loading...| Preferred Name |
Dermatomyositis |
|
| Synonyms |
Adult dermatomyositis |
|
| Definitions |
A rare idiopathic inflammatory myopathy (IIM) characterized by evocative skin lesions, muscle involvement with symmetrical proximal muscle weakness, and specific histological features. The clinical subtypes are defined by the presence of myositis-specific antibodies (anti-Mi2, anti-NXP2, anti-TIF1-γ, anti-MDA5, or anti-SAE antibodies) and are associated with specific clinical phenotypes and prognosis. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_221 |
|
| alternative_term |
Adult dermatomyositis |
|
| definition |
A rare idiopathic inflammatory myopathy (IIM) characterized by evocative skin lesions, muscle involvement with symmetrical proximal muscle weakness, and specific histological features. The clinical subtypes are defined by the presence of myositis-specific antibodies (anti-Mi2, anti-NXP2, anti-TIF1-γ, anti-MDA5, or anti-SAE antibodies) and are associated with specific clinical phenotypes and prognosis. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=221 |
|
| has_age_of_onset |
Adult Elderly |
|
| has_inheritance |
Not applicable |
|
| hasDbXref |
ICD-11:4A41.00 UMLS:C0011633 ICD-10:M33.1 MedDRA:10012503 MeSH:D003882 ICD-10:M33.0 |
|
| label |
Dermatomyositis |
|
| notation |
ORPHA:221 |
|
| part_of |
http://www.orpha.net/ORDO/Orphanet_98482 |
|
| prefixIRI |
ORDO:Orphanet_221 |
|
| prefLabel |
Dermatomyositis |
|
| present_in |
Norway AND has_point_prevalence_average_value : 4.9172 AND has_point_prevalence_range : 1-9 / 100 000 Germany AND has_annual_incidence_average_value : 3.5 AND has_annual_incidence_range : 1-9 / 100 000 Germany AND has_point_prevalence_average_value : 11.3 AND has_point_prevalence_range : 1-5 / 10 000 Spain AND has_annual_incidence_average_value : 0.49 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_average_value : 7.5312 AND has_point_prevalence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 13.0 AND has_point_prevalence_range : 1-5 / 10 000 Argentina AND has_point_prevalence_average_value : 10.2 AND has_point_prevalence_range : 1-5 / 10 000 Sweden AND has_annual_incidence_average_value : 0.32 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_annual_incidence_average_value : 0.9704 AND has_annual_incidence_range : 1-9 / 100 000 Argentina AND has_annual_incidence_average_value : 0.32 AND has_annual_incidence_range : 1-9 / 1 000 000 United States AND has_annual_incidence_average_value : 1.1 AND has_annual_incidence_range : 1-9 / 100 000 Australia AND has_point_prevalence_average_value : 1.97 AND has_point_prevalence_range : 1-9 / 100 000 Australia AND has_annual_incidence_average_value : 0.1 AND has_annual_incidence_range : 1-9 / 1 000 000 Sweden AND has_point_prevalence_average_value : 3.8 AND has_point_prevalence_range : 1-9 / 100 000 |
|
| treeView |
http://www.orpha.net/ORDO/Orphanet_98482 |
|
| subClassOf |