Enthesitis-related juvenile idiopathic arthritis
disease diseaseOn this page
Also known as enthesitis related arthritis, juvenileenthesitis-related arthritisenthesitis-related JIAERAjuvenile enthesitis-related arthritisjuvenile spondylarthropathy
Summary
Enthesitis-related juvenile idiopathic arthritis (MONDO:0019437) is a disease and 11 clinical trials. Top therapeutic interventions include adalimumab, secukinumab, and bimekizumab. A subtype of juvenile idiopathic arthritis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 100 000 (Europe)
- Phenotypes (HPO): 34
- Clinical trials: 11
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 5.7 | Europe | Not yet validated |
Signs & symptoms
Clinical features (HPO)
34 HPO clinical features (Orphanet curated; top 34 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0001369 | Arthritis | Very frequent (80-99%) |
| HP:0002829 | Arthralgia | Very frequent (80-99%) |
| HP:0100686 | Enthesitis | Very frequent (80-99%) |
| HP:0040313 | Oligoarthritis | Frequent (30-79%) |
| HP:0100536 | Abnormality of the fascia | Frequent (30-79%) |
| HP:0100712 | Abnormal lumbar spine morphology | Frequent (30-79%) |
| HP:0000925 | Abnormality of the vertebral column | Frequent (30-79%) |
| HP:0001384 | Abnormality of the hip joint | Frequent (30-79%) |
| HP:0003028 | Abnormality of the ankles | Frequent (30-79%) |
| HP:0003418 | Back pain | Frequent (30-79%) |
| HP:0004690 | Thickened Achilles tendon | Frequent (30-79%) |
| HP:0005086 | Knee osteoarthritis | Frequent (30-79%) |
| HP:0008364 | Abnormality of the calcaneus | Frequent (30-79%) |
| HP:0008843 | Hip osteoarthritis | Frequent (30-79%) |
| HP:0012317 | Sacroiliac arthritis | Frequent (30-79%) |
| HP:0001627 | Abnormal heart morphology | Occasional (5-29%) |
| HP:0001760 | Abnormal foot morphology | Occasional (5-29%) |
| HP:0001832 | Abnormal metatarsal morphology | Occasional (5-29%) |
| HP:0003019 | Abnormality of the wrist | Occasional (5-29%) |
| HP:0003043 | Abnormality of the shoulder | Occasional (5-29%) |
| HP:0003319 | Abnormality of the cervical spine | Occasional (5-29%) |
| HP:0003366 | Abnormality of the femoral neck or head region | Occasional (5-29%) |
| HP:0006217 | Limited mobility of proximal interphalangeal joint | Occasional (5-29%) |
| HP:0007813 | Nongranulomatous uveitis | Occasional (5-29%) |
| HP:0009811 | Abnormality of the elbow | Occasional (5-29%) |
| HP:0011842 | Abnormality of skeletal morphology | Occasional (5-29%) |
| HP:0011911 | Abnormality of metacarpophalangeal joint | Occasional (5-29%) |
| HP:0012122 | Anterior uveitis | Occasional (5-29%) |
| HP:0031090 | Finger dactylitis | Occasional (5-29%) |
| HP:0100711 | Abnormality of the thoracic spine | Occasional (5-29%) |
| HP:0000794 | IgA deposition in the glomerulus | Very rare (<1-4%) |
| HP:0001634 | Mitral valve prolapse | Very rare (<1-4%) |
| HP:0001659 | Aortic regurgitation | Very rare (<1-4%) |
| HP:0006698 | Dilatation of the ventricular cavity | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | enthesitis-related juvenile idiopathic arthritis |
| Mondo ID | MONDO:0019437 |
| EFO | EFO:0009732 |
| Orphanet | 85438 |
| ICD-11 | 1128255226 |
| NCIT | C119024 |
| SNOMED CT | 410801005 |
| UMLS | C3495919 |
| MedGen | 854059 |
| GARD | 0010969 |
| Is cancer (heuristic) | no |
Also known as: enthesitis related arthritis, juvenile · enthesitis-related arthritis · enthesitis-related JIA · ERA · era · juvenile enthesitis-related arthritis · juvenile spondylarthropathy
Disease family
This is a subtype of juvenile idiopathic arthritis. 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 › juvenile idiopathic arthritis › enthesitis-related juvenile idiopathic arthritis
Related subtypes (5): polyarticular juvenile idiopathic arthritis, oligoarticular juvenile idiopathic arthritis, systemic-onset juvenile idiopathic arthritis, psoriasis-related juvenile idiopathic arthritis, unspecified juvenile idiopathic arthritis
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: 11.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 6 |
| PHASE3 | 5 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT04527380 | PHASE3 | ACTIVE_NOT_RECRUITING | A Study of Ixekizumab (LY2439821) in Children With Juvenile Idiopathic Arthritis Categories of Enthesitis-related Arthritis (Including Juvenile Onset Ankylosing Spondylitis) and Juvenile Psoriatic Arthritis |
| NCT06668181 | PHASE3 | RECRUITING | An Open-label Study to Evaluate the Pharmacokinetics and Safety of Bimekizumab in Pediatric Study Participants With Active Juvenile Idiopathic Arthritis Subtypes Enthesitis-related Arthritis (Including Juvenile-onset Ankylosing Spondylitis) and Juvenile Psoriatic Arthritis |
| NCT01166282 | PHASE3 | COMPLETED | A Study of the Efficacy and Safety of Adalimumab in Pediatric Subjects With Enthesitis Related Arthritis |
| NCT03031782 | PHASE3 | COMPLETED | Secukinumab Safety and Efficacy in Juvenile Psoriatic Arthritis (JPsA) and Enthesitis-related Arthritis (ERA) |
| NCT03769168 | PHASE3 | COMPLETED | An Extension Study of Subcutaneous Secukinumab in Patients With Juvenile Psoriatic Arthritis (JPsA) and Enthesitis Related Arthritis (ERA) |
| NCT06766552 | Not specified | NOT_YET_RECRUITING | A Registry Study Assessing PRO, Dosing Patterns, and Safety of Vunakizumab in Patients With General Rheumatic Diseases. |
| NCT07190053 | Not specified | NOT_YET_RECRUITING | Long Term Treatment Strategies and Effectiveness of Secukinumab in Patient With JIA Subtypes of JPsA and ERA: Study From German BIKER Registry. |
| NCT02684695 | Not specified | UNKNOWN | Juvenile Arthritis Quantitative Imaging |
| NCT03858504 | Not specified | COMPLETED | Yoga Versus Home Exercise Program in Children With Enthesitis Related Arthritis |
| NCT03949114 | Not specified | UNKNOWN | Rehabilitation Intervention on Prevention of Postoperative Delirium |
| NCT06383195 | Not specified | UNKNOWN | The Reliability and Validity of the Ankylosing Spondylitis Performance Index (ASPI) in Enthesitis-Related Arthritis |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| ADALIMUMAB | 4 | 2 |
| SECUKINUMAB | 4 | 2 |
| BIMEKIZUMAB | 4 | 1 |
| IXEKIZUMAB | 4 | 1 |
Related Atlas pages
- Drugs: Adalimumab, Secukinumab, Bimekizumab, Ixekizumab