Diagnosis and classification of Goodpasture's disease (anti-GBM).
Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli where the inflammation usually results in a severe rapidly progressive glomerulonephritis. Despite modern treatment less than one third of the patients survive with a preserved kidney function after 6 months follow-up. Frequencies vary from 0.5 to 1 cases per million inhabitants per year and there is a strong genetic linkage to HLA-DRB1(∗)1501 and DRB1(∗)1502. Essentially, anti-GBM disease is now a preferred term for what was earlier called Goodpasture's syndrome or Goodpasture's disease; anti-GBM disease is now classified as small vessel vasculitis caused by in situ immune complex formation; the diagnosis relies on the detection of anti-GBM in tissues or circulation in conjunction with alveolar or glomerular disease; therapy is effective only when detected at an early stage, making a high degree of awareness necessary to find these rare cases; 20-35% have anti-GBM and MPO-ANCA simultaneously, which necessitates testing for anti-GBM whenever acute test for ANCA is ordered in patients with renal disease.
Department of Nephrology, Clinical Sciences in Lund, BMC-B13, Lund University, 221 84 Lund, Sweden. Electronic address: Thomas.Hellmark@med.lu.se.
Thomas
T
Hellmark
Division of Drug Research/Nephrology, Department of Medical and Health Sciences, Linköping University, 581 85 Linköping, Sweden. Electronic address: marten.segelmark@liu.se.
Mårten
M
Segelmark
D019867:Anti-Glomerular Basement Membrane Disease / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology; D042241:Early Diagnosis; D006801:Humans; D007249:Inflammation / Q000145:classification / Q000175:diagnosis / Q000276:immunology; D007678:Kidney Glomerulus / Q000098:blood supply / Q000276:immunology / Q000473:pathology; D014657:Vasculitis / Q000175:diagnosis
Anti-GBM disease; Autoantibodies; Glomerulonephritis; Goodpasture's disease; Vasculitis