MCID: SYS034
MIFTS: 53

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 49
Systemic Onset Juvenile Rheumatoid Arthritis 49
Systemic Onset Juvenile Chronic Arthritis 69
Systemic Juvenile Idiopathic Arthritis 49

Classifications:



External Ids:

UMLS 69 C1384600

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

NIH Rare Diseases : 49 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 macrophage activation syndrome and arthritis. 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 Methotrexate 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 184)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 32.6 PRF1 TNF
2 arthritis 30.8 IL18 IL1B TNF
3 hemophagocytic lymphohistiocytosis 29.5 IL18 PRF1 TNF
4 rheumatoid arthritis 29.1 IL18 IL1B TNF
5 adult-onset still's disease 28.7 IL18 IL1B MEFV TNF
6 rheumatoid arthritis, systemic juvenile 12.1
7 kawasaki disease 10.4
8 autoinflammation, panniculitis, and dermatosis syndrome 10.3 MEFV TNF
9 erysipelas 10.3 MEFV TNF
10 relapsing fever 10.3 MEFV TNF
11 listeria meningitis 10.3 IL18 TNF
12 periodic fever, familial, autosomal dominant 10.3 MEFV TNF
13 inflammatory bowel disease 3 10.2 IL18 TNF
14 pleural tuberculosis 10.2 IL18 TNF
15 pyoderma gangrenosum 10.2 MEFV TNF
16 pyoderma 10.2 MEFV TNF
17 leukomalacia 10.2 IL18 TNF
18 fasciitis 10.2 IL18 TNF
19 rasmussen encephalitis 10.2 IL18 TNF
20 pleurisy 10.2 MEFV TNF
21 acute graft versus host disease 10.2 IL18 TNF
22 cutaneous lupus erythematosus 10.2 IL18 TNF
23 paracoccidioidomycosis 10.2 IL18 TNF
24 melioidosis 10.2 IL18 TNF
25 smallpox 10.2 IL18 TNF
26 pulmonary sarcoidosis 10.2 IL18 TNF
27 listeriosis 10.2 PRF1 TNF
28 bacteriuria 10.1 IL18 PRF1
29 amyloidosis 10.1
30 plasmodium falciparum malaria 10.1 IL18 TNF
31 relapsing-remitting multiple sclerosis 10.1 IL18 TNF
32 polyposis, gastric 10.0 IL1B TNF
33 staphylococcal toxic shock syndrome 10.0 IL1B TNF
34 kashin-beck disease 10.0 IL1B TNF
35 vulvar vestibulitis syndrome 10.0 IL1B TNF
36 burns 10.0 IL1B TNF
37 laryngitis 10.0 IL1B TNF
38 cerebral artery occlusion 10.0 IL1B TNF
39 haemophilus influenzae 10.0 IL1B TNF
40 retinitis pigmentosa 60 10.0 IL1B TNF
41 acute vascular insufficiency of intestine 10.0 IL1B TNF
42 actinic prurigo 10.0 IL1B TNF
43 poems syndrome 10.0 IL1B TNF
44 irritant dermatitis 10.0 IL1B TNF
45 crohn's colitis 10.0 IL1B TNF
46 burning mouth syndrome 10.0 IL1B TNF
47 central nervous system vasculitis 10.0 IL1B TNF
48 dental pulp disease 10.0 IL1B TNF
49 osteosclerotic myeloma 10.0 IL1B TNF
50 jejunoileitis 10.0 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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.55 IL18 IL1B MEFV PRF1 TNF
2 immune system MP:0005387 9.35 IL18 IL1B MEFV PRF1 TNF
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 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
3
Alendronate Approved Phase 2, Phase 3 121268-17-5, 66376-36-1 2088
4
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
5
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
6
Tofacitinib Approved, Investigational Phase 3 477600-75-2
7
Etanercept Approved, Investigational Phase 3 185243-69-0
8
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
9 tannic acid Approved, Nutraceutical Phase 3
10
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
11 Analgesics Phase 3
12 glucocorticoids Phase 2, Phase 3
13 Folic Acid Antagonists Phase 3
14 Analgesics, Non-Narcotic Phase 3
15 Nucleic Acid Synthesis Inhibitors Phase 3
16 Dermatologic Agents Phase 3
17 Vitamin B Complex Phase 3
18 Peripheral Nervous System Agents Phase 3
19 Antibodies, Monoclonal Phase 3,Phase 1,Phase 2
20 Pharmaceutical Solutions Phase 3,Phase 2,Phase 1
21 Immunoglobulins Phase 3,Phase 1,Phase 2
22 Immunosuppressive Agents Phase 3
23 Antimetabolites Phase 3
24 Anti-Inflammatory Agents Phase 3
25 Antimetabolites, Antineoplastic Phase 3
26 Interleukin 1 Receptor Antagonist Protein Phase 2, Phase 3
27 Anti-Inflammatory Agents, Non-Steroidal Phase 3
28 Antibodies Phase 3,Phase 1,Phase 2
29 Bone Density Conservation Agents Phase 2, Phase 3
30 Antirheumatic Agents Phase 2, Phase 3
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
37 interferons Phase 2
38 Interferon-gamma Phase 2

Interventional clinical trials:

(show all 35)

# Name Status NCT ID Phase Drugs
1 A Study of Tocilizumab in Chinese Participants With Systemic Juvenile Idiopathic Arthritis (sJIA) Recruiting NCT03301883 Phase 4 Tocilizumab;NSAIDs;CSs;MTX
2 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
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 ß-SPECIFIC 4 Patients: Study of Pediatric EffiCacy and Safety wIth FIrst-line Use of Canakinumab Completed NCT02296424 Phase 3 ACZ885 150 mg (Canakinumab)
10 Alendronate Osteoporosis Study Completed NCT00277251 Phase 2, Phase 3 Alendronate
11 Study of MRA for Polyarticular Juvenile Idiopathic Arthritis (pJIA) Completed NCT00144664 Phase 3 MRA(Tocilizumab)
12 A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD) Recruiting NCT03265132 Phase 3 Placebo
13 A Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis or Hereditary Periodic Fevers Who Participated in the CACZ885G2301E1, CACZ885G2306 or CACZ885N2301 Studies Active, not recruiting NCT02334748 Phase 3 canakinumab
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 Safety and Effectiveness of Rilonacept for Treating Systemic Juvenile Idiopathic Arthritis in Children and Young Adults Completed NCT00534495 Phase 2
20 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
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 Tocilizumab in Patients With Schnitzler's Syndrome Recruiting NCT03046381 Phase 2 Tocilizumab
25 A Repeated Dose-finding Study of Sarilumab in Children and Adolescents With Systemic Juvenile Idiopathic Arthritis (sJIA) Suspended NCT02991469 Phase 2 Sarilumab SAR153191 (REGN88)
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 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
31 Extension Study Evaluating the Safety and Efficacy of Subcutaneous Tocilizumab (RoActemra/Actemra) Administration in Systemic and Polyarticular-Course Juvenile Idiopathic Arthritis Active, not recruiting NCT02165345 Phase 1 Tocilizumab
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:

38
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 123)
# Title Authors Year
1
Neutrophils in systemic onset Juvenile Idiopathic Arthritis display sepsis-like features which can be reverted by IL-1 blockade. ( 29426067 )
2018
2
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
3
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
4
Canakinumab for first line steroid-free treatment in a child with systemic-onset juvenile idiopathic arthritis. ( 28266235 )
2017
5
A Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack. ( 28416939 )
2017
6
Systemic-onset juvenile idiopathic arthritis and incomplete Kawasaki disease may belong to a single clinical syndrome within a spectrum of severity. ( 28980895 )
2017
7
Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis. ( 28229369 )
2017
8
Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases. ( 28225869 )
2017
9
Reasons for inactive disease and flare in systemic onset juvenile idiopathic arthritis patients during tocilizumab treatment. ( 29303703 )
2017
10
Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry. ( 29166924 )
2017
11
Systemic-onset juvenile idiopathic arthritis or incomplete Kawasaki disease: a diagnostic challenge. ( 27782865 )
2016
12
Assessment of vascular function in systemic onset juvenile idiopathic arthritis. ( 27075461 )
2016
13
IL-6 blockers in systemic onset juvenile idiopathic arthritis. ( 26642378 )
2016
14
Systemic onset juvenile idiopathic arthritis and exposure to fine particulate air pollution. ( 27607024 )
2016
15
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
16
Systemic-onset juvenile idiopathic arthritis. ( 27392503 )
2016
17
Operative stabilization of the remaining mobile segment in ankylosed cervical spine in systemic onset - juvenile idiopathic arthritis: A case report. ( 27458558 )
2016
18
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
19
Diagnosis of Systemic-Onset Juvenile Idiopathic Arthritis after Treatment for Presumed Kawasaki Disease. ( 25771391 )
2015
20
Myositis as a presenting feature of systemic onset juvenile idiopathic arthritis. ( 25677488 )
2015
21
Bullous Erythema Nodosum Leprosum masquerading as systemic onset juvenile idiopathic arthritis: A case report. ( 26964436 )
2015
22
Successful treatment of macrophage activation syndrome due to systemic onset juvenile idiopathic arthritis with antithymocyte globulin. ( 26210998 )
2015
23
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease. ( 27411422 )
2015
24
Potential Effects of Interleukins on the Pathogenesis of Systemic Onset Juvenile Idiopathic Arthritis. ( 26810447 )
2015
25
A safety evaluation of canakinumab for the treatment of systemic onset juvenile idiopathic arthritis. ( 26568054 )
2015
26
Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature. ( 25469247 )
2015
27
Effect of etanercept on refractory systemic-onset juvenile idiopathic arthritis. ( 25754755 )
2015
28
MEFV mutations in egyptian children with systemic-onset juvenile idiopathic arthritis. ( 24862656 )
2014
29
Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis. ( 24793086 )
2014
30
Atopy in children with systemic onset juvenile idiopathic arthritis (SoJIA) is associated with a worse outcome. ( 24611751 )
2014
31
Discrepancy between clinical and radiological responses to tocilizumab treatment in patients with systemic-onset juvenile idiopathic arthritis. ( 24786929 )
2014
32
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
33
Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis. ( 24417800 )
2014
34
A130: Is the CCR5-delta32 Mutation Protective Against Systemic-Onset Juvenile Idiopathic Arthritis? ( 24677884 )
2014
35
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. ( 21132551 )
2013
36
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. ( 24315465 )
2013
37
Incomplete Kawasaki disease followed by systemic onset juvenile idiopathic arthritis- the diagnostic dilemma. ( 23054859 )
2013
38
Congestive heart failure: an uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). ( 22161583 )
2013
39
AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. ( 23664546 )
2013
40
Anakinra pharmacokinetics in children and adolescents with systemic-onset juvenile idiopathic arthritis and autoinflammatory syndromes. ( 23915458 )
2013
41
Thalidomide for systemic onset juvenile idiopathic arthritis. ( 23474929 )
2013
42
Systemic-onset juvenile idiopathic arthritis and HLA-B27 juvenile-onset undifferentiated spondyloarthritis in the same patient. ( 23295075 )
2013
43
Fatal myocarditis in a child with systemic onset juvenile idiopathic arthritis during treatment with an interleukin 1 receptor antagonist. ( 22490470 )
2012
44
Anakinra: a safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA). ( 22083619 )
2012
45
ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis. ( 22189036 )
2012
46
Leflunomide in systemic onset Juvenile idiopathic arthritis. ( 23024081 )
2012
47
Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India. ( 21221591 )
2012
48
Acute liver failure in young children with systemic-onset juvenile idiopathic arthritis without macrophage activation syndrome: report of two cases. ( 22050445 )
2012
49
Safety and response to influenza vaccine in patients with systemic-onset juvenile idiopathic arthritis receiving tocilizumab. ( 22322589 )
2012
50
Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease. ( 22573189 )
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
# 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 38)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.67 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
12.45 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
11.69 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 10.75 IL18 IL1B TNF
36 10.64 IL18 IL1B TNF
37 10.36 IL18 IL1B
38 10.14 IL18 IL1B TNF

GO Terms for Systemic Onset Juvenile Idiopathic Arthritis

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

# Name GO ID Score Top Affiliating Genes
1 autophagosome GO:0005776 8.62 IL1B MEFV

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

(show all 30)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.82 IL18 IL1B TNF
2 regulation of receptor activity GO:0010469 9.81 IL18 IL1B TNF
3 positive regulation of gene expression GO:0010628 9.8 IL18 IL1B TNF
4 MAPK cascade GO:0000165 9.76 IL18 IL1B TNF
5 inflammatory response GO:0006954 9.71 IL18 IL1B MEFV TNF
6 positive regulation of JNK cascade GO:0046330 9.65 IL1B TNF
7 regulation of insulin secretion GO:0050796 9.64 IL1B TNF
8 positive regulation of smooth muscle cell proliferation GO:0048661 9.63 IL18 TNF
9 positive regulation of interleukin-6 production GO:0032755 9.62 IL1B TNF
10 protein kinase B signaling GO:0043491 9.61 IL1B TNF
11 positive regulation of nitric oxide biosynthetic process GO:0045429 9.61 IL1B TNF
12 negative regulation of extrinsic apoptotic signaling pathway in absence of ligand GO:2001240 9.6 IL1B TNF
13 positive regulation of phagocytosis GO:0050766 9.59 IL1B TNF
14 positive regulation of interleukin-8 production GO:0032757 9.58 IL1B TNF
15 negative regulation of myoblast differentiation GO:0045662 9.57 IL18 TNF
16 negative regulation of lipid catabolic process GO:0050995 9.56 IL1B TNF
17 positive regulation of membrane protein ectodomain proteolysis GO:0051044 9.55 IL1B TNF
18 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.54 IL1B TNF
19 cellular response to organic cyclic compound GO:0071407 9.54 IL18 IL1B TNF
20 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.52 IL1B TNF
21 positive regulation of granulocyte macrophage colony-stimulating factor production GO:0032725 9.51 IL18 IL1B
22 positive regulation of chemokine biosynthetic process GO:0045080 9.49 IL1B TNF
23 regulation of establishment of endothelial barrier GO:1903140 9.48 IL1B TNF
24 interleukin-6 production GO:0032635 9.46 IL18 IL1B
25 positive regulation of fever generation GO:0031622 9.43 IL1B TNF
26 positive regulation of interferon-gamma production GO:0032729 9.33 IL18 IL1B TNF
27 sequestering of triglyceride GO:0030730 9.32 IL1B TNF
28 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.26 IL1B TNF
29 lipopolysaccharide-mediated signaling pathway GO:0031663 9.13 IL18 IL1B TNF
30 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:

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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