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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
Joint Authors
Hellmark, Thomas
Wieslander, Jorgen
Rajora, Nilum
Armstead, Sumiko I.
Zhou, Xin J.
Saxena, Ramesh
Source
Case Reports in Transplantation
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-12-02
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Armstead, Sumiko I.& Hellmark, Thomas& Wieslander, Jorgen& Zhou, Xin J.& Saxena, Ramesh& Rajora, Nilum. 2013. 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،Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-450968
Modern Language Association (MLA)
Armstead, Sumiko I.…[et al.]. 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 No. 2013 (2013), pp.1-5.
https://search.emarefa.net/detail/BIM-450968
American Medical Association (AMA)
Armstead, Sumiko I.& Hellmark, Thomas& Wieslander, Jorgen& Zhou, Xin J.& Saxena, Ramesh& Rajora, Nilum. 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. 2013. Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-450968
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-450968