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Orphanet Rare Disease Ontology
Last uploaded:
December 4, 2024
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Id | http://www.orpha.net/ORDO/Orphanet_375
http://www.orpha.net/ORDO/Orphanet_375
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Preferred Name | Anti-glomerular basement membrane disease |
Definitions |
A rare, fulminant small vessel vasculitis that affects the capillary beds of the kidneys and lungs and characterized by the presence of anti-glomerular basement membrane (GBM) and, in its full-blown form, anti-alveolar basement membrane (ABM) antibodies. Consequently, it may manifest as a rapidly progressive, isolated glomerulonephritis (anti-GBM nephritis) or as a pulmonary-renal syndrome with severe lung hemorrhage.
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Synonyms |
Goodpasture syndrome
Anti-GBM syndrome
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Type | http://www.w3.org/2002/07/owl#Class |
All Properties
definition_citation | Orphanet |
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definition | A rare, fulminant small vessel vasculitis that affects the capillary beds of the kidneys and lungs and characterized by the presence of anti-glomerular basement membrane (GBM) and, in its full-blown form, anti-alveolar basement membrane (ABM) antibodies. Consequently, it may manifest as a rapidly progressive, isolated glomerulonephritis (anti-GBM nephritis) or as a pulmonary-renal syndrome with severe lung hemorrhage. |
label |
Anti-glomerular basement membrane disease
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prefLabel |
Anti-glomerular basement membrane disease
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hasDbXref |
OMIM:233450
ICD-10:N08.5*
ICD-11:MF85
UMLS:C0403529
MeSH:D019867
MedDRA:10018620
ICD-10:M31.0+
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notation |
ORPHA:375
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alternative_term |
Goodpasture syndrome
Anti-GBM syndrome
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present_in |
China AND has_annual_incidence_average_value : 0.06 AND has_annual_incidence_range : <1 / 1 000 000
Europe AND has_annual_incidence_average_value : 0.08 AND has_annual_incidence_range : <1 / 1 000 000
Europe AND has_point_prevalence_average_value : 0.2 AND has_point_prevalence_range : 1-9 / 1 000 000
New Zealand AND has_annual_incidence_average_value : 0.179 AND has_annual_incidence_range : 1-9 / 1 000 000
Worldwide AND has_point_prevalence_range : Unknown
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part_of | |
prefixIRI |
ORDO:Orphanet_375
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expertlink | |
subClassOf | |
type | |
has_inheritance |
Not applicable
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has_age_of_onset |
Adolescent
Adult
Elderly
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