MCID: RHM037
MIFTS: 34

Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis

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

Aliases & Classifications for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

MalaCards integrated aliases for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:

Name: Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis 58
Polyarticular Juvenile Idiopathic Arthritis, Rheumatoid Factor Negative 17
Juvenile Rheumatoid Factor-Negative Polyarthritis 58
Juvenile Polyarthritis Without Rheumatoid Factor 58
Rheumatoid Factor-Negative Polyarticular Jia 58

Characteristics:

Orphanet epidemiological data:

58
rheumatoid factor-negative polyarticular juvenile idiopathic arthritis
Inheritance: Multigenic/multifactorial; 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 M08.3
Orphanet 58 ORPHA85408

Summaries for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

MalaCards based summary : Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis, also known as polyarticular juvenile idiopathic arthritis, rheumatoid factor negative, is related to juvenile rheumatoid arthritis and rheumatoid factor-negative juvenile idiopathic arthritis. An important gene associated with Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis is ANKRD55 (Ankyrin Repeat Domain 55), and among its related pathways/superpathways are PAK Pathway and Th17 cell differentiation. The drugs Adalimumab and Abatacept have been mentioned in the context of this disorder. Affiliated tissues include eye, bone and thymus, and related phenotypes are dental malocclusion and arthralgia

Related Diseases for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Diseases in the Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis family:

Rheumatoid Factor-Positive Polyarticular Juvenile Idiopathic Arthritis

Diseases related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 33)
# Related Disease Score Top Affiliating Genes
1 juvenile rheumatoid arthritis 32.3 STAT4 LACC1 IL2RB IL2RA
2 rheumatoid factor-negative juvenile idiopathic arthritis 11.8
3 rheumatoid factor-negative juvenile idiopathic arthritis without anti-nuclear antibodies 11.8
4 rheumatoid factor-negative juvenile idiopathic arthritis with anti-nuclear antibodies 11.8
5 juvenile arthritis 10.5
6 polyarticular juvenile idiopathic arthritis 10.5
7 arthritis 10.3
8 acute graft versus host disease 10.0 IL2RB IL2RA
9 b-cell expansion with nfkb and t-cell anergy 10.0 IL2RB IL2RA
10 t-cell large granular lymphocyte leukemia 9.9 IL2RB IL2RA
11 posterior uveitis 9.9 IL2RB IL2RA
12 severe combined immunodeficiency, x-linked 9.9 IL2RB IL2RA
13 type 1 diabetes mellitus 18 9.8 PTPN22 IL2RA
14 type 1 diabetes mellitus 3 9.8 PTPN22 IL2RA
15 tropical spastic paraparesis 9.7 IL2RB IL2RA
16 autoimmune disease of endocrine system 9.7 PTPN22 IL2RA
17 common variable immunodeficiency 9.6 IL2RB IL2RA CD247
18 sezary's disease 9.6 STAT4 IL2RB IL2RA
19 vogt-koyanagi-harada disease 9.6 PTPN22 IL2RA
20 severe combined immunodeficiency 9.6 IL2RB IL2RA CD247
21 lymphadenitis 9.5 IL2RB IL2RA
22 immune deficiency disease 9.5 IL2RB IL2RA CD247
23 crohn's disease 9.4 PTPN22 PTPN2 LACC1
24 lymphopenia 9.4 PTPN22 IL2RB IL2RA
25 behcet syndrome 9.4 STAT4 PTPN22 IL2RA
26 primary biliary cirrhosis 9.2 STAT4 PTPN22 IL2RB
27 aplastic anemia 9.1 STAT4 PTPN22 IL2RA CD247
28 rheumatoid arthritis 9.0 STAT4 PTPN22 IL2RB IL2RA
29 diabetes mellitus, type i 9.0 PTPN22 PTPN2 IL2RA
30 celiac disease 1 8.7 STAT4 PTPN22 PTPN2 IL2RA ANKRD55
31 systemic lupus erythematosus 8.6 STAT4 PTPN22 IL2RB IL2RA CD247
32 inflammatory bowel disease 8.3 STAT4 PTPN22 PTPN2 LACC1 IL2RB IL2RA
33 oligoarticular juvenile idiopathic arthritis 8.0 STAT4 PTPN22 PTPN2 IL2RB IL2RA CD247

Graphical network of the top 20 diseases related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:



Diseases related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis

Symptoms & Phenotypes for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Human phenotypes related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:

58 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dental malocclusion 58 Very frequent (99-80%)
2 arthralgia 58 Very frequent (99-80%)
3 joint dislocation 58 Very frequent (99-80%)
4 joint swelling 58 Very frequent (99-80%)
5 limitation of joint mobility 58 Very frequent (99-80%)
6 elevated c-reactive protein level 58 Very frequent (99-80%)
7 elevated erythrocyte sedimentation rate 58 Very frequent (99-80%)
8 apraxia 58 Very frequent (99-80%)
9 juvenile rheumatoid arthritis 58 Very frequent (99-80%)
10 antinuclear antibody positivity 58 Very frequent (99-80%)
11 polyarticular arthritis 58 Very frequent (99-80%)

MGI Mouse Phenotypes related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.63 CD247 IL2RA IL2RB PTPN2 PTPN22 STAT4
2 hematopoietic system MP:0005397 9.5 CD247 IL2RA IL2RB LACC1 PTPN2 PTPN22
3 immune system MP:0005387 9.17 CD247 IL2RA IL2RB LACC1 PTPN2 PTPN22

Drugs & Therapeutics for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Drugs for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Adalimumab Approved Phase 3 331731-18-1 16219006
2
Abatacept Approved Phase 3 332348-12-6 10237
3
tannic acid Approved Phase 3 1401-55-4
4
Etanercept Approved, Investigational Phase 3 185243-69-0
5
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
6 Immunologic Factors Phase 3
7 Analgesics, Non-Narcotic Phase 3
8 Rheumatoid Factor Phase 3
9 Analgesics Phase 3
10 Immunosuppressive Agents Phase 3
11 Anti-Inflammatory Agents Phase 3
12 Gastrointestinal Agents Phase 3
13 Antirheumatic Agents Phase 3
14 Anti-Inflammatory Agents, Non-Steroidal Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Treatment Tapering in Oligoarticular or Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis With Inactive Disease on Biologic Therapy Active, not recruiting NCT02840175 Phase 3 etanercept;adalimumab;Abatacept;Tocilizumab

Search NIH Clinical Center for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis

Genetic Tests for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Anatomical Context for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

MalaCards organs/tissues related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:

40
Eye, Bone, Thymus, T Cells

Publications for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Articles related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis:

(show all 12)
# Title Authors PMID Year
1
Biallelic loss-of-function LACC1/FAMIN Mutations Presenting as Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis. 61
30872671 2019
2
Predictors of Flare Following Etanercept Withdrawal in Patients with Rheumatoid Factor-negative Juvenile Idiopathic Arthritis Who Reached Remission while Taking Medication. 61
29717035 2018
3
Allogeneic hematopoietic stem cell transplantation for severe, refractory juvenile idiopathic arthritis. 61
29618462 2018
4
Safety of weekly adalimumab in the treatment of juvenile idiopathic arthritis and pediatric chronic uveitis. 61
29103180 2018
5
T Cell Receptor-Independent, CD31/IL-17A-Driven Inflammatory Axis Shapes Synovitis in Juvenile Idiopathic Arthritis. 61
30127787 2018
6
Later-onset rheumatoid factor negative polyarticular juvenile idiopathic arthritis (JIA): a unique patient group? 61
23622111 2013
7
Dense genotyping of immune-related disease regions identifies 14 new susceptibility loci for juvenile idiopathic arthritis. 61
23603761 2013
8
Biologic similarities based on age at onset in oligoarticular and polyarticular subtypes of juvenile idiopathic arthritis. 61
20662067 2010
9
Juvenile idiopathic arthritis and HLA class I and class II interactions and age-at-onset effects. 61
20191588 2010
10
Decreased recent thymus emigrant number in rheumatoid factor-negative polyarticular juvenile idiopathic arthritis. 61
20460033 2010
11
Tumour necrosis factor alpha promoter polymorphisms and etanercept therapy in juvenile idiopathic arthritis. 61
16951943 2007
12
Juvenile idiopathic arthritis (JIA) is primarily associated with HLA-DR8 but not DQ4 on the DR8-DQ4 haplotype. 61
11874841 2002

Variations for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Expression for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Search GEO for disease gene expression data for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis.

Pathways for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Pathways related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

(show all 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.93 PTPN22 PTPN2 IL2RB IL2RA
2
Show member pathways
12.24 STAT4 IL2RB IL2RA CD247
3
Show member pathways
12.13 STAT4 PTPN2 IL2RB IL2RA
4
Show member pathways
12.03 IL2RB IL2RA CD247
5 11.87 STAT4 PTPN22 PTPN2 IL2RB IL2RA
6
Show member pathways
11.77 STAT4 IL2RB IL2RA
7
Show member pathways
11.51 STAT4 IL2RB IL2RA CD247
8 11.47 IL2RB IL2RA
9 11.4 PTPN22 CD247
10
Show member pathways
11.36 IL2RB IL2RA
11 11.2 STAT4 IL2RA
12 11.16 STAT4 IL2RB IL2RA
13
Show member pathways
11.07 IL2RB IL2RA
14 11.01 IL2RB IL2RA
15
Show member pathways
10.92 STAT4 IL2RA
16
Show member pathways
10.9 IL2RB IL2RA
17 10.88 STAT4 IL2RA CD247
18
Show member pathways
10.71 STAT4 IL2RB IL2RA CD247

GO Terms for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

Cellular components related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 interleukin-2 receptor complex GO:0005893 8.62 IL2RB IL2RA

Biological processes related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.43 STAT4 IL2RB IL2RA
2 T cell differentiation GO:0030217 9.16 PTPN22 PTPN2
3 negative regulation of T cell receptor signaling pathway GO:0050860 8.96 PTPN22 PTPN2
4 interleukin-2-mediated signaling pathway GO:0038110 8.62 IL2RB IL2RA

Molecular functions related to Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein tyrosine kinase binding GO:1990782 9.26 PTPN22 CD247
2 non-membrane spanning protein tyrosine phosphatase activity GO:0004726 9.16 PTPN22 PTPN2
3 interleukin-2 binding GO:0019976 8.96 IL2RB IL2RA
4 interleukin-2 receptor activity GO:0004911 8.62 IL2RB IL2RA

Sources for Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic...

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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