ORPHA:3099
Rheumatic fever
Synonyms: Acute rheumatic fever
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
Rheumatic fever (RF) is a multisystem inflammatory disease occurring as a post-infectious, nonsuppurative sequela of untreated streptococcus pyogenes (Group A streptococcus [GAS]) pharyngitis, and mainly occurs in individuals aged 5 to 15 years. The most common presenting signs are fever, migratory polyarthritis and carditis.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Rheumatic fever | Acute rheumatic fever |
| fr | Rhumatisme articulaire aigu | |
| de | Rheumatisches Fieber | Akutes rheumatisches Fieber |
| es | Fiebre reumática | Fiebre reumática aguda |
| it | Febbre reumatica | Febbre reumatica acuta |
| nl | Reumatische koorts | Acute reumatische koorts |
| pt | Reumatismo articular agudo | Febre reumática |
| pl | Gorączka reumatyczna | Ostra gorączka reumatyczna |
| cs | Revmatická horečka | Akutní revmatická horečka |
| uk | Ревматична лихоманка | Гостра ревматична лихоманка |
| zh | 风湿热 | 急性风湿热 |
| tr | Romatizmal ateş | Akut romatizmal ateş |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 268240 | BTNT | validated | |
| ICD-10 | I00 | BTNT | specific | validated |
| ICD-10 | I01.0 | BTNT | specific | validated |
| ICD-10 | I01.1 | BTNT | specific | validated |
| ICD-10 | I01.2 | BTNT | specific | validated |
| ICD-10 | I01.8 | BTNT | specific | validated |
| ICD-10 | I01.9 | BTNT | specific | validated |
| ICD-11 | 1B40 | BTNT | specific | validated |
| ICD-11 | 1B41 | BTNT | specific | validated |
| MONDO | 0017767 | E | validated | |
| MeSH | D012213 | E | validated | |
| UMLS | C0035436 | E | validated | |
| GARD | 5699 | E | validated | |
| MedDRA | 10039054 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:182222Rare systemic disease
rare systemic and rheumatological diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
9 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 5 / 100 000 | Europe | Value and class | Validated | 21386976[PMID]_[EXPERT] |
| Annual incidence | 1-5 / 10 000 | 12.5 / 100 000 | Canada | Value and class | Validated | 21386976[PMID]_[EXPERT]_ ORPHANET |
| Annual incidence | 1-5 / 10 000 | 12.5 / 100 000 | Spain | Value and class | Validated | 21386976[PMID]_[EXPERT]_ ORPHANET |
| Annual incidence | >1 / 1000 | 100 / 100 000 | Australia | Value and class | Validated | 21386976[PMID]_[EXPERT] |
| Annual incidence | 1-5 / 10 000 | 30 / 100 000 | India | Value and class | Validated | 21386976[PMID]_[EXPERT]_ ORPHANET |
| Annual incidence | >1 / 1000 | 100 / 100 000 | Mexico | Value and class | Validated | 21386976[PMID]_[EXPERT] |
| Annual incidence | 1-9 / 100 000 | 5 / 100 000 | Latin America | Value and class | Validated | 21386976[PMID]_[EXPERT] |
| Annual incidence | 1-9 / 100 000 | 7.6 / 100 000 | New Zealand | Value and class | Validated | 23254495[PMID]_21386976[PMID]_ [EXPERT] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | 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.
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HPO phenotypes
36 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002039 | Anorexia | |
| HP:0001369 | Arthritis | |
| HP:0012733 | Macule | |
| HP:0002017 | Nausea and vomiting | |
| HP:0001482 | Subcutaneous nodule | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001646 | Abnormal aortic valve morphology | |
| HP:0001633 | Abnormal mitral valve morphology | |
| HP:0011675 | Arrhythmia | |
| HP:0002829 | Arthralgia | |
| HP:0100749 | Chest pain | |
| HP:0002072 | Chorea | |
| HP:0100584 | Endocarditis | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0012819 | Myocarditis | |
| HP:0000980 | Pallor | |
| HP:0100776 | Recurrent pharyngitis | |
| HP:0000246 | Sinusitis | |
| Occasional (29-5%) | ||
| HP:0001654 | Abnormal heart valve morphology | |
| HP:0002167 | Abnormality of speech or vocalization | |
| HP:0002103 | Abnormality of the pleura | |
| HP:0010318 | Aplasia/Hypoplasia of the abdominal wall musculature | |
| HP:0000708 | Atypical behavior | |
| HP:0002019 | Constipation | |
| HP:0010526 | Dysgraphia | |
| HP:0010522 | Dyslexia | |
| HP:0000421 | Epistaxis | |
| HP:0010783 | Erythema | |
| HP:0002380 | Fasciculations | |
| HP:0001288 | Gait disturbance | |
| HP:0100248 | Hemiballismus | |
| HP:0002076 | Migraine | |
| HP:0000100 | Nephrotic syndrome | |
| HP:0001701 | Pericarditis | |
| HP:0002093 | Respiratory insufficiency | |
Run the differential on the 5 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3099
- This record as JSON: /data/d/3099.json