Polymyositis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare idiopathic inflammatory myopathy (IIM) historically characterized by symmetric proximal muscle weakness, elevated muscle enzymes (creatine kinase), myopathic findings on electromyography, and muscle biopsy showing endomyial infiltration composed mainly of macrophages and lymphocytes. The features are non-specific, thus the disease should be distinguished from similar entities with specific clinical, immunological, histological features, notably dermatomyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, inclusion body myositis, and myositis associated with other connective tissue disorder.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Polymyositis | |
| fr | Polymyosite | |
| de | Polymyositis | |
| es | Polimiositis | |
| it | Polimiosite | PM |
| nl | Polymyositis | |
| pt | Polimiosite | |
| pl | Zapalenie wielomięśniowe | PM |
| cs | Polymyozitida | PM |
| uk | Поліміозит | |
| zh | 多发性肌炎 | |
| tr | Polimiyozit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M33.2 | E | specific | validated |
| ICD-11 | 4A41.1 | E | specific | validated |
| MONDO | 0019127 | E | validated | |
| MeSH | D017285 | E | validated | |
| UMLS | C0085655 | E | validated | |
| GARD | 7425 | E | validated | |
| MedDRA | 10036102 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:98482Idiopathic 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
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
8 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.585 / 100 000 | Europe | Value and class | Validated | ORPHANET |
| Point prevalence | 1-9 / 100 000 | 7.1 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.75 / 100 000 | Argentina | Value and class | Validated | 23965482[PMID] |
| Point prevalence | 1-9 / 100 000 | 7.2 / 100 000 | Argentina | Value and class | Validated | 23965482[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.41 / 100 000 | Australia | Value and class | Validated | 23981756[PMID] |
| Point prevalence | 1-9 / 100 000 | 7.2 / 100 000 | Australia | Value and class | Validated | 23981756[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.39 / 100 000 | Spain | Value and class | Validated | 18053482[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.76 / 100 000 | Sweden | Value and class | Validated | 9137324[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
60 annotations · validated 2024-06-27| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004303 | Abnormal muscle fiber morphology | |
| HP:0002829 | Arthralgia | |
| HP:0002960 | Autoimmunity | |
| HP:0012735 | Cough | |
| HP:0003457 | EMG abnormality | |
| HP:0012544 | Elevated circulating aldolase concentration | |
| HP:0003236 | Elevated circulating creatine kinase concentration | |
| HP:0001252 | Hypotonia | |
| HP:0003701 | Proximal muscle weakness | |
| Frequent (79-30%) | ||
| HP:0006530 | Abnormal pulmonary interstitial morphology | |
| HP:0002039 | Anorexia | |
| HP:0034145 | Anti-alanyl-tRNA synthetase antibody positivity | |
| HP:0034147 | Anti-aminoacyl-tRNA synthetase antibody positivity | |
| HP:0034151 | Anti-asparaginyl-tRNA synthetase antibody positivity | |
| HP:0034146 | Anti-glycyl tRNA-synthetase antibody positivity | |
| HP:0034152 | Anti-histidyl tRNA synthetase antibody positivity | |
| HP:0034148 | Anti-isoleucyl tRNA-synthetase antibody positivity | |
| HP:0034149 | Anti-phenylalanyl tRNA synthetase antibody positivity | |
| HP:0033713 | Anti-signal recognition particle antibody positivity | |
| HP:0034143 | Anti-threonyl-tRNA synthetase antibody positivity | |
| HP:0034150 | Anti-tyrosyl-tRNA synthetase antibody positivity | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0001369 | Arthritis | |
| HP:0002019 | Constipation | |
| HP:0002875 | Exertional dyspnea | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0003326 | Myalgia | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0005150 | Abnormal atrioventricular conduction | |
| HP:0001633 | Abnormal mitral valve morphology | |
| HP:0000091 | Abnormal renal tubule morphology | |
| HP:0001608 | Abnormality of the voice | |
| HP:0011675 | Arrhythmia | |
| HP:0100595 | Camptocormia | |
| HP:0031352 | Chest tightness | |
| HP:0000934 | Chondrocalcinosis | |
| HP:0001635 | Congestive heart failure | |
| HP:0001644 | Dilated cardiomyopathy | |
| HP:0001618 | Dysphonia | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0001288 | Gait disturbance | |
| HP:0002020 | Gastroesophageal reflux | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0002240 | Hepatomegaly | |
| HP:0001611 | Hypernasal speech | |
| HP:0001639 | Hypertrophic cardiomyopathy | |
| HP:0100827 | Lymphocytosis | |
| HP:0001658 | Myocardial infarction | |
| HP:0002068 | Neuromuscular dysphagia | |
| HP:0001701 | Pericarditis | |
| HP:0002206 | Pulmonary fibrosis | |
| HP:0030880 | Raynaud phenomenon | |
| HP:0001315 | Reduced tendon reflexes | |
| HP:0000988 | Skin rash | |
| HP:0001894 | Thrombocytosis | |
| HP:0002633 | Vasculitis | |
| HP:0004936 | Venous thrombosis | |
Run the differential on the 9 obligate / very frequent terms
Annotation source: 33085276[PMID]_37329941[PMID]_36556960[PMID]_29632291[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/732
- This record as JSON: /data/d/732.json