A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin
Posttransplant antiglomerular basement membrane (anti-GBM) disease occurs in approximately 5% of Alport patients and usually ends in irreversible graft failure. Recent research has focused on characterizing the structure of the anti-GBM alloepitope. Here we present a case of a 22-year-old male with...
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| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Transplantation |
| Online Access: | http://dx.doi.org/10.1155/2013/164016 |
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| author | Sumiko I. Armstead Thomas Hellmark Jorgen Wieslander Xin J. Zhou Ramesh Saxena Nilum Rajora |
| author_facet | Sumiko I. Armstead Thomas Hellmark Jorgen Wieslander Xin J. Zhou Ramesh Saxena Nilum Rajora |
| author_sort | Sumiko I. Armstead |
| collection | DOAJ |
| description | Posttransplant antiglomerular basement membrane (anti-GBM) disease occurs in approximately 5% of Alport patients and usually ends in irreversible graft failure. Recent research has focused on characterizing the structure of the anti-GBM alloepitope. Here we present a case of a 22-year-old male with end-stage renal disease secondary to Alport syndrome, with a previously failed renal allograft, who received a second deceased-donor kidney transplant. Six days after transplantation, he developed acute kidney injury. The serum anti-GBM IgG was negative by enzyme immunoassay (EIA). On biopsy, he had crescentic glomerulonephritis with linear GBM fixation of IgG. With further analysis by western blotting, we were able to detect antibodies to an unidentified protein from the basement membrane. This patient was treated with plasmapheresis twice per week and monthly intravenous immunoglobulin (IVIG) for a total of five months. At the end of treatment, these unknown antibodies were no longer detected. His renal function improved, and he has not required dialysis. We conclude that anti-GBM disease in patients with Alport Syndrome may be caused by circulating antibodies to other components of the basement membrane that are undetectable by routine anti-GBM EIA and may respond to treatment with plasmapheresis and IVIG. |
| format | Article |
| id | doaj-art-ea03525d059c4cd7b3339a4065a49515 |
| institution | Kabale University |
| issn | 2090-6943 2090-6951 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
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| series | Case Reports in Transplantation |
| spelling | doaj-art-ea03525d059c4cd7b3339a4065a495152025-02-03T01:03:06ZengWileyCase Reports in Transplantation2090-69432090-69512013-01-01201310.1155/2013/164016164016A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous ImmunoglobulinSumiko I. Armstead0Thomas Hellmark1Jorgen Wieslander2Xin J. Zhou3Ramesh Saxena4Nilum Rajora5Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390, USAThe Kidney Research Lab, Lund University, Lund, SwedenThe Kidney Research Lab, Lund University, Lund, SwedenDepartment of Pathology, Baylor University Medical Center at Dallas, Dallas, TX 75246, USADivision of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390, USADivision of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390, USAPosttransplant antiglomerular basement membrane (anti-GBM) disease occurs in approximately 5% of Alport patients and usually ends in irreversible graft failure. Recent research has focused on characterizing the structure of the anti-GBM alloepitope. Here we present a case of a 22-year-old male with end-stage renal disease secondary to Alport syndrome, with a previously failed renal allograft, who received a second deceased-donor kidney transplant. Six days after transplantation, he developed acute kidney injury. The serum anti-GBM IgG was negative by enzyme immunoassay (EIA). On biopsy, he had crescentic glomerulonephritis with linear GBM fixation of IgG. With further analysis by western blotting, we were able to detect antibodies to an unidentified protein from the basement membrane. This patient was treated with plasmapheresis twice per week and monthly intravenous immunoglobulin (IVIG) for a total of five months. At the end of treatment, these unknown antibodies were no longer detected. His renal function improved, and he has not required dialysis. We conclude that anti-GBM disease in patients with Alport Syndrome may be caused by circulating antibodies to other components of the basement membrane that are undetectable by routine anti-GBM EIA and may respond to treatment with plasmapheresis and IVIG.http://dx.doi.org/10.1155/2013/164016 |
| spellingShingle | Sumiko I. Armstead Thomas Hellmark Jorgen Wieslander Xin J. Zhou Ramesh Saxena Nilum Rajora A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin Case Reports in Transplantation |
| title | A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin |
| title_full | A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin |
| title_fullStr | A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin |
| title_full_unstemmed | A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin |
| title_short | A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin |
| title_sort | case of alport syndrome with posttransplant antiglomerular basement membrane disease despite negative antiglomerular basement membrane antibodies by eia treated with plasmapheresis and intravenous immunoglobulin |
| url | http://dx.doi.org/10.1155/2013/164016 |
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