ANTIGLOMERULAR AND ANTI-TUBULAR BASEMENT-MEMBRANE NEPHRITIS IN A RENAL-ALLOGRAFT RECIPIENT WITH ALPORTS-SYNDROME

A 28-year-old deaf, white man with a clinical diagnosis of Alport's sy ndrome since 4 years of age experienced renal failure, fever, and ment al status changes suggestive of vasculitis following his first cadaver ic renal transplant; these symptoms and changes resolved after removal of the allograft. Immunohistological stains demonstrated intense line ar deposition of IgG and C3 in glomerular and tubular basement membran es in the absence of glomerular crescents. One year later, a second re nal transplant led to similar symptoms. A biopsy was performed 14 days after engraftment, which demonstrated intense linear deposition of Ig G in glomerular and tubular basement membranes, but cellular crescents were not present. A serologic profile was ordered to evaluate the pat ient further for vasculitis, and during the evaluation, circulating an ti-glomerular basement membrane and anti-tubular basement membrane ant ibodies were identified by indirect immunofluorescence microscopy (tit er, >1:320). An open biopsy specimen obtained during repair of a renal laceration demonstrated a crescentic glomerulonephritis with immunohi stologic findings identical to those of previous biopsies. Anti-glomer ular basement membrane nephritis should be suspected in any patient wi th Alport's syndrome in whom progressive renal failure develops follow ing renal transplantation. Detection of anti-glomerular basement membr ane/anti-tubular basement membrane antibodies will assure the diagnosi s, and early initiation of plasmapheresis may be helpful to prevent fu rther renal damage.