MCID: ENT010
MIFTS: 33

Enthesitis-Related Juvenile Idiopathic Arthritis

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

Aliases & Classifications for Enthesitis-Related Juvenile Idiopathic Arthritis

MalaCards integrated aliases for Enthesitis-Related Juvenile Idiopathic Arthritis:

Name: Enthesitis-Related Juvenile Idiopathic Arthritis 54 60 17
Enthesitis-Related Arthritis 54 74
Enthesitis-Related Jia 54 60
Era 54 60
Enthesitis Related Arthritis, Juvenile 54
Juvenile Enthesitis-Related Arthritis 54
Juvenile Spondylarthropathy 54

Characteristics:

Orphanet epidemiological data:

60
enthesitis-related juvenile idiopathic arthritis
Age of onset: Childhood; Age of death: normal life expectancy;

Classifications:



External Ids:

ICD10 via Orphanet 35 M08.8
UMLS via Orphanet 75 C3495919
Orphanet 60 ORPHA85438
UMLS 74 C3495919

Summaries for Enthesitis-Related Juvenile Idiopathic Arthritis

NIH Rare Diseases : 54 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.

MalaCards based summary : Enthesitis-Related Juvenile Idiopathic Arthritis, also known as enthesitis-related arthritis, is related to arthritis and juvenile rheumatoid arthritis. The drugs Adalimumab and Etanercept have been mentioned in the context of this disorder. Affiliated tissues include bone, eye and t cells, and related phenotypes are joint swelling and elevated c-reactive protein level

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:

(show all 20)
# Related Disease Score Top Affiliating Genes
1 arthritis 10.3
2 juvenile rheumatoid arthritis 10.3
3 psoriatic arthritis 10.3
4 spondylarthropathy 10.3
5 crohn's disease 10.2
6 psoriasis 10.2
7 atlantoaxial subluxation 10.2
8 rheumatoid arthritis 10.1
9 spondyloarthropathy 10.1
10 spondyloarthropathy 1 10.1
11 ige responsiveness, atopic 10.1
12 agammaglobulinemia, x-linked 10.1
13 body mass index quantitative trait locus 1 10.1
14 crohn's colitis 10.1
15 agammaglobulinemia 10.1
16 pustulosis of palm and sole 10.1
17 reactive arthritis 10.1
18 spondylitis 10.1
19 psoriatic juvenile idiopathic arthritis 10.1
20 oligoarticular juvenile idiopathic arthritis 10.1

Graphical network of the top 20 diseases related to Enthesitis-Related Juvenile Idiopathic Arthritis:



Diseases related to Enthesitis-Related Juvenile Idiopathic Arthritis

Symptoms & Phenotypes for Enthesitis-Related Juvenile Idiopathic Arthritis

Human phenotypes related to Enthesitis-Related Juvenile Idiopathic Arthritis:

60 33 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 joint swelling 60 33 hallmark (90%) Very frequent (99-80%) HP:0001386
2 elevated c-reactive protein level 60 33 hallmark (90%) Very frequent (99-80%) HP:0011227
3 elevated erythrocyte sedimentation rate 60 33 hallmark (90%) Very frequent (99-80%) HP:0003565
4 juvenile rheumatoid arthritis 60 33 hallmark (90%) Very frequent (99-80%) HP:0005681
5 anterior uveitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0012122
6 sacroiliac arthritis 60 33 hallmark (90%) Very frequent (99-80%) HP:0012317
7 dental malocclusion 60 33 frequent (33%) Frequent (79-30%) HP:0000689
8 cartilage destruction 60 33 frequent (33%) Frequent (79-30%) HP:0100773
9 enthesitis 60 33 frequent (33%) Frequent (79-30%) HP:0100686
10 increased inflammatory response 60 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 19)
# 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 Pharmaceutical Solutions Phase 3
6 Antirheumatic Agents Phase 3
7 Anti-Inflammatory Agents Phase 3
8 Anti-Inflammatory Agents, Non-Steroidal Phase 3
9 Analgesics Phase 3
10 Immunologic Factors Phase 3
11 Gastrointestinal Agents Phase 3
12 Peripheral Nervous System Agents Phase 3
13 Immunosuppressive Agents Phase 3
14 Analgesics, Non-Narcotic Phase 3
15 Antibodies Phase 3
16 Antibodies, Monoclonal Phase 3
17 Immunoglobulins Phase 3
18 Protein Kinase Inhibitors Phase 3
19 Rheumatoid Factor

Interventional clinical trials:

(show all 14)
# 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 A Study of Baricitinib in Participants From 2 Years to Less Than 18 Years Old With Juvenile Idiopathic Arthritis Recruiting NCT03773978 Phase 3 Baricitinib;Placebo
5 A Study of Baricitinib in Participants From 1 Year to Less Than 18 Years Old With Juvenile Idiopathic Arthritis Recruiting NCT03773965 Phase 3 Baricitinib
6 Long-Term Safety Study Of Tofacinib In Patients With Juvenile Idiopathic Arthritis Recruiting NCT01500551 Phase 3 Tofacitinib;Tofacitinib;Tofacitinib
7 Extension Study Evaluating Etanercept in 3 Subtypes of Childhood Arthritis Active, not recruiting NCT01421069 Phase 3 etanercept
8 Pediatric Arthritis Study of Certolizumab Pegol Active, not recruiting NCT01550003 Phase 3 Certolizumab Pegol (CZP)
9 An Extension Study of Subcutaneous Secukinumab in Patients With Juvenile Psoriatic Arthritis (JPsA) and Enthesitis Related Arthritis (ERA) Not yet recruiting NCT03769168 Phase 3 AIN457
10 Surveillance of Humira in Korean JIA Patients Completed NCT02141984
11 Juvenile Arthritis Quantitative Imaging Recruiting NCT02684695
12 Natural History and Development of Spondyloarthritis Recruiting NCT01422694
13 Observational Study of Pediatric Rheumatic Diseases: The CARRA Registry Recruiting NCT02418442
14 Yoga Versus Home Exercise Program in Children With Enthesitis Related Arthritis Not yet recruiting NCT03858504 Not Applicable

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:

42
Bone, Eye, T Cells, Monocytes

Publications for Enthesitis-Related Juvenile Idiopathic Arthritis

Articles related to Enthesitis-Related Juvenile Idiopathic Arthritis:

(show top 50) (show all 82)
# Title Authors Year
1
Evaluation of factors affecting the duration of disease-modifying anti-rheumatic drugs application in patients with enthesitis-related arthritis. ( 31070580 )
2019
2
Enthesitis-Related Juvenile Idiopathic Arthritis. ( 31043447 )
2019
3
Clinical correlates of HLA-B*27 and its subtypes in enthesitis-related arthritis variant of juvenile idiopathic arthritis in south Indian Tamil patients. ( 30884197 )
2019
4
Enthesitis-related arthritis: current perspectives. ( 30774484 )
2019
5
Evidence for M2 macrophage activation in patients with enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 30734215 )
2019
6
Spondyloarthritis Research Consortium of Canada Scoring System for Sacroiliitis in Juvenile Spondyloarthritis/Enthesitis-related Arthritis: A Reliability, Validity, and Responsiveness Study. ( 30709956 )
2019
7
Prospective validation of the Juvenile Spondyloarthritis Disease Activity Index in children with enthesitis-related arthritis. ( 30107576 )
2018
8
Safety of Adalimumab in Pediatric Patients with Polyarticular Juvenile Idiopathic Arthritis, Enthesitis-Related Arthritis, Psoriasis, and Crohn's Disease. ( 30054164 )
2018
9
Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre. ( 29609643 )
2018
10
Comparative Effectiveness of Tumor Necrosis Factor Agents and Disease-modifying Antirheumatic Therapy in Children with Enthesitis-related Arthritis: The First Year after Diagnosis. ( 28916542 )
2018
11
Prominent midfoot involvement in children with enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 28634696 )
2017
12
Intermediate monocytes are increased in enthesitis-related arthritis, a category of juvenile idiopathic arthritis. ( 27706807 )
2017
13
Gut microbiome in children with enthesitis-related arthritis in a developing country and the effect of probiotic administration. ( 27861762 )
2017
14
Diffusion-weighted imaging is a sensitive biomarker of response to biologic therapy in enthesitis-related arthritis. ( 27994095 )
2017
15
ERAP1 rs30187 single nucleotide polymorphism does not confer disease susceptibility in North Indian children with enthesitis-related arthritis. ( 28161768 )
2017
16
Multicenter inception cohort of enthesitis-related arthritis: variation in disease characteristics and treatment approaches. ( 28464909 )
2017
17
Clinical predictors of magnetic resonance imaging-detected sacroiliitis in children with enthesitis related arthritis. ( 28757344 )
2017
18
Two-year Efficacy and Safety of Etanercept in Pediatric Patients with Extended Oligoarthritis, Enthesitis-related Arthritis, or Psoriatic Arthritis. ( 26932344 )
2016
19
IL-27 levels are low in enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 27050079 )
2016
20
Effect of probiotics on clinical and immune parameters in enthesitis-related arthritis category of juvenile idiopathic arthritis. ( 27238895 )
2016
21
A diffusion-based quantification technique for assessment of sacroiliitis in adolescents with enthesitis-related arthritis. ( 26642308 )
2016
22
Sonologic enthesitis in children with enthesitis-related arthritis. ( 26812448 )
2016
23
Inactive Disease in Enthesitis-related Arthritis: Association of Increased Body Mass Index. ( 26980582 )
2016
24
Clinical Usefulness of 18F-fluorodeoxyglucose Positron Emission Tomography for Enthesitis-related Arthritis Diagnosis. ( 27371647 )
2016
25
Discordant inflammatory changes in the apophyseal and sacroiliac joints: serial observations in enthesitis-related arthritis. ( 27376529 )
2016
26
Ustekinumab as a Therapeutic Option for Children With Refractory Enthesitis-Related Arthritis. ( 27464779 )
2016
27
Update on enthesitis-related arthritis. ( 27466726 )
2016
28
HLA-B27 subtypes in enthesitis-related arthritis category of juvenile idiopathic arthritis and ankylosing spondylitis in northern India. ( 26314893 )
2015
29
Atopy in children with the enthesitis-related arthritis (ERA) subtype of juvenile idiopathic arthritis is associated with a worse outcome. ( 25935591 )
2015
30
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
31
Efficacy and safety of etanercept in patients with the enthesitis-related arthritis category of juvenile idiopathic arthritis: results from a phase III randomized, double-blind study. ( 25891010 )
2015
32
Fever as an initial manifestation of enthesitis-related arthritis subtype of juvenile idiopathic arthritis: retrospective study. ( 26030261 )
2015
33
Natural killer cell and gamma delta T cell alterations in enthesitis related arthritis category of juvenile idiopathic arthritis. ( 26244610 )
2015
34
Safety and efficacy of etanercept in children with the JIA categories extended oligoarthritis, enthesitis-related arthritis and psoriasis arthritis. ( 25034081 )
2015
35
A Randomized, Double-Blind, Placebo-Controlled Multicenter Study of Adalimumab in Pediatric Patients With Enthesitis-Related Arthritis. ( 26223543 )
2015
36
Enthesitis-related arthritis. ( 26233720 )
2015
37
Diagnositic value of pelvic enthesitis on MRI of the sacroiliac joints in enthesitis related arthritis. ( 26554668 )
2015
38
Evaluation and Treatment of Childhood Enthesitis-Related Arthritis. ( 26824032 )
2015
39
Early atlantoaxial subluxation in enthesitis-related arthritis. ( 24882848 )
2014
40
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 )
2014
41
Association of microRNA-146a and its target gene IRAK1 polymorphism with enthesitis related arthritis category of juvenile idiopathic arthritis. ( 24719227 )
2014
42
Inflammatory changes of the lumbar spine in children and adolescents with enthesitis-related arthritis: magnetic resonance imaging findings. ( 24127385 )
2014
43
TLR4 endogenous ligand MRP8/14 level in enthesitis-related arthritis and its association with disease activity and TLR4 expression. ( 24231443 )
2014
44
Detection of enthesitis in children with enthesitis-related arthritis: dolorimetry compared to ultrasonography. ( 24449586 )
2014
45
Are MEFV mutations susceptibility factors in enthesitis-related arthritis patients in the eastern Mediterranean? ( 24564907 )
2014
46
Enthesitis-related arthritis: time to re-define? ( 25366933 )
2014
47
Altered microbiota associated with abnormal humoral immune responses to commensal organisms in enthesitis-related arthritis. ( 25434931 )
2014
48
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
49
Evaluation and Treatment of Enthesitis-Related Arthritis. ( 24403667 )
2013
50
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

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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75 UMLS via Orphanet
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