ERA
MCID: ENT010
MIFTS: 29

Enthesitis-Related Juvenile Idiopathic Arthritis (ERA)

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 52 58 17
Enthesitis-Related Arthritis 52 71
Enthesitis-Related Jia 52 58
Era 52 58
Enthesitis Related Arthritis, Juvenile 52
Juvenile Enthesitis-Related Arthritis 52
Juvenile Spondylarthropathy 52

Characteristics:

Orphanet epidemiological data:

58
enthesitis-related juvenile idiopathic arthritis
Age of onset: Childhood; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare eye diseases
Rare respiratory diseases
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 M08.8
UMLS via Orphanet 72 C3495919
Orphanet 58 ORPHA85438
UMLS 71 C3495919

Summaries for Enthesitis-Related Juvenile Idiopathic Arthritis

NIH Rare Diseases : 52 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 juvenile rheumatoid arthritis and spondyloarthropathy 1. The drugs Adalimumab and Tofacitinib have been mentioned in the context of this disorder. Affiliated tissues include bone and eye, 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 top 50) (show all 58)
# Related Disease Score Top Affiliating Genes
1 juvenile rheumatoid arthritis 12.0
2 spondyloarthropathy 1 10.6
3 inflammatory spondylopathy 10.6
4 spondylitis 10.6
5 uveitis 10.6
6 spondyloarthropathy 10.5
7 spondylarthropathy 10.4
8 iridocyclitis 10.4
9 juvenile spondyloarthropathy 10.4
10 reactive arthritis 10.3
11 back pain 10.3
12 autoimmune disease 10.2
13 familial mediterranean fever 10.2
14 rheumatic disease 10.2
15 gastroenteritis 10.2
16 synovitis 10.2
17 arthropathy 10.2
18 pustulosis of palm and sole 10.2
19 crohn's disease 10.2
20 psoriasis 10.2
21 polyarticular juvenile idiopathic arthritis 10.2
22 oligoarticular juvenile idiopathic arthritis 10.2
23 psoriatic arthritis 10.2
24 arthritis 10.2
25 ige responsiveness, atopic 10.1
26 rheumatoid arthritis 10.1
27 agammaglobulinemia, x-linked 10.1
28 body mass index quantitative trait locus 1 10.1
29 leukemia, acute lymphoblastic 3 10.1
30 juvenile ankylosing spondylitis 10.1
31 inflammatory bowel disease 10.1
32 crohn's colitis 10.1
33 bacterial infectious disease 10.1
34 nasopharyngitis 10.1
35 neutropenia 10.1
36 bronchopneumonia 10.1
37 balanitis 10.1
38 urethritis 10.1
39 lymphadenitis 10.1
40 enthesopathy 10.1
41 ankylosis 10.1
42 pharyngitis 10.1
43 agammaglobulinemia 10.1
44 rhinitis 10.1
45 bronchitis 10.1
46 conjunctivitis 10.1
47 osteoarthritis 10.1
48 herpes zoster 10.1
49 measles 10.1
50 chickenpox 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:

58 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 joint swelling 58 31 hallmark (90%) Very frequent (99-80%) HP:0001386
2 elevated c-reactive protein level 58 31 hallmark (90%) Very frequent (99-80%) HP:0011227
3 elevated erythrocyte sedimentation rate 58 31 hallmark (90%) Very frequent (99-80%) HP:0003565
4 juvenile rheumatoid arthritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0005681
5 anterior uveitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012122
6 sacroiliac arthritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012317
7 dental malocclusion 58 31 frequent (33%) Frequent (79-30%) HP:0000689
8 cartilage destruction 58 31 frequent (33%) Frequent (79-30%) HP:0100773
9 enthesitis 58 31 frequent (33%) Frequent (79-30%) HP:0100686
10 increased inflammatory response 58 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
Tofacitinib Approved, Investigational Phase 3 477600-75-2
3
Certolizumab pegol Approved Phase 3 428863-50-7
4
Etanercept Approved, Investigational Phase 3 185243-69-0
5 Protein Kinase Inhibitors Phase 3
6 Pharmaceutical Solutions Phase 3
7 Immunosuppressive Agents Phase 3
8 Antirheumatic Agents Phase 3
9 Immunologic Factors Phase 3
10 Anti-Inflammatory Agents Phase 3
11 Antibodies, Monoclonal Phase 3
12 Antibodies Phase 3
13 Immunoglobulins Phase 3
14 Gastrointestinal Agents Phase 3
15 Analgesics, Non-Narcotic Phase 3
16 Analgesics Phase 3
17 Anti-Inflammatory Agents, Non-Steroidal Phase 3
18 Rheumatoid Factor

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 A Double-blind, Placebo-Controlled, Multicenter Study of the Efficacy and Safety of Adalimumab in Pediatric Subjects With Enthesitis Related Arthritis Completed NCT01166282 Phase 3
2 A 2-Part Open-Label Study to Assess the Clinical Benefit and Long-Term Safety of Etanercept in Children and Adolescents With Extended Oligoarticular Juvenile Idiopathic Arthritis, Enthesitis-Related Arthritis, or Psoriatic Arthritis Completed NCT00962741 Phase 3 Etanercept
3 EFFICACY, SAFETY AND TOLERABILITY OF TOFACITINIB FOR TREATMENT OF POLYARTICULAR COURSE JUVENILE IDIOPATHIC ARTHRITIS (JIA) IN CHILDREN AND ADOLESCENT SUBJECTS Completed NCT02592434 Phase 3 CP-690,550 (tofacitinib)
4 A Randomized, Double-Blind, Placebo-Controlled, Withdrawal, Safety and Efficacy Study of Oral Baricitinib in Patients From 2 Years to Less Than 18 Years Old With Juvenile Idiopathic Arthritis (JIA) Recruiting NCT03773978 Phase 3 Baricitinib;Placebo
5 A Phase 3 Multicenter Study to Evaluate the Long-Term Safety and Efficacy of Baricitinib in Patients From 1 Year to <18 Years of Age With Juvenile Idiopathic Arthritis (JIA) Recruiting NCT03773965 Phase 3 Baricitinib
6 An Extension Study of Subcutaneous Secukinumab to Evaluate the Long-term Efficacy, Safety and Tolerability up to 4 Years in Patients With Juvenile Idiopathic Arthritis Subtypes of Juvenile Psoriatic Arthritis and Enthesitis Related Arthritis Recruiting NCT03769168 Phase 3 AIN457
7 A Multicenter, Open-label Study to Assess the Pharmacokinetics, Safety and Efficacy of Certolizumab Pegol in Children and Adolescents With Moderately to Severely Active Polyarticular-course Juvenile Idiopathic Arthritis (JIA) Active, not recruiting NCT01550003 Phase 3 Certolizumab Pegol (CZP)
8 A Three-part Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Secukinumab Treatment in Juvenile Idiopathic Arthritis Subtypes of Psoriatic and Enthesitis-related Arthritis Active, not recruiting NCT03031782 Phase 3 AIN457
9 AN OPEN-LABEL EXTENSION STUDY TO ASSESS THE LONG-TERM SAFETY AND CLINICAL BENEFIT OF ETANERCEPT IN CHILDREN AND ADOLESCENTS WITH EXTENDED OLIGOARTICULAR JUVENILE IDIOPATHIC ARTHRITIS, ENTHESITIS-RELATED ARTHRITIS, OR PSORIATIC ARTHRITIS WHO WERE PREVIOUSLY ENROLLED IN PROTOCOL 0881A1-3338-WW(B1801014) Active, not recruiting NCT01421069 Phase 3 etanercept
10 Studies on the Natural History and Pathogenesis of Spondyloarthritis Recruiting NCT01422694
11 Observational Study of Pediatric Rheumatic Diseases: The CARRA Registry Recruiting NCT02418442
12 The Development and Validation of Quantitative Imaging Biomarkers as a Measure of Disease Activity in Juvenile Idiopathic Arthritis Recruiting NCT02684695
13 The Comparison of the Effects of Yoga and Home Exercise Program in Children With Enthesitis Related Arthritis Not yet recruiting NCT03858504

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:

40
Bone, Eye

Publications for Enthesitis-Related Juvenile Idiopathic Arthritis

Articles related to Enthesitis-Related Juvenile Idiopathic Arthritis:

(show all 14)
# Title Authors PMID Year
1
Characteristics of coexisting localized scleroderma and inflammatory arthritis. 61
31804172 2019
2
[Serum lipid profile in children with different subtypes of juvenile idiopathic arthritis]. 61
31208507 2019
3
Enthesitis-Related Juvenile Idiopathic Arthritis. 61
31043447 2019
4
Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre. 61
29609643 2018
5
Predictors of the response to etanercept in patients with juvenile idiopathic arthritis without systemic manifestations within 12 months: results of an open-label, prospective study conducted at the National Scientific and Practical Center of Children's Health, Russia. 61
28615036 2017
6
Outcome of Juvenile Idiopathic Arthritis Associated Uveitis in Two Disease Subtypes. 61
30375520 2017
7
[Serum levels of Th1/Th2 cytokines in children with non-systemic juvenile idiopathic arthritis]. 61
27651193 2016
8
Rationale, design and baseline data of a mixed methods study examining the clinical impact of a brief transition programme for young people with juvenile idiopathic arthritis: the DON'T RETARD project. 61
24302502 2013
9
Meta-analysis confirms association between TNFA-G238A variant and JIA, and between PTPN22-C1858T variant and oligoarticular, RF-polyarticular and RF-positive polyarticular JIA. 61
24160187 2013
10
Lipid profile in adult patients with idiopathic juvenile arthritis. 61
24217671 2013
11
Pediatric rheumatology in Southeast Asia: insights from the Singapore experience. 61
21181313 2011
12
Factors influencing the efficacy of intra-articular steroid injections in patients with juvenile idiopathic arthritis. 61
17562077 2008
13
Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort: ethnicity as a risk factor. 61
17530723 2007
14
[Clinical analysis of 25 cases of enthesitis related juvenile idiopathic arthritis]. 61
17306087 2007

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

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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