MCID: SYS034
MIFTS: 52

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 juvenile rheumatoid 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 Pathway and Allograft rejection. The drugs Liver Extracts and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include testes, neutrophil and monocytes, 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 119)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 32.7 PRF1 TNF
2 juvenile rheumatoid arthritis 31.6 IL18 TNF
3 arthritis 31.3 IL18 MIF TNF
4 vasculitis 29.9 MEFV TNF
5 rheumatoid arthritis 29.8 IL18 MIF TNF
6 periodic fever, familial, autosomal dominant 29.8 MEFV TNF
7 blau syndrome 29.8 MEFV TNF
8 pericarditis 29.7 MEFV TNF
9 familial mediterranean fever 29.7 MEFV TNF
10 hemophagocytic lymphohistiocytosis 29.7 IL18 PRF1 TNF
11 peritonitis 29.7 MEFV TNF
12 adult-onset still's disease 29.4 IL18 MEFV MIF TNF
13 rheumatoid arthritis, systemic juvenile 11.9
14 kawasaki disease 10.5
15 amyloidosis 10.2
16 listeria meningitis 10.1 IL18 TNF
17 melanoma metastasis 10.1 IL18 TNF
18 rheumatoid vasculitis 10.1 MIF TNF
19 enthesopathy 10.1
20 stachybotrys chartarum 10.1 IL18 TNF
21 arthus reaction 10.1 MIF TNF
22 inflammatory bowel disease 3 10.1 IL18 TNF
23 mycoplasma pneumoniae pneumonia 10.1 IL18 TNF
24 oligoarticular juvenile idiopathic arthritis 10.1 MIF TNF
25 cardiac tamponade 10.1
26 pleural tuberculosis 10.1 IL18 TNF
27 fasciitis 10.1 IL18 TNF
28 rasmussen encephalitis 10.1 IL18 TNF
29 acute graft versus host disease 10.1 IL18 TNF
30 cutaneous lupus erythematosus 10.1 IL18 TNF
31 lepromatous leprosy 10.1 MIF TNF
32 cysticercosis 10.1 IL18 MIF
33 melioidosis 10.1 IL18 TNF
34 smallpox 10.1 IL18 TNF
35 pulmonary sarcoidosis 10.1 IL18 TNF
36 listeriosis 10.1 PRF1 TNF
37 bacterial infectious disease 10.1 IL18 TNF
38 lymphadenitis 10.1 IL18 TNF
39 cytokine deficiency 10.1
40 bacterial vaginosis 10.0 PRF1 TNF
41 relapsing-remitting multiple sclerosis 10.0 IL18 TNF
42 synovitis 10.0 IL18 TNF
43 proteasome-associated autoinflammatory syndrome 1 10.0 IL18 TNF
44 pulmonary tuberculosis 10.0 IL18 TNF
45 torticollis 10.0
46 diabetes mellitus 10.0
47 thrombocytopenia 10.0
48 lymphoma 10.0
49 respiratory failure 10.0
50 encephalitis 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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.72 IL18 MEFV MIF PRF1 TNF
2 hematopoietic system MP:0005397 9.65 IL18 MEFV MIF PRF1 TNF
3 homeostasis/metabolism MP:0005376 9.55 IL18 MEFV MIF PRF1 TNF
4 immune system MP:0005387 9.35 IL18 MEFV MIF PRF1 TNF
5 integument MP:0010771 8.92 IL18 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
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
3
tannic acid Approved Phase 3 1401-55-4
4
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
5
leucovorin Approved Phase 3 58-05-9 143 6006
6
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 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, Monoclonal Phase 3,Phase 1,Phase 2,Not Applicable
14 Antibodies Phase 3,Phase 1,Phase 2,Not Applicable
15 Immunologic Factors Phase 3,Phase 1,Phase 2
16 Immunoglobulins Phase 3,Phase 1,Phase 2,Not Applicable
17 Anti-Inflammatory Agents, Non-Steroidal Phase 3
18 Vitamin B9 Phase 3
19 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1
20 Folate Phase 3
21 Immunosuppressive Agents Phase 3
22 Folic Acid Antagonists Phase 3
23 Peripheral Nervous System Agents Phase 3
24 Dermatologic Agents Phase 3
25 Analgesics Phase 3
26 Analgesics, Non-Narcotic Phase 3
27 Nucleic Acid Synthesis Inhibitors Phase 3
28 Vitamin B Complex Phase 3
29 Antimetabolites, Antineoplastic Phase 3
30 Antimetabolites 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 39)
# 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 Single Escalating Dose Pilot Trial of Canakinumab (ILARIS®) in Duchenne Muscular Dystrophy Recruiting NCT03936894 Phase 1, Phase 2 Canakinumab Injection [Ilaris]
27 Tocilizumab in Patients With Schnitzler's Syndrome Recruiting NCT03046381 Phase 2 Tocilizumab
28 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)
29 A Study of Subcutaneously Administered Tocilizumab in Participants With Systemic Juvenile Idiopathic Arthritis Completed NCT01904292 Phase 1 Tocilizumab
30 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
31 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
32 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
33 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
34 New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
35 Ultrasonography in Juvenile Idiopathic Arthritis Completed NCT01304420
36 The CARRA Registry Completed NCT01697254
37 Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still's Disease, and Related Conditions Recruiting NCT03510442
38 Long-term Safety Study of Kineret® in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Enrolling by invitation NCT03932344 Anakinra
39 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
Testes, Neutrophil, Monocytes, T Cells, Endothelial, Myeloid, Bone Marrow

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 410)
# 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
Achromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis. ( 31102385 )
2019
3
Molecular signature characterisation of different inflammatory phenotypes of systemic juvenile idiopathic arthritis. ( 31005900 )
2019
4
Clinical significance of serum CXCL9 levels as a biomarker for systemic juvenile idiopathic arthritis associated macrophage activation syndrome. ( 30952065 )
2019
5
Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis. ( 30948682 )
2019
6
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
7
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
8
Serum Leucine-Rich α2-Glycoprotein as a Biomarker for Monitoring Disease Activity in Patients with Systemic Juvenile Idiopathic Arthritis. ( 30863782 )
2019
9
A Bullseye for Children with Systemic Juvenile Idiopathic Arthritis. ( 30802004 )
2019
10
PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY. ( 30810692 )
2019
11
Treatment of patients with systemic-onset juvenile idiopathic arthritis with tacrolimus. ( 30783487 )
2019
12
The role of extracellular histones in systemic-onset juvenile idiopathic arthritis. ( 30642364 )
2019
13
Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis. ( 30647180 )
2019
14
No preventive or therapeutic efficacy of infliximab against macrophage activation syndrome due to systemic juvenile idiopathic arthritis. ( 30406708 )
2019
15
S100A12 and vascular endothelial growth factor can differentiate Blau syndrome and familial Mediterranean fever from systemic juvenile idiopathic arthritis. ( 30406853 )
2019
16
Short-term outcomes in patients with systemic juvenile idiopathic arthritis treated with either tocilizumab or anakinra. ( 30137641 )
2019
17
Risk factors for hypersensitivity reactions to tocilizumab introduction in systemic juvenile idiopathic arthritis. ( 29578355 )
2019
18
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
19
Primary varicella infection in children with systemic juvenile idiopathic arthritis under tocilizumab therapy. ( 27846755 )
2019
20
Drug Retention Rate and Predictive Factors of Drug Survival for Interleukin-1 Inhibitors in Systemic Juvenile Idiopathic Arthritis. ( 30670972 )
2018
21
Neutrophils From Children With Systemic Juvenile Idiopathic Arthritis Exhibit Persistent Proinflammatory Activation Despite Long-Standing Clinically Inactive Disease. ( 30619348 )
2018
22
Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience. ( 30505008 )
2018
23
Methotrexate-Induced Vasculitis in a Child with Systemic Onset Juvenile Idiopathic Arthritis. ( 30431484 )
2018
24
Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience. ( 30431724 )
2018
25
Transient natural killer cell dysfunction associated with interleukin-18 overproduction in systemic juvenile idiopathic arthritis. ( 30345698 )
2018
26
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
27
Insufficient IL-10 Production as a Mechanism Underlying the Pathogenesis of Systemic Juvenile Idiopathic Arthritis. ( 30266771 )
2018
28
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
29
Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis. ( 30238379 )
2018
30
Thrombocytopaenia in Systemic Juvenile Idiopathic Arthritis: Not Always Macrophage Activation Syndrome! ( 30178590 )
2018
31
Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis. ( 28499329 )
2018
32
Characteristic elevation of soluble TNF receptor II : I ratio in macrophage activation syndrome with systemic juvenile idiopathic arthritis. ( 28815559 )
2018
33
Validation of Classification Criteria of Macrophage Activation Syndrome in Japanese Patients With Systemic Juvenile Idiopathic Arthritis. ( 29195002 )
2018
34
Brief Report: Novel UNC13D Intronic Variant Disrupting an NF-κB Enhancer in a Patient With Recurrent Macrophage Activation Syndrome and Systemic Juvenile Idiopathic Arthritis. ( 29409136 )
2018
35
Systemic juvenile idiopathic arthritis and macrophage activation syndrome: update on pathogenesis and treatment. ( 29870499 )
2018
36
Sustained hyperferritinemia in a child with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis - perforinopathy: case based review. ( 30968645 )
2018
37
Remission of Refractory Systemic-Onset Juvenile Idiopathic Arthritis After Treatment With Siltuximab. ( 29485546 )
2018
38
Reasons for inactive disease and flare in systemic onset juvenile idiopathic arthritis patients during tocilizumab treatment. ( 29303703 )
2018
39
Reversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis. ( 29426067 )
2018
40
Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. ( 29574969 )
2018
41
The burden of systemic juvenile idiopathic arthritis for patients and caregivers: an international survey and retrospective chart review. ( 29600940 )
2018
42
IL1RN Variation Influences Both Disease Susceptibility and Response to Recombinant Human Interleukin-1 Receptor Antagonist Therapy in Systemic Juvenile Idiopathic Arthritis. ( 29609200 )
2018
43
IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry). ( 29622022 )
2018
44
Soluble CD163, a unique biomarker to evaluate the disease activity, exhibits macrophage activation in systemic juvenile idiopathic arthritis. ( 29801971 )
2018
45
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
46
Early reduction of serum interleukin-6 levels as a predictor of clinical remission in systemic juvenile idiopathic arthritis. ( 29888930 )
2018
47
The role of IL-1 inhibition in systemic juvenile idiopathic arthritis: current status and future perspectives. ( 29922038 )
2018
48
Interleukin-1 in monocyte activation phenotypes in systemic juvenile idiopathic arthritis: Observations from a clinical trial of rilonacept, an interleukin-1 inhibitor. ( 29928998 )
2018
49
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
50
Impact of systemic juvenile idiopathic arthritis/Still's disease on adolescents as evidenced through social media posts. ( 29942167 )
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:

(show all 20)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.68 IL18 MEFV TNF
2
Show member pathways
12.46 IL18 PRF1 TNF
3 11.71 MIF TNF
4
Show member pathways
11.7 IL18 TNF
5 11.66 MIF TNF
6 11.61 IL18 TNF
7 11.5 IL18 TNF
8 11.43 IL18 TNF
9 11.36 IL18 TNF
10
Show member pathways
11.29 MIF TNF
11
Show member pathways
11.26 PRF1 TNF
12 11.2 IL18 TNF
13 11.12 IL18 TNF
14 11.09 IL18 TNF
15 11.05 IL18 TNF
16 10.93 IL18 MEFV
17 10.82 IL18 TNF
18 10.66 IL18 TNF
19 10.4 IL18 PRF1
20 9.97 IL18 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 15)
# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.65 IL18 MIF TNF
2 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.55 MIF TNF
3 positive regulation of inflammatory response GO:0050729 9.54 IL18 TNF
4 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.52 IL18 TNF
5 cellular response to organic cyclic compound GO:0071407 9.51 IL18 TNF
6 positive regulation of MAP kinase activity GO:0043406 9.49 MIF TNF
7 positive regulation of smooth muscle cell proliferation GO:0048661 9.46 IL18 TNF
8 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.43 IL18 TNF
9 positive regulation of interferon-gamma production GO:0032729 9.4 IL18 TNF
10 lipopolysaccharide-mediated signaling pathway GO:0031663 9.37 IL18 TNF
11 positive regulation of cytokine secretion GO:0050715 9.32 MIF TNF
12 negative regulation of myoblast differentiation GO:0045662 9.26 IL18 TNF
13 positive regulation of neuroinflammatory response GO:0150078 9.16 IL18 TNF
14 positive regulation of chemokine (C-X-C motif) ligand 2 production GO:2000343 8.96 MIF TNF
15 inflammatory response GO:0006954 8.92 IL18 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 IL18 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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