Are there any special complications in patients with Alport syndrome following kidney transplantation?
Patients with XLAS who undergo transplant have survival rates similar to or better than those with other inherited kidney diseases.
However, 3% to 5% of transplanted males with Alport syndrome develop post-transplant anti-GBM nephritis, leading to rapid decline in allograft function despite aggressive therapy.
This condition results from an immunologic response by the recipient to the alpha-5 chain of type IV collagen, a previously unrecognized antigen in the transplant recipient.
The subset of patients with Alport syndrome at highest risk for this complication includes males with significant deafness and the onset of ESKD before the age of 30 years.
