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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]
On electron microscopy, subepithelial deposits that nestle against the glomerular basement membrane seems to be the cause of the thickening. Also, the podocytes lose their foot processes . As the disease progresses, the deposits will eventually be cleared, leaving cavities in the basement membrane.
Video explanation. Author: Tanner Marshall Editor: Rishi Desai, MD, MPH Membranous glomerulonephritis, also known as membranous nephropathy, is where the glomerular basement membrane, or GBM, which lines the glomeruli in the kidney, becomes inflamed and damaged, which results in increased permeability and proteins being able to filter through into the urine, causing nephrotic syndrome.
Goodpasture syndrome - This is a rare autoimmune disease where autoantibodies are produced that target the glomerular basement membrane in both the lungs and the kidneys. The damage to the basement membrane causes bleeding, and the disease often presents in patients as hematuria and haemoptysis (coughing up blood).
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 presentation.
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]
Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis caused by deposits in the kidney glomerular mesangium and basement membrane thickening, [2] activating the complement system and damaging the glomeruli. MPGN accounts for approximately 4% of primary renal causes of nephrotic syndrome in children and 7% in adults. [3]
Pulmonary-renal syndromes are most commonly caused by an underlying autoimmune disease. PRS is most commonly due to ANCA-associated vasculitides (e.g., granulomatosis with polyangiitis) or due to anti-basement membrane diseases (e.g., Goodpasture's syndrome).