Atypical antiglomerular basement membrane disease associated with thin membrane nephropathy.
Antiglomerular basement membrane (anti-GBM) disease is characteristically described with linear deposition of IgG along the GBM. We report two unusual cases of IgA and IgM anti-GBM disease associated with diffuse thinning of the GMB, and review the literature on atypical immunoglobulin species in this disorder. Both patients were male, aged 55 and 49 years, and presented with isolated microscopic haematuria, neither having shown evidence of impaired renal or pulmonary function on follow-up for 4 and 6 years respectively. Renal histology revealed minor focal mesangial changes only, but immunoperoxidase preparations demonstrated intense linear staining of the GBM with IgA in one case, and IgM with C3 in the other. On electron-microscopy there was diffuse thinning of the GBM in both cases, mean thickness 220 and 295 nm respectively (normal range 350-450 nm). Antinuclear antibodies were not detected and their glucose tolerance tests were normal. Assays for circulating IgG anti-GBM antibodies using indirect immunofluorescence (IF) and radioimmunoassay (RIA) were negative in both patients, although IgA anti-GBM antibodies with specificity confirmed by inhibition studies were identified in the first case. Thin GBMs in these patients may expose the Goodpasture antigen to toxic or infectious insults, thus altering its antigenic profile and promoting this unusual immune response.
de Caestecker
M P
MP
Department of Nephrology, Royal United Hospital, Bath, UK.
Hall
C L
CL
MacIver
A G
AG
D001327:Autoimmune Diseases / Q000276:immunology*; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D007075:Immunoglobulin M / Q000276:immunology*; D007518:Isoantibodies / Q000032:analysis; D007674:Kidney Diseases / Q000276:immunology / Q000473:pathology*; D007678:Kidney Glomerulus / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged
D007070:Immunoglobulin A; D007075:Immunoglobulin M; D007518:Isoantibodies