loading...| Preferred Name | anti-glomerular basement membrane disease | |
| Synonyms |
rapidly progressive glomerulonephritis with pulmonary hemorrhage pulmonary renal syndrome anti-glomerular basement membrane antibody disease glomerulonephritis - pulmonary hemorrhage Goodpasture syndrome anti-GBM syndrome anti-glomerular basement membrane disease |
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| Definitions |
Goodpasture syndrome is 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. |
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| ID |
http://purl.obolibrary.org/obo/MONDO_0009303 |
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| closeMatch |
http://identifiers.org/snomedct/50581000 http://identifiers.org/snomedct/155445002 http://identifiers.org/snomedct/266323005 http://identifiers.org/snomedct/195351002 |
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| database_cross_reference |
SCTID:236432001 ICD10:M31.0+ ICD9:446.21 UMLS:C0403529 MESH:D019867 ICD10:N08.5* DOID:9808 NCIT:C84566 OMIM:233450 GARD:0002551 Orphanet:375 EFO:0007290 ICD10:M31.0 MedDRA:10018620 |
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| disease has feature | ||
| exactMatch |
http://purl.obolibrary.org/obo/DOID_9808 http://identifiers.org/mesh/D019867 http://www.orpha.net/ORDO/Orphanet_375 http://purl.obolibrary.org/obo/NCIT_C84566 http://identifiers.org/snomedct/236432001 http://identifiers.org/omim/233450 |
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| has exact synonym |
Goodpasture syndrome anti-GBM syndrome anti-glomerular basement membrane disease |
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| has related synonym |
rapidly progressive glomerulonephritis with pulmonary hemorrhage pulmonary renal syndrome anti-glomerular basement membrane antibody disease glomerulonephritis - pulmonary hemorrhage |
|
| id |
MONDO:0009303 |
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| imported from | ||
| in_subset | ||
| label |
anti-glomerular basement membrane disease |
|
| notation |
MONDO:0009303 |
|
| prefLabel |
anti-glomerular basement membrane disease |
|
| see also |
https://rarediseases.info.nih.gov/diseases/2551/goodpasture-syndrome |
|
| textual definition |
Goodpasture syndrome is 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. |
|
| subClassOf |
http://purl.obolibrary.org/obo/MONDO_0030703 http://purl.obolibrary.org/obo/MONDO_0019724 |