Anti glomerular basement membrane disease in a renal transplant patient with Alport syndrome

We report a case of anti GBM disease that developed in the renal graft of a patient with Alport syndrome. After reaching abnormal values of creatinine , the patient presented with deteriorating renal function three months afte r a cadaver transplant and the biopsy showed crescent formation, and linear IF deposits. Circulating antibodies against alpha 5 chain of type IV colla gen were found and plasmaphereses stabilized the condition for one year unt il a lung infection led to withdrawal of the immunosuppressive drugs and th e patient returned to dialysis. We discuss the possible mechanisms underlyi ng the specificity of the circulating antibodies in this case, which differ s from the target characteristic of the idiopathic form of anti GEM disease , the alpha 3 (IV) chain.