Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature
The simultaneous occurrence of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and light chain proximal tubulopathy (LCPT) presents a unique diagnostic and therapeutic challenge. PGNMID is characterized by monoclonal immunoglobulin deposition in glomeruli, leading t...
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Frontiers Media S.A.
2025-01-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2025.1502798/full |
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| author | Jingdong Zhang Yang Liu Fengyan Jin Jia Li Hui Wang Fuzhe Ma Ye Jia Jinyu Yu Shan Wu Shaojie Fu Zhonggao Xu Hao Wu |
| author_facet | Jingdong Zhang Yang Liu Fengyan Jin Jia Li Hui Wang Fuzhe Ma Ye Jia Jinyu Yu Shan Wu Shaojie Fu Zhonggao Xu Hao Wu |
| author_sort | Jingdong Zhang |
| collection | DOAJ |
| description | The simultaneous occurrence of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and light chain proximal tubulopathy (LCPT) presents a unique diagnostic and therapeutic challenge. PGNMID is characterized by monoclonal immunoglobulin deposition in glomeruli, leading to proliferative glomerular pathology, while LCPT involves monoclonal light chain deposition in proximal tubular cells, causing tubulointerstitial damage. Both conditions are classified under monoclonal gammopathy of renal significance (MGRS), but their coexistence in a single patient is exceedingly rare. This case report details the presentation of a patient with nephrotic syndrome and renal insufficiency, where renal biopsy revealed both PGNMID and LCPT. Treatment with bortezomib, cyclophosphamide, and dexamethasone achieved clinical remission and significant renal function recovery. This case emphasizes the critical role of renal biopsy in the diagnosis, particularly in the absence of detectable monoclonal proteins, and demonstrates the efficacy of targeted therapy in managing such complex renal pathologies. These findings contribute to a better understanding of MGRS and may guide future therapeutic strategies for similar cases. |
| format | Article |
| id | doaj-art-282f791c701f4e07b95edb5129683b7c |
| institution | Kabale University |
| issn | 2296-858X |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj-art-282f791c701f4e07b95edb5129683b7c2025-01-31T06:39:49ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2025-01-011210.3389/fmed.2025.15027981502798Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literatureJingdong Zhang0Yang Liu1Fengyan Jin2Jia Li3Hui Wang4Fuzhe Ma5Ye Jia6Jinyu Yu7Shan Wu8Shaojie Fu9Zhonggao Xu10Hao Wu11Department of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Hematology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaLaboratory of Electron Microscopy, Peking University First Hospital, Beijing, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaDepartment of Nephrology, The First Hospital of Jilin University, Changchun, ChinaThe simultaneous occurrence of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and light chain proximal tubulopathy (LCPT) presents a unique diagnostic and therapeutic challenge. PGNMID is characterized by monoclonal immunoglobulin deposition in glomeruli, leading to proliferative glomerular pathology, while LCPT involves monoclonal light chain deposition in proximal tubular cells, causing tubulointerstitial damage. Both conditions are classified under monoclonal gammopathy of renal significance (MGRS), but their coexistence in a single patient is exceedingly rare. This case report details the presentation of a patient with nephrotic syndrome and renal insufficiency, where renal biopsy revealed both PGNMID and LCPT. Treatment with bortezomib, cyclophosphamide, and dexamethasone achieved clinical remission and significant renal function recovery. This case emphasizes the critical role of renal biopsy in the diagnosis, particularly in the absence of detectable monoclonal proteins, and demonstrates the efficacy of targeted therapy in managing such complex renal pathologies. These findings contribute to a better understanding of MGRS and may guide future therapeutic strategies for similar cases.https://www.frontiersin.org/articles/10.3389/fmed.2025.1502798/fullproliferative glomerulonephritis with monoclonal immunoglobulin depositslight chain proximal tubulopathymonoclonal gammopathynephrotic syndromerenal biopsycase report |
| spellingShingle | Jingdong Zhang Yang Liu Fengyan Jin Jia Li Hui Wang Fuzhe Ma Ye Jia Jinyu Yu Shan Wu Shaojie Fu Zhonggao Xu Hao Wu Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature Frontiers in Medicine proliferative glomerulonephritis with monoclonal immunoglobulin deposits light chain proximal tubulopathy monoclonal gammopathy nephrotic syndrome renal biopsy case report |
| title | Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature |
| title_full | Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature |
| title_fullStr | Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature |
| title_full_unstemmed | Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature |
| title_short | Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature |
| title_sort | concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy a case report and review of the literature |
| topic | proliferative glomerulonephritis with monoclonal immunoglobulin deposits light chain proximal tubulopathy monoclonal gammopathy nephrotic syndrome renal biopsy case report |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2025.1502798/full |
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