MCID: SYS034
MIFTS: 44

Systemic Onset Juvenile Idiopathic Arthritis malady

Rare diseases, Bone diseases categories
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Summaries for Systemic Onset Juvenile Idiopathic Arthritis

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33MalaCards
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MalaCards: Systemic Onset Juvenile Idiopathic Arthritis, also known as systemic onset juvenile rheumatoid arthritis, is related to arthritis and sepsis. 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

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43NIH Rare Diseases, 45Novoseek, 62UMLS
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Classifications:

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


Aliases & Descriptions:

systemic onset juvenile idiopathic arthritis 43
systemic onset juvenile rheumatoid arthritis 43
systemic onset juvenile chronic arthritis 62
systemic juvenile idiopathic arthritis 43
juvenile-onset still disease 62
still's disease 43
stills disease 45


Related Diseases for Systemic Onset Juvenile Idiopathic Arthritis

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17GeneCards, 18GeneDecks
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Diseases related to Systemic Onset Juvenile Idiopathic Arthritis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 78)
idRelated DiseaseScoreTop Affiliating Genes
1arthritis31.8IL10, MEFV, TNF
2sepsis30.4IL10, TNF
3peritonitis30.4MEFV, TNF
4rheumatoid arthritis30.4IL10, TNF, MEFV
5macrophage activation syndrome10.9
6kawasaki disease10.6
7cardiac tamponade10.5
8amyloidosis10.5
9blindness10.5
10diabetes mellitus10.3
11encephalitis10.3
12hodgkin's lymphoma10.3
13respiratory failure10.3
14acute liver failure10.3
15pure red-cell aplasia10.3
16juvenile rheumatoid arthritis10.3
17autoimmune hepatitis10.3
18hemophagocytic lymphohistiocytosis10.3
19systemic lupus erythematosus10.3
20adult-onset still's disease10.3
21congestive heart failure10.3
22glomerulonephritis10.3
23hepatitis10.3
24influenza10.3
25leukemia10.3
26lupus erythematosus10.3
27lymphoblastic leukemia10.3
28myocarditis10.3
29pericardial effusion10.3
30renal cell carcinoma10.3
31germinoma10.3
32nodular regenerative hyperplasia10.3
33autoimmune lymphoproliferative syndrome10.2
34gastric ulcer10.2
35cervicitis10.2
36gastroenteritis10.2
37pericarditis10.2
38castleman's disease10.2
39endophthalmitis10.1PRF1
40meningitis10.1PRF1
41erysipelas10.1MEFV
42palindromic rheumatism10.1MEFV
43leishmaniasis10.1IL10
44blau syndrome10.0MEFV, TNF
45periodic fever, familial, autosomal dominant10.0TNF, MEFV
46familial mediterranean fever10.0MEFV, TNF
47behcet's disease10.0MEFV, TNF
48rheumatic disease10.0TNF, MEFV
49vasculitis10.0TNF, MEFV
50alcoholic hepatitis10.0IL10, TNF

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

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Drugs & Therapeutics for Systemic Onset Juvenile Idiopathic Arthritis

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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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

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Anatomical Context for Systemic Onset Juvenile Idiopathic Arthritis

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33MalaCards
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MalaCards organs/tissues related to Systemic Onset Juvenile Idiopathic Arthritis:

33
Liver, Heart, Myeloid, B cells

Animal Models for Systemic Onset Juvenile Idiopathic Arthritis or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

37 (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

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52PubMed
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Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50)    (show all 87)
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
AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. (23664546)
2013
3
Anakinra pharmacokinetics in children and adolescents with systemic-onset juvenile idiopathic arthritis and autoinflammatory syndromes. (23915458)
2013
4
Congestive heart failure: an uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). (22161583)
2013
5
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. (24315465)
2013
6
Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India. (21221591)
2012
7
Leflunomide in systemic onset Juvenile idiopathic arthritis. (23024081)
2012
8
Anakinra: a safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA). (22083619)
2012
9
Development of germinoma during the treatment of systemic-onset juvenile idiopathic arthritis with infliximab. (22072226)
2012
10
Pure red cell aplasia in systemic onset juvenile idiopathic arthritis. (23448798)
2012
11
Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease. (22573189)
2012
12
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
13
De novo 19q13.42 duplications involving NLRP gene cluster in a patient with systemic-onset juvenile idiopathic arthritis. (21326309)
2011
14
Safety and efficacy of an oral histone deacetylase inhibitor in systemic-onset juvenile idiopathic arthritis. (21538322)
2011
15
IL10 promoter polymorphisms are associated with systemic onset juvenile idiopathic arthritis (SoJIA). (21205466)
2010
16
Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. (20019066)
2010
17
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
18
Incomplete Kawasaki syndrome followed by systemic onset-juvenile idiopathic arthritis mimicking Kawasaki syndrome. (19455336)
2010
19
Bicipital synovial cyst in systemic-onset juvenile idiopathic arthritis. (20338579)
2010
20
Perioperative anesthetic concerns in a child with systemic onset juvenile idiopathic arthritis syndrome. (20670244)
2010
21
Fever of unknown origin in a patient of systemic onset juvenile idiopathic arthritis. (22918076)
2010
22
Macrophage activation syndrome in an inadequately treated patient with systemic onset juvenile idiopathic arthritis. (20502084)
2009
23
Rituximab therapy for refractory systemic-onset juvenile idiopathic arthritis. (19286909)
2009
24
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
25
Nodulosis in systemic onset juvenile idiopathic arthritis: an uncommon event with spontaneous resolution. (19840317)
2009
26
Successful treatment with B-cell depleting therapy for refractory systemic onset juvenile idiopathic arthritis: a case report. (19164423)
2009
27
Macrophage activation syndrome associated with etanercept in a child with systemic onset juvenile idiopathic arthritis. (20077954)
2009
28
Genetic loci contributing to hemophagocytic lymphohistiocytosis do not confer susceptibility to systemic-onset juvenile idiopathic arthritis. (18311812)
2008
29
Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis. (18661762)
2008
30
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
31
Systemic onset juvenile idiopathic arthritis--its unusual presentation. (18536898)
2008
32
Is tocilizumab a good therapeutic option for RA and systemic-onset juvenile idiopathic arthritis? (18779846)
2008
33
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
34
The role of gene transcript signature in diagnosing systemic onset juvenile idiopathic arthritis. (18460268)
2008
35
Anakinra treatment for systemic onset juvenile idiopathic arthritis (SOJIA). (18321945)
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

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Expression for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

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2BioGPS, 15Gene Expression Omnibus DataSets
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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

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50PathCards, 55Reactome, 38NCBI BioSystems Database, 60Thomson Reuters, 30KEGG, 53QIAGEN, 54R&D Systems
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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 Signaling38
Apoptosis38
9.4TNF, PRF1
2
Show member pathways
Immune response CD16 signaling in NK cells60
9.4PRF1, TNF
3
Show member pathways
9.4PRF1, TNF
4
Show member pathways
Ras signaling in the CD4+ TCR pathway38
9.4TNF, PRF1
5
Show member pathways
FAS pathway and Stress induction of HSP regulation38
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis38
9.4PRF1, TNF
6
Show member pathways
9.3TNF, MEFV
7
Show member pathways
9.1TNF, IL10
89.1TNF, IL10
99.1TNF, IL10
10
Show member pathways
BCR signaling pathway38
TCR Signaling Pathway38
9.1TNF, IL10
11
Show member pathways
Toll-like receptor signaling pathway38
Regulation of toll-like receptor signaling pathway38
9.1TNF, IL10
129.1TNF, IL10
13
Show member pathways
9.1IL10, TNF
14
Show member pathways
IL23-mediated signaling events38
Immune response IL 10 signaling pathway60
Angiopoietin receptor Tie2-mediated signaling38
Development PDGF signaling via STATs and NF kB60
Development Angiopoietin Tie2 signaling60
9.1IL10, TNF
159.1IL10, TNF
169.1IL10, TNF
179.1IL10, TNF
18
Show member pathways
IL27-mediated signaling events38
9.1IL10, TNF
199.1TNF, IL10
209.1IL10, TNF
219.1TNF, IL10
22
Show member pathways
8.6IL10, TNF, PRF1

Compounds for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

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45Novoseek, 11DrugBank, 51PharmGKB, 61Tocris Bioscience, 29IUPHAR, 3BitterDB, 24HMDB
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Compounds related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

(show all 50)
idCompoundScoreTop Affiliating Genes
1cdp571459.6IL10, TNF
2interferon gamma459.6IL10, TNF
3blys459.6IL10, TNF
4gabexate mesilate459.6IL10, TNF
5ulinastatin459.6TNF, IL10
6toxin-1459.6TNF, IL10
7mip 1alpha459.6IL10, TNF
8natalizumab45 1110.6IL10, TNF
95 aminosalicylic acid459.6TNF, IL10
10il-12459.6TNF, IL10
11adalimumab45 51 1111.6IL10, TNF
12indoleamine459.6TNF, IL10
13imiquimod45 61 29 1112.5IL10, TNF
14cyclosporine51 1110.5IL10, TNF
15polymyxin b459.5TNF, IL10
16okt3459.5IL10, TNF
17groalpha459.5IL10, TNF
18gm-csf45 2910.5TNF, IL10
19lipoteichoic acid459.5IL10, TNF
208-isoprostane459.5IL10, TNF
212,5-oligoadenylate459.5TNF, IL10
22polyinosinic-polycytidylic acid459.5TNF, IL10
23rolipram45 61 2911.5IL10, TNF
24etanercept45 51 1111.5IL10, TNF
25mycophenolate mofetil45 51 1111.5IL10, TNF
26il 10459.5IL10, TNF
27tarc459.5TNF, IL10
28infliximab45 51 1111.5TNF, IL10
29peptidoglycan45 2910.5IL10, TNF
30ivig459.5TNF, IL10
31pentoxifylline45 1110.5IL10, TNF
32ccl345 2910.4IL10, TNF
33neopterin459.4TNF, IL10
3415-deoxy-delta-12,14-prostaglandin j2459.4IL10, TNF
35rituximab45 51 1111.4IL10, TNF
36chloroquine45 3 51 29 1113.4TNF, IL10
37thalidomide45 51 61 1112.4TNF, IL10
38prednisolone45 29 1111.4IL10, TNF
39atorvastatin45 51 29 24 1113.4IL10, TNF
40hydrocortisone45 3 61 1112.3TNF, IL10
41tgf beta1459.3IL10, TNF
42simvastatin45 51 61 29 24 1114.3IL10, TNF
43ionomycin459.2TNF, IL10
44prostacyclin459.2IL10, TNF
45tacrolimus45 51 1111.1IL10, TNF
46rantes459.1IL10, TNF
47calcitriol45 61 24 1112.0TNF, IL10
48cycloheximide459.0TNF, PRF1, IL10
49endotoxin459.0TNF, MEFV, IL10
50methotrexate51 45 1110.8IL10, TNF

GO Terms for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

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16Gene Ontology
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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

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Sources for Systemic Onset Juvenile Idiopathic Arthritis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet