Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis?
In light chain amyloidosis (LA), the massive glomerular and vascular amyloid deposition leading to interstitial fibrosis/tubular atrophy (IFTA) is thought to be responsible for renal failure. The amyloid deposition in the interstitium and the tubular basement membrane (TBM) has received less attenti...
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2023-12-01
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| Series: | Renal Failure |
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| Online Access: | https://www.tandfonline.com/doi/10.1080/0886022X.2023.2203776 |
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| author | Csilla Markóth László Bidiga László Váróczy Ibolya File József Balla János Mátyus |
| author_facet | Csilla Markóth László Bidiga László Váróczy Ibolya File József Balla János Mátyus |
| author_sort | Csilla Markóth |
| collection | DOAJ |
| description | In light chain amyloidosis (LA), the massive glomerular and vascular amyloid deposition leading to interstitial fibrosis/tubular atrophy (IFTA) is thought to be responsible for renal failure. The amyloid deposition in the interstitium and the tubular basement membrane (TBM) has received less attention in the study of LA. We, therefore, collected clinical and laboratory data on patients diagnosed with LA in our Nephrology Department and studied amyloid deposition in the TBM. Twelve LA patients were diagnosed by renal biopsy during a seven-year period. In 4 of the 12, amyloid deposition could also be detected in the TBM. In our first case of a patient with diabetes mellitus, non-amyloid fibrils resembling ‘diabetic fibrillosis’ were also seen by electron microscopy. Despite the double damage, IFTA was negligible, blood vessels were unaffected, and the glomerular deposition was segmental. In the other three cases, significant (>50%) IFTA and a severely reduced estimated glomerular filtration rate were already detected at the time of diagnosis and amyloid deposition was also observed in the blood vessels. These findings indicate the importance of TBM amyloid deposition in the progression of renal disease. This may represent a late-stage presentation of the disease with a heavy LC burden. |
| format | Article |
| id | doaj-art-5cb133e45f0b4ec8ae7007e93039c1d9 |
| institution | Kabale University |
| issn | 0886-022X 1525-6049 |
| language | English |
| publishDate | 2023-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Renal Failure |
| spelling | doaj-art-5cb133e45f0b4ec8ae7007e93039c1d92025-08-20T03:48:57ZengTaylor & Francis GroupRenal Failure0886-022X1525-60492023-12-0145110.1080/0886022X.2023.2203776Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis?Csilla Markóth0László Bidiga1László Váróczy2Ibolya File3József Balla4János Mátyus5Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, HungaryKálmán Laki Doctoral School, University of Debrecen, Debrecen, HungaryDivision of Hematology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, HungaryDivision of Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, HungaryDivision of Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, HungaryDivision of Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, HungaryIn light chain amyloidosis (LA), the massive glomerular and vascular amyloid deposition leading to interstitial fibrosis/tubular atrophy (IFTA) is thought to be responsible for renal failure. The amyloid deposition in the interstitium and the tubular basement membrane (TBM) has received less attention in the study of LA. We, therefore, collected clinical and laboratory data on patients diagnosed with LA in our Nephrology Department and studied amyloid deposition in the TBM. Twelve LA patients were diagnosed by renal biopsy during a seven-year period. In 4 of the 12, amyloid deposition could also be detected in the TBM. In our first case of a patient with diabetes mellitus, non-amyloid fibrils resembling ‘diabetic fibrillosis’ were also seen by electron microscopy. Despite the double damage, IFTA was negligible, blood vessels were unaffected, and the glomerular deposition was segmental. In the other three cases, significant (>50%) IFTA and a severely reduced estimated glomerular filtration rate were already detected at the time of diagnosis and amyloid deposition was also observed in the blood vessels. These findings indicate the importance of TBM amyloid deposition in the progression of renal disease. This may represent a late-stage presentation of the disease with a heavy LC burden.https://www.tandfonline.com/doi/10.1080/0886022X.2023.2203776Proteinurialight chain amyloidosistubular basement membranemonoclonal gammopathy of renal significancemultiple myeloma |
| spellingShingle | Csilla Markóth László Bidiga László Váróczy Ibolya File József Balla János Mátyus Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? Renal Failure Proteinuria light chain amyloidosis tubular basement membrane monoclonal gammopathy of renal significance multiple myeloma |
| title | Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? |
| title_full | Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? |
| title_fullStr | Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? |
| title_full_unstemmed | Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? |
| title_short | Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis? |
| title_sort | tubular basement membrane amyloid deposition is it an indicator of renal progression in light chain amyloidosis |
| topic | Proteinuria light chain amyloidosis tubular basement membrane monoclonal gammopathy of renal significance multiple myeloma |
| url | https://www.tandfonline.com/doi/10.1080/0886022X.2023.2203776 |
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