MCID: SYS034
MIFTS: 44

Systemic Onset Juvenile Idiopathic Arthritis malady

Rare diseases, Bone diseases categories
Download this MalaCard

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

About this section


Fully expand this MalaCard
MalaCards based summary: Systemic Onset Juvenile Idiopathic Arthritis, also known as systemic onset juvenile rheumatoid arthritis, is related to arthritis and peritonitis. An important gene associated with Systemic Onset Juvenile Idiopathic Arthritis is PRF1 (perforin 1 (pore forming protein)), and among its related pathways are Apoptosis and Immune response Role of DAP12 receptors in NK cells. The compounds cdp571 and interferon gamma have been mentioned in the context of this disorder. Affiliated tissues include liver, heart and myeloid, and related mouse phenotypes are tumorigenesis and skeleton.

Aliases & Classifications for Systemic Onset Juvenile Idiopathic Arthritis

About this section
Sources:
42NIH Rare Diseases, 44Novoseek, 62UMLS
See all sources

Systemic Onset Juvenile Idiopathic Arthritis, Aliases & Descriptions:

Name: Systemic Onset Juvenile Idiopathic Arthritis 42
Systemic Onset Juvenile Rheumatoid Arthritis 42 62
Systemic Onset Juvenile Chronic Arthritis 62
Systemic Juvenile Idiopathic Arthritis 42
 
Juvenile-Onset Still Disease 62
Still's Disease 42
Stills Disease 44


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Bone diseases


Related Diseases for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Graphical network of the top 20 diseases related to Systemic Onset Juvenile Idiopathic Arthritis:



Diseases related to systemic onset juvenile idiopathic arthritis

Symptoms for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Drugs & Therapeutics for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Drug clinical trials:

Search ClinicalTrials for Systemic Onset Juvenile Idiopathic Arthritis

Search NIH Clinical Center for Systemic Onset Juvenile Idiopathic Arthritis

Genetic Tests for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Anatomical Context for Systemic Onset Juvenile Idiopathic Arthritis

About this section

MalaCards organs/tissues related to Systemic Onset Juvenile Idiopathic Arthritis:

32
Liver, Heart, Myeloid, B cells

Animal Models for Systemic Onset Juvenile Idiopathic Arthritis or affiliated genes

About this section

MGI Mouse Phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

36 (show all 12)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.7IL10, PRF1, TNF
2MP:00053908.5IL10, MEFV, TNF
3MP:00053798.3TNF, PRF1, IL10
4MP:00053708.2IL10, MEFV, PRF1, TNF
5MP:00107718.2TNF, PRF1, MEFV, IL10
6MP:00053898.2TNF, PRF1, MEFV, IL10
7MP:00053978.2IL10, MEFV, PRF1, TNF
8MP:00053878.2TNF, PRF1, MEFV, IL10
9MP:00053848.1TNF, PRF1, MEFV, IL10
10MP:00053788.1TNF, PRF1, MEFV, IL10
11MP:00053768.0TNF, PRF1, MEFV, IL10
12MP:00107687.9IL10, MEFV, PRF1, TNF

Publications for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50)    (show all 94)
idTitleAuthorsYear
1
Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis. (24417800)
2014
2
Pathogenesis of systemic inflammatory diseases in childhood: "Lessons from clinical trials of anti-cytokine monoclonal antibodies for Kawasaki disease, systemic onset juvenile idiopathic arthritis, and cryopyrin-associated periodic fever syndrome" (24842480)
2014
3
A130: Is the CCR5-delta32 Mutation Protective Against Systemic-Onset Juvenile Idiopathic Arthritis? (24677884)
2014
4
Atopy in children with systemic onset juvenile idiopathic arthritis (SoJIA) is associated with a worse outcome. (24611751)
2014
5
AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. (23664546)
2013
6
Anakinra pharmacokinetics in children and adolescents with systemic-onset juvenile idiopathic arthritis and autoinflammatory syndromes. (23915458)
2013
7
Congestive heart failure: an uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). (22161583)
2013
8
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. (24315465)
2013
9
Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India. (21221591)
2012
10
Leflunomide in systemic onset Juvenile idiopathic arthritis. (23024081)
2012
11
Anakinra: a safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA). (22083619)
2012
12
Development of germinoma during the treatment of systemic-onset juvenile idiopathic arthritis with infliximab. (22072226)
2012
13
Pure red cell aplasia in systemic onset juvenile idiopathic arthritis. (23448798)
2012
14
Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease. (22573189)
2012
15
A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial). (21173013)
2011
16
De novo 19q13.42 duplications involving NLRP gene cluster in a patient with systemic-onset juvenile idiopathic arthritis. (21326309)
2011
17
Safety and efficacy of an oral histone deacetylase inhibitor in systemic-onset juvenile idiopathic arthritis. (21538322)
2011
18
IL10 promoter polymorphisms are associated with systemic onset juvenile idiopathic arthritis (SoJIA). (21205466)
2010
19
Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. (20019066)
2010
20
Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis. (20472718)
2010
21
Peripheral ulcerative keratitis in a patient with systemic-onset juvenile idiopathic arthritis. (21162464)
2010
22
Incomplete Kawasaki syndrome followed by systemic onset-juvenile idiopathic arthritis mimicking Kawasaki syndrome. (19455336)
2010
23
Bicipital synovial cyst in systemic-onset juvenile idiopathic arthritis. (20338579)
2010
24
Perioperative anesthetic concerns in a child with systemic onset juvenile idiopathic arthritis syndrome. (20670244)
2010
25
Fever of unknown origin in a patient of systemic onset juvenile idiopathic arthritis. (22918076)
2010
26
Macrophage activation syndrome in an inadequately treated patient with systemic onset juvenile idiopathic arthritis. (20502084)
2009
27
Rituximab therapy for refractory systemic-onset juvenile idiopathic arthritis. (19286909)
2009
28
The myeloid-related proteins 8 and 14 complex, a novel ligand of toll-like receptor 4, and interleukin-1beta form a positive feedback mechanism in systemic-onset juvenile idiopathic arthritis. (19248102)
2009
29
Nodulosis in systemic onset juvenile idiopathic arthritis: an uncommon event with spontaneous resolution. (19840317)
2009
30
Genetic loci contributing to hemophagocytic lymphohistiocytosis do not confer susceptibility to systemic-onset juvenile idiopathic arthritis. (18311812)
2008
31
Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis. (18661762)
2008
32
Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. (17947302)
2008
33
Systemic onset juvenile idiopathic arthritis--its unusual presentation. (18536898)
2008
34
Is tocilizumab a good therapeutic option for RA and systemic-onset juvenile idiopathic arthritis? (18779846)
2008
35
Efficacy and safety of tocilizumab in patients with systemic-onset juvenile idiopathic arthritis: a randomised, double-blind, placebo-controlled, withdrawal phase III trial. (18358927)
2008
36
Large pericardial effusion requiring pericardiocentesis as cardinal sign of macrophage activation syndrome in systemic onset-juvenile idiopathic arthritis. (17160383)
2007
37
Treatment of difficult cases of systemic-onset juvenile idiopathic arthritis with tacrolimus. (17187256)
2007
38
Failure to distinguish systemic-onset juvenile idiopathic arthritis from incomplete Kawasaki disease in an infant. (17854457)
2007
39
Systemic lupus erythematosus with major organ involvement in a patient with systemic-onset juvenile idiopathic arthritis. (17407252)
2007
40
Blood leukocyte microarrays to diagnose systemic onset juvenile idiopathic arthritis and follow the response to IL-1 blockade. (17724127)
2007
41
Systemic onset juvenile idiopathic arthritis: update on pathogenesis and treatment. (20477675)
2007
42
Developing a disease activity tool for systemic-onset juvenile idiopathic arthritis by international consensus using the Delphi approach. (16159950)
2005
43
Therapeutic efficacy of humanized recombinant anti-interleukin-6 receptor antibody in children with systemic-onset juvenile idiopathic arthritis. (15751095)
2005
44
Role of interleukin-1 (IL-1) in the pathogenesis of systemic onset juvenile idiopathic arthritis and clinical response to IL-1 blockade. (15851489)
2005
45
Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis? (15956091)
2005
46
Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis. (15930186)
2005
47
Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis. (12746913)
2003
48
Infliximab for systemic onset juvenile idiopathic arthritis: experience in 3 children. (12022340)
2002
49
Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years. (11196535)
2001
50
Macrophage activation syndrome as the initial manifestation of systemic onset juvenile idiopathic arthritis. (11791663)
2001

Variations for Systemic Onset Juvenile Idiopathic Arthritis

About this section

Expression for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

About this section
Expression patterns in normal tissues for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

Search GEO for disease gene expression data for Systemic Onset Juvenile Idiopathic Arthritis.

Pathways for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

About this section

Pathways related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

(show all 22)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Apoptosis Modulation and Signaling37
Apoptosis37
9.4TNF, PRF1
2
Show member pathways
Immune response CD16 signaling in NK cells60
9.4TNF, PRF1
3
Show member pathways
9.4PRF1, TNF
4
Show member pathways
Ras signaling in the CD4+ TCR pathway37
9.4TNF, PRF1
5
Show member pathways
FAS pathway and Stress induction of HSP regulation37
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis37
9.4TNF, PRF1
6
Show member pathways
9.3MEFV, TNF
7
Show member pathways
9.1TNF, IL10
89.1IL10, TNF
99.1IL10, TNF
10
Show member pathways
BCR signaling pathway37
TCR Signaling Pathway37
9.1IL10, TNF
11
Show member pathways
Toll-like receptor signaling pathway37
Regulation of toll-like receptor signaling pathway37
9.1IL10, TNF
129.1IL10, TNF
13
Show member pathways
9.1TNF, IL10
14
Show member pathways
IL23-mediated signaling events37
Immune response IL 10 signaling pathway60
Angiopoietin receptor Tie2-mediated signaling37
Development PDGF signaling via STATs and NF kB60
Development Angiopoietin Tie2 signaling60
9.1IL10, TNF
159.1IL10, TNF
169.1TNF, IL10
179.1IL10, TNF
18
Show member pathways
IL27-mediated signaling events37
9.1IL10, TNF
199.1IL10, TNF
209.1TNF, IL10
219.1IL10, TNF
22
Show member pathways
8.6IL10, PRF1, TNF

Compounds for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

About this section

Compounds related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

(show all 50)
idCompoundScoreTop Affiliating Genes
1cdp571449.6IL10, TNF
2interferon gamma449.6IL10, TNF
3blys449.6IL10, TNF
4gabexate mesilate449.6IL10, TNF
5ulinastatin449.6TNF, IL10
6toxin-1449.6TNF, IL10
7mip 1alpha449.6IL10, TNF
8natalizumab44 1110.6IL10, TNF
95 aminosalicylic acid449.6TNF, IL10
10il-12449.6TNF, IL10
11adalimumab44 50 1111.6IL10, TNF
12indoleamine449.6TNF, IL10
13imiquimod44 61 28 1112.5IL10, TNF
14cyclosporine50 1110.5IL10, TNF
15polymyxin b449.5TNF, IL10
16okt3449.5IL10, TNF
17groalpha449.5IL10, TNF
18gm-csf44 2810.5TNF, IL10
19lipoteichoic acid449.5IL10, TNF
208-isoprostane449.5IL10, TNF
212,5-oligoadenylate449.5TNF, IL10
22polyinosinic-polycytidylic acid449.5TNF, IL10
23rolipram44 61 2811.5IL10, TNF
24etanercept44 50 1111.5IL10, TNF
25mycophenolate mofetil44 50 1111.5IL10, TNF
26il 10449.5IL10, TNF
27tarc449.5TNF, IL10
28infliximab44 50 1111.5TNF, IL10
29peptidoglycan44 2810.5IL10, TNF
30ivig449.5TNF, IL10
31pentoxifylline44 1110.5IL10, TNF
32ccl344 2810.4IL10, TNF
33neopterin449.4TNF, IL10
3415-deoxy-delta-12,14-prostaglandin j2449.4IL10, TNF
35rituximab44 50 1111.4IL10, TNF
36chloroquine44 2 50 28 1113.4TNF, IL10
37thalidomide44 50 61 1112.4TNF, IL10
38prednisolone44 28 1111.4IL10, TNF
39atorvastatin44 50 28 24 1113.4IL10, TNF
40hydrocortisone44 2 61 1112.3TNF, IL10
41tgf beta1449.3IL10, TNF
42simvastatin44 50 61 28 24 1114.3IL10, TNF
43ionomycin449.2TNF, IL10
44prostacyclin449.2IL10, TNF
45tacrolimus44 50 1111.1IL10, TNF
46rantes449.1IL10, TNF
47calcitriol44 61 24 1112.0TNF, IL10
48cycloheximide449.0TNF, PRF1, IL10
49endotoxin449.0TNF, MEFV, IL10
50methotrexate50 44 1110.8IL10, TNF

GO Terms for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

About this section

Biological processes related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:0328009.4IL10, TNF
2negative regulation of cytokine secretion involved in immune responseGO:0027409.3IL10, TNF
3negative regulation of growth of symbiont in hostGO:0441309.3IL10, TNF
4negative regulation of interleukin-12 productionGO:0326959.2IL10, MEFV
5negative regulation of interleukin-6 productionGO:0327159.2IL10, TNF
6positive regulation of cytokine secretionGO:0507159.1IL10, TNF
7positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.1IL10, TNF
8response to activityGO:0148239.0TNF, IL10
9response to glucocorticoidGO:0513848.8IL10, TNF
10inflammatory responseGO:0069548.6TNF, MEFV, IL10

Molecular functions related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.1IL10, TNF

Products for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Systemic Onset Juvenile Idiopathic Arthritis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet