Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
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

http://www.orpha.net/ORDO/Orphanet_567558

http://www.orpha.net/ORDO/Orphanet_290836

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

http://www.orpha.net/ORDO/Orphanet_567558

http://www.orpha.net/ORDO/Orphanet_290836

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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Delete Mapping To Ontology Source
http://www.owl-ontologies.com/unnamed.owl#RID14482 DERMLEX LOOM
http://www.ebi.ac.uk/efo/EFO_0000398 CCONT LOOM
http://www.ebi.ac.uk/efo/EFO_0000398 HSPO LOOM
http://www.ebi.ac.uk/efo/EFO_0000398 EFO LOOM
http://www.ebi.ac.uk/efo/EFO_0000398 EFO LOOM
http://purl.bioontology.org/ontology/ICD9CM/710.3 ICD9CM LOOM
http://purl.obolibrary.org/obo/MONDO_0016367 GCBO LOOM
http://purl.obolibrary.org/obo/MONDO_0016367 MONDO LOOM
http://purl.obolibrary.org/obo/MONDO_0016367 DOVES LOOM
http://purl.obolibrary.org/obo/MONDO_0016367 KTAO LOOM
http://purl.obolibrary.org/obo/MONDO_0016367 OBA LOOM
http://purl.bioontology.org/ontology/CST/DERMATOMYOSITIS COSTART LOOM
http://purl.bioontology.org/ontology/CSP/1982-9738 CRISP LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C10.668.491.562.575.500 RH-MESH LOOM
http://purl.obolibrary.org/obo/DOID_10223 DOID LOOM
http://purl.obolibrary.org/obo/DOID_10223 BAO LOOM
http://purl.obolibrary.org/obo/DOID_10223 HHEAR LOOM
http://purl.obolibrary.org/obo/DOID_10223 DDSS LOOM
http://purl.obolibrary.org/obo/DOID_10223 NIFSTD LOOM
http://purl.obolibrary.org/obo/DOID_10223 MIDO LOOM
http://purl.obolibrary.org/obo/DOID_10223 FNS-H LOOM
https://github.com/sap218/ocimido/blob/master/ontology/ocimido.owl#OCIMIDO_00064 OCIMIDO LOOM
http://www.limics.fr/ontologies/ontolurgences#Dermatomyosite ONTOLURGENCES LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C05.651.594.819.500 RH-MESH LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#D003882 RH-MESH LOOM
http://www.semanticweb.org/ontologies/2012/5/Ontology1338526551855.owl#Dermatomyositis RPO LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C17.300.250 RH-MESH LOOM
http://purl.bioontology.org/ontology/SNMI/D1-10300 SNMI LOOM
http://www.phoc.org.cn/pmo/class/PMO_00037746 PMAPP-PMO LOOM
http://www.co-ode.org/ontologies/galen#Dermatomyositis GALEN LOOM
http://purl.obolibrary.org/obo/NCIT_C26744 BERO LOOM
http://purl.bioontology.org/ontology/RCD/XE1DH RCD LOOM
http://purl.obolibrary.org/obo/DERMO_0001883 DERMO LOOM
http://purl.bioontology.org/ontology/ICPC2P/L99048 ICPC2P LOOM
http://www.owl-ontologies.com/NPOntology.owl#DOID_10223 NATPRO LOOM
http://purl.bioontology.org/ontology/MEDDRA/10012503 MEDDRA LOOM
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#Dermatomyositis CSEO LOOM
rgo:03312 GAMUTS LOOM
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C26744 NCIT LOOM
http://purl.bioontology.org/ontology/OMIM/MTHU018873 OMIM LOOM
http://bmi.utah.edu/ontologies/hfontology/C0011633 HFO LOOM
http://nanbyodata.jp/ontology/NANDO_1200274 NANDO LOOM
http://sbmi.uth.tmc.edu/ontology/ochv#3800 OCHV LOOM
http://purl.bioontology.org/ontology/MEDLINEPLUS/C0011633 MEDLINEPLUS LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C05.651.594.297 RH-MESH LOOM
http://radlex.org/RID/RID34666 RADLEX LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C17.800.185 RH-MESH LOOM
http://purl.bioontology.org/ontology/MESH/D003882 MESH LOOM
http://sbmi.uth.tmc.edu/ontology/ochv#C0011633 OCHV LOOM
http://purl.obolibrary.org/obo/OMIT_0005158 OMIT LOOM
http://purl.bioontology.org/ontology/SNOMEDCT/396230008 SNOMEDCT LOOM
http://www.limics.org/hrdo/rdfns#pat_id_701 HRDO LOOM
http://purl.bioontology.org/ontology/RCTV2/N003.00 RCTV2 LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C10.668.491.562.150 RH-MESH LOOM
http://purl.jp/bio/4/id/200906047537122067 IOBC LOOM
http://localhost/plosthes.2017-1#3723 PLOSTHES LOOM
http://www.gamuts.net/entity#dermatomyositis GAMUTS REST