loading...| Preferred Name |
Sarcoidosis |
|
| Synonyms |
Besnier-Boeck-Schaumann disease |
|
| Definitions |
A rare multisystemic, autoinflammatory disorder of unknown etiology characterized by the formation of immune, non-caseating granulomas in any organ(s), leading to variable clinical symptoms and severity. Clinical presentation is typically with persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and Löfgren syndrome. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_797 |
|
| alternative_term |
Besnier-Boeck-Schaumann disease Boeck sarcoid |
|
| definition |
A rare multisystemic, autoinflammatory disorder of unknown etiology characterized by the formation of immune, non-caseating granulomas in any organ(s), leading to variable clinical symptoms and severity. Clinical presentation is typically with persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and Löfgren syndrome. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=797 |
|
| has_age_of_onset |
Adolescent Adult Childhood Elderly |
|
| has_inheritance |
Multigenic/multifactorial |
|
| hasDbXref |
ICD-11:4B20.2 ICD-11:4B20.1 ICD-11:4B20.3 ICD-11:4B20.4 ICD-11:4B20.5 OMIM:612387 OMIM:612388 ICD-10:D86.0 ICD-10:D86.8 OMIM:181000 ICD-10:D86.9 MedDRA:10039486 ICD-10:D86.1 ICD-10:D86.3 ICD-11:4B20 MeSH:D012507 ICD-10:D86.2 UMLS:C0036202 |
|
| label |
Sarcoidosis |
|
| notation |
ORPHA:797 |
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| part_of |
http://www.orpha.net/ORDO/Orphanet_324930 http://www.orpha.net/ORDO/Orphanet_95617 http://www.orpha.net/ORDO/Orphanet_182222 http://www.orpha.net/ORDO/Orphanet_280933 http://www.orpha.net/ORDO/Orphanet_280930 http://www.orpha.net/ORDO/Orphanet_280926 http://www.orpha.net/ORDO/Orphanet_209007 http://www.orpha.net/ORDO/Orphanet_264745 http://www.orpha.net/ORDO/Orphanet_166484 http://www.orpha.net/ORDO/Orphanet_567558 |
|
| prefixIRI |
ORDO:Orphanet_797 |
|
| prefLabel |
Sarcoidosis |
|
| present_in |
Finland AND has_point_prevalence_average_value : 28.2 AND has_point_prevalence_range : 1-5 / 10 000 Guadeloupe AND has_annual_incidence_average_value : 2.3 AND has_annual_incidence_range : 1-9 / 100 000 Greece AND has_point_prevalence_average_value : 5.89 AND has_point_prevalence_range : 1-9 / 100 000 Greece AND has_annual_incidence_average_value : 1.07 AND has_annual_incidence_range : 1-9 / 100 000 Denmark AND has_annual_incidence_average_value : 4.68 AND has_annual_incidence_range : 1-9 / 100 000 Czech Republic AND has_point_prevalence_average_value : 63.0 AND has_point_prevalence_range : 6-9 / 10 000 Taiwan, Province of China AND has_point_prevalence_average_value : 2.17 AND has_point_prevalence_range : 1-9 / 100 000 Czech Republic AND has_annual_incidence_average_value : 4.0 AND has_annual_incidence_range : 1-9 / 100 000 Norway AND has_annual_incidence_average_value : 14.4 AND has_annual_incidence_range : 1-5 / 10 000 Iceland AND has_annual_incidence_average_value : 3.84 AND has_annual_incidence_range : 1-9 / 100 000 Korea, Republic of AND has_annual_incidence_average_value : 0.125 AND has_annual_incidence_range : 1-9 / 1 000 000 Europe AND has_point_prevalence_average_value : 20.0 AND has_point_prevalence_range : 1-5 / 10 000 Italy AND has_point_prevalence_average_value : 49.0 AND has_point_prevalence_range : 1-5 / 10 000 United Kingdom AND has_annual_incidence_average_value : 5.0 AND has_annual_incidence_range : 1-9 / 100 000 Worldwide AND has_point_prevalence_range : 1-5 / 10 000 France AND has_point_prevalence_average_value : 30.2 AND has_point_prevalence_range : 1-5 / 10 000 Switzerland AND has_point_prevalence_range : 6-9 / 10 000 Canada AND has_point_prevalence_average_value : 10.5 AND has_point_prevalence_range : 1-5 / 10 000 Sweden AND has_point_prevalence_average_value : 160.0 AND has_point_prevalence_range : >1 / 1000 Singapore AND has_annual_incidence_average_value : 0.56 AND has_annual_incidence_range : 1-9 / 1 000 000 Australia AND has_annual_incidence_average_value : 5.35 AND has_annual_incidence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 60.0 AND has_point_prevalence_range : 6-9 / 10 000 Uruguay AND has_annual_incidence_average_value : 0.4 AND has_annual_incidence_range : 1-9 / 1 000 000 Japan AND has_point_prevalence_average_value : 3.7 AND has_point_prevalence_range : 1-9 / 100 000 Switzerland AND has_annual_incidence_average_value : 7.0 AND has_annual_incidence_range : 1-9 / 100 000 France AND has_annual_incidence_average_value : 4.9 AND has_annual_incidence_range : 1-9 / 100 000 Norway AND has_point_prevalence_average_value : 26.7 AND has_point_prevalence_range : 1-5 / 10 000 Croatia AND has_annual_incidence_average_value : 3.3 AND has_annual_incidence_range : 1-9 / 100 000 Guadeloupe AND has_point_prevalence_average_value : 21.0 AND has_point_prevalence_range : 1-5 / 10 000 Spain AND has_annual_incidence_average_value : 4.5 AND has_annual_incidence_range : 1-9 / 100 000 Sweden AND has_annual_incidence_average_value : 11.5 AND has_annual_incidence_range : 1-5 / 10 000 Japan AND has_annual_incidence_average_value : 1.01 AND has_annual_incidence_range : 1-9 / 100 000 Ireland AND has_point_prevalence_average_value : 48.1 AND has_point_prevalence_range : 1-5 / 10 000 Portugal AND has_point_prevalence_average_value : 0.2 AND has_point_prevalence_range : 1-9 / 1 000 000 |
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| treeView |
http://www.orpha.net/ORDO/Orphanet_324930 http://www.orpha.net/ORDO/Orphanet_95617 http://www.orpha.net/ORDO/Orphanet_182222 http://www.orpha.net/ORDO/Orphanet_280933 http://www.orpha.net/ORDO/Orphanet_280930 http://www.orpha.net/ORDO/Orphanet_280926 http://www.orpha.net/ORDO/Orphanet_209007 http://www.orpha.net/ORDO/Orphanet_264745 http://www.orpha.net/ORDO/Orphanet_166484 http://www.orpha.net/ORDO/Orphanet_567558 |
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| subClassOf |