MCID: SYS034
MIFTS: 47

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 52
Systemic Juvenile Idiopathic Arthritis 52 17
Systemic Onset Juvenile Rheumatoid Arthritis 52
Systemic-Onset Juvenile Idiopathic Arthritis 52
Systemic Onset Juvenile Chronic Arthritis 71
Systemic Polyarthritis 52
Systemic-Onset Jia 52
Still Disease 52

Classifications:



External Ids:

UMLS 71 C1384600

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 85414 Definition Systemic-onset juvenile idiopathic arthritis is marked by the severity of the extra-articular manifestations (fever, cutaneous eruptions) and by an equal sex ratio. Epidemiology It 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 description Onset 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. Etiology The 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 methods The 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 diagnosis The differential diagnosis should include fever associated with infection, connective tissue disease (notably lupus), acute leukaemia and other autoinflammatory diseases. Management and treatment Management 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. Prognosis The 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 Cytokine Signaling in Immune system and Measles. The drugs Liver Extracts and Alendronate have been mentioned in the context of this disorder. Affiliated tissues include bone, testes and liver.

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 490)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 33.0 TNF IL18
2 juvenile rheumatoid arthritis 30.6 TNF IL1RN IL1B IL18
3 exanthem 30.6 TNF IL1RN IL1B
4 kawasaki disease 30.5 TNF IL1B IL18
5 spondylitis 30.3 TNF IL1RN
6 pericarditis 30.1 TNF IL1RN IL1B
7 arthritis 30.1 TNF IL1RN IL1B IL18
8 acute graft versus host disease 30.1 TNF IL18
9 brucellosis 30.0 TNF IL1RN IL18
10 pyogenic sterile arthritis, pyoderma gangrenosum, and acne 30.0 IL1RN IL1B
11 perinatal necrotizing enterocolitis 29.9 TNF IL1B
12 myocarditis 29.9 TNF IL1B IL18
13 amyloidosis 29.9 TNF IL1RN IL1B
14 cellulitis 29.9 TNF IL1B
15 appendicitis 29.8 TNF IL1B
16 neutropenia 29.8 TNF IL1RN IL1B
17 wells syndrome 29.8 IL1RAPL2 IL1B
18 pleurisy 29.7 TNF IL1RN
19 interstitial lung disease 29.7 TNF IL1B IL18
20 visceral leishmaniasis 29.6 TNF IL1B IL18
21 leishmaniasis 29.6 TNF IL1B IL18
22 gastroenteritis 29.6 TNF IL1RN IL1B
23 celiac disease 1 29.5 TNF IL1B IL18
24 familial mediterranean fever 29.5 TNF IL1RN IL1B IL18
25 periodic fever, familial, autosomal dominant 29.5 TNF IL1RN IL1B
26 blau syndrome 29.5 TNF IL1RN IL1B
27 gastric ulcer 29.5 TNF IL1RN IL1B
28 ulcerative colitis 29.4 TNF IL1RN IL1B
29 subacute thyroiditis 29.4 TNF IL1B
30 osteoarthritis 29.4 TNF IL1RN IL1B
31 acute myocarditis 29.4 TNF IL1B
32 skin disease 29.4 TNF IL1B IL18
33 hidradenitis 29.4 TNF IL1B
34 cytomegalovirus infection 29.3 TNF IL1B
35 histiocytosis 29.3 TNF IL1RN
36 spondyloarthropathy 1 29.3 TNF IL1RN IL1RAPL2
37 thrombocytopenia 29.3 TNF IL1RN IL1B IL18
38 meningoencephalitis 29.3 TNF IL1B
39 graft-versus-host disease 29.3 TNF IL1B
40 pharyngitis 29.2 TNF IL1RN IL1B
41 lung disease 29.2 TNF IL1B IL18
42 hepatitis a 29.2 TNF IL1B
43 clostridium difficile colitis 29.0 IL1RAPL2 IL1B
44 bacterial infectious disease 29.0 TNF IL1RN IL1B IL18
45 leprosy 3 28.9 TNF IL1B
46 meningitis 28.9 TNF IL1B IL18
47 aseptic meningitis 28.9 TNF IL1RN IL1B
48 chikungunya 28.9 TNF IL1B IL18
49 synovitis 28.9 TNF IL1RN IL1B IL18
50 peritonitis 28.9 TNF IL1B IL18

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

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 43)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Alendronate Approved Phase 2, Phase 3 66376-36-1, 121268-17-5 2088
3
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
4
tannic acid Approved Phase 3 1401-55-4
5
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
6
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
7
leucovorin Approved Phase 3 58-05-9 6006 143
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
BCG vaccine Investigational Phase 3
12 glucocorticoids Phase 2, Phase 3
13 Folic Acid Antagonists Phase 3
14 Vitamin B Complex Phase 3
15 Folate Phase 3
16 Dermatologic Agents Phase 3
17 Antimetabolites Phase 3
18 Vitamin B9 Phase 3
19 Fluorodeoxyglucose F18 Phase 3
20 Pharmaceutical Solutions Phase 3
21 Protein Kinase Inhibitors Phase 3
22 Interleukin 1 Receptor Antagonist Protein Phase 3
23 Antirheumatic Agents Phase 3
24 Anti-Inflammatory Agents Phase 3
25 Gastrointestinal Agents Phase 3
26 Analgesics, Non-Narcotic Phase 3
27 Analgesics Phase 3
28 Immunosuppressive Agents Phase 3
29 Anti-Inflammatory Agents, Non-Steroidal Phase 3
30
Rilonacept Approved, Investigational Phase 2 501081-76-1 104924
31 Histone Deacetylase Inhibitors Phase 2
32 Givinostat hydrochloride Phase 2
33 interferons Phase 2
34 Interferon-gamma Phase 2
35
Prednisolone phosphate Approved, Vet_approved Phase 1 302-25-0
36
Methylprednisolone Approved, Vet_approved Phase 1 83-43-2 6741
37
Methylprednisolone hemisuccinate Approved Phase 1 2921-57-5
38 Prednisolone acetate Approved, Vet_approved Phase 1 52-21-1
39
Prednisolone Approved, Vet_approved Phase 1 50-24-8 5755
40
Tyrosine Approved, Investigational, Nutraceutical Phase 1 60-18-4 6057
41
Prednisolone hemisuccinate Experimental Phase 1 2920-86-7
42 Immunoglobulins, Intravenous Phase 1
43 Methylprednisolone Acetate Phase 1

Interventional clinical trials:

(show top 50) (show all 52)
# Name Status NCT ID Phase Drugs
1 A Phase IV Study to Evaluate Decreased Dose Frequency in Patients With Systemic Juvenile Arthritis (SJIA) Who Experience Laboratory Abnormalities During Treatment With Tocilizumab Completed NCT01734382 Phase 4 Tocilizumab
2 A Phase IV, Multicenter, Single-Arm, Open-Label Study to Assess the Efficacy and Safety of Tocilizumab in Chinese Patients With Systemic Juvenile Idiopathic Arthritis Recruiting NCT03301883 Phase 4 Tocilizumab;NSAIDs;CSs;MTX
3 Double Blind Controlled Trial of Alendronate for the Treatment of Childhood and Adolescent Glucocorticoid-Associated Osteopenia and Osteoporosis Unknown status NCT00209469 Phase 2, Phase 3 alendronate sodium
4 ANAkinra in Severe Juvenile Idiopathic Arthritis of Systemic Onset (ANAJIS) Completed NCT00339157 Phase 2, Phase 3 Anakinra
5 Double-Blinded Controlled Trial of Alendronate for the Treatment of Childhood and Adolescent Glucocorticoid- Associated Osteopenia and Osteoporosis Completed NCT00277251 Phase 2, Phase 3 Alendronate
6 An Open-label Extension Study of Canakinumab (ACZ885) in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) and Active Systemic Manifestations Who Participated in Studies ACZ885G2301 and ACZ885G2305; and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Without Fever Completed NCT00891046 Phase 3 Canakinumab
7 An Open-label, Single-arm, Active-treatment, Efficacy and Safety Study of Canakinumab (ACZ885) Administered for at Least 48 Weeks in Japanese Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT02396212 Phase 3
8 A Randomized, Double-blind, Placebo Controlled, Withdrawal Study of Flare Prevention of Canakinumab (ACZ885) in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) and Active Systemic Manifestations Completed NCT00889863 Phase 3 canakinumab;placebo
9 An Open-Label, Phase III Study to Evaluate the Efficacy, Safety and PK of MRA in Patients With pJIA Completed NCT00144664 Phase 3 MRA(Tocilizumab)
10 A Double-Blind, Phase III Study to Evaluate the Efficacy, Safety and PK of MRA in Patients With sJIA Completed NCT00144599 Phase 3 MRA(Tocilizumab);placebo
11 An Open-label, Extension, Phase III Study to Evaluate the Long-term Safety and Efficacy of MRA in Patients With sJIA Who Participated in Studies MRA011JP or MRA316JP Completed NCT00144612 Phase 3 MRA(Tocilizumab)
12 β-SPECIFIC 4 Patients: Study of Pediatric EffiCacy and Safety wIth FIrst-line Use of Canakinumab An Open-label Canakinumab (ACZ885) Dose Reduction or Dose Interval Prolongation Efficacy and Safety Study in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT02296424 Phase 3 ACZ885 150 mg (Canakinumab)
13 A French Open-label Extension Study of Canakinumab in Patients Who Participated in International Phase III Studies CACZ885G2301E1 or CACZ885G2306 in Systemic Juvenile Idiopathic Arthritis and CACZ885N2301 in Hereditary Periodic Fevers (TRAPS, HIDS, or crFMF) Completed NCT02334748 Phase 3 canakinumab
14 A Randomized, Placebo-controlled Study to Evaluate the Effect of Tocilizumab on Disease Response in Patients With Active Systemic Juvenile Idiopathic Arthritis (JIA), With an Open-label Extension to Examine the Long Term Use of Tocilizumab Completed NCT00642460 Phase 3 tocilizumab [RoActemra/Actemra];Placebo;Non-steroidal anti-inflammatory drugs (NSAIDs);methotrexate;corticosteroids
15 Utility of FDG-PET Scan on the Selection of Patients for Resection of Hepatic Colorectal Metastases Completed NCT00119899 Phase 3
16 Randomized, Double-Blind, Placebo-Controlled, Withdrawal, Safety and Efficacy Study of Oral Baricitinib in Patients From 1 Year to Less Than 18 Years Old With Systemic Juvenile Idiopathic Arthritis Recruiting NCT04088396 Phase 3 Baricitinib;Placebo
17 EFFICACY, SAFETY, TOLERABILITY AND PHARMACOKINETICS OF TOFACITINIB FOR TREATMENT OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS (SJIA) WITH ACTIVE SYSTEMIC FEATURES IN CHILDREN AND ADOLESCENT SUBJECTS Recruiting NCT03000439 Phase 3 In open-label phase: treatment with tofacitinib;In double-blind phase: treatment with tofacitinib or placebo in 1:1 ratio
18 Open-Label, Multicenter, Ph 3 Study to Evaluate the Efficacy and Tolerability of Intravesical Vicinium™ in Subjects With Non Muscle-Invasive Carcinoma in Situ and/or High-Grade Papillary Disease of the Bladder Treated With BCG Active, not recruiting NCT02449239 Phase 3
19 A Randomized, Double-blind, Placebo Controlled, Single-dose Study to Assess the Initial Efficacy of Canakinumab (ACZ885) With Respect to the Adapted ACR Pediatric 30 Criteria in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) and Active Systemic Manifestations Terminated NCT00886769 Phase 3 Canakinumab;Placebo
20 A Phase 3 Safety and Efficacy Study of Etanercept In Children With Systemic Onset Juvenile Rheumatoid Arthritis Terminated NCT00078806 Phase 3 Etanercept;Placebo
21 A Randomized, Double-blind, Placebo-controlled, Multicenter, Phase 3 Efficacy and Safety Study of 2 Dose Levels of Subcutaneous Anakinra (Kineret®) in Patients With Still's Disease (SJIA and AOSD) Terminated NCT03265132 Phase 3 Placebo
22 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
23 Efficacy and Safety of Tocilizumab (a Monoclonal Antibody to Receptor of IL-6) in the Treatment of Adult's Still Disease Unknown status NCT01002781 Phase 2 Tocilizumab
24 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
25 Tocilizumab in the Treatment of Refractory Polymyositis and Dermatomyositis Completed NCT02043548 Phase 2 tocilizumab;placebo
26 Ilaris (Canakinumab) in the Schnitzler Syndrome. A Case Series. Completed NCT01245127 Phase 2 Ilaris
27 Safety, Efficacy and Pharmacokinetics of Subcutaneous ACZ885 in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT00426218 Phase 1, Phase 2 ACZ885
28 Randomized Placebo Phase Study of Rilonacept in the Treatment of Systemic Juvenile Idiopathic Arthritis (RAPPORT) Completed NCT00534495 Phase 2
29 Efficacy and Safety of Canakinumab in Schnitzler Syndrome Completed NCT01276522 Phase 2 Canakinumab
30 A Pilot Open-label Study to Assess the Efficacy and Safety of Tocilizumab in Patients With Active Schnitzler's Syndrome Completed NCT03046381 Phase 2 Tocilizumab
31 A Single Escalating Dose Pilot Trial of Canakinumab (ILARIS®) Assessing Safety and Biomarker Changes in Boys With Duchenne Muscular Dystrophy Recruiting NCT03936894 Phase 1, Phase 2 Canakinumab Injection [Ilaris]
32 An Open-label, Sequential, Ascending, Repeated Dose-finding Study of Sarilumab, Administered With Subcutaneous (SC) Injection, in Children and Adolescents, Aged 1 to 17 Years, With Systemic Juvenile Idiopathic Arthritis (sJIA), Followed by an Extension Phase Recruiting NCT02991469 Phase 2 Sarilumab SAR153191 (REGN88)
33 A Pilot, Open-label, Single Arm, Multicenter Study to Evaluate Safety, Tolerability, Pharmacokinetics and Efficacy of Intravenous Administrations of NI-0501, an Anti-interferon Gamma (Anti-IFNγ) Monoclonal Antibody, in Patients With Systemic Juvenile Idiopathic Arthritis (sJIA) Developing Macrophage Activation Syndrome/Secondary HLH (MAS/sHLH) Active, not recruiting NCT03311854 Phase 2 NI-0501
34 Early Phase Administration of Anakinra as a Rescue Treatment for Inhaled Allergen Challenge-Induced Airway Inflammation Enrolling by invitation NCT03513471 Phase 1, Phase 2 Dermatophagoides Farinae
35 Late Phase Administration Anakinra as a Rescue Treatment for Inhaled Allergen Challenge-Induced Airway Inflammation Enrolling by invitation NCT03513458 Phase 1, Phase 2 Dermatophagoides Farinae
36 A Pilot, Multi-center, Randomized, Double-Blind, Placebo-Controlled, Ascending Dose, Safety, Tolerability and Preliminary Efficacy Study of Two Dose Levels of IL-1 Trap Administered Subcutaneously in Pediatric Subjects With Active Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT01803321 Phase 1 rilonacept (IL-1 Trap)
37 A Phase Ib, Open-Label, Multicenter Study to Investigate the Pharmacokinetics, Pharmacodynamics, and Safety of Tocilizumab Following Subcutaneous Administration to Patients With Systemic Juvenile Idiopathic Arthritis Completed NCT01904292 Phase 1 Tocilizumab
38 A Phase I Pharmacokinetic and Safety Study of Tocilizumab (TCZ) in Patients Less Than 2 Years Old With Active Systemic Juvenile Idiopathic Arthritis (sJIA) Completed NCT01455701 Phase 1 Tocilizumab
39 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
40 Randomized Placebo Controlled Trial of Subcutaneous rhIL-1A (Anakinra) in the Management of Hospitalized Pediatric and Adult Patients With Macrophage Activation Syndrome Recruiting NCT02780583 Phase 1 kineret;placebo
41 A Randomized Controlled Trial of Adjunctive Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
42 Long-Term Extension Study to Evaluate the Safety and Efficacy of Subcutaneous Tocilizumab in Patients With Polyarticular-Course and Systemic Juvenile Idiopathic Arthritis Active, not recruiting NCT02165345 Phase 1 Tocilizumab
43 Phase I Study of Itacitinib in Combination With Tocilizumab for the Treatment of Steroid Refractory Acute Graft Versus Host Disease Not yet recruiting NCT04070781 Phase 1 Itacitinib;Tocilizumab
44 A Phase I Study of PR1-Specific Cytotoxic T-Lymphocyte Infusion for Patients With Recurrent CML After Allogeneic Hematopoietic Transplantation Withdrawn NCT00866346 Phase 1
45 Comparison of Predictive Ability of New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
46 The Immune Signature of Palmoplantar Pustulosis Completed NCT01780857
47 A Non-interventional, Post-authorization Safety Study (PASS) to Evaluate Long-term Safety of Anakinra (Kineret®) in Patients With Systemic Juvenile Idiopathic Arthritis Completed NCT03932344 Anakinra
48 Admission of Adult-onset Still Disease Patients in the ICU: a French Retrospective Multicentric Study Completed NCT03276650
49 Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still s Disease and Related Inflammatory Conditions Recruiting NCT03510442
50 Arthritis in Assiut University Children Hospital Not yet recruiting NCT04326582

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:

40
Bone, Testes, Liver, Neutrophil, Monocytes, Lung, T Cells

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 733)
# Title Authors PMID Year
1
Rheumatologists' perspective on coronavirus disease 19 (COVID-19) and potential therapeutic targets. 61
32277367 2020
2
Interleukin-1 Blockade in Systemic Juvenile Idiopathic Arthritis. 61
32291603 2020
3
Macrophage activation syndrome as a complication of dermatomyositis: A case report. 61
32548165 2020
4
Inflammatory Bowel Disease in Children with Systemic Juvenile Idiopathic Arthritis. 61
32541073 2020
5
Systemic juvenile idiopathic arthritis and recurrent macrophage activation syndrome due to a CASP1 variant causing inflammasome hyperactivation. 61
32556329 2020
6
Efficacy and safety of canakinumab in systemic juvenile idiopathic arthritis: 48-week results from an open-label Phase III study in Japanese patients. 61
32552266 2020
7
Role of FDG-PET/CT in children with fever of unknown origin. 61
32030452 2020
8
Macrophage activation syndrome in juvenile dermatomyositis: a systematic review. 61
31529231 2020
9
Experience with Cidofovir as an adjunctive therapy in a patient of adenovirus-induced macrophage activation syndrome in systemic arthritis. 61
32418043 2020
10
Tocilizumab-induced anaphylaxis in patients with adult-onset Still's disease and systemic juvenile idiopathic arthritis: a case-based review. 61
31598752 2020
11
A case of life-threatening valvulitis mimicking infective endocarditis in systemic juvenile idiopathic arthritis. 61
32290893 2020
12
Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome. 61
32373790 2020
13
The association of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis. 61
32398039 2020
14
Discontinuation of Canakinumab following Clinical Disease Remission Is Feasible in Patients with Systemic Juvenile Idiopathic Arthritis. 61
32062604 2020
15
Successful treatment of plasma exchange for refractory systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome and severe lung disease. 61
32245894 2020
16
Response to: 'Successful treatment of plasma exchange for refractory systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome and severe lung disease' by Sato et al. 61
32317313 2020
17
FUNCTIONAL AND GENETIC TESTING IN ADULTS WITH HLH DO NOT REVEAL A CYTOTOXICITY DEFECT BUT RATHER AN INFLAMMATORY PROFILE. 61
32356861 2020
18
Treatment of Systemic JIA: When do we need a biologic? Real world data of a single center. 61
32343644 2020
19
Macrophage activation syndrome complicating rheumatic diseases in adults: case-based review. 61
31367795 2020
20
[Still's disease as biphasic disorder : Current knowledge on pathogenesis and novel treatment approaches]. 61
32253510 2020
21
Severe sepsis caused by serious gastrointestinal infection in sJIA patients treated with IL-6 receptor antagonist: a case report. 61
32188441 2020
22
Comparison of serum biomarkers for the diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis during tocilizumab therapy. 61
32184444 2020
23
Autoinflammatory diseases in childhood, part 2: polygenic syndromes. 61
32065273 2020
24
Autoinflammatory diseases in childhood, part 1: monogenic syndromes. 61
32065272 2020
25
Th17 reprogramming of T cells in systemic juvenile idiopathic arthritis. 61
32213704 2020
26
Impact of IL1RN Variants on Response to Interleukin-1 Blocking Therapy in Systemic Juvenile Idiopathic Arthritis. 61
31599092 2020
27
Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis. 61
31707357 2020
28
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management. 61
31732958 2020
29
Clinical and laboratory features, treatment, and outcomes of macrophage activation syndrome in 80 children: a multi-center study in China. 61
31612427 2020
30
IL-18 as therapeutic target in a patient with resistant systemic juvenile idiopathic arthritis and recurrent macrophage activation syndrome. 61
31359055 2020
31
IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome. 61
31326996 2020
32
Innately Adaptive or Truly Autoimmune: Is There Something Unique About Systemic Juvenile Idiopathic Arthritis? 61
31524322 2020
33
Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis. 61
31513353 2020
34
Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. 61
31924225 2020
35
Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis. 61
31273457 2020
36
Clinical and associated inflammatory biomarker features predictive of short-term outcomes in non-systemic juvenile idiopathic arthritis. 61
31919503 2020
37
Macrophage activation syndrome as a complication of rheumatologic disorders, a report from Iran. 61
31995957 2020
38
Severe Recurrent Fever Episodes With Clinical Diagnosis of Hemophagocytic Lymphohistiocytosis, Incomplete Kawasaki Disease and Systemic-Onset Juvenile Idiopathic Arthritis: A Case Report and Literature Review. 61
32211357 2020
39
Height and sexual maturation in girls with juvenile idiopathic arthritis. 61
30339783 2020
40
Canakinumab in colchicine resistant familial Mediterranean fever and other pediatric rheumatic diseases. 61
32419407 2020
41
Diacylglycerol Kinase ζ Regulates Macrophage Responses in Juvenile Arthritis and Cytokine Storm Syndrome Mouse Models. 61
31801815 2020
42
Emergent high fatality lung disease in systemic juvenile arthritis. 61
31562126 2019
43
Transcriptome-Guided Drug Repositioning. 61
31842375 2019
44
Long-term surveillance of biologic therapies in systemic-onset juvenile idiopathic arthritis: data from the German BIKER registry. 61
31846042 2019
45
Regulatory Role for NK Cells in a Mouse Model of Systemic Juvenile Idiopathic Arthritis. 61
31676671 2019
46
Population pharmacokinetics of tacrolimus in pediatric patients with systemic-onset juvenile idiopathic arthritis: Initial dosage recommendations. 61
31772640 2019
47
Nonspecific interstitial pneumonia in refractory systemic juvenile idiopathic arthritis responded to tocilizumab treatment. 61
31837211 2019
48
Management of adult-onset Still's disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts. 61
31829244 2019
49
Pediatric macrophage activation syndrome, recognizing the tip of the Iceberg. 61
31804174 2019
50
Anakinra in children and adults with Still's disease. 61
31769856 2019

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 37)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.07 TNF IL1RN IL1B IL18
2
Show member pathways
12.56 TNF IL1B IL18
3
Show member pathways
12.39 TNF IL1RN IL1B IL18
4
Show member pathways
12.36 TNF IL1B IL18
5
Show member pathways
12.34 TNF IL1B IL18
6 12.32 TNF IL1RN IL1B
7 12.16 TNF IL1B IL18
8 12.08 TNF IL1B IL18
9 12.04 TNF IL1B IL18
10
Show member pathways
12.02 TNF IL1B IL18
11 12 TNF IL1B IL18
12 11.99 TNF IL1B IL18
13 11.93 TNF IL1RN IL1B
14 11.79 TNF IL1B IL18
15 11.76 TNF IL1B IL18
16
Show member pathways
11.7 TNF IL1B IL18
17 11.62 TNF IL1B IL18
18 11.51 TNF IL1B IL18
19
Show member pathways
11.47 TNF IL1B
20 11.47 TNF IL1B
21 11.34 TNF IL1B
22 11.34 TNF IL1B IL18
23 11.31 TNF IL1B
24
Show member pathways
11.28 TNF IL1B
25 11.26 IL1B IL18
26 11.24 TNF IL1B IL18
27 11.18 TNF IL1B IL18
28 11.17 TNF IL1B
29 11.11 TNF IL1B
30 11.07 TNF IL1B
31 11 IL1B IL18
32 11 TNF IL1B IL18
33 10.84 TNF IL1B IL18
34 10.78 TNF IL1B
35 10.61 TNF IL1RN IL1B IL18
36 10.47 TNF IL1B IL18
37 10.36 IL1B IL18

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 41)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.85 TNF IL1B IL18
2 immune response GO:0006955 9.83 TNF IL1RN IL1B IL18
3 MAPK cascade GO:0000165 9.81 TNF IL1B IL18
4 inflammatory response GO:0006954 9.76 TNF IL1RN IL1B IL18
5 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.74 TNF IL1B IL18
6 positive regulation of inflammatory response GO:0050729 9.69 TNF IL1B IL18
7 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.68 TNF IL18
8 regulation of insulin secretion GO:0050796 9.68 TNF IL1B
9 response to glucocorticoid GO:0051384 9.68 TNF IL1RN
10 positive regulation of smooth muscle cell proliferation GO:0048661 9.67 TNF IL18
11 positive regulation of interleukin-6 production GO:0032755 9.67 TNF IL1B
12 positive regulation of phagocytosis GO:0050766 9.67 TNF IL1B
13 positive regulation of nitric oxide biosynthetic process GO:0045429 9.66 TNF IL1B
14 positive regulation of interleukin-6 secretion GO:2000778 9.66 TNF IL1B
15 protein kinase B signaling GO:0043491 9.65 TNF IL1B
16 negative regulation of extrinsic apoptotic signaling pathway in absence of ligand GO:2001240 9.65 TNF IL1B
17 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.65 TNF IL1B
18 positive regulation of interleukin-8 production GO:0032757 9.64 TNF IL1B
19 negative regulation of neurogenesis GO:0050768 9.64 TNF IL1B
20 inflammatory response to antigenic stimulus GO:0002437 9.63 IL1RN IL1B
21 negative regulation of myoblast differentiation GO:0045662 9.62 TNF IL18
22 positive regulation of glial cell proliferation GO:0060252 9.62 TNF IL1B
23 negative regulation of lipid catabolic process GO:0050995 9.61 TNF IL1B
24 astrocyte activation GO:0048143 9.61 TNF IL1B
25 positive regulation of membrane protein ectodomain proteolysis GO:0051044 9.6 TNF IL1B
26 positive regulation of interleukin-6 biosynthetic process GO:0045410 9.59 TNF IL1B
27 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.58 TNF IL1B
28 positive regulation of chemokine biosynthetic process GO:0045080 9.58 TNF IL1B
29 cellular response to organic cyclic compound GO:0071407 9.58 TNF IL1B IL18
30 positive regulation of granulocyte macrophage colony-stimulating factor production GO:0032725 9.56 IL1B IL18
31 interleukin-6 production GO:0032635 9.55 IL1B IL18
32 regulation of establishment of endothelial barrier GO:1903140 9.54 TNF IL1B
33 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.54 TNF IL1B IL18
34 positive regulation of T-helper 1 cell cytokine production GO:2000556 9.49 IL1B IL18
35 positive regulation of fever generation GO:0031622 9.48 TNF IL1B
36 sequestering of triglyceride GO:0030730 9.43 TNF IL1B
37 positive regulation of interferon-gamma production GO:0032729 9.43 TNF IL1B IL18
38 lipopolysaccharide-mediated signaling pathway GO:0031663 9.33 TNF IL1B IL18
39 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.32 TNF IL1B
40 positive regulation of neuroinflammatory response GO:0150078 9.13 TNF IL1B IL18
41 cytokine-mediated signaling pathway GO:0019221 9.02 TNF IL1RN IL1RAPL2 IL1B IL18

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

# Name GO ID Score Top Affiliating Genes
1 interleukin-1 receptor binding GO:0005149 8.96 IL1RN IL1B
2 cytokine activity GO:0005125 8.92 TNF IL1RN IL1B IL18

Sources for Systemic Onset Juvenile Idiopathic Arthritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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