MCID: SYS034
MIFTS: 44

Systemic Onset Juvenile Idiopathic Arthritis malady

Rare diseases, Bone diseases categories

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

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34MalaCards
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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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44NIH Rare Diseases, 46Novoseek, 63UMLS
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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 44
systemic onset juvenile rheumatoid arthritis 44
systemic onset juvenile chronic arthritis 63
systemic juvenile idiopathic arthritis 44
juvenile-onset still disease 63
still's disease 44
stills disease 46


Related Diseases for Systemic Onset Juvenile Idiopathic Arthritis

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18GeneCards, 19GeneDecks
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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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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Systemic Onset Juvenile Idiopathic Arthritis

Drug clinical trials:

Search ClinicalTrials for Systemic Onset Juvenile Idiopathic Arthritis

Search NIH Clinical Center for Systemic Onset Juvenile Idiopathic Arthritis

Search CenterWatch 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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34MalaCards
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MalaCards organs/tissues related to Systemic Onset Juvenile Idiopathic Arthritis:

34
Liver, Heart, Myeloid, B cells

Animal Models for Systemic Onset Juvenile Idiopathic Arthritis or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

38 (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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53PubMed
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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, 16Gene 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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Sources:
51PathCards, 56Reactome, 39NCBI BioSystems Database, 61Thomson Reuters, 31KEGG, 54QIAGEN, 55R&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 Signaling39
Apoptosis39
9.4TNF, PRF1
2
Show member pathways
Immune response CD16 signaling in NK cells61
9.4PRF1, TNF
3
Show member pathways
9.4PRF1, TNF
4
Show member pathways
Ras signaling in the CD4+ TCR pathway39
9.4TNF, PRF1
5
Show member pathways
FAS pathway and Stress induction of HSP regulation39
Apoptosis and survival FAS signaling cascades61
Caspase cascade in apoptosis39
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 pathway39
TCR Signaling Pathway39
9.1TNF, IL10
11
Show member pathways
Toll-like receptor signaling pathway39
Regulation of toll-like receptor signaling pathway39
9.1TNF, IL10
129.1TNF, IL10
13
Show member pathways
9.1IL10, TNF
14
Show member pathways
IL23-mediated signaling events39
Immune response IL 10 signaling pathway61
Angiopoietin receptor Tie2-mediated signaling39
Development PDGF signaling via STATs and NF kB61
Development Angiopoietin Tie2 signaling61
9.1IL10, TNF
159.1IL10, TNF
169.1IL10, TNF
179.1IL10, TNF
18
Show member pathways
IL27-mediated signaling events39
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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46Novoseek, 12DrugBank, 52PharmGKB, 62Tocris Bioscience, 30IUPHAR, 3BitterDB, 25HMDB
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Compounds related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards/GeneDecks:

(show all 50)
idCompoundScoreTop Affiliating Genes
1cdp571469.6IL10, TNF
2interferon gamma469.6IL10, TNF
3blys469.6IL10, TNF
4gabexate mesilate469.6IL10, TNF
5ulinastatin469.6TNF, IL10
6toxin-1469.6TNF, IL10
7mip 1alpha469.6IL10, TNF
8natalizumab46 1210.6IL10, TNF
95 aminosalicylic acid469.6TNF, IL10
10il-12469.6TNF, IL10
11adalimumab46 52 1211.6IL10, TNF
12indoleamine469.6TNF, IL10
13imiquimod46 62 30 1212.5IL10, TNF
14cyclosporine52 1210.5IL10, TNF
15polymyxin b469.5TNF, IL10
16okt3469.5IL10, TNF
17groalpha469.5IL10, TNF
18gm-csf46 3010.5TNF, IL10
19lipoteichoic acid469.5IL10, TNF
208-isoprostane469.5IL10, TNF
212,5-oligoadenylate469.5TNF, IL10
22polyinosinic-polycytidylic acid469.5TNF, IL10
23rolipram46 62 3011.5IL10, TNF
24etanercept46 52 1211.5IL10, TNF
25mycophenolate mofetil46 52 1211.5IL10, TNF
26il 10469.5IL10, TNF
27tarc469.5TNF, IL10
28infliximab46 52 1211.5TNF, IL10
29peptidoglycan46 3010.5IL10, TNF
30ivig469.5TNF, IL10
31pentoxifylline46 1210.5IL10, TNF
32ccl346 3010.4IL10, TNF
33neopterin469.4TNF, IL10
3415-deoxy-delta-12,14-prostaglandin j2469.4IL10, TNF
35rituximab46 52 1211.4IL10, TNF
36chloroquine46 3 52 30 1213.4TNF, IL10
37thalidomide46 52 62 1212.4TNF, IL10
38prednisolone46 30 1211.4IL10, TNF
39atorvastatin46 52 30 25 1213.4IL10, TNF
40hydrocortisone46 3 62 1212.3TNF, IL10
41tgf beta1469.3IL10, TNF
42simvastatin46 52 62 30 25 1214.3IL10, TNF
43ionomycin469.2TNF, IL10
44prostacyclin469.2IL10, TNF
45tacrolimus46 52 1211.1IL10, TNF
46rantes469.1IL10, TNF
47calcitriol46 62 25 1212.0TNF, IL10
48cycloheximide469.0TNF, PRF1, IL10
49endotoxin469.0TNF, MEFV, IL10
50methotrexate52 46 1210.8IL10, TNF

GO Terms for genes affiliated with Systemic Onset Juvenile Idiopathic Arthritis

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17Gene 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
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet