Anti-glomerular and anti-tubular basement membrane nephritis in a renal allograft recipient with Alport's syndrome.
A 28-year-old deaf, white man with a clinical diagnosis of Alport's syndrome since 4 years of age experienced renal failure, fever, and mental status changes suggestive of vasculitis following his first cadaveric renal transplant; these symptoms and changes resolved after removal of the allograft. Immunohistological stains demonstrated intense linear deposition of IgG and C3 in glomerular and tubular basement membranes in the absence of glomerular crescents. One year later, a second renal transplant led to similar symptoms. A biopsy was performed 14 days after engraftment, which demonstrated intense linear deposition of IgG in glomerular and tubular basement membranes, but cellular crescents were not present. A serologic profile was ordered to evaluate the patient further for vasculitis, and during the evaluation, circulating anti-glomerular basement membrane and anti-tubular basement membrane antibodies were identified by indirect immunofluorescence microscopy (titer, > 1:320). An open biopsy specimen obtained during repair of a renal laceration demonstrated a crescentic glomerulonephritis with immunohistologic findings identical to those of previous biopsies. Anti-glomerular basement membrane nephritis should be suspected in any patient with Alport's syndrome in whom progressive renal failure develops following renal transplantation. Detection of anti-glomerular basement membrane/anti-tubular basement membrane antibodies will assure the diagnosis, and early initiation of plasmapheresis may be helpful to prevent further renal damage.
Diaz
J I
JI
Department of Pathology, H. Lee Moffitt Cancer Center, University of South Florida Health Sciences Center, Tampa 33612-0179.
Valenzuela
R
R
Gephardt
G
G
Novick
A
A
Tubbs
R R
RR
D000328:Adult; D000917:Antibody Formation; D001485:Basement Membrane / Q000276:immunology; D006801:Humans; D007678:Kidney Glomerulus / Q000276:immunology*; D016030:Kidney Transplantation / Q000276:immunology* / Q000473:pathology; D007684:Kidney Tubules / Q000276:immunology*; D008297:Male; D008856:Microscopy, Fluorescence; D009393:Nephritis / Q000209:etiology / Q000276:immunology*; D009394:Nephritis, Hereditary / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000601:surgery