Recurrence of Atypical Anti-Glomerular Basement Membrane Disease in Kidney Transplantation

Anti-glomerular basement membrane (anti-GBM) disease is a rare entity. The classic presentation includes circulating autoantibodies and rapidly progressive glomerulonephritis. However, there are atypical variants, which constitute 5% to 10% of cases. These variants are characterized by the absence of circulating antibodies, a more indolent clinical course, and the potential to recur in a kidney transplant. We present the case of a woman with chronic kidney disease initially diagnosed as unclassified glomerulonephritis. After the first transplant, a biopsy revealed linear IgG deposits with lambda chain restriction, leading to a diagnosis of atypical anti-GBM disease. The patient developed progressive chronic allograft dysfunction, which required a return to dialysis and, subsequently, a second transplant. The disease recurred again. This case highlights the diagnostic difficulty of atypical anti-GBM disease and the possibility of its recurrence after kidney transplantation. The recurrence of the monotypic variant may be associated with a plasma cell clone that is undetectable by conventional methods, suggesting a lower sensitivity to immunosuppression. The case also demonstrates the slow evolution of this disease, which underscores the need for long-term histological follow-up.