MCID: SYS034
MIFTS: 51

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 54
Systemic Juvenile Idiopathic Arthritis 54 17
Systemic Onset Juvenile Rheumatoid Arthritis 54
Systemic Onset Juvenile Chronic Arthritis 74

Classifications:



External Ids:

UMLS 74 C1384600

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

NIH Rare Diseases : 54 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 juvenile idiopathic 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 Benzocaine 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 118)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 32.7 PRF1 TNF
2 arthritis 31.1 IL1RN MIF TNF
3 juvenile rheumatoid arthritis 30.7 IL1RN TNF
4 vasculitis 30.2 MEFV TNF
5 periodic fever, familial, autosomal dominant 30.1 MEFV TNF
6 blau syndrome 30.0 MEFV TNF
7 peritonitis 29.8 MEFV TNF
8 pericarditis 29.6 IL1RN MEFV TNF
9 brucellosis 29.6 IL1RN MEFV TNF
10 familial mediterranean fever 29.6 IL1RN MEFV TNF
11 hemophagocytic lymphohistiocytosis 29.4 PRF1 TNF
12 rheumatoid arthritis 29.3 IL1RN MIF TNF
13 adult-onset still's disease 29.3 IL1RN MEFV MIF TNF
14 rheumatoid arthritis, systemic juvenile 11.9
15 kawasaki disease 10.5
16 amyloidosis 10.2
17 spondylitis 10.2 IL1RN TNF
18 stomatitis 10.2 MEFV TNF
19 autoinflammation, panniculitis, and dermatosis syndrome 10.2 MEFV TNF
20 scleral disease 10.1 IL1RN TNF
21 pustulosis of palm and sole 10.1 IL1RN TNF
22 vulvar vestibulitis syndrome 10.1 IL1RN TNF
23 erysipelas 10.1 MEFV TNF
24 relapsing fever 10.1 MEFV TNF
25 osteosclerotic myeloma 10.1 IL1RN TNF
26 hypersensitivity reaction type iii disease 10.1 MEFV TNF
27 cardiac tamponade 10.1
28 aseptic meningitis 10.1 IL1RN TNF
29 pyoderma gangrenosum 10.1 MEFV TNF
30 pyoderma 10.1 MEFV TNF
31 joint disorders 10.1 IL1RN TNF
32 hypersensitivity reaction type iv disease 10.1 IL1RN TNF
33 dengue shock syndrome 10.1 IL1RN TNF
34 palindromic rheumatism 10.1 MEFV TNF
35 exanthem 10.1 IL1RN TNF
36 idiopathic neutropenia 10.1 IL1RN TNF
37 pericardium disease 10.1 IL1RN MEFV
38 pleurisy 10.1 MEFV TNF
39 peptic ulcer disease 10.1 IL1RN TNF
40 hydrarthrosis 10.1 IL1RN MEFV
41 temporal arteritis 10.1 IL1RN TNF
42 cinca syndrome 10.1 IL1RN MEFV
43 silicosis 10.1 IL1RN TNF
44 alopecia areata 10.1 IL1RN TNF
45 atrophic gastritis 10.1 IL1RN TNF
46 listeriosis 10.1 PRF1 TNF
47 aggressive periodontitis 10.1 IL1RN TNF
48 duodenal ulcer 10.1 IL1RN TNF
49 chorioamnionitis 10.1 IL1RN TNF
50 primary bacterial infectious disease 10.1 MEFV 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:

47
# 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 40)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
3
tannic acid Approved Phase 3 1401-55-4
4
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
5
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
6
leucovorin Approved Phase 3 58-05-9 6006 143
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 Immunoglobulins Phase 3,Phase 1,Phase 2,Not Applicable
14 Antibodies, Monoclonal Phase 3,Phase 1,Phase 2,Not Applicable
15 Antibodies Phase 3,Phase 1,Phase 2,Not Applicable
16 Immunologic Factors Phase 3,Phase 1,Phase 2
17 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1
18 Peripheral Nervous System Agents Phase 3
19 Antimetabolites Phase 3
20 Vitamin B Complex Phase 3
21 Anti-Inflammatory Agents, Non-Steroidal Phase 3
22 Immunosuppressive Agents Phase 3
23 Antimetabolites, Antineoplastic Phase 3
24 Nucleic Acid Synthesis Inhibitors Phase 3
25 Vitamin B9 Phase 3
26 Analgesics Phase 3
27 Folate Phase 3
28 Dermatologic Agents Phase 3
29 Folic Acid Antagonists Phase 3
30 Analgesics, Non-Narcotic 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
Rilonacept Approved, Investigational Phase 2,Phase 1 501081-76-1 104924
37 Histone Deacetylase Inhibitors Phase 2
38 Givinostat hydrochloride Phase 2
39 Interferon-gamma Phase 2
40 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:

42
Bone, Testes, Neutrophil, Monocytes, T Cells, Endothelial, Myeloid

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 406)
# Title Authors Year
1
Comparative study of Interleukin-18 (IL-18) serum levels in adult onset Still's disease (AOSD) and systemic onset juvenile idiopathic arthritis (sJIA) and its use as a biomarker for diagnosis and evaluation of disease activity. ( 30886992 )
2019
2
Serum Leucine-Rich α2-Glycoprotein as a Biomarker for Monitoring Disease Activity in Patients with Systemic Juvenile Idiopathic Arthritis. ( 30863782 )
2019
3
Early Treatment of Systemic Juvenile Idiopathic Arthritis with Canakinumab and Complete Remission After 2 Years of Treatment Suspension: Case Report of an Adolescent Girl. ( 30847869 )
2019
4
Treat-to-target using first-line recombinant interleukin-1 receptor antagonist monotherapy in new-onset systemic juvenile idiopathic arthritis: results from a five year follow-up study. ( 30848528 )
2019
5
A Bullseye for Children with Systemic Juvenile Idiopathic Arthritis. ( 30802004 )
2019
6
PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY. ( 30810692 )
2019
7
Treatment of patients with systemic-onset juvenile idiopathic arthritis with tacrolimus. ( 30783487 )
2019
8
The role of extracellular histones in systemic-onset juvenile idiopathic arthritis. ( 30642364 )
2019
9
Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis. ( 30647180 )
2019
10
Decreased PD-1 expression on circulating CD4+T cell and PD-L1 expression on myeloid dendritic cell correlate with clinical manifestations in systemic juvenile idiopathic arthritis. ( 29609005 )
2019
11
Risk factors for hypersensitivity reactions to tocilizumab introduction in systemic juvenile idiopathic arthritis. ( 29578355 )
2019
12
Remission of Refractory Systemic-Onset Juvenile Idiopathic Arthritis After Treatment With Siltuximab. ( 29485546 )
2018
13
Novel UNC13D intronic variant disrupting a NFI_B enhancer in a patient with recurrent macrophage activation syndrome and systemic juvenile idiopathic arthritis. ( 29409136 )
2018
14
Neutrophils in systemic onset Juvenile Idiopathic Arthritis display sepsis-like features which can be reverted by IL-1 blockade. ( 29426067 )
2018
15
Systemic juvenile idiopathic arthritis and macrophage activation syndrome: update on pathogenesis and treatment. ( 29870499 )
2018
16
Drug Retention Rate and Predictive Factors of Drug Survival for Interleukin-1 Inhibitors in Systemic Juvenile Idiopathic Arthritis. ( 30670972 )
2018
17
Neutrophils From Children With Systemic Juvenile Idiopathic Arthritis Exhibit Persistent Proinflammatory Activation Despite Long-Standing Clinically Inactive Disease. ( 30619348 )
2018
18
Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience. ( 30505008 )
2018
19
Methotrexate-Induced Vasculitis in a Child with Systemic Onset Juvenile Idiopathic Arthritis. ( 30431484 )
2018
20
Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience. ( 30431724 )
2018
21
No preventive or therapeutic efficacy of infliximab against macrophage activation syndrome due to systemic juvenile idiopathic arthritis. ( 30406708 )
2018
22
S100A12 and vascular endothelial growth factor can differentiate Blau syndrome and familial Mediterranean fever from systemic juvenile idiopathic arthritis. ( 30406853 )
2018
23
Transient natural killer cell dysfunction associated with interleukin-18 overproduction in systemic juvenile idiopathic arthritis. ( 30345698 )
2018
24
Plasma interleukin-37 is increased and inhibits the production of inflammatory cytokines in peripheral blood mononuclear cells in systemic juvenile idiopathic arthritis patients. ( 30305171 )
2018
25
Insufficient IL-10 Production as a Mechanism Underlying the Pathogenesis of Systemic Juvenile Idiopathic Arthritis. ( 30266771 )
2018
26
Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials. ( 30269054 )
2018
27
Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis. ( 30238379 )
2018
28
Thrombocytopaenia in Systemic Juvenile Idiopathic Arthritis: Not Always Macrophage Activation Syndrome! ( 30178590 )
2018
29
Short-term outcomes in patients with systemic juvenile idiopathic arthritis treated with either tocilizumab or anakinra. ( 30137641 )
2018
30
Systemic Juvenile Idiopathic Arthritis. ( 30031494 )
2018
31
The burden of systemic juvenile idiopathic arthritis for patients and caregivers: an international survey and retrospective chart review. ( 29600940 )
2018
32
IL1RN Variation Influences Both Disease Susceptibility and Response to Recombinant Human Interleukin-1 Receptor Antagonist Therapy in Systemic Juvenile Idiopathic Arthritis. ( 29609200 )
2018
33
IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry). ( 29622022 )
2018
34
Soluble CD163, a unique biomarker to evaluate the disease activity, exhibits macrophage activation in systemic juvenile idiopathic arthritis. ( 29801971 )
2018
35
Transcription factor motif enrichment in whole transcriptome analysis identifies STAT4 and BCL6 as the most prominent binding motif in systemic juvenile idiopathic arthritis. ( 29848367 )
2018
36
Early reduction of serum interleukin-6 levels as a predictor of clinical remission in systemic juvenile idiopathic arthritis. ( 29888930 )
2018
37
The role of IL-1 inhibition in systemic juvenile idiopathic arthritis: current status and future perspectives. ( 29922038 )
2018
38
Interleukin-1 in monocyte activation phenotypes in systemic juvenile idiopathic arthritis: Observations from a clinical trial of rilonacept, an interleukin-1 inhibitor. ( 29928998 )
2018
39
Growth Failure in Children with Systemic Juvenile Idiopathic Arthritis and Prolonged Inflammation despite Treatment with Biologicals: Late Normalization of Height by Combined Hormonal Therapies. ( 29940586 )
2018
40
Impact of systemic juvenile idiopathic arthritis/Still's disease on adolescents as evidenced through social media posts. ( 29942167 )
2018
41
Filling the Gap: Toward a Disease Activity Tool for Systemic Juvenile Idiopathic Arthritis. ( 29292315 )
2018
42
Patient characteristics associated with response to NSAID monotherapy in children with systemic juvenile idiopathic arthritis. ( 29304824 )
2018
43
MicroRNA networks associated with active systemic juvenile idiopathic arthritis regulate CD163 expression and anti-inflammatory functions in macrophages through two distinct mechanisms. ( 29345059 )
2018
44
Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany. ( 29357887 )
2018
45
Application of the international league against rheumatism classification criteria for systemic juvenile idiopathic arthritis as a prognostic factor in patients with adults-onset Still's disease. ( 29370863 )
2018
46
Efficacy and safety of canakinumab in patients with Still's disease: exposure-response analysis of pooled systemic juvenile idiopathic arthritis data by age groups. ( 29533755 )
2018
47
Bayesian comparative effectiveness study of four consensus treatment plans for initial management of systemic juvenile idiopathic arthritis: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST). ( 29542334 )
2018
48
Editorial: Toward Personalized Treatment for Systemic Juvenile Idiopathic Arthritis. ( 29569852 )
2018
49
Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. ( 29574969 )
2018
50
Proposed Core Set of Items for Measuring Disease Activity in Systemic Juvenile Idiopathic Arthritis. ( 28765240 )
2018

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 cytokine secretion GO:0050715 9.32 MIF TNF
8 positive regulation of chemokine (C-X-C motif) ligand 2 production GO:2000343 9.16 MIF TNF
9 positive regulation of JUN kinase activity GO:0043507 9.07 TNF
10 carboxylic acid metabolic process GO:0019752 8.96 MIF
11 inflammatory response GO:0006954 8.92 IL1RN MEFV MIF TNF
12 chronic inflammatory response to antigenic stimulus GO:0002439 8.65 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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