MCID: SYS034
MIFTS: 48

Systemic Onset Juvenile Idiopathic Arthritis

Categories: Rare diseases, Bone diseases

Aliases & Classifications for Systemic Onset Juvenile Idiopathic Arthritis

MalaCards integrated aliases for Systemic Onset Juvenile Idiopathic Arthritis:

Name: Systemic Onset Juvenile Idiopathic Arthritis 50
Systemic Onset Juvenile Rheumatoid Arthritis 50
Systemic Onset Juvenile Chronic Arthritis 69
Systemic Juvenile Idiopathic Arthritis 50

Classifications:



Summaries for Systemic Onset Juvenile Idiopathic Arthritis

NIH Rare Diseases : 50 the following summary is from orphanet, a european reference portal for information on rare diseases and orphan drugs.orpha number: 85414disease definitionsystemic-onset juvenile idiopathic arthritis is marked by the severity of the extra-articular manifestations (fever, cutaneous eruptions) and by an equal sex ratio.epidemiologyit represents 10-11% of cases of juvenile idiopathic arthritis (jia). the prevalence has been estimated at 1-10 in 30,000 children with an annual incidence of 1-20 in 900,000 children.clinical descriptiononset usually occurs between 3 and 5 years of age. the clinical signs include fever with oscillating temperatures over a 24-hour period and peaks of over 39┬░c or more. these fever peaks are associated with transient cutaneous eruptions and diffuse erythematosis or urticarial-like lesions. the presence of arthritis is essential for diagnosis but may appear later in the disease course. the number of sites affected is variable (mono-, oligo- or polyarthritis) affecting both the small and large joints in a nearly symmetrical manner. this characteristic diagnostic triad may also be associated with an adenopathy and hepatosplenomegaly. visceral complications (pericarditis, pleural effusion or serous peritonitis with abdominal pain) may be present. there are no specific biological signs but the inflammatory disease is severe with a large increase on the level of ferritin and a decrease in the percentage of glycosylated ferritin.etiologythe underlying mechanisms and triggering factors have not yet been identified, but the disease can be clearly distinguished from other forms of jia. the disease may represent an autoimmune disorder rather than an autoinflammatory disease (as for periodic fever and cinca/nomid).diagnostic methodsthe clinical triad of daily fever (lasting more than 2 weeks), arthritis and transient cutaneous eruptions is vital for diagnosis (criteria established at in 2001 at the last international meeting in edmonton). in the absence of cutaneous eruptions, the presence of an adenopathy, hepatosplenomegaly or serous effusion also confirm the diagnosis. there is no specific biological sign. exclusion criteria are the presence of systemic arthritis or psoriasis in the patient, or a family history of psoriasis in one of the parents or a first-degree relative, hla b27-positivity in males with onset of arthritis after 6 years of age and detection of rheumatoid factor igm in two test samples taken three months apart. other exclusion criteria include: the presence of ankylosing spondylarthritis, enthesitis and arthritis, sacroiliitis with an inflammatory enteropathy or acute anterior uveitis in the patient or a family history of one of these conditions in a parent or first-degree relative.differential diagnosisthe differential diagnosis should include fever associated with infection, connective tissue disease (notably lupus), acute leukaemia and other autoinflammatory diseases.management and treatmentmanagement should be carried out at a specialised centre. high doses of aspirin or nonsteroidal anti-inflammatory drugs (nsaids) constitute the first-line treatment. in cases refractory to treatment over a period of 2-6 weeks, high-dose corticotherapy should be used. disease-modifying antirheumatic drugs (methotrexate and biotherapy) may be recommended in case of corticoresistance but their effectiveness varies. other drug treatments (thalidomide, interlukin-1 receptor antagonists (anakinra) and monoclonal anti-interleukin-6 monoclonal antibody (mra) therapy) are available or currently under investigation for corticoresistant patients. in certain cases, intraarticular injection may be proposed.prognosisthe disease resolves before adulthood in around half of patients. in the remaining cases, the arthritis persists, with or without fever and cutaneous eruption. severe sequelae are present in 20% of cases and involve growth delay, bone and cartilage erosion with functional handicap, and a risk of osteopaenia.visit the orphanet disease page for more resources. last updated: 1/22/2007

MalaCards based summary : Systemic Onset Juvenile Idiopathic Arthritis, also known as systemic onset juvenile rheumatoid arthritis, is related to rheumatoid arthritis and macrophage activation syndrome. An important gene associated with Systemic Onset Juvenile Idiopathic Arthritis is TNF (Tumor Necrosis Factor), and among its related pathways/superpathways are Toll-Like receptor Signaling Pathways and Allograft rejection. The drugs Liver Extracts and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include bone, testes and liver, and related phenotypes are hematopoietic system and immune system

Related Diseases for Systemic Onset Juvenile Idiopathic Arthritis

Diseases related to Systemic Onset Juvenile Idiopathic Arthritis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 181)
id Related Disease Score Top Affiliating Genes
1 rheumatoid arthritis 28.9 IL18 IL1B TNF
2 macrophage activation syndrome 11.4
3 rheumatoid arthritis, systemic juvenile 11.2
4 arthritis 10.8
5 craniometaphyseal dysplasia 10.4 MEFV TNF
6 tetanus 10.4 MEFV TNF
7 mechanical lagophthalmos 10.4 MEFV TNF
8 cauda equina syndrome 10.4 IL18 TNF
9 multiple sclerosis 5 10.4 MEFV TNF
10 dermatomycosis 10.4 MEFV TNF
11 deafness, autosomal recessive 67 10.4 IL18 TNF
12 steatorrhea 10.4 IL18 TNF
13 lip cancer 10.4 MEFV TNF
14 myopathy 10.4 MEFV TNF
15 levator syndrome 10.4 IL18 TNF
16 acute monoblastic leukemia 10.4 IL18 TNF
17 reardon wilson cavanagh syndrome 10.4 IL18 TNF
18 esophagus leiomyoma 10.4 IL18 TNF
19 louping ill 10.4 MEFV TNF
20 tick-borne encephalitis 10.4 IL18 TNF
21 melioidosis 10.3 IL18 TNF
22 erythema infectiosum 10.3 IL18 TNF
23 macular dystrophy, atypical vitelliform 10.3 PRF1 TNF
24 renal tubular acidosis 10.3 IL18 TNF
25 phlyctenulosis 10.3 PRF1 TNF
26 kawasaki disease 10.3
27 geniculate ganglionitis 10.2 IL18 TNF
28 neonatal jaundice 10.2 IL18 TNF
29 sternal cleft 10.1 IL1B TNF
30 pulmonary hypertension 10.1 IL1B TNF
31 walker dyson syndrome 10.1 IL1B TNF
32 cataract 10.1 IL1B TNF
33 scrotal carcinoma 10.1 IL1B TNF
34 hypotrichosis-lymphedema-telangiectasia-renal defect syndrome 10.1 IL1B TNF
35 upper gum cancer 10.1 IL1B TNF
36 moved to 244450 10.1 IL1B TNF
37 bipolar i disorder 10.1 IL1B TNF
38 lung sarcoma 10.1 IL1B TNF
39 amyotrophic lateral sclerosis type 10 10.1 IL1B TNF
40 tinea favosa 10.1 IL1B TNF
41 central nervous system leiomyosarcoma 10.1 IL1B TNF
42 epulis 10.1 IL1B TNF
43 indolent myeloma 10.1 IL1B TNF
44 ileocolitis 10.1 IL1B TNF
45 early-onset zonular cataract 10.1 IL1B TNF
46 senile reticular retinal degeneration 10.1 IL1B TNF
47 acquired thrombocytopenia 10.1 IL1B TNF
48 ampulla of vater neoplasm 10.1 IL1B TNF
49 megaloblastic anemia 10.1 IL1B TNF
50 arthus reaction 10.1 IL1B TNF

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



Diseases related to Systemic Onset Juvenile Idiopathic Arthritis

Symptoms & Phenotypes for Systemic Onset Juvenile Idiopathic Arthritis

MGI Mouse Phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.55 TNF IL18 IL1B MEFV PRF1
2 immune system MP:0005387 9.35 TNF IL18 IL1B MEFV PRF1
3 integument MP:0010771 9.02 IL18 IL1B MEFV PRF1 TNF

Drugs & Therapeutics for Systemic Onset Juvenile Idiopathic Arthritis

Drugs for Systemic Onset Juvenile Idiopathic Arthritis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 36)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Benzocaine Approved Phase 3 1994-09-7, 94-09-7 2337
3
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
4
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
5
Alendronate Approved Phase 2, Phase 3 121268-17-5, 66376-36-1 2088
6
Tofacitinib Approved, Investigational Phase 3 477600-75-2
7
Etanercept Approved, Investigational Phase 3 185243-69-0
8 tannic acid Approved, Nutraceutical Phase 3
9
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
10
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
11 Antirheumatic Agents Phase 2, Phase 3
12 Interleukin 1 Receptor Antagonist Protein Phase 2, Phase 3
13 Antibodies Phase 3,Phase 1,Phase 2
14 Antibodies, Monoclonal Phase 3,Phase 1,Phase 2
15 Immunoglobulins Phase 3,Phase 1,Phase 2
16 Analgesics Phase 3
17 Analgesics, Non-Narcotic Phase 3
18 Anti-Inflammatory Agents Phase 3
19 Anti-Inflammatory Agents, Non-Steroidal Phase 3
20 Antimetabolites Phase 3
21 Antimetabolites, Antineoplastic Phase 3
22 Dermatologic Agents Phase 3
23 Folic Acid Antagonists Phase 3
24 Immunosuppressive Agents Phase 3
25 Nucleic Acid Synthesis Inhibitors Phase 3
26 Peripheral Nervous System Agents Phase 3
27 Vitamin B Complex Phase 3
28 Bone Density Conservation Agents Phase 2, Phase 3
29 glucocorticoids Phase 2, Phase 3
30 Pharmaceutical Solutions Phase 3,Phase 2,Phase 1
31 Protein Kinase Inhibitors Phase 3
32 Gastrointestinal Agents Phase 3
33 Folate Nutraceutical Phase 3
34 Vitamin B9 Nutraceutical Phase 3
35 Givinostat hydrochloride Phase 2
36 Histone Deacetylase Inhibitors Phase 2

Interventional clinical trials:

(show all 35)

id Name Status NCT ID Phase Drugs
1 A Study of Decreased Dose Frequency in Participants With Systemic Juvenile Arthritis Who Experience Laboratory Abnormalities During Treatment With RoActemra/Actemra (Tocilizumab) Recruiting NCT01734382 Phase 4 Tocilizumab
2 A Study of Tocilizumab in Chinese Participants With Systemic Juvenile Idiopathic Arthritis (sJIA) Not yet recruiting NCT03301883 Phase 4 Tocilizumab;NSAIDs;CSs;MTX
3 Interleukin-1 Receptor Antagonist (IL-1RA) (ANAKINRA) IN SEVERE SYSTEMIC-ONSET JUVENILE IDIOPATHIC ARTHRITIS Completed NCT00339157 Phase 2, Phase 3 Anakinra
4 An Open-label Extension Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis and Active Systemic Manifestations Manifestations and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Wi Completed NCT00891046 Phase 3 Canakinumab
5 Flare Prevention Study of Canakinumab in Patients With Active Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT00889863 Phase 3 canakinumab;placebo
6 A Study of RoActemra/Actemra (Tocilizumab) in Patients With Active Systemic Juvenile Idiopathic Arthritis (JIA) Completed NCT00642460 Phase 3 tocilizumab [RoActemra/Actemra];Placebo;Non-steroidal anti-inflammatory drugs (NSAIDs);methotrexate;corticosteroids
7 Study of MRA for Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT00144599 Phase 3 MRA(Tocilizumab);placebo
8 Long-term Treatment Study of MRA for Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT00144612 Phase 3 MRA(Tocilizumab)
9 Alendronate Osteoporosis Study Completed NCT00277251 Phase 2, Phase 3 Alendronate
10 Study of MRA for Polyarticular Juvenile Idiopathic Arthritis (pJIA) Completed NCT00144664 Phase 3 MRA(Tocilizumab)
11 A Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis or Hereditary Periodic Fevers Who Participated in the CACZ885G2301E1, CACZ885G2306 or CACZ885N2301 Studies Recruiting NCT02334748 Phase 3 canakinumab
12 ß-SPECIFIC 4 Patients: Study of Pediatric EffiCacy and Safety wIth FIrst-line Use of Canakinumab Recruiting NCT02296424 Phase 3 ACZ885 150 mg (Canakinumab)
13 A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD) Recruiting NCT03265132 Phase 3 Placebo
14 Study of Efficacy and Safety of Canakinumab in Japanese Patients With SJIA Active, not recruiting NCT02396212 Phase 3
15 A Safety, Efficacy And Pharmacokinetics Study Of Tofacitinib In Pediatric Patients With sJIA Not yet recruiting NCT03000439 Phase 3 In open-label phase: treatment with tofacitinib;In double-blind phase: treatment with tofacitinib or placebo in 1:1 ratio
16 Safety and Efficacy Study of Etanercept (Enbrel®) In Children With Systemic Onset Juvenile Rheumatoid Arthritis Terminated NCT00078806 Phase 3 Enbrel;Placebo
17 Single-dose Study to Assess Efficacy of Canakinumab (ACZ885) in Patients With Active Juvenile Idiopathic Arthritis (SJIA) Terminated NCT00886769 Phase 3 Canakinumab;Placebo
18 An Open-label, Multi-arm, Non-comparative Safety and Tolerability Study of Canakinumab (ACZ885) in Patients With Active Systemic Juvenile Idiopathic Arthritis (SJIA) Withdrawn NCT01676948 Phase 3 ACZ885;Canakinumab;Canakinumab;Canakinumab
19 Phase II, Open Label, International, Multicentre Clinical Trial to Investigate Safety and Efficacy of Oral ITF2357 in Patients With Active Systemic Onset Juvenile Idiopathic Arthritis (SOJIA) Completed NCT00570661 Phase 2 ITF2357
20 Safety and Effectiveness of Rilonacept for Treating Systemic Juvenile Idiopathic Arthritis in Children and Young Adults Completed NCT00534495 Phase 2
21 Safety, Efficacy and Pharmacokinetics of Subcutaneous ACZ885 in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT00426218 Phase 1, Phase 2 ACZ885
22 Ilaris (Canakinumab) in the Schnitzler Syndrome Completed NCT01245127 Phase 2 Ilaris
23 A Study to Investigate the Safety and Efficacy of an Anti-IFN-gamma mAb in Children With Systemic Juvenile Idiopathic Arthritis (sJIA) Developing Macrophage Activation Syndrome/ Secondary Hemophagocytic Lymphohistiocytosis (MAS/sHLH) Recruiting NCT03311854 Phase 2 NI-0501
24 A Repeated Dose-finding Study of Sarilumab in Children and Adolescents With Systemic Juvenile Idiopathic Arthritis (sJIA) Recruiting NCT02991469 Phase 2 Sarilumab SAR153191 (REGN88)
25 Tocilizumab in Patients With Schnitzler's Syndrome Recruiting NCT03046381 Phase 2 Tocilizumab
26 Study to Evaluate the Safety and Tolerability of Two Doses of Rilonacept in Pediatric Subjects With Active Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT01803321 Phase 1 rilonacept (IL-1 Trap)
27 A Study of Subcutaneously Administered Tocilizumab in Participants With Systemic Juvenile Idiopathic Arthritis Completed NCT01904292 Phase 1 Tocilizumab
28 A Study to Evaluate Pharmacokinetics and Safety of Tocilizumab (RoActemra/Actemra) in Participants Less Than 2 Years Old With Active Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT01455701 Phase 1 Tocilizumab
29 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
30 Extension Study Evaluating the Safety and Efficacy of Subcutaneous Tocilizumab (RoActemra/Actemra) Administration in Systemic and Polyarticular-Course Juvenile Idiopathic Arthritis Recruiting NCT02165345 Phase 1 Tocilizumab
31 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
32 New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
33 Ultrasonography in Juvenile Idiopathic Arthritis Completed NCT01304420 Early Phase 1
34 The CARRA Registry Completed NCT01697254
35 Single Patient Use of Tocilizumab in Systemic Onset Juvenile Idiopathic Arthritis Terminated NCT00868751

Search NIH Clinical Center for Systemic Onset Juvenile Idiopathic Arthritis

Genetic Tests for Systemic Onset Juvenile Idiopathic Arthritis

Anatomical Context for Systemic Onset Juvenile Idiopathic Arthritis

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

39
Bone, Testes, Liver, Neutrophil, B Cells, Myeloid, Heart

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 119)
id Title Authors Year
1
Clinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review. ( 28499374 )
2017
2
Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases. ( 28225869 )
2017
3
A Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack. ( 28416939 )
2017
4
Canakinumab for first line steroid-free treatment in a child with systemic-onset juvenile idiopathic arthritis. ( 28266235 )
2017
5
Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis. ( 28229369 )
2017
6
The novel use of combined IL-1 and IL-6 inhibition in a patient with severe, aggressive, erosive, systemic-onset juvenile idiopathic arthritis. ( 28293458 )
2017
7
Assessment of vascular function in systemic onset juvenile idiopathic arthritis. ( 27075461 )
2016
8
Systemic-onset juvenile idiopathic arthritis or incomplete Kawasaki disease: a diagnostic challenge. ( 27782865 )
2016
9
IL-6 blockers in systemic onset juvenile idiopathic arthritis. ( 26642378 )
2016
10
Systemic-onset juvenile idiopathic arthritis. ( 27392503 )
2016
11
Systemic onset juvenile idiopathic arthritis and exposure to fine particulate air pollution. ( 27607024 )
2016
12
Should 2016 Criteria for Macrophage Activation Syndrome be applied in children with Kawasaki disease, as well as with systemic-onset juvenile idiopathic arthritis? ( 26965982 )
2016
13
Operative stabilization of the remaining mobile segment in ankylosed cervical spine in systemic onset - juvenile idiopathic arthritis: A case report. ( 27458558 )
2016
14
Successful treatment with tocilizumab every 4A weeks of a low disease activity group who achieve a drug-free remission in patients with systemic-onset juvenile idiopathic arthritis. ( 25685108 )
2015
15
Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature. ( 25469247 )
2015
16
A safety evaluation of canakinumab for the treatment of systemic onset juvenile idiopathic arthritis. ( 26568054 )
2015
17
Effect of etanercept on refractory systemic-onset juvenile idiopathic arthritis. ( 25754755 )
2015
18
Potential Effects of Interleukins on the Pathogenesis of Systemic Onset Juvenile Idiopathic Arthritis. ( 26810447 )
2015
19
Diagnosis of Systemic-Onset Juvenile Idiopathic Arthritis after Treatment for Presumed Kawasaki Disease. ( 25771391 )
2015
20
Bullous Erythema Nodosum Leprosum masquerading as systemic onset juvenile idiopathic arthritis: A case report. ( 26964436 )
2015
21
Successful treatment of macrophage activation syndrome due to systemic onset juvenile idiopathic arthritis with antithymocyte globulin. ( 26210998 )
2015
22
Myositis as a presenting feature of systemic onset juvenile idiopathic arthritis. ( 25677488 )
2015
23
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease. ( 27411422 )
2015
24
Atopy in children with systemic onset juvenile idiopathic arthritis (SoJIA) is associated with a worse outcome. ( 24611751 )
2014
25
A130: Is the CCR5-delta32 Mutation Protective Against Systemic-Onset Juvenile Idiopathic Arthritis? ( 24677884 )
2014
26
MEFV mutations in egyptian children with systemic-onset juvenile idiopathic arthritis. ( 24862656 )
2014
27
Discrepancy between clinical and radiological responses to tocilizumab treatment in patients with systemic-onset juvenile idiopathic arthritis. ( 24786929 )
2014
28
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
29
Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis. ( 24417800 )
2014
30
Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis. ( 24793086 )
2014
31
AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. ( 23664546 )
2013
32
Thalidomide for systemic onset juvenile idiopathic arthritis. ( 23474929 )
2013
33
Incomplete Kawasaki disease followed by systemic onset juvenile idiopathic arthritis- the diagnostic dilemma. ( 23054859 )
2013
34
Systemic-onset juvenile idiopathic arthritis and HLA-B27 juvenile-onset undifferentiated spondyloarthritis in the same patient. ( 23295075 )
2013
35
Congestive heart failure: an uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). ( 22161583 )
2013
36
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. ( 21132551 )
2013
37
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. ( 24315465 )
2013
38
Anakinra pharmacokinetics in children and adolescents with systemic-onset juvenile idiopathic arthritis and autoinflammatory syndromes. ( 23915458 )
2013
39
Systemic-onset juvenile idiopathic arthritis complicated by early onset amyloidosis in a patient carrying a mutation in the MEFV gene. ( 20044784 )
2012
40
Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India. ( 21221591 )
2012
41
Development of germinoma during the treatment of systemic-onset juvenile idiopathic arthritis with infliximab. ( 22072226 )
2012
42
Anakinra: a safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA). ( 22083619 )
2012
43
Initial manifestations and clinical course of systemic onset juvenile idiopathic arthritis: a ten-year retrospective study. ( 23089689 )
2012
44
Recurrent cardiac tamponade in a child with newly diagnosed systemic-onset juvenile idiopathic arthritis. ( 22955481 )
2012
45
Leflunomide in systemic onset Juvenile idiopathic arthritis. ( 23024081 )
2012
46
Pure red cell aplasia in systemic onset juvenile idiopathic arthritis. ( 23448798 )
2012
47
The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis. ( 22267331 )
2012
48
ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis. ( 22189036 )
2012
49
Fatal myocarditis in a child with systemic onset juvenile idiopathic arthritis during treatment with an interleukin 1 receptor antagonist. ( 22490470 )
2012
50
Acute liver failure in young children with systemic-onset juvenile idiopathic arthritis without macrophage activation syndrome: report of two cases. ( 22050445 )
2012

Variations for Systemic Onset Juvenile Idiopathic Arthritis

Expression for Systemic Onset Juvenile Idiopathic Arthritis

LifeMap Discovery
Genes differentially expressed in tissues of Systemic Onset Juvenile Idiopathic Arthritis patients vs. healthy controls: 35
id Gene Description Tissue Up/Dn Fold Change (log2) P value
1 PRTN3 proteinase 3 Blood + 3.61 0.000
2 AZU1 azurocidin 1 Blood + 3.54 0.000
3 MPO myeloperoxidase Blood + 3.34 0.000
4 ELANE elastase, neutrophil expressed Blood + 3.31 0.000
5 DEFA4 defensin, alpha 4, corticostatin Blood + 3.24 0.000
6 GPER1 G protein-coupled estrogen receptor 1 Blood + 3.07 0.000
Search GEO for disease gene expression data for Systemic Onset Juvenile Idiopathic Arthritis.

Pathways for Systemic Onset Juvenile Idiopathic Arthritis

Pathways related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

(show all 37)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.69 IL18 IL1B MEFV TNF
2
Show member pathways
12.58 IL1B PRF1 TNF
3
Show member pathways
12.53 IL18 IL1B TNF
4
Show member pathways
12.46 IL18 IL1B TNF
5
Show member pathways
12.34 IL18 IL1B TNF
6
Show member pathways
12.33 IL18 IL1B TNF
7 11.99 IL18 IL1B TNF
8
Show member pathways
11.92 IL18 IL1B TNF
9 11.76 IL18 IL1B TNF
10
Show member pathways
11.7 IL18 IL1B TNF
11 11.61 IL18 IL1B
12 11.61 IL18 IL1B TNF
13 11.55 IL1B TNF
14 11.54 IL1B TNF
15 11.54 IL18 IL1B TNF
16 11.45 IL1B TNF
17
Show member pathways
11.45 IL1B TNF
18
Show member pathways
11.43 PRF1 TNF
19 11.35 IL18 IL1B TNF
20 11.32 IL1B TNF
21 11.29 IL1B TNF
22 11.27 IL18 IL1B TNF
23
Show member pathways
11.26 IL1B TNF
24 11.23 IL18 IL1B TNF
25 11.18 IL18 IL1B TNF
26 11.16 IL1B TNF
27 11.1 IL1B TNF
28 11.08 IL1B TNF
29 11.05 IL18 IL1B MEFV
30 11.04 IL18 PRF1
31 10.98 IL18 IL1B
32 10.98 IL18 IL1B TNF
33 10.88 IL18 IL1B TNF
34 10.77 IL1B TNF
35 10.64 IL18 IL1B TNF
36 10.36 IL18 IL1B
37 10.14 IL18 IL1B TNF

GO Terms for Systemic Onset Juvenile Idiopathic Arthritis

Biological processes related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

(show all 28)
id Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.81 IL18 IL1B TNF
2 MAPK cascade GO:0000165 9.75 IL18 IL1B TNF
3 inflammatory response GO:0006954 9.71 IL18 IL1B MEFV TNF
4 positive regulation of JNK cascade GO:0046330 9.64 IL1B TNF
5 regulation of insulin secretion GO:0050796 9.63 IL1B TNF
6 positive regulation of smooth muscle cell proliferation GO:0048661 9.62 IL18 TNF
7 positive regulation of interleukin-6 production GO:0032755 9.62 IL1B TNF
8 positive regulation of gene expression GO:0010628 9.61 IL1B TNF
9 positive regulation of nitric oxide biosynthetic process GO:0045429 9.61 IL1B TNF
10 negative regulation of extrinsic apoptotic signaling pathway in absence of ligand GO:2001240 9.6 IL1B TNF
11 protein kinase B signaling GO:0043491 9.59 IL1B TNF
12 positive regulation of phagocytosis GO:0050766 9.58 IL1B TNF
13 positive regulation of interleukin-8 production GO:0032757 9.57 IL1B TNF
14 negative regulation of myoblast differentiation GO:0045662 9.56 IL18 TNF
15 negative regulation of lipid catabolic process GO:0050995 9.55 IL1B TNF
16 positive regulation of membrane protein ectodomain proteolysis GO:0051044 9.54 IL1B TNF
17 cellular response to organic cyclic compound GO:0071407 9.54 IL18 IL1B TNF
18 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.52 IL1B TNF
19 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.51 IL1B TNF
20 positive regulation of granulocyte macrophage colony-stimulating factor production GO:0032725 9.49 IL18 IL1B
21 positive regulation of chemokine biosynthetic process GO:0045080 9.48 IL1B TNF
22 regulation of establishment of endothelial barrier GO:1903140 9.46 IL1B TNF
23 positive regulation of fever generation GO:0031622 9.43 IL1B TNF
24 positive regulation of interferon-gamma production GO:0032729 9.33 IL18 IL1B TNF
25 sequestering of triglyceride GO:0030730 9.32 IL1B TNF
26 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.26 IL1B TNF
27 lipopolysaccharide-mediated signaling pathway GO:0031663 9.13 IL18 IL1B TNF
28 positive regulation of NF-kappaB import into nucleus GO:0042346 8.8 IL18 IL1B TNF

Molecular functions related to Systemic Onset Juvenile Idiopathic Arthritis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 8.8 IL18 IL1B TNF

Sources for Systemic Onset Juvenile Idiopathic Arthritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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