MCID: ENT010
MIFTS: 30

Enthesitis-Related Juvenile Idiopathic Arthritis

Categories: Rare diseases, Eye diseases, Respiratory diseases, Bone diseases

Aliases & Classifications for Enthesitis-Related Juvenile Idiopathic Arthritis

MalaCards integrated aliases for Enthesitis-Related Juvenile Idiopathic Arthritis:

Name: Enthesitis-Related Juvenile Idiopathic Arthritis 49 55
Enthesitis-Related Arthritis 49 69
Enthesitis-Related Jia 49 55
Era 49 55
Enthesitis Related Arthritis, Juvenile 49
Juvenile Enthesitis-Related Arthritis 49
Juvenile Spondylarthropathy 49

Characteristics:

Orphanet epidemiological data:

55
enthesitis-related juvenile idiopathic arthritis
Age of onset: Childhood; Age of death: normal life expectancy;

Classifications:



External Ids:

Orphanet 55 ORPHA85438
UMLS via Orphanet 70 C3495919
ICD10 via Orphanet 33 M08.8
UMLS 69 C3495919

Summaries for Enthesitis-Related Juvenile Idiopathic Arthritis

NIH Rare Diseases : 49 Enthesitis-related juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis that is characterized by both arthritis and inflammation of an enthesitis site (the point at which a ligament, tendon, or joint capsule attaches to the bone). Signs and symptoms generally develop in late childhood or early adolescence and include pain, tenderness, and swelling in joints and at the enthesis. The knee and the back of the ankle (at the Achilles tendon) are the most commonly affected parts of the body. The underlying cause of enthesitis-related juvenile idiopathic arthritis is currently unknown (idiopathic). It is very rare for more than one member of a family to have juvenile arthritis; however, research suggests that having a family member with juvenile arthritis or any autoimmune disease may increase the risk of having juvenile arthritis, in general. Treatment usually involves different types of medications to help manage symptoms and/or physical therapy. Last updated: 11/23/2015

MalaCards based summary : Enthesitis-Related Juvenile Idiopathic Arthritis, also known as enthesitis-related arthritis, is related to spondylarthropathy and rheumatoid arthritis, and has symptoms including dental malocclusion, joint swelling and elevated erythrocyte sedimentation rate. The drugs Adalimumab and Etanercept have been mentioned in the context of this disorder. Affiliated tissues include bone and eye.

Related Diseases for Enthesitis-Related Juvenile Idiopathic Arthritis

Diseases related to Enthesitis-Related Juvenile Idiopathic Arthritis via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 spondylarthropathy 10.0
2 rheumatoid arthritis 9.9
3 arthritis 9.9

Symptoms & Phenotypes for Enthesitis-Related Juvenile Idiopathic Arthritis

Human phenotypes related to Enthesitis-Related Juvenile Idiopathic Arthritis:

55 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dental malocclusion 55 31 frequent (33%) Frequent (79-30%) HP:0000689
2 joint swelling 55 31 hallmark (90%) Very frequent (99-80%) HP:0001386
3 elevated erythrocyte sedimentation rate 55 31 hallmark (90%) Very frequent (99-80%) HP:0003565
4 juvenile rheumatoid arthritis 55 31 hallmark (90%) Very frequent (99-80%) HP:0005681
5 elevated c-reactive protein level 55 31 hallmark (90%) Very frequent (99-80%) HP:0011227
6 anterior uveitis 55 31 hallmark (90%) Very frequent (99-80%) HP:0012122
7 sacroiliac arthritis 55 31 hallmark (90%) Very frequent (99-80%) HP:0012317
8 enthesitis 55 31 frequent (33%) Frequent (79-30%) HP:0100686
9 cartilage destruction 55 31 frequent (33%) Frequent (79-30%) HP:0100773
10 increased inflammatory response 55 Very frequent (99-80%)

Drugs & Therapeutics for Enthesitis-Related Juvenile Idiopathic Arthritis

Drugs for Enthesitis-Related Juvenile Idiopathic Arthritis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Adalimumab Approved Phase 3 331731-18-1 16219006
2
Etanercept Approved, Investigational Phase 3 185243-69-0
3
Tofacitinib Approved, Investigational Phase 3 477600-75-2
4
Certolizumab pegol Approved Phase 3 428863-50-7
5 Analgesics Phase 3
6 Gastrointestinal Agents Phase 3
7 Analgesics, Non-Narcotic Phase 3
8 Peripheral Nervous System Agents Phase 3
9 Pharmaceutical Solutions Phase 3
10 Immunosuppressive Agents Phase 3
11 Anti-Inflammatory Agents Phase 3
12 Anti-Inflammatory Agents, Non-Steroidal Phase 3
13 Antirheumatic Agents Phase 3
14 Antibodies, Monoclonal Phase 3
15 Immunoglobulins Phase 3
16 Protein Kinase Inhibitors Phase 3
17 Antibodies Phase 3
18 Rheumatoid Factor

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Study of the Efficacy and Safety of Adalimumab in Pediatric Subjects With Enthesitis Related Arthritis Completed NCT01166282 Phase 3
2 Study Evaluating Etanercept in 3 Subtypes of Childhood Arthritis Completed NCT00962741 Phase 3 Etanercept
3 Secukinumab Safety and Efficacy in JPsA and ERA Recruiting NCT03031782 Phase 3 AIN457
4 Long-Term Safety Study Of Tofacinib In Patients With Juvenile Idiopathic Arthritis Recruiting NCT01500551 Phase 3 Tofacitinib;Tofacitinib;Tofacitinib
5 Extension Study Evaluating Etanercept in 3 Subtypes of Childhood Arthritis Active, not recruiting NCT01421069 Phase 3 etanercept
6 Pediatric Arthritis Study of Certolizumab Pegol Active, not recruiting NCT01550003 Phase 3 Certolizumab Pegol (CZP)
7 Surveillance of Humira in Korean JIA Patients Completed NCT02141984
8 Juvenile Arthritis Quantitative Imaging Recruiting NCT02684695
9 Natural History and Development of Spondyloarthritis Recruiting NCT01422694
10 Observational Study of Pediatric Rheumatic Diseases: The CARRA Registry Recruiting NCT02418442

Search NIH Clinical Center for Enthesitis-Related Juvenile Idiopathic Arthritis

Genetic Tests for Enthesitis-Related Juvenile Idiopathic Arthritis

Anatomical Context for Enthesitis-Related Juvenile Idiopathic Arthritis

MalaCards organs/tissues related to Enthesitis-Related Juvenile Idiopathic Arthritis:

38
Bone, Eye

Publications for Enthesitis-Related Juvenile Idiopathic Arthritis

Articles related to Enthesitis-Related Juvenile Idiopathic Arthritis:

(show top 50) (show all 66)
# Title Authors Year
1
Intermediate monocytes are increased in enthesitis-related arthritis, a category of juvenile idiopathic arthritis. ( 27706807 )
2017
2
Inactive Disease in Enthesitis-related Arthritis: Association of Increased Body Mass Index. ( 26980582 )
2016
3
Two-year Efficacy and Safety of Etanercept in Pediatric Patients with Extended Oligoarthritis, Enthesitis-related Arthritis, or Psoriatic Arthritis. ( 26932344 )
2016
4
IL-27 levels are low in enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 27050079 )
2016
5
Effect of probiotics on clinical and immune parameters in enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 27238895 )
2016
6
Update on enthesitis-related arthritis. ( 27466726 )
2016
7
Sonologic enthesitis in children with enthesitis-related arthritis. ( 26812448 )
2016
8
Ustekinumab as a Therapeutic Option for Children With Refractory Enthesitis-Related Arthritis. ( 27464779 )
2016
9
A diffusion-based quantification technique for assessment of sacroiliitis in adolescents with enthesitis-related arthritis. ( 26642308 )
2016
10
Discordant inflammatory changes in the apophyseal and sacroiliac joints: serial observations in enthesitis-related arthritis. ( 27376529 )
2016
11
Clinical Usefulness of 18F-fluorodeoxyglucose Positron Emission Tomography for Enthesitis-related Arthritis Diagnosis. ( 27371647 )
2016
12
A Randomized, Double-Blind, Placebo-Controlled Multicenter Study of Adalimumab in Pediatric Patients With Enthesitis-Related Arthritis. ( 26223543 )
2015
13
Tenascin-C Levels, A Toll-like Receptor 4 Ligand, in Enthesitis-related Arthritis Category of Juvenile Idiopathic Arthritis: A Cross-sectional and Longitudinal Study. ( 25774061 )
2015
14
Enthesitis-related arthritis. ( 26233720 )
2015
15
Atopy in children with the enthesitis-related arthritis (ERA) subtype of juvenile idiopathic arthritis is associated with a worse outcome. ( 25935591 )
2015
16
Evaluation and Treatment of Childhood Enthesitis-Related Arthritis. ( 26824032 )
2015
17
HLA-B27 subtypes in enthesitis-related arthritis category of juvenile idiopathic arthritis and ankylosing spondylitis in northern India. ( 26314893 )
2015
18
Diagnositic value of pelvic enthesitis on MRI of the sacroiliac joints in enthesitis related arthritis. ( 26554668 )
2015
19
Natural killer cell and gamma delta T cell alterations in enthesitis related arthritis category of juvenile idiopathic arthritis. ( 26244610 )
2015
20
Efficacy and safety of etanercept in enthesitis-related arthritis juvenile idiopathic arthritis: Results from a phase 3 randomized double-blind study. ( 25891010 )
2015
21
Fever as an initial manifestation of enthesitis-related arthritis subtype of juvenile idiopathic arthritis: retrospective study. ( 26030261 )
2015
22
Detection of enthesitis in children with enthesitis-related arthritis: dolorimetry compared to ultrasonography. ( 24449586 )
2014
23
Association of microRNA-146a and its target gene IRAK1 polymorphism with enthesitis related arthritis category of juvenile idiopathic arthritis. ( 24719227 )
2014
24
Safety and efficacy of etanercept in children with the JIA categories extended oligoarthritis, enthesitis-related arthritis and psoriasis arthritis. ( 25034081 )
2014
25
Altered microbiota associated with abnormal humoral immune responses to commensal organisms in enthesitis-related arthritis. ( 25434931 )
2014
26
Early atlantoaxial subluxation in enthesitis-related arthritis. ( 24882848 )
2014
27
Enthesitis-Related Arthritis: Time to Re-define? ( 25366933 )
2014
28
Inflammatory changes of the lumbar spine in children and adolescents with enthesitis-related arthritis: magnetic resonance imaging findings. ( 24127385 )
2014
29
TLR4 endogenous ligand MRP8/14 level in enthesitis-related arthritis and its association with disease activity and TLR4 expression. ( 24231443 )
2014
30
Are MEFV mutations susceptibility factors in enthesitis-related arthritis patients in the eastern Mediterranean? ( 24564907 )
2014
31
Detection of enthesitis in children with Enthesitis-related arthritis: Dolorimeter examination compared to ultrasonography. ( 24114526 )
2013
32
Efficacy and safety of open-label etanercept on extended oligoarticular juvenile idiopathic arthritis, enthesitis-related arthritis and psoriatic arthritis: part 1 (week 12) of the CLIPPER study. ( 23696632 )
2013
33
Evaluation and Treatment of Enthesitis-Related Arthritis. ( 24403667 )
2013
34
Lack of association of single nucleotide polymorphisms in toll-like receptors 2 and 4 with enthesitis-related arthritis category of juvenile idiopathic arthritis in Indian population. ( 22453525 )
2013
35
Membrane-bound toll-like receptors are overexpressed in peripheral blood and synovial fluid mononuclear cells of enthesitis-related arthritis category of juvenile idiopathic arthritis (JIAa89ERA) patients and lead to secretion of inflammatory mediators. ( 22302567 )
2012
36
Utility of magnetic resonance imaging when following up sacroiliitis in enthesitis-related arthritis. ( 22310966 )
2012
37
Enthesitis-related arthritis is associated with higher pain intensity and poorer health status in comparison with other categories of juvenile idiopathic arthritis: the Childhood Arthritis and Rheumatology Research Alliance Registry. ( 23070991 )
2012
38
Synovial fluid mononuclear cell gene expression profiling suggests dysregulation of innate immune genes in enthesitis-related arthritis patients. ( 22763987 )
2012
39
A modified juvenile arthritis damage index to improve articular damage assessment in juvenile idiopathic arthritis--enthesitis-related arthritis (JIA-ERA). ( 22234493 )
2012
40
IL1 gene polymorphisms in enthesitis related arthritis category of juvenile idiopathic arthritis (ERA-JIA). ( 22124790 )
2012
41
Diagnosis and treatment of enthesitis-related arthritis. ( 23236258 )
2012
42
Subtype specific genetic associations for juvenile idiopathic arthritis: ERAP1 with the enthesitis related arthritis subtype and IL23R with juvenile psoriatic arthritis. ( 21281511 )
2011
43
Tumor necrosis factor-blocking agents for children with enthesitis-related arthritis--data from the dutch arthritis and biologicals in children register, 1999-2010. ( 21844151 )
2011
44
Evaluation of MR imaging guided steroid injection of the sacroiliac joints for the treatment of children with refractory enthesitis-related arthritis. ( 21046404 )
2011
45
Fecal calprotectin in children with the enthesitis-related arthritis subtype of juvenile idiopathic arthritis. ( 21965700 )
2011
46
Enthesitis in an inception cohort of enthesitis-related arthritis. ( 21618453 )
2011
47
Attainment of inactive disease status following initiation of TNF-I+ inhibitor therapy for juvenile idiopathic arthritis: enthesitis-related arthritis predicts persistent active disease. ( 22089470 )
2011
48
Evidence of cellular immune response to outer membrane protein of Salmonella typhimurium in patients with enthesitis-related arthritis subtype of juvenile idiopathic arthritis. ( 21041273 )
2011
49
Levels of serum matrix metalloproteinase-3 correlate with disease activity in the enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 21885500 )
2011
50
Nutritional supplementation and dietary restriction in the resolution of enthesitis-related arthritis. ( 21541080 )
2011

Variations for Enthesitis-Related Juvenile Idiopathic Arthritis

Expression for Enthesitis-Related Juvenile Idiopathic Arthritis

Search GEO for disease gene expression data for Enthesitis-Related Juvenile Idiopathic Arthritis.

Pathways for Enthesitis-Related Juvenile Idiopathic Arthritis

GO Terms for Enthesitis-Related Juvenile Idiopathic Arthritis

Sources for Enthesitis-Related Juvenile Idiopathic Arthritis

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70 UMLS via Orphanet
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