MCID: SYS034
MIFTS: 49

Systemic Onset Juvenile Idiopathic Arthritis

Categories: Bone diseases, Rare diseases

Aliases & Classifications for Systemic Onset Juvenile Idiopathic Arthritis

MalaCards integrated aliases for Systemic Onset Juvenile Idiopathic Arthritis:

Name: Systemic Onset Juvenile Idiopathic Arthritis 53
Systemic Onset Juvenile Rheumatoid Arthritis 53
Systemic Onset Juvenile Chronic Arthritis 73
Systemic Juvenile Idiopathic Arthritis 53

Classifications:



External Ids:

UMLS 73 C1384600

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

NIH Rare Diseases : 53 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.

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 Adipogenesis and Spinal Cord Injury. The drugs Liver Extracts and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include bone, testes and neutrophil, and related phenotypes are cellular and hematopoietic 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 112)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 32.7 PRF1 TNF
2 arthritis 31.3 IL1RN MIF TNF
3 juvenile rheumatoid arthritis 30.6 IL1RN TNF
4 vasculitis 29.9 MEFV TNF
5 periodic fever, familial, autosomal dominant 29.8 MEFV TNF
6 blau syndrome 29.8 MEFV TNF
7 peritonitis 29.7 MEFV TNF
8 hemophagocytic lymphohistiocytosis 29.7 PRF1 TNF
9 rheumatoid arthritis 29.7 IL1RN MIF TNF
10 adult-onset still's disease 29.5 IL1RN MEFV MIF TNF
11 pericarditis 29.5 IL1RN MEFV TNF
12 familial mediterranean fever 29.5 IL1RN MEFV TNF
13 inflammatory bowel disease 29.4 IL1RN MIF TNF
14 rheumatoid arthritis, systemic juvenile 11.9
15 kawasaki disease 10.5
16 amyloidosis 10.2
17 cardiac tamponade 10.1
18 spondylitis 10.1 IL1RN TNF
19 scleral disease 10.1 IL1RN TNF
20 pustulosis of palm and sole 10.1 IL1RN TNF
21 vulvar vestibulitis syndrome 10.1 IL1RN TNF
22 osteosclerotic myeloma 10.1 IL1RN TNF
23 listeriosis 10.1 PRF1 TNF
24 aseptic meningitis 10.1 IL1RN TNF
25 joint disorders 10.1 IL1RN TNF
26 hypersensitivity reaction type iv disease 10.1 IL1RN TNF
27 dengue shock syndrome 10.1 IL1RN TNF
28 exanthem 10.1 IL1RN TNF
29 idiopathic neutropenia 10.1 IL1RN TNF
30 bacterial vaginosis 10.1 PRF1 TNF
31 peptic ulcer disease 10.1 IL1RN TNF
32 temporal arteritis 10.1 IL1RN TNF
33 silicosis 10.1 IL1RN TNF
34 atrophic gastritis 10.1 IL1RN TNF
35 aggressive periodontitis 10.1 IL1RN TNF
36 rheumatoid vasculitis 10.1 MIF TNF
37 duodenal ulcer 10.1 IL1RN TNF
38 chorioamnionitis 10.1 IL1RN TNF
39 oligoarticular juvenile idiopathic arthritis 10.0 MIF TNF
40 osteomyelitis 10.0 IL1RN TNF
41 bone inflammation disease 10.0 IL1RN TNF
42 lepromatous leprosy 10.0 MIF TNF
43 alopecia areata 10.0 IL1RN TNF
44 synovitis 10.0 IL1RN TNF
45 plasmodium falciparum malaria 10.0 MIF TNF
46 proteasome-associated autoinflammatory syndrome 1 10.0 IL1RN TNF
47 stomatitis 10.0 MEFV TNF
48 autoinflammation, panniculitis, and dermatosis syndrome 10.0 MEFV TNF
49 torticollis 10.0
50 diabetes mellitus 10.0

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.77 IL1RN MEFV MIF PRF1 TNF
2 hematopoietic system MP:0005397 9.72 IL1RN MEFV MIF PRF1 TNF
3 homeostasis/metabolism MP:0005376 9.65 IL1RN MEFV MIF PRF1 TNF
4 immune system MP:0005387 9.55 IL1RN MEFV MIF PRF1 TNF
5 integument MP:0010771 9.26 IL1RN MEFV PRF1 TNF
6 reproductive system MP:0005389 8.92 IL1RN 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 39)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
3 tannic acid Approved Phase 3
4
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
5
leucovorin Approved Phase 3 58-05-9 6006 143
6
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
7
Alendronate Approved Phase 2, Phase 3 66376-36-1, 121268-17-5 2088
8
Tofacitinib Approved, Investigational Phase 3 477600-75-2
9
Etanercept Approved, Investigational Phase 3 185243-69-0
10
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
11 Antirheumatic Agents Phase 2, Phase 3,Phase 3
12 Interleukin 1 Receptor Antagonist Protein Phase 2, Phase 3,Phase 3
13 Antibodies Phase 3,Phase 1,Phase 2,Not Applicable
14 Immunoglobulins Phase 3,Phase 1,Phase 2,Not Applicable
15 Immunologic Factors Phase 3,Phase 1,Phase 2
16 Antibodies, Monoclonal Phase 3,Phase 1,Phase 2,Not Applicable
17 Folate Phase 3
18 Anti-Inflammatory Agents, Non-Steroidal Phase 3
19 Anti-Inflammatory Agents Phase 3
20 Folic Acid Antagonists Phase 3
21 Vitamin B Complex Phase 3
22 Dermatologic Agents Phase 3
23 Peripheral Nervous System Agents Phase 3
24 Analgesics Phase 3
25 Nucleic Acid Synthesis Inhibitors Phase 3
26 Immunosuppressive Agents Phase 3
27 Antimetabolites Phase 3
28 Analgesics, Non-Narcotic Phase 3
29 Antimetabolites, Antineoplastic Phase 3
30 Vitamin B9 Phase 3
31 Bone Density Conservation Agents Phase 2, Phase 3
32 glucocorticoids Phase 2, Phase 3
33 Pharmaceutical Solutions Phase 3,Phase 2,Phase 1
34 Protein Kinase Inhibitors Phase 3
35 Gastrointestinal Agents Phase 3
36 Givinostat hydrochloride Phase 2
37 Histone Deacetylase Inhibitors Phase 2
38 Interferon-gamma Phase 2
39 interferons Phase 2

Interventional clinical trials:

(show all 37)
# 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) Active, not 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 Without Fever. 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 Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis or Hereditary Periodic Fevers Who Participated in the CACZ885G2301E1, CACZ885G2306 or CACZ885N2301 Studies Completed NCT02334748 Phase 3 canakinumab
7 Study of Efficacy and Safety of Canakinumab in Japanese Patients With SJIA Completed NCT02396212 Phase 3
8 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
9 Study of MRA for Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT00144599 Phase 3 MRA(Tocilizumab);placebo
10 Long-term Treatment Study of MRA for Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT00144612 Phase 3 MRA(Tocilizumab)
11 ß-SPECIFIC 4 Patients: Study of Pediatric EffiCacy and Safety wIth FIrst-line Use of Canakinumab Completed NCT02296424 Phase 3 ACZ885 150 mg (Canakinumab)
12 Alendronate Osteoporosis Study Completed NCT00277251 Phase 2, Phase 3 Alendronate
13 Study of MRA for Polyarticular Juvenile Idiopathic Arthritis (pJIA) Completed NCT00144664 Phase 3 MRA(Tocilizumab)
14 A Safety, Efficacy And Pharmacokinetics Study Of Tofacitinib In Pediatric Patients With sJIA Recruiting NCT03000439 Phase 3 In open-label phase: treatment with tofacitinib;In double-blind phase: treatment with tofacitinib or placebo in 1:1 ratio
15 A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD) Recruiting NCT03265132 Phase 3 Placebo
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 Extension Study to WA19977 in Patients With Active Polyarticular-Course Juvenile Idiopathic Arthritis Terminated NCT01575769 Phase 3 RoActemra/Actemra (tocilizumab)
19 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
20 Safety and Effectiveness of Rilonacept for Treating Systemic Juvenile Idiopathic Arthritis in Children and Young Adults Completed NCT00534495 Phase 2
21 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
22 Safety, Efficacy and Pharmacokinetics of Subcutaneous ACZ885 in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT00426218 Phase 1, Phase 2 ACZ885
23 Ilaris (Canakinumab) in the Schnitzler Syndrome Completed NCT01245127 Phase 2 Ilaris
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 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
26 Tocilizumab in Patients With Schnitzler's Syndrome Recruiting NCT03046381 Phase 2 Tocilizumab
27 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)
28 A Study of Subcutaneously Administered Tocilizumab in Participants With Systemic Juvenile Idiopathic Arthritis Completed NCT01904292 Phase 1 Tocilizumab
29 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
30 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
31 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
32 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
33 New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
34 Ultrasonography in Juvenile Idiopathic Arthritis Completed NCT01304420
35 The CARRA Registry Completed NCT01697254
36 Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still's Disease, and Related Conditions Recruiting NCT03510442
37 Single Patient Use of Tocilizumab in Systemic Onset Juvenile Idiopathic Arthritis Terminated NCT00868751 Not Applicable

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:

41
Bone, Testes, Neutrophil, Liver, Heart, Lung, Lymph Node

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 182)
# Title Authors Year
1
Remission of Refractory Systemic-Onset Juvenile Idiopathic Arthritis After Treatment With Siltuximab. ( 29485546 )
2018
2
Neutrophils in systemic onset Juvenile Idiopathic Arthritis display sepsis-like features which can be reverted by IL-1 blockade. ( 29426067 )
2018
3
Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience. ( 30505008 )
2018
4
Methotrexate-Induced Vasculitis in a Child with Systemic Onset Juvenile Idiopathic Arthritis. ( 30431484 )
2018
5
Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis. ( 30238379 )
2018
6
A Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack. ( 28416939 )
2017
7
Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases. ( 28225869 )
2017
8
Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry. ( 29166924 )
2017
9
Reasons for inactive disease and flare in systemic onset juvenile idiopathic arthritis patients during tocilizumab treatment. ( 29303703 )
2017
10
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
11
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
12
Canakinumab for first line steroid-free treatment in a child with systemic-onset juvenile idiopathic arthritis. ( 28266235 )
2017
13
Systemic-onset juvenile idiopathic arthritis and incomplete Kawasaki disease may belong to a single clinical syndrome within a spectrum of severity. ( 28980895 )
2017
14
Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis. ( 28229369 )
2017
15
Systemic-onset juvenile idiopathic arthritis. ( 27392503 )
2016
16
Systemic-onset juvenile idiopathic arthritis or incomplete Kawasaki disease: a diagnostic challenge. ( 27782865 )
2016
17
Systemic onset juvenile idiopathic arthritis and exposure to fine particulate air pollution. ( 27607024 )
2016
18
Operative stabilization of the remaining mobile segment in ankylosed cervical spine in systemic onset - juvenile idiopathic arthritis: A case report. ( 27458558 )
2016
19
Assessment of vascular function in systemic onset juvenile idiopathic arthritis. ( 27075461 )
2016
20
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
21
IL-6 blockers in systemic onset juvenile idiopathic arthritis. ( 26642378 )
2016
22
Myositis as a presenting feature of systemic onset juvenile idiopathic arthritis. ( 25677488 )
2015
23
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
24
A safety evaluation of canakinumab for the treatment of systemic onset juvenile idiopathic arthritis. ( 26568054 )
2015
25
Bullous Erythema Nodosum Leprosum masquerading as systemic onset juvenile idiopathic arthritis: A case report. ( 26964436 )
2015
26
Diagnosis of Systemic-Onset Juvenile Idiopathic Arthritis after Treatment for Presumed Kawasaki Disease. ( 25771391 )
2015
27
Potential Effects of Interleukins on the Pathogenesis of Systemic Onset Juvenile Idiopathic Arthritis. ( 26810447 )
2015
28
Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature. ( 25469247 )
2015
29
Successful treatment of macrophage activation syndrome due to systemic onset juvenile idiopathic arthritis with antithymocyte globulin. ( 26210998 )
2015
30
Effect of etanercept on refractory systemic-onset juvenile idiopathic arthritis. ( 25754755 )
2015
31
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease. ( 27411422 )
2015
32
Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis. ( 24793086 )
2014
33
Discrepancy between clinical and radiological responses to tocilizumab treatment in patients with systemic-onset juvenile idiopathic arthritis. ( 24786929 )
2014
34
A130: Is the CCR5-delta32 Mutation Protective Against Systemic-Onset Juvenile Idiopathic Arthritis? ( 24677884 )
2014
35
MEFV mutations in egyptian children with systemic-onset juvenile idiopathic arthritis. ( 24862656 )
2014
36
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
37
Atopy in children with systemic onset juvenile idiopathic arthritis (SoJIA) is associated with a worse outcome. ( 24611751 )
2014
38
Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis. ( 24417800 )
2014
39
Congestive heart failure: an uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). ( 22161583 )
2013
40
AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. ( 23664546 )
2013
41
Anakinra pharmacokinetics in children and adolescents with systemic-onset juvenile idiopathic arthritis and autoinflammatory syndromes. ( 23915458 )
2013
42
Incomplete Kawasaki disease followed by systemic onset juvenile idiopathic arthritis- the diagnostic dilemma. ( 23054859 )
2013
43
Systemic-onset juvenile idiopathic arthritis and HLA-B27 juvenile-onset undifferentiated spondyloarthritis in the same patient. ( 23295075 )
2013
44
Thalidomide for systemic onset juvenile idiopathic arthritis. ( 23474929 )
2013
45
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. ( 24315465 )
2013
46
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. ( 21132551 )
2013
47
ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis. ( 22189036 )
2012
48
Systemic-onset juvenile idiopathic arthritis complicated by early onset amyloidosis in a patient carrying a mutation in the MEFV gene. ( 20044784 )
2012
49
Initial manifestations and clinical course of systemic onset juvenile idiopathic arthritis: a ten-year retrospective study. ( 23089689 )
2012
50
Development of germinoma during the treatment of systemic-onset juvenile idiopathic arthritis with infliximab. ( 22072226 )
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:

# Super pathways Score Top Affiliating Genes
1 11.49 MIF TNF
2 11.4 MIF TNF
3 11.11 IL1RN TNF
4
Show member pathways
10.96 MIF TNF
5
Show member pathways
10.74 PRF1 TNF
6 10.31 IL1RN 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 12)
# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.58 IL1RN MIF TNF
2 positive regulation of JNK cascade GO:0046330 9.51 IL1RN TNF
3 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.49 MIF TNF
4 response to glucocorticoid GO:0051384 9.48 IL1RN TNF
5 positive regulation of MAP kinase activity GO:0043406 9.46 MIF TNF
6 positive regulation of interleukin-6 production GO:0032755 9.43 IL1RN TNF
7 positive regulation of JUN kinase activity GO:0043507 9.37 IL1RN TNF
8 positive regulation of cytokine secretion GO:0050715 9.32 MIF TNF
9 carboxylic acid metabolic process GO:0019752 9.26 IL1RN MIF
10 positive regulation of chemokine (C-X-C motif) ligand 2 production GO:2000343 9.16 MIF TNF
11 chronic inflammatory response to antigenic stimulus GO:0002439 8.96 IL1RN TNF
12 inflammatory response GO:0006954 8.92 IL1RN MEFV MIF TNF

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 MEFV MIF PRF1 TNF
2 protease binding GO:0002020 9.16 MIF TNF
3 cytokine activity GO:0005125 8.8 IL1RN MIF TNF

Sources for Systemic Onset Juvenile Idiopathic Arthritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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