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Goodpasture syndrome (GPS), also known as anti–glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs, glomerulonephritis, [1] and kidney failure. [2]
Anti-glomerular basement membrane antibody (anti-GBM Ab) is an antibody which is found in Goodpasture's syndrome but not found in microscopic polyangiitis. Some sources consider "anti-GBM disease" and "Goodpasture disease" to be synonymous terms describing histological presentation, reserving the term "Goodpasture syndrome" for clinical ...
The presence of anti-glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum. [2]
Leukopenia, thrombocytopenia, Coombs' test, complement activation: low serum concentrations of C3 and C4, positive immunofluorescence using Crithidia luciliae as substrate, antiphospholipid antibodies (i.e. anticardiolipin, lupus anticoagulant, false-positive VDRL) Goodpasture's disease: Anti-glomerular basement membrane antibody
Immune complexes (black) are deposited in a thickened basement membrane creating a "spike and dome" appearance on electron microscopy. MGN is caused by immune complex formation in the glomerulus. The immune complexes are formed by binding of antibodies to antigens in the glomerular basement membrane. The antigens may be part of the basement ...
Malaise, arthralgia, sinusitis, and rhinitis are typically present at the beginning of Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Prodromes often occur weeks or months before pulmonary-renal syndrome. [3] Anti-glomerular basement membrane (anti-GBM) vasculitis patients usually report sudden onset of anuria or oliguria.
Antiglomerular basement membrane (anti-GBM) antibody - If positive, this is highly indicative of Goodpasture's syndrome and can be used to guide treatment. [9] Antineutrophil cytoplasmic antibody (ANCA) - If positive, this indicates that there is likely an underlying vasculitis that may be causing the acute nephritic syndrome. [36]
The glomerular basement membrane of the kidney is the basal lamina layer of the glomerulus.The glomerular endothelial cells, the glomerular basement membrane, and the filtration slits between the podocytes perform the filtration function of the glomerulus, separating the blood in the capillaries from the filtrate that forms in Bowman's capsule. [1]