Go back to clinical information and images

Diagnosis: Post-Transplant Anti Glomerular Basement Membrane Disease (in patient with Alport syndrome)

The glomerular basement membrane of patients with Alport syndrome lacks of normal alpha3-alpha4-alpha5 structure of the type IV collagen. Posttransplant anti-GBM nephritis is an allogeneic response to antigens present in the transplanted kidney but absent in the native kidneys of the Alport recipient. When patients with Alport syndrome receive renal transplants, posttransplant anti-GBM nephritis occurs in 3–5% of patients. In X-linked Alport syndrome, target alloantibodies are to the alpha5(IV) noncollagenous 1 domain primarily and commercial assays are relatively insensitive to these antibodies (Kashtan CE. Pediatr Transplant. 2006;10(6):651-7. [PubMed link]). Current treatment options include methylprednisolone pulse therapy, plasmapheresis, cyclophosphamide, mycophenolate, IVIG, and rituximab, and recent studies indicate bortezomib may be effective as well. Graft failure approaches 90% regardless of the specific therapy used [7] (Armstead SI, et al. Case Rep Transplant. 2013;2013:164016. [PubMed link]).

See the chapter: Hereditary diseases of our Tutorial (only Spanish version).

Go back to clinical information and images

References

• Kelly YP, Patil A, Wallis L, Murray S, Kant S, Kaballo MA, Casserly L, Doyle B, Dorman A, O'Kelly P, Conlon PJ. Outcomes of kidney transplantation in Alport syndrome compared with other forms of renal disease. Ren Fail. 2017;39(1):290-293. [PubMed link]
• Yilmaz VT, Dinckan A, Yilmaz F, Suleymanlar G, Kocak H. Outcomes of Renal Transplantation in Patients With Alport Syndrome. Transplant Proc. 2015;47(5):1377-81. [PubMed link]
• Olaru F, Luo W, Wang XP, Ge L, Hertz JM, Kashtan CE, Sado Y, Segal Y, Hudson BG, Borza DB. Quaternary epitopes of α345(IV) collagen initiate Alport post-transplant anti-GBM nephritis. J Am Soc Nephrol. 2013;24(6):889-95. [PubMed link]
• Armstead SI, Hellmark T, Wieslander J, Zhou XJ, Saxena R, Rajora N. 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 Rep Transplant. 2013;2013:164016. [PubMed link]
• Kashtan CE. Renal transplantation in patients with Alport syndrome. Pediatr Transplant. 2006;10(6):651-7. [PubMed link]

[Top]

Go back to clinical information and images