Rheumatic fever

Summary

Rheumatic fever (MONDO:0017767) is a disease and 32 clinical trials. Top therapeutic interventions include etanercept. A subtype of rheumatic disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 36
• Clinical trials: 32

Clinical features

Epidemiology

Prevalence records

8 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 100 000	5	Europe	Validated
Annual incidence	1-5 / 10 000	12.5	Canada	Validated
Annual incidence	1-5 / 10 000	12.5	Spain	Validated
Annual incidence	>1 / 1000	100	Australia	Validated
Annual incidence	1-5 / 10 000	30	India	Validated
Annual incidence	>1 / 1000	100	Mexico	Validated
Annual incidence	1-9 / 100 000	5	Latin America	Validated
Annual incidence	1-9 / 100 000	7.6	New Zealand	Validated

Signs & symptoms

Clinical features (HPO)

36 HPO clinical features (Orphanet curated; top 36 by frequency):

HPO ID	Term	Frequency
HP:0001369	Arthritis	Very frequent (80-99%)
HP:0001482	Subcutaneous nodule	Very frequent (80-99%)
HP:0002017	Nausea and vomiting	Very frequent (80-99%)
HP:0002039	Anorexia	Very frequent (80-99%)
HP:0012733	Macule	Very frequent (80-99%)
HP:0000246	Sinusitis	Frequent (30-79%)
HP:0000980	Pallor	Frequent (30-79%)
HP:0001633	Abnormal mitral valve morphology	Frequent (30-79%)
HP:0001646	Abnormal aortic valve morphology	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0002027	Abdominal pain	Frequent (30-79%)
HP:0002072	Chorea	Frequent (30-79%)
HP:0002829	Arthralgia	Frequent (30-79%)
HP:0011675	Arrhythmia	Frequent (30-79%)
HP:0012378	Fatigue	Frequent (30-79%)
HP:0012819	Myocarditis	Frequent (30-79%)
HP:0100584	Endocarditis	Frequent (30-79%)
HP:0100749	Chest pain	Frequent (30-79%)
HP:0100776	Recurrent pharyngitis	Frequent (30-79%)
HP:0000100	Nephrotic syndrome	Occasional (5-29%)
HP:0000421	Epistaxis	Occasional (5-29%)
HP:0000708	Atypical behavior	Occasional (5-29%)
HP:0001288	Gait disturbance	Occasional (5-29%)
HP:0001654	Abnormal heart valve morphology	Occasional (5-29%)
HP:0001701	Pericarditis	Occasional (5-29%)
HP:0002019	Constipation	Occasional (5-29%)
HP:0002076	Migraine	Occasional (5-29%)
HP:0002093	Respiratory insufficiency	Occasional (5-29%)
HP:0002103	Abnormality of the pleura	Occasional (5-29%)
HP:0002167	Abnormality of speech or vocalization	Occasional (5-29%)
HP:0002380	Fasciculations	Occasional (5-29%)
HP:0010318	Aplasia/Hypoplasia of the abdominal wall musculature	Occasional (5-29%)
HP:0010522	Dyslexia	Occasional (5-29%)
HP:0010526	Dysgraphia	Occasional (5-29%)
HP:0010783	Erythema	Occasional (5-29%)
HP:0100248	Hemiballismus	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	rheumatic fever
Mondo ID	MONDO:0017767
EFO	EFO:1001160
MeSH	D012213
Orphanet	3099
DOID	DOID:1586
ICD-10-CM	I00-I02
NCIT	C34984
SNOMED CT	58718002
UMLS	C0035436
MedGen	48448
GARD	0005699
MedDRA	10039054
NORD	1668
Is cancer (heuristic)	no

Also known as: acute rheumatic fever · ARF · inflammatory rheumatism

Disease family

This is a subtype of rheumatic disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › connective tissue disorder › rheumatic disorder › rheumatic fever

Related subtypes (29): palindromic rheumatism, rheumatic pulmonary valve disease, lupus erythematosus, mixed connective tissue disease, Reye syndrome, Wissler syndrome, acroosteolysis dominant type, chondrocalcinosis 2, Gorham-Stout disease, rheumatoid arthritis, camptodactyly-arthropathy-coxa vara-pericarditis syndrome, juvenile idiopathic arthritis, sweet syndrome, dermatomyositis, IL10-related early-onset inflammatory bowel disease, unexplained long-lasting fever/inflammatory syndrome, myalgia-eosinophilia syndrome associated with tryptophan, reactive arthritis, intermittent hydrarthrosis, fibroblastic rheumatism, interstitial granulomatous dermatitis with arthritis, scleroderma, idiopathic juvenile osteoporosis, polymyalgia rheumatica, autoinflammatory syndrome, progeria-associated arthropathy, LAMA5-related multisystemic syndrome, rheumatic disease of mitral valve, isolated sternocostoclavicular hyperostosis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 32.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	29
PHASE3	2
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06259006	PHASE3	RECRUITING	TREAT-SC: Early, Short Course Oral Dexamethasone for the Treatment of Sydenham Chorea in Children
NCT02164214	PHASE3	COMPLETED	Does Etanercept Influence Tweak Modulation of Inflammation During Inflammatory Rheumatisms (Psoriatic Arthritis and Rheumatoid Arthritis)?
NCT03998592	PHASE1	WITHDRAWN	Clinical Trial to Assess Safety and Immunogenicity of a Synthetic Vaccine Against Streptococcus Pyogenes
NCT02719314	Not specified	RECRUITING	Glucocorticoid-induced Osteoporosis in Patients With Chronic Inflammatory Rheumatic Diseases or Psoriasis
NCT04602091	Not specified	ACTIVE_NOT_RECRUITING	Observational Study in Patients Treated With JAK Inhibitors for Inflammatory Rheumatism (MAJIK)
NCT04664465	Not specified	RECRUITING	PRediction of DIverse Glucocorticoids ToxIcity OUtcomeS
NCT04733079	Not specified	ACTIVE_NOT_RECRUITING	Treat-to-target by Email During Urate-lowering Therapy in Gout
NCT06395883	Not specified	RECRUITING	Dynamics in Bone Turnover Markers During and After Short-term Glucocorticoid Treatment in Patients With an Inflammatory Joint Disease
NCT06400628	Not specified	RECRUITING	Effectiveness of Chronic Self-management Education on Behavioral and Clinical Outcomes
NCT06558357	Not specified	NOT_YET_RECRUITING	Diagnostic Performance of 18-FDG PET-CT Scores for the Diagnosis of Polymyalgia Rheumatica
NCT06600958	Not specified	RECRUITING	IDENTIFICATION OF BIOMARKERS ASSOCIATED WITH THE SEVERITY AND RADIO-CLINICAL PROGRESSION OF KNEE OSTEOARTHRITIS
NCT07132164	Not specified	NOT_YET_RECRUITING	Justification of the Initial Diagnosis and Evaluation of the Overall Evolution of a Cohort of Recent Polymyalgia Rheumatica (JADORE)
NCT01511341	Not specified	COMPLETED	Impact of a Telenursing Service on Satisfaction and Health Outcomes of Children With Inflammatory Rheumatic Diseases
NCT01886846	Not specified	COMPLETED	Plasma Adiponectin Levels and Relations With Cytokines in Children With Acute Rheumatic Fever
NCT02124109	Not specified	COMPLETED	The Genetic Basis of Acquired Heart Disease in Africa
NCT02353663	Not specified	COMPLETED	Rheumatic Heart Disease in Peru: Prevalence and Cardiovascular Outcomes Among Schoolchildren
NCT02407106	Not specified	UNKNOWN	Efficacy of BLIS K12 as Preventive Measure for Rheumatic Children
NCT03216616	Not specified	COMPLETED	Guided Self-Management Intervention Targeting Fatigue in Rheumatic Inflammatory Diseases
NCT03281863	Not specified	COMPLETED	Clinical Audit on the Managment in Assiut University Children Hospital
NCT03306784	Not specified	COMPLETED	Pain Catastrophizing and Routine Assessment of Patient Index Data 3(RAPID3) in Ten Categories of Rheumatology Outpatients
NCT04174092	Not specified	COMPLETED	Catastrophism in Chronic Inflammatory Rheumatism
NCT04316897	Not specified	UNKNOWN	Prevalence of Rheumatic Heart Disease According to Revised Jones Criteria (2015) in Assiut Governate
NCT04353609	Not specified	COMPLETED	Covid-19 in Patients With Chronic Inflammatory Rheumatism, Auto-immune or Auto-inflammatory Rare and Non-rare Diseases
NCT04510467	Not specified	TERMINATED	Mental Health Consequences of COVID19 Infection in the French RMD Cohort
NCT04530682	Not specified	COMPLETED	COVID-19 in Rheumatic Inflammatory Diseases Under Immuno-suppressive Drugs
NCT04649528	Not specified	COMPLETED	HIIT for Inflammatory Rheumatic Disease: Man vs Machine
NCT04749875	Not specified	UNKNOWN	Study of Leukocyte Populations in Patients With Chronic Inflammatory
NCT04798053	Not specified	COMPLETED	Impact of COVID-19 Pandemic and Social Distancing on Mental Health of Chronic Inflammatory Rheumatism Affected Patients
NCT04806867	Not specified	COMPLETED	Selection of Patients With Chronic Inflammatory Rheumatism Requiring Management During the COVID-19 Pandemia
NCT04998955	Not specified	COMPLETED	MST in Patients With Inflammatory Rheumatic Disease
NCT05026892	Not specified	UNKNOWN	Identification of Factors Associated With the Occurrence of Severe Forms of COVID-19 Infection in Patients With Inflammatory Rheumatism or Autoimmune Diseases
NCT05283096	Not specified	UNKNOWN	Survey About Diet in Chronic Inflammatory Rheumatic Diseases

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ETANERCEPT	4	1