loading...| Preferred Name |
Autoimmune pulmonary alveolar proteinosis |
| ID |
http://www.orpha.net/ORDO/Orphanet_747 |
| alternative_term |
aPAP Autoimmune PAP |
| definition |
A rare primary interstitial lung disease characterized by the accumulation of lipids and proteins related to surfactant in the alveoli in association with the presence of antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). The disease leads to a progressive impairment of gas exchange and respiratory insufficiency. |
| definition_citation |
Orphanet |
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=747 |
| has_age_of_onset |
Adult Adolescent Childhood |
| has_inheritance |
Not applicable Multigenic/multifactorial |
| hasDbXref |
UMLS:C1970472 ICD-11:CB04.31 OMIM:610910 ICD-10:J84.0 |
| label |
Autoimmune pulmonary alveolar proteinosis |
| notation |
ORPHA:747 |
| part_of | |
| prefixIRI |
ORDO:Orphanet_747 |
| prefLabel |
Autoimmune pulmonary alveolar proteinosis |
| present_in |
Japan AND has_annual_incidence_average_value : 0.165 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_average_value : 2.66 AND has_point_prevalence_range : 1-9 / 1 000 000 United States AND has_point_prevalence_average_value : 0.687 AND has_point_prevalence_range : 1-9 / 1 000 000 Japan AND has_point_prevalence_average_value : 2.66 AND has_point_prevalence_range : 1-9 / 1 000 000 |
| treeView | |
| subClassOf |
| Delete | Mapping To | Ontology | Source |
|---|---|---|---|
| http://purl.obolibrary.org/obo/MONDO_0012579 | Mondo Disease Ontology / Mondo疾病本体 | LOOM |