MCID: SYS034
MIFTS: 53

Systemic Onset Juvenile Idiopathic Arthritis

Categories: Bone diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Systemic Onset Juvenile Idiopathic Arthritis

MalaCards integrated aliases for Systemic Onset Juvenile Idiopathic Arthritis:

Name: Systemic Onset Juvenile Idiopathic Arthritis 20
Systemic-Onset Juvenile Idiopathic Arthritis 20 58
Systemic Juvenile Idiopathic Arthritis 20 17
Systemic-Onset Jia 20 58
Still Disease 20 58
Systemic Onset Juvenile Rheumatoid Arthritis 20
Systemic Onset Juvenile Chronic Arthritis 70
Systemic Polyarthritis 20

Characteristics:

Orphanet epidemiological data:

58
systemic-onset juvenile idiopathic arthritis
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/100000 (Europe); Age of onset: Childhood; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare respiratory diseases
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 M08.2
UMLS via Orphanet 71 C0087031 C1384600
Orphanet 58 ORPHA85414
UMLS 70 C1384600

Summaries for Systemic Onset Juvenile Idiopathic Arthritis

GARD : 20 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.

MalaCards based summary : Systemic Onset Juvenile Idiopathic Arthritis, also known as systemic-onset juvenile idiopathic arthritis, is related to macrophage activation syndrome and juvenile rheumatoid arthritis. An important gene associated with Systemic Onset Juvenile Idiopathic Arthritis is LACC1 (Laccase Domain Containing 1), and among its related pathways/superpathways are Allograft rejection and NOD-like receptor signaling pathway. The drugs tannic acid and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, myeloid and t cells, and related phenotypes are fever and autoimmunity

Wikipedia : 73 Systemic-onset juvenile idiopathic arthritis (or the juvenile onset form of Still's disease) is a type... more...

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 435)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 32.3 TNF RAB27A PRF1
2 juvenile rheumatoid arthritis 31.3 TNF MIF IL6 HLA-DRB1
3 exanthem 31.1 TNF MEFV IL6
4 pericarditis 30.6 TNF MEFV IL6
5 rheumatoid arthritis, systemic juvenile 30.6 MIF IL6
6 familial mediterranean fever 30.6 TNF MEFV IL6
7 adult-onset still's disease 30.4 TNF MEFV IL6
8 juvenile arthritis 30.4 MIF LACC1 IL6
9 brucellosis 30.4 TNF MEFV IL6
10 vasculitis 30.3 TNF MEFV IL6
11 amyloidosis 30.3 TNF MEFV IL6
12 hemophagocytic lymphohistiocytosis 30.2 TNF RAB27A PRF1 GZMB
13 post-transplant lymphoproliferative disease 30.2 TNF IL6
14 enterocolitis 30.2 TNF IL6
15 tenosynovitis 30.2 TNF IL6
16 bursitis 30.2 TNF IL6
17 polyarticular juvenile idiopathic arthritis 30.2 TNF LACC1
18 visceral leishmaniasis 30.2 TNF MIF HLA-DRB1
19 bacterial infectious disease 30.1 TNF MIF IL6
20 arthritis 30.1 TNF MIF MEFV LACC1 IL6 HLA-DRB1
21 erythema nodosum 30.1 TNF MIF IL6
22 myositis 30.1 TNF IL6 HLA-DRB1
23 chickenpox 30.1 TNF PRF1 IL6
24 keratitis, hereditary 30.1 TNF MIF IL6
25 enthesopathy 30.1 TNF MEFV
26 childhood type dermatomyositis 30.1 TNF IL6
27 adult respiratory distress syndrome 30.0 TNF MIF IL6
28 pancytopenia 30.0 RAB27A PRF1 IL6
29 psoriatic arthritis 30.0 TNF IL6 HLA-DRB1
30 periodic fever, familial, autosomal dominant 29.9 TNF MEFV IL6
31 proteasome-associated autoinflammatory syndrome 1 29.9 TNF MEFV IL6
32 mixed connective tissue disease 29.9 TNF IL6 HLA-DRB1
33 leishmaniasis 29.9 TNF IL6 HLA-DRB1
34 neutrophilic dermatosis, acute febrile 29.8 TNF MEFV
35 sarcoidosis 1 29.7 TNF MIF HLA-DRB1
36 pneumonia 29.7 TNF PRF1 IL6
37 cardiogenic shock 29.7 TNF IL6
38 purpura 29.6 TNF MEFV IL6
39 subacute thyroiditis 29.6 TNF IL6
40 bacterial sepsis 29.5 TNF MIF IL6
41 autoimmune disease 29.4 TNF PRF1 MIF IL6 HLA-DRB1
42 peritonitis 29.4 TNF MEFV IL6
43 pharyngitis 29.4 TNF MEFV IL6 HLA-DRB1
44 erythema multiforme 29.3 TNF IL6 GZMB
45 thyroiditis 29.3 TNF IL6 HLA-DRB1
46 celiac disease 1 29.2 TNF PRF1 IL6 HLA-DRB1 GZMB
47 viral hepatitis 29.2 TNF IL6 HLA-DRB1
48 chronic granulomatous disease 29.2 TNF PRF1 LACC1
49 ulcerative colitis 29.1 TNF LACC1 IL6 HLA-DRB1
50 graft-versus-host disease 28.9 TNF IL6 HLA-DRB1 GZMB

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

Human phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

58 31 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 31 hallmark (90%) Very frequent (99-80%) HP:0001945
2 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
3 arthralgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002829
4 joint swelling 58 31 hallmark (90%) Very frequent (99-80%) HP:0001386
5 skin rash 58 31 hallmark (90%) Very frequent (99-80%) HP:0000988
6 elevated c-reactive protein level 58 31 hallmark (90%) Very frequent (99-80%) HP:0011227
7 elevated erythrocyte sedimentation rate 58 31 hallmark (90%) Very frequent (99-80%) HP:0003565
8 juvenile rheumatoid arthritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0005681
9 lymphadenopathy 58 31 frequent (33%) Frequent (79-30%) HP:0002716
10 splenomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0001744
11 hepatomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0002240
12 abdominal pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0002027
13 pericarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001701
14 pleural effusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0002202
15 anterior uveitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012122

MGI Mouse Phenotypes related to Systemic Onset Juvenile Idiopathic Arthritis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.23 IL6 LACC1 MEFV MIF PRF1 RAB27A

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
tannic acid Approved Phase 3 1401-55-4
2
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
3
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
4
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
5
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
6
Tofacitinib Approved, Investigational Phase 3 477600-75-2
7
Etanercept Approved, Investigational Phase 3 185243-69-0
8
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
9 Folic Acid Antagonists Phase 3
10 Vitamin B9 Phase 3
11 Vitamin B Complex Phase 3
12 Folate Phase 3
13 Dermatologic Agents Phase 3
14 Antimetabolites Phase 3
15 Protein Kinase Inhibitors Phase 3
16 Pharmaceutical Solutions Phase 3
17 Antirheumatic Agents Phase 3
18 Interleukin 1 Receptor Antagonist Protein Phase 3
19 Analgesics, Non-Narcotic Phase 3
20 Gastrointestinal Agents Phase 3
21 Immunosuppressive Agents Phase 3
22 Analgesics Phase 3
23 Anti-Inflammatory Agents Phase 3
24 Anti-Inflammatory Agents, Non-Steroidal Phase 3
25
Rilonacept Approved, Investigational Phase 2 501081-76-1 104924
26
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
27
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
28
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
29
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
30
Azathioprine Approved Phase 2 446-86-6 2265
31
Leflunomide Approved, Investigational Phase 2 75706-12-6 3899
32
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
33
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
34 Givinostat hydrochloride Phase 2
35 Histone Deacetylase Inhibitors Phase 2
36 Methylprednisolone Acetate Phase 2
37 interferons Phase 2
38 Interferon-gamma Phase 2
39
Tyrosine Approved, Investigational, Nutraceutical Phase 1 60-18-4 6057

Interventional clinical trials:

(show all 40)
# Name Status NCT ID Phase Drugs
1 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
2 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
3 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
4 ANAkinra in Severe Juvenile Idiopathic Arthritis of Systemic Onset (ANAJIS) Completed NCT00339157 Phase 2, Phase 3 Anakinra
5 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)
6 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)
7 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)
8 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
9 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
10 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
11 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
12 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
13 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
14 An Open-label, Single-arm, Active-treatment Study to Evaluate Efficacy and Safety of Canakinumab (ACZ885) Administered for at Least 48 Weeks in Japanese Patients With Adult Onset Still's Disease (AOSD) Not yet recruiting NCT04717635 Phase 3
15 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
16 A Phase 3 Safety and Efficacy Study of Etanercept In Children With Systemic Onset Juvenile Rheumatoid Arthritis Terminated NCT00078806 Phase 3 Etanercept;Placebo
17 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
18 An Open-label, Multi-arm, Non-comparative Safety and Tolerability Study of Canakinumab (ACZ885) in Patients With Active Systemic Juvenile Idiopathic Arthritis (SJIA) Withdrawn NCT01676948 Phase 3 ACZ885;Canakinumab;Canakinumab;Canakinumab
19 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
20 Phase II, Open Label, International, Multicentre Clinical Trial to Investigate Safety and Efficacy of Oral ITF2357 in Patients With Active Systemic Onset Juvenile Idiopathic Arthritis (SOJIA) Completed NCT00570661 Phase 2 ITF2357
21 Safety, Efficacy and Pharmacokinetics of Subcutaneous ACZ885 in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) Completed NCT00426218 Phase 1, Phase 2 ACZ885
22 Randomized Placebo Phase Study of Rilonacept in the Treatment of Systemic Juvenile Idiopathic Arthritis (RAPPORT) Completed NCT00534495 Phase 2
23 An Open, Randomized Study Treating Refractory Adult-onset Still's Disease With Interleukin-1 Receptor Antagonist Anakinra (Kineret), Compared to an Established, Single Anti-rheumatic Drug Treatment Completed NCT01033656 Phase 2 anakinra;comparators
24 Open-label, Multicenter, Dose-escalating Phase II Study to Investigate the Safety, Tolerability, and Early Signs of Efficacy of Subcutaneous Administrations of Tadekinig Alfa (IL-18BP) in Patients With Adult -Onset Still's Disease (AoSD) During 12 Weeks Completed NCT02398435 Phase 2
25 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)
26 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
27 Study Protocol for a Multi-Centre, Placebo-Controlled Phase II Study of Canakinumab for the Treatment of Adult-onset Still's Disease (AOSD) Including an Open-label Long Term Extension Terminated NCT02204293 Phase 2 Canakinumab;Placebo
28 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)
29 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
30 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
31 Phase I Study of Itacitinib in Combination With Tocilizumab for the Treatment of Steroid Refractory Acute Graft Versus Host Disease Recruiting NCT04070781 Phase 1 Itacitinib;Tocilizumab
32 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
33 A Phase 1b, Multicenter, Open-Label Study to Evaluate the Safety and Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of AEVI-007 in Subjects With Adult Onset Still's Disease Not yet recruiting NCT04752371 Phase 1 AEVI-007 (CERC-007)
34 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
35 Evaluation of Adult Onset Still Disease Classification Criteria and Differential Diagnosis at Eighteen Months of Follow-up Completed NCT04750863
36 Admission of Adult-onset Still Disease Patients in the ICU: a French Retrospective Multicentric Study Completed NCT03276650
37 Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still s Disease and Related Inflammatory Conditions Recruiting NCT03510442
38 Multi-center Registration Study of Adult Onset Still's Disease in Chinese Population Recruiting NCT03651193
39 Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes. Recruiting NCT02143986
40 Single Patient Use of Tocilizumab for Treatment of Steroid Dependent, Active Systemic Onset Juvenile Idiopathic Arthritis Terminated NCT00868751

Search NIH Clinical Center for Systemic Onset Juvenile Idiopathic Arthritis

Genetic Tests for Systemic Onset Juvenile Idiopathic Arthritis

Anatomical Context for Systemic Onset Juvenile Idiopathic Arthritis

MalaCards organs/tissues related to Systemic Onset Juvenile Idiopathic Arthritis:

40
Neutrophil, Myeloid, T Cells

Publications for Systemic Onset Juvenile Idiopathic Arthritis

Articles related to Systemic Onset Juvenile Idiopathic Arthritis:

(show top 50) (show all 244)
# Title Authors PMID Year
1
Macrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities. 61
33388903 2021
2
Pulmonary Manifestations of Rheumatic Diseases in Children. 61
33228929 2021
3
Efficacy of moderately dosed etoposide in macrophage activation syndrome - hemophagocytic lymphohistiocytosis (MAS-HLH). 61
33589555 2021
4
Adult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach. 61
33673234 2021
5
Hemophagocytic Lymphohistiocytosis in a PICU of a Developing Economy: Clinical Profile, Intensive Care Needs, Outcome, and Predictors of Mortality. 61
33031348 2021
6
Safety and efficacy of anakinra as first-line or second-line therapy for systemic onset juvenile idiopathic arthritis - data from the German BIKER registry. 61
33148061 2021
7
Outcome of PatientsWith Systemic Onset Juvenile Idiopathic Arthritis With Macrophage Activation Syndrome at Onset. 61
33452788 2021
8
Aiming high: quantifying inflammation in systemic onset juvenile idiopathic arthritis (sJIA), a multi-faceted and complex inflammatory disease. 61
32844227 2020
9
Kawasaki disease mimickers. 61
33249696 2020
10
Long-term surveillance of biologic therapies in systemic-onset juvenile idiopathic arthritis: data from the German BIKER registry. 61
31846042 2020
11
Perimyocarditis as first sign of systemic onset juvenile idiopathic arthritis treated successfully with anakinra: a case-based review. 61
33525302 2020
12
Communicating Calf Swelling in Systemic-Onset Juvenile Idiopathic Arthritis. 61
32833912 2020
13
Rheumatologists' perspective on coronavirus disease 19 (COVID-19) and potential therapeutic targets. 61
32277367 2020
14
Autoinflammatory diseases in childhood, part 1: monogenic syndromes. 61
32065272 2020
15
Autoinflammatory diseases in childhood, part 2: polygenic syndromes. 61
32065273 2020
16
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
17
Case Report: Life-Threatening Macrophage Activation Syndrome With Fulminant Myocarditis Successfully Rescued by High Dose Intravenous Anakinra. 61
33537271 2020
18
Population pharmacokinetics of tacrolimus in pediatric patients with systemic-onset juvenile idiopathic arthritis: Initial dosage recommendations. 61
31772640 2019
19
Systemic-onset juvenile idiopathic arthritis and incomplete Kawasaki disease may belong to a single clinical syndrome within a spectrum of severity. 61
28980895 2019
20
Characterization of microbiota in systemic-onset juvenile idiopathic arthritis with different disease severities. 61
31616689 2019
21
Cyclosporine for Systemic Onset Juvenile Idiopathic Arthritis: Current Stand and Future Directions. 61
31154576 2019
22
Remission of Refractory Systemic-Onset Juvenile Idiopathic Arthritis After Treatment With Siltuximab. 61
29485546 2019
23
Extreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report. 61
31234906 2019
24
Achromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis. 61
31102385 2019
25
Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis. 61
30647180 2019
26
Treatment of patients with systemic-onset juvenile idiopathic arthritis with tacrolimus. 61
30783487 2019
27
Novel presentations of periodic fever syndromes: Discrepancies between genetic and clinical diagnoses. 61
30407166 2019
28
The role of extracellular histones in systemic-onset juvenile idiopathic arthritis. 61
30642364 2019
29
Systemic onset juvenile idiopathic arthritis: a single center experience. 61
32134578 2019
30
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. 61
30886992 2019
31
Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis. 61
30238379 2018
32
Tocilizumab in Giant Cell Arteritis. 61
29570475 2018
33
Methotrexate-Induced Vasculitis in a Child with Systemic Onset Juvenile Idiopathic Arthritis. 61
30431484 2018
34
Molecular genetic analysis for periodic fever syndromes: a supplemental role for the diagnosis of adult-onset Still's disease. 61
29909561 2018
35
Reversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis. 61
29426067 2018
36
Reasons for inactive disease and flare in systemic onset juvenile idiopathic arthritis patients during tocilizumab treatment. 61
29303703 2018
37
[Recognition of systemic-onset juvenile idiopathic arthritis at the genetic level]. 61
29518839 2018
38
[Systemic juvenile onset idiopathic arthritis and adult onset still disease]. 61
29461751 2018
39
Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience. 61
30505008 2018
40
Proteinuria in children with juvenile idiopathic arthritis: Making the case for early urinary screening. 61
29265055 2017
41
Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry. 61
29166924 2017
42
Canakinumab for first line steroid-free treatment in a child with systemic-onset juvenile idiopathic arthritis. 61
28266235 2017
43
Proinflammatory Cytokine Environments Can Drive Interleukin-17 Overexpression by γ/δ T Cells in Systemic Juvenile Idiopathic Arthritis. 61
28296284 2017
44
Protecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations. 61
28290112 2017
45
Clinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review. 61
28499374 2017
46
Systemic-onset juvenile idiopathic arthritis or incomplete Kawasaki disease: a diagnostic challenge. 61
27782865 2017
47
Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis. 61
28229369 2017
48
[Correctly adDRESS the cause of hemophagocytic lymphohistiocytosis]. 61
28161232 2017
49
The novel use of combined IL-1 and IL-6 inhibition in a patient with severe, aggressive, erosive, systemic-onset juvenile idiopathic arthritis. 61
28293458 2017
50
A Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack. 61
28416939 2017

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 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 15)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.1 TNF PRF1 IL6 HLA-DRB1 GZMB
2 11.97 TNF MEFV IL6
3 11.96 TNF IL6 HLA-DRB1
4
Show member pathways
11.91 TNF PRF1 GZMB
5 11.77 TNF MEFV IL6
6 11.73 TNF MIF IL6
7 11.66 TNF MIF IL6
8 11.54 TNF IL6 HLA-DRB1
9 11.47 TNF IL6 HLA-DRB1
10
Show member pathways
11.23 TNF MIF IL6
11
Show member pathways
11.12 TNF PRF1 GZMB
12 10.99 PRF1 HLA-DRB1
13 10.85 TNF IL6
14 10.77 TNF IL6
15 10.26 TNF IL6 HLA-DRB1

GO Terms for Systemic Onset Juvenile Idiopathic Arthritis

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of neurogenesis GO:0050768 9.48 TNF IL6
2 positive regulation of cytokine production involved in inflammatory response GO:1900017 9.46 TNF IL6
3 cytolysis GO:0019835 9.43 PRF1 GZMB
4 positive regulation of glial cell proliferation GO:0060252 9.4 TNF IL6
5 positive regulation of leukocyte adhesion to vascular endothelial cell GO:1904996 9.37 TNF IL6
6 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.33 TNF MIF IL6
7 positive regulation of neuroinflammatory response GO:0150078 9.32 TNF IL6
8 negative regulation of lipid storage GO:0010888 9.26 TNF IL6
9 inflammatory response GO:0006954 9.02 TNF MIF MEFV LACC1 IL6
10 vascular endothelial growth factor production GO:0010573 8.96 TNF IL6

Sources for Systemic Onset Juvenile Idiopathic Arthritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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