuria and was diagnosed with immune complex–mediated glomerulonephritis, and lupus nephritis was suspected. This patient progressed to end-stage renal disease after 18 years and received an allogeneic kidney transplant. However, proteinuria recurred 27 months after kidney transplantation. The renal biopsy found extensive deposition in glomeruli, and the patient was diagnosed with FNG using mass spectrometry analysis and confirmed by immunohistochemistry in both the native and transplanted kidneys. Gene sequencing revealed that a missense mutation in the fibronectin 1 (FN1) gene caused reduced binding to heparin, endothelial cells, and podocytes and impaired stress fiber formation. The patient had stable renal function but persistent nephrotic proteinuria after 6 months of follow-up. Given the persistence of abnormal circulating fibronectin levels, FNG can relapse following renal transplantation. The circulating fibronectin deposits on grafts, and renal function progressively deteriorates after recurrence. Therefore, whether renal transplantation is an acceptable treatment for FNG is still debatable. |
| first_indexed | 2024-04-13T21:42:15Z |
| format | Article |
| id | doaj.art-10e601bc714e4b079a8a3c275d0004dc |
| institution | Directory Open Access Journal |
| issn | 1664-8021 |
| language | English |
| last_indexed | 2024-04-13T21:42:15Z |
| publishDate | 2022-06-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Genetics |
| spelling | doaj.art-10e601bc714e4b079a8a3c275d0004dc2022-12-22T02:28:42ZengFrontiers Media S.A.Frontiers in Genetics1664-80212022-06-011310.3389/fgene.2022.839703839703Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native KidneysXiaona Wei0Xiangdong Wang1Rui Zhang2Peifen Liang3Bo Liu4Lin Wang5Shuling Yue6Xiaojuan Li7Wenfang Chen8Qiongqiong Yang9Department of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaDepartment of Pathology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, ChinaDepartment of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaDepartment of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaDepartment of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaDepartment of Renal Pathology, King Medical Diagnostics Center, Guangzhou, ChinaDepartment of Renal Pathology, King Medical Diagnostics Center, Guangzhou, ChinaCellular and Molecular Diagnostics Center, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaDepartment of Pathology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, ChinaDepartment of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, ChinaFibronectin glomerulopathy (FNG) is a rare inherited kidney disease characterized by extensive deposition of fibronectin in the glomeruli, especially in the mesangial and subendothelial regions. The disease progresses slowly and eventually leads to kidney failure in 15–20 years. Here, we report an interesting case. The patient presented with proteinuria and was diagnosed with immune complex–mediated glomerulonephritis, and lupus nephritis was suspected. This patient progressed to end-stage renal disease after 18 years and received an allogeneic kidney transplant. However, proteinuria recurred 27 months after kidney transplantation. The renal biopsy found extensive deposition in glomeruli, and the patient was diagnosed with FNG using mass spectrometry analysis and confirmed by immunohistochemistry in both the native and transplanted kidneys. Gene sequencing revealed that a missense mutation in the fibronectin 1 (FN1) gene caused reduced binding to heparin, endothelial cells, and podocytes and impaired stress fiber formation. The patient had stable renal function but persistent nephrotic proteinuria after 6 months of follow-up. Given the persistence of abnormal circulating fibronectin levels, FNG can relapse following renal transplantation. The circulating fibronectin deposits on grafts, and renal function progressively deteriorates after recurrence. Therefore, whether renal transplantation is an acceptable treatment for FNG is still debatable.https://www.frontiersin.org/articles/10.3389/fgene.2022.839703/fullrenal allograftfibronectin glomerulopathyfibronectin 1 mutationrecurrent post-transplantationphenotypic heterogeneity of FN1 mutation |
| spellingShingle | Xiaona Wei Xiangdong Wang Rui Zhang Peifen Liang Bo Liu Lin Wang Shuling Yue Xiaojuan Li Wenfang Chen Qiongqiong Yang Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys Frontiers in Genetics renal allograft fibronectin glomerulopathy fibronectin 1 mutation recurrent post-transplantation phenotypic heterogeneity of FN1 mutation |
| title | Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys |
| title_full | Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys |
| title_fullStr | Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys |
| title_full_unstemmed | Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys |
| title_short | Case Report: Recurrent Deposition in Renal Allografts: A Rare Case of Fibronectin Glomerulopathy Overlooked in Native Kidneys |
| title_sort | case report recurrent deposition in renal allografts a rare case of fibronectin glomerulopathy overlooked in native kidneys |
| topic | renal allograft fibronectin glomerulopathy fibronectin 1 mutation recurrent post-transplantation phenotypic heterogeneity of FN1 mutation |
| url | https://www.frontiersin.org/articles/10.3389/fgene.2022.839703/full |
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