anti-glomerular basement membrane disease
An autoimmune disease that affects the lungs and kidneys and is characterized by pulmonary alveolar hemorrhage (bleeding in the lungs) and a kidney disease known as glomerulonephritis. Some use the term 'Goodpasture syndrome' for the findings of glomerulonephritis and pulmonary hemorrhage and the term 'Goodpasture disease' for those patients with glomerulonephritis, pulmonary hemorrhage, and anti-GBM antibodies. Currently, the preferred term for both conditions is “ anti-GBM antibody disease ”. Circulating antibodies are directed against the collagen of the part of the kidney known as the glomerular basement membrane (GBM), resulting in acute or rapidly progressive glomerulonephritis. Antibodies also attack the collagen of the air sacs of the lung (alveoli) resulting in bleeding of the lung (pulmonary hemorrhage). Symptoms may include general body discomfort or pain, bleeding from the nose and/or blood in the urine, respiratory problems, anemia, chest pain, and kidney failure. Anti-GBM disease is thought to result from an environmental insult (smoking, infections, exposure to certain drugs) in a person with genetic susceptibility, such as a specific human leukocyte antigen (HLA) type. Diagnosis is confirmed with the presence of anti-GBM antibody in the blood or in the kidney. The treatment of choice is plasmapheresis in conjunction with prednisone and cyclophosphamide.
MONDO:0015490
obsolete predominantly small-vessel vasculitis (obsolete MONDO:0015490)
excluded_subClassOf
MONDO:0017035
obsolete secondary interstitial lung disease in childhood and adulthood associated with a systemic disease (obsolete MONDO:0017035)
excluded_subClassOf
MONDO:0019724
obsolete secondary glomerular disease (obsolete MONDO:0019724)
excluded_subClassOf
MONDO:0007179
autoimmune disease
is_a
Goodpasture Syndrome
exact
Goodpasture syndrome
exact
anti-GBM syndrome
exact
anti-glomerular basement membrane antibody disease
related
anti-glomerular basement membrane disease
exact
glomerulonephritis - pulmonary haemorrhage
related
glomerulonephritis - pulmonary hemorrhage
related
pulmonary renal syndrome
related
rapidly progressive glomerulonephritis with pulmonary haemorrhage
related
rapidly progressive glomerulonephritis with pulmonary hemorrhage
related