ORPHA:93568
Juvenile polymyositis
Synonyms: Juvenile PM
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Childhood
- Status
- active
A rare type of juvenile idiopathic inflammatory myopathy (IIM) characterized by an onset before 18 years of age of chronic skeletal muscle inflammation, manifesting as progressive, proximal and distal muscle weakness and atrophy.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Juvenile polymyositis | Juvenile PM |
| fr | Polymyosite juvénile | PM juvénile |
| de | Polymyositis, juvenile | |
| es | Polimiositis juvenil | PM juvenil |
| it | Polimiosite giovanile | PM giovanile |
| nl | Juveniele polymyositis | Juveniele PM |
| pt | Polimiosite juvenil | |
| pl | Młodzieńcze zapalenie wielomięśniowe | Młodzieńcze PM |
| cs | Juvenilní polymyozitida | Juvenilní PM |
| uk | Ювенільний поліміозит | Ювенільний PM |
| zh | 幼年型多发性肌炎 | 幼年型PM |
| tr | Juvenil polimiyozit | jüvenil PM |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M33.2 | NTBT | attributed | validated |
| ICD-11 | 4A41.10 | E | specific | validated |
| MONDO | 0019734 | E | validated | |
| UMLS | C3826988 | E | validated | |
| GARD | 12742 | E | validated | |
| MedDRA | 10076673 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (4):
- ORPHA:329888Juvenile idiopathic inflammatory myopathy
rare neurological diseases; rare systemic and rheumatological diseases - ORPHA:567558Non-genetic systemic disease with glomerulopathy as a major feature
rare renal diseases; rare transplant-related disorders - ORPHA:280373Rare pediatric systemic disease
rare systemic or rheumatologic diseases of childhood - ORPHA:264704Secondary interstitial lung disease specific to childhood associated with a connective tissue disease
rare respiratory diseases; rare transplant-related disorders
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 39 cases | Worldwide | Case(s) | Validated | 23925923[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 23925923[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93568
- This record as JSON: /data/d/93568.json