AOSD
MCID: ADL030
MIFTS: 58

Adult-Onset Still's Disease (AOSD)

Categories: Bone diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Adult-Onset Still's Disease

MalaCards integrated aliases for Adult-Onset Still's Disease:

Name: Adult-Onset Still's Disease 12 74 52 15 17
Adult-Onset Still Disease 12 58 71 32
Still's Disease, Adult-Onset 43
Adult Onset Still's Disease 12
Still's Disease Adult Onset 52
Stills Disease Adult-Onset 54
Adult Onset Still Disease 36
Wissler-Fanconi Syndrome 58
Adult Still's Disease 52
Wissler's Syndrome 71
Aosd 58

Characteristics:

Orphanet epidemiological data:

58
adult-onset still disease
Inheritance: Not applicable; Prevalence: 1-9/1000000 (France),1-9/1000000 (Japan),1-9/1000000 (Norway),1-9/100000 (Norway),1-9/100000 (Europe),1-9/1000000 (Turkey),1-9/100000 (Turkey); Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare renal diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:14256
KEGG 36 H01516
MeSH 43 D016706
SNOMED-CT 67 239920006
ICD10 32 M06.1
MESH via Orphanet 44 D014924 D016706
ICD10 via Orphanet 33 M06.1
UMLS via Orphanet 72 C0043195 C0085253
Orphanet 58 ORPHA829
UMLS 71 C0043195 C0085253

Summaries for Adult-Onset Still's Disease

NIH Rare Diseases : 52 Adult-onset Still's disease (AOSD) is an inflammatory disease characterized by high fevers, rash, sore throat, and joint pain. Several joints are often affected at the same time, and the joints are often stiff for several hours in the morning. Fevers develop each day (usually in the afternoon or evening), and the rash comes and goes with the fever. Other signs and symptoms may include abdominal pain, pleurisy , swollen lymph nodes , and weight loss. In some cases, the lungs and heart become inflamed. As it progresses, the disease may lead to chronic arthritis and other complications. The cause of AOSD is unknown, and no risk factors for the disease have been identified. It is diagnosed based on symptoms and the results of several blood tests. Blood test results may show a high white blood cell count , high C-reactive protein and ESR (measures of inflammation), high ferritin level, high fibrinogen level, and high levels of AST and ALT on liver function tests. AOSD is only diagnosed when other conditions such as infections and cancer are ruled out. Treatment aims to control inflammation, and prevent joint damage and other complications. Depending on severity and whether long-term management is needed, medications used may include non-steroidal inflammatory drugs, glucocorticoids, nonbiologic or biologic disease-modifying antirheumatic drugs (DMARDs ), or other biologic agents. The long-term outlook depends on severity, whether the disease is chronic (long-lasting), and what organs or parts of the body are involved. Of note, Still's disease which occurs in children (those under the age of 16) is known as systemic onset juvenile rheumatoid arthritis (JRA).

MalaCards based summary : Adult-Onset Still's Disease, also known as adult-onset still disease, is related to macrophage activation syndrome and disseminated intravascular coagulation, and has symptoms including exanthema An important gene associated with Adult-Onset Still's Disease is IL18 (Interleukin 18), and among its related pathways/superpathways are Innate Immune System and PEDF Induced Signaling. The drugs Interleukin 1 Receptor Antagonist Protein and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, testes and skin, and related phenotypes are splenomegaly and hepatomegaly

Disease Ontology : 12 A rheumatoid arthritis that is characterized by high fevers, rash, sore throat and joint pain.

KEGG : 36 Adult-onset Still disease (AOSD) is a systemic inflammatory disorder. The disease manifestations are protean ranging from high fever, arthralgia, skin rash, sore throat, lymphadenopathy, and hepatosplenomegaly accompanied by systemic manifestations. Complications of AOSD include transient pulmonary hypertension, macrophage activation syndrome, diffuse alveolar hemorrhage, thrombotic thrombocytopenic purpura and amyloidosis. Common laboratory abnormalities include neutrophilic leukocytosis, abnormal liver function tests, and elevated acute-phase reactants (ESR, CRP, ferritin). The exact pathogenesis of AOSD is unknown. Several factors such as genetics, infectious (bacterial and viral) agents, and environmental factors have been thought to play a causative role. Although no familial trend has been reported in AOSD, some studies have reported an association between AOSD and gene polymorphism of HLA, IL-18, and MEFV. Recent advances have revealed a pivotal role of proinflammatory cytokines such as TNF-alpha, IL-1, IL-6, IL-8, and IL-18 in disease pathogenesis. Treatment consists of anti-inflammatory medications. Non-steroidal anti-inflammatory drugs have limited efficacy, and corticosteroid therapy and disease-modifying anti-rheumatic drugs are usually required.

Wikipedia : 74 Adult-onset Still's disease (AOSD) is a form of Still's disease, a rare systemic autoinflammatory... more...

Related Diseases for Adult-Onset Still's Disease

Diseases related to Adult-Onset Still's Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 814)
# Related Disease Score Top Affiliating Genes
1 macrophage activation syndrome 33.2 TNFRSF1A TNF IL18
2 disseminated intravascular coagulation 31.4 TNF IL6 CRP
3 vasculitis 31.3 TNF MEFV IL6 CRP
4 purpura 31.2 TNF MEFV IL6 CRP
5 myocarditis 31.2 TNF IL6 IL1B IL18
6 enthesopathy 31.1 TNF MEFV CRP
7 hemophagocytic lymphohistiocytosis 31.0 TNF IL18 CXCL8
8 systemic onset juvenile idiopathic arthritis 31.0 TNF IL1RN IL1RAPL2 IL1B IL18
9 amyloidosis aa 31.0 MEFV CRP
10 herpes zoster 30.9 TNF HLA-DRB1 CRP
11 systemic autoimmune disease 30.9 IL6 CRP
12 urticaria 30.8 TNF NLRP3 IL6 CRP
13 rheumatic fever 30.8 TNF IL6 HLA-DRB1
14 inflammatory myopathy with abundant macrophages 30.7 TNFRSF1A MEFV
15 rubella 30.7 TNF IL6 IL1B HLA-DRB1
16 iron deficiency anemia 30.6 TNF IL6 CRP
17 juvenile arthritis 30.6 MIF IL6 IL1RAPL2 IL1R1 CXCL8 CRP
18 cryptogenic organizing pneumonia 30.6 TNF CXCL8 CRP
19 spondylitis 30.6 TNF IL6 IL1RN CRP
20 drug reaction with eosinophilia and systemic symptoms 30.6 TNF CRP
21 polyarteritis nodosa 30.5 MEFV IL6 HLA-DRB1 CRP
22 endocarditis 30.5 TNF IL6 CXCL8 CRP
23 whipple disease 30.5 TNF HLA-DRB1 CRP
24 syphilis 30.5 TNF IL6 HLA-DRB1 CRP
25 exanthem 30.5 TNF NLRP3 MEFV IL6 IL1RN IL1B
26 thrombocytosis 30.5 IL6 IL1B CRP
27 infective endocarditis 30.5 TNF IL6 CRP
28 scleritis 30.5 TNF IL1RN IL1RAPL2 CRP
29 bursitis 30.5 TNF IL1B CRP
30 meningoencephalitis 30.5 TNF IL6 IL1B CRP
31 acute myocarditis 30.4 TNF IL1B CRP
32 hepatic encephalopathy 30.4 TNF IL6 IL18
33 cytomegalovirus infection 30.4 TNF IL6 IL1B CXCL8
34 listeriosis 30.4 TNF IL6 IL1B
35 lymphadenitis 30.4 TNF IL1B IL18 CXCL8 CRP
36 extrapulmonary tuberculosis 30.4 TNF IL1RN IL1B CRP
37 hepatitis a 30.4 TNF IL1B CXCL8
38 uremia 30.4 TNF IL6 CRP
39 erythema multiforme 30.3 TNF IL6 CXCL8
40 adult respiratory distress syndrome 30.3 TNF IL6 IL1R1 IL1B CXCL8
41 chickenpox 30.3 TNF IL6 CRP
42 pericardial effusion 30.3 TNFRSF1A NLRP3 IL6 IL1RN CRP
43 lepromatous leprosy 30.3 TNF MIF IL18
44 guillain-barre syndrome 30.3 TNFRSF1A TNF HLA-DRB1
45 lung disease 30.3 TNF IL6 IL1B IL18 CXCL8 CRP
46 panuveitis 30.3 TNF IL6 HLA-DRB1 CRP
47 pulmonary hypertension 30.3 TNF IL6 IL1B CXCL8 CRP
48 crohn's colitis 30.2 TNF IL1B CXCL8
49 candidiasis 30.2 TNF IL6 IL1B CXCL8
50 pleurisy 30.2 TNF MEFV IL1RN CXCL8 CRP

Graphical network of the top 20 diseases related to Adult-Onset Still's Disease:



Diseases related to Adult-Onset Still's Disease

Symptoms & Phenotypes for Adult-Onset Still's Disease

Human phenotypes related to Adult-Onset Still's Disease:

58 31 (show all 29)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 splenomegaly 58 31 hallmark (90%) Very frequent (99-80%) HP:0001744
2 hepatomegaly 58 31 hallmark (90%) Very frequent (99-80%) HP:0002240
3 arthritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0001369
4 fatigue 58 31 hallmark (90%) Very frequent (99-80%) HP:0012378
5 fever 58 31 hallmark (90%) Very frequent (99-80%) HP:0001945
6 skin rash 58 31 hallmark (90%) Very frequent (99-80%) HP:0000988
7 pruritus 58 31 hallmark (90%) Very frequent (99-80%) HP:0000989
8 arthralgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002829
9 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
10 restrictive ventilatory defect 58 31 hallmark (90%) Very frequent (99-80%) HP:0002091
11 joint swelling 58 31 hallmark (90%) Very frequent (99-80%) HP:0001386
12 elevated c-reactive protein level 58 31 hallmark (90%) Very frequent (99-80%) HP:0011227
13 elevated erythrocyte sedimentation rate 58 31 hallmark (90%) Very frequent (99-80%) HP:0003565
14 leukocytosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0001974
15 neutrophilia 58 31 hallmark (90%) Very frequent (99-80%) HP:0011897
16 pericarditis 58 31 frequent (33%) Frequent (79-30%) HP:0001701
17 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
18 myalgia 58 31 frequent (33%) Frequent (79-30%) HP:0003326
19 pleuritis 58 31 frequent (33%) Frequent (79-30%) HP:0002102
20 generalized lymphadenopathy 58 31 frequent (33%) Frequent (79-30%) HP:0008940
21 hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012115
22 elevated hepatic transaminase 58 31 occasional (7.5%) Occasional (29-5%) HP:0002910
23 myocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012819
24 recurrent pharyngitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100776
25 meningitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001287
26 cartilage destruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0100773
27 bone marrow hypocellularity 58 31 occasional (7.5%) Occasional (29-5%) HP:0005528
28 abnormal circulating lipid concentration 31 occasional (7.5%) HP:0003119
29 abnormality of lipid metabolism 58 Occasional (29-5%)

UMLS symptoms related to Adult-Onset Still's Disease:


exanthema

MGI Mouse Phenotypes related to Adult-Onset Still's Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.13 IL18 IL18BP IL1B IL1R1 IL1RN IL6
2 homeostasis/metabolism MP:0005376 10.07 CRP IL18 IL18BP IL1B IL1R1 IL1RN
3 immune system MP:0005387 10 CRP IL18 IL18BP IL1B IL1R1 IL1RN
4 integument MP:0010771 9.61 IL18 IL1B IL1R1 IL1RN IL6 MEFV
5 liver/biliary system MP:0005370 9.17 IL18BP IL1R1 IL6 MEFV NLRP3 TNF

Drugs & Therapeutics for Adult-Onset Still's Disease

Drugs for Adult-Onset Still's Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 30)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Interleukin 1 Receptor Antagonist Protein Phase 3
2 Antirheumatic Agents Phase 3
3
Leflunomide Approved, Investigational Phase 2 75706-12-6 3899
4
leucovorin Approved Phase 2 58-05-9 6006 143
5
Methotrexate Approved Phase 2 59-05-2, 1959-05-2 126941
6
Azathioprine Approved Phase 2 446-86-6 2265
7
Rilonacept Approved, Investigational Phase 2 501081-76-1 104924
8
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9 Vitamin B Complex Phase 2
10 Folic Acid Antagonists Phase 2
11 Folate Phase 2
12 Immunosuppressive Agents Phase 2
13 Dermatologic Agents Phase 2
14 Antimetabolites Phase 2
15 Vitamin B9 Phase 2
16 Anti-Inflammatory Agents Phase 2
17 interferons Phase 2
18 Interferon-gamma Phase 2
19 Janus Kinase Inhibitors Phase 2
20 Immunologic Factors Phase 2
21 Immunoglobulins Phase 2
22 Antibodies Phase 2
23 Antibodies, Monoclonal Phase 2
24
Methylprednisolone Approved, Vet_approved Phase 1 83-43-2 6741
25
Prednisolone phosphate Approved, Vet_approved Phase 1 302-25-0
26
Methylprednisolone hemisuccinate Approved Phase 1 2921-57-5
27 Prednisolone acetate Approved, Vet_approved Phase 1 52-21-1
28
Prednisolone Approved, Vet_approved Phase 1 50-24-8 5755
29
Prednisolone hemisuccinate Experimental Phase 1 2920-86-7
30 Methylprednisolone Acetate Phase 1

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 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
2 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
3 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
4 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
5 Continuation of a Pilot Open-Label Study of IL 1 Trap in Adult Subjects With Autoinflammatory Diseases: A Therapeutic Approach to Study Pathogenesis Completed NCT00094900 Phase 2 IL-1 Trap
6 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
7 Interleukin-1 (IL-1) and Interferon Gamma (IFNg) Inhibition During COVID 19 Inflammation: Randomized, Controlled Study Assessing Efficacy and Safety of Anakinra and Ruxolitinib Not yet recruiting NCT04366232 Phase 2 Anakinra alone (stages 2b/3);Anakinra and Ruxolitinib (overcome stage 3)
8 Canakinumab for Treatment of Adult Onset Still's Disease to Achieve Reduction of Arthritic Manifestation Terminated NCT02204293 Phase 2 Canakinumab;Placebo Injection;Canakinumab
9 A Dose-Block Randomized, Placebo Controlled (Double-blind), Active Controlled(Open-label), Dose-escalation Study to Investigate the Tolerability, and Pharmacokinetics/Pharmacodynamics of HL2351 After a Single Subcutaneous Administration in Healthy Male Subjects Completed NCT02175056 Phase 1
10 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
11 Admission of Adult-onset Still Disease Patients in the ICU: a French Retrospective Multicentric Study Completed NCT03276650
12 Rheumatology Patient Registry and Biorepository Recruiting NCT04402086
13 Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes. Recruiting NCT02143986
14 Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still s Disease and Related Inflammatory Conditions Recruiting NCT03510442
15 Multi-center Registration Study of Adult Onset Still's Disease in Chinese Population Not yet recruiting NCT03651193

Search NIH Clinical Center for Adult-Onset Still's Disease

Cochrane evidence based reviews: still's disease, adult-onset

Genetic Tests for Adult-Onset Still's Disease

Anatomical Context for Adult-Onset Still's Disease

MalaCards organs/tissues related to Adult-Onset Still's Disease:

40
Liver, Testes, Skin, Neutrophil, Lymph Node, Lung, Heart

Publications for Adult-Onset Still's Disease

Articles related to Adult-Onset Still's Disease:

(show top 50) (show all 1351)
# Title Authors PMID Year
1
[Adult-onset Still's disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood]. 61 54
19838717 2009
2
Functional association of interleukin 18 gene -607 (C/A) promoter polymorphisms with disease course in Chinese patients with adult-onset Still's disease. 61 54
19684151 2009
3
Lymph node IL-18 expression in adult-onset Still's disease. 54 61
19213748 2009
4
A case report of a patient with refractory adult-onset Still's disease who was successfully treated with tocilizumab over 6 years. 54 61
18762861 2009
5
Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. 54 61
17947302 2008
6
The future of the IL-1 receptor antagonist anakinra: from rheumatoid arthritis to adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis. 61 54
18321234 2008
7
Steroid-resistant adult-onset Still's disease which showed a quick response to methotrexate. 61 54
18271807 2008
8
Increased apoptosis of peripheral blood lymphocytes and its association with interleukin-18 in patients with active untreated adult-onset Still's disease. 54 61
18050226 2007
9
Severe systemic inflammatory response syndrome in a patient with adult onset Still's disease treated with the anti-IL1 drug anakinra: a case report. 54 61
18078628 2007
10
Association of interleukin 18 polymorphisms with adult onset Still's disease in Korea. 54 61
17611971 2007
11
Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? 54 61
17538564 2007
12
Interleukin 1 receptor antagonist therapy-induced thrombocytopenia in adult onset Still's disease. 54 61
17407250 2007
13
Interleukin-18 in chronic joint diseases. 61 54
17317618 2007
14
[Blood cytokine levels as a clinical laboratory test]. 61 54
17441472 2007
15
Cardiac death in a patient with adult-onset Still's disease treated with the interleukin 1 receptor inhibitor anakinra. 54 61
17179171 2007
16
A promoter haplotype of the interleukin-18 gene is associated with juvenile idiopathic arthritis in the Japanese population. 54 61
16563174 2006
17
-137 (G/C) IL-18 promoter polymorphism in patients with inflammatory bowel disease. 54 61
16316892 2005
18
Association of intercellular adhesion molecule-1 with clinical manifestations and interleukin-18 in patients with active, untreated adult-onset Still's disease. 54 61
15934126 2005
19
Pathogenesis of hemophagocytic syndrome (HPS). 54 61
15003190 2004
20
Interleukin 18 and hepatocyte growth factor in fulminant hepatic failure of adult onset Still's disease. 54 61
12734913 2003
21
Association between adult-onset Still's disease and interleukin-18 gene polymorphisms. 54 61
12424620 2002
22
Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still's disease. 61 54
11465725 2001
23
Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still's disease. 54 61
11263769 2001
24
A shock associated with adult-onset Still's disease. 54 61
11235787 2001
25
High serum level of macrophage-colony stimulating factor (M-CSF) in adult-onset Still's disease. 61 54
10371293 1999
26
Interleukin 6: a possible marker of disease activity in adult onset Still's disease. 54 61
8978961 1996
27
COVID-19 gone bad: A new character in the spectrum of the hyperferritinemic syndrome? 61
32387470 2020
28
Clinical characteristics and comorbidities in adult-onset Still's disease using a large US administrative claims database. 61
31965185 2020
29
Rheumatologists' perspective on coronavirus disease 19 (COVID-19) and potential therapeutic targets. 61
32277367 2020
30
Comment on: Clinical characteristics and comorbidities in adult-onset Still's disease using a large US administrative claims database: reply. 61
32375168 2020
31
Comment on: Clinical characteristics and comorbidities in adult-onset Still's disease using a large US administrative claims database. 61
32375171 2020
32
Adult Onset Still's Disease and Radiotherapy treatment for breast cancer: Case report about management of this rare association and literature review. 61
32477018 2020
33
Translating IL-6 biology into effective treatments. 61
32327746 2020
34
Severe pyrexia from nivolumab-resistant advanced melanoma after successful combined therapy with encorafenib plus binimetinib. 61
32293049 2020
35
Tofacitinib in refractory adult-onset Still's disease: 14 cases from a single centre in China. 61
32079571 2020
36
Purtscher-like retinopathy in adult-onset Still's disease, complicated by treatment-related central serous chorioretinopathy. 61
32140617 2020
37
Elevated plasma galectin-3 levels and their correlation with disease activity in adult-onset Still's disease. 61
31960208 2020
38
Treatment of refractory adult onset Still's disease with tocilizumab-a single centre experience and literature review. 61
32506202 2020
39
Parenchymal lung disease in adult onset Still's disease: an emergent marker of disease severity-characterisation and predictive factors from Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort of patients. 61
32571407 2020
40
Parvovirus-Induced Adult-Onset Still Disease Presenting With Elevated Liver Transaminases. 61
32541612 2020
41
Combination value of biomarkers in discriminating adult onset still's disease and sepsis. 61
32430612 2020
42
Anakinra in COVID-19 therapy: what have we learned from adult-onset Still's disease? 61
32456767 2020
43
Tocilizumab-induced anaphylaxis in patients with adult-onset Still's disease and systemic juvenile idiopathic arthritis: a case-based review. 61
31598752 2020
44
Storm, typhoon, cyclone or hurricane in patients with COVID-19? Beware of the same storm that has a different origin. 61
32423970 2020
45
Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome. 61
32373790 2020
46
The treatment of adult-onset Still's disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature. 61
32452353 2020
47
Anxiety and depression in adult-onset Still's disease patients and associations with health-related quality of life. 61
32447600 2020
48
Interleukin-18 and fibroblast growth factor 2 in combination is a useful diagnostic biomarker to distinguish adult-onset Still's disease from sepsis. 61
32381117 2020
49
Canakinumab for Treatment of Adult-Onset Still's Disease to Achieve Reduction of Arthritic Manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial. 61
32404342 2020
50
[Still's disease as biphasic disorder : Current knowledge on pathogenesis and novel treatment approaches]. 61
32253510 2020

Variations for Adult-Onset Still's Disease

Expression for Adult-Onset Still's Disease

Search GEO for disease gene expression data for Adult-Onset Still's Disease.

Pathways for Adult-Onset Still's Disease

Pathways related to Adult-Onset Still's Disease according to GeneCards Suite gene sharing:

(show top 50) (show all 75)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.86 TNFRSF1A TNF TLR7 NLRP3 MIF MEFV
2
Show member pathways
13.72 TNFRSF1A TNF MIF IL6 IL37 IL1RN
3
Show member pathways
13.58 TNFRSF1A TNF MAP4K3 IL6 IL1R1 IL1B
4
Show member pathways
13.45 TNFRSF1A TNF TLR7 IL6 IL1R1 IL1B
5
Show member pathways
13.35 TNFRSF1A TNF IL6 IL37 IL1RN IL1R1
6
Show member pathways
13.34 TNFRSF1A TNF IL6 IL1R1 IL1B IL18
7
Show member pathways
13.1 TNFRSF1A TNF TLR7 IL6 IL1B CXCL8
8
Show member pathways
12.99 TNFRSF1A TNF IL6 IL1R1 IL1B CXCL8
9
Show member pathways
12.89 TNF IL6 IL1B HLA-DRB1 CXCL8
10 12.84 TNFRSF1A TNF IL6 IL1B HLA-DRB1
11 12.81 TNFRSF1A TNF MAP4K3 IL1R1 IL1B
12
Show member pathways
12.77 TNFRSF1A TNF TLR7 IL6 IL1R1 IL1B
13
Show member pathways
12.76 TNFRSF1A TNF TLR7 NLRP3 IL6 IL1B
14
Show member pathways
12.67 TNF IL6 IL1R1 IL1B IL18 HLA-DRB1
15
Show member pathways
12.64 TNFRSF1A TNF TLR7 IL1R1 IL1B
16 12.62 TNFRSF1A TNF TLR7 IL1RN IL1B
17 12.45 TNFRSF1A TNF NLRP3 IL1R1 IL1B IL18
18
Show member pathways
12.42 TNF MIF IL6 IL1R1 IL1B CXCL8
19
Show member pathways
12.42 TNFRSF1A TNF IL6 IL37 IL18 CXCL8
20 12.41 TNFRSF1A TNF IL6 IL1R1 HLA-DRB1
21
Show member pathways
12.38 TNF IL6 IL1B CXCL8
22
Show member pathways
12.38 TNF IL6 IL1B CRP
23 12.34 TNFRSF1A TNF NLRP3 IL6 IL1B IL18
24
Show member pathways
12.33 TNF TLR7 NLRP3 IL6 IL37 IL1RN
25
Show member pathways
12.32 TNF TLR7 IL6 CXCL8
26
Show member pathways
12.32 TNF IL6 IL1B IL18 CXCL8
27 12.32 TNFRSF1A TNF IL6 IL1B IL18 HLA-DRB1
28
Show member pathways
12.31 TNFRSF1A TNF IL1B HLA-DRB1
29
Show member pathways
12.27 TNF NLRP3 IL6 IL1B
30
Show member pathways
12.26 TNFRSF1A TNF NLRP3 IL1B
31 12.26 TNF IL6 IL1RN IL1B CXCL8
32
Show member pathways
12.26 TNF TLR7 IL6 IL1R1 IL1B CXCL8
33 12.24 TNFRSF1A TNF TLR7 IL6
34 12.23 TNF NLRP3 MEFV IL6 IL1B IL18
35 12.15 TNFRSF1A TNF IL1R1 IL1B
36 12.11 TNFRSF1A TNF IL1R1 IL1B
37 12.1 TNF IL6 IL1B IL18 CXCL8
38 12.07 TNF MIF IL6 IL1R1 IL1B CXCL8
39
Show member pathways
12.06 TNF TLR7 IL6 IL1B IL18
40
Show member pathways
12.05 IL1R1 IL1B IL18 HLA-DRB1
41 12.04 TNFRSF1A TNF IL6 IL1B
42 12.04 TNFRSF1A TNF IL1R1 IL1B CXCL8
43 12.04 TNFRSF1A TNF NLRP3 IL6 IL1R1 IL1B
44 12.03 TNF IL6 IL1R1 IL1B CXCL8
45 12 TNF IL6 IL1R1 IL1B HLA-DRB1
46 11.99 TNF IL6 IL1B CXCL8
47
Show member pathways
11.94 TLR7 IL6 IL1R1 IL1B
48 11.9 TNF IL6 IL1B IL18 HLA-DRB1 CXCL8
49 11.88 TNF IL1R1 IL1B IL18
50 11.88 TNF NLRP3 MEFV IL6 IL1B IL18

GO Terms for Adult-Onset Still's Disease

Cellular components related to Adult-Onset Still's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.77 TNFRSF1A TNF NLRP3 MIF IL6 IL37
2 extracellular space GO:0005615 9.4 TNFRSF1A TNF MIF IL6 IL37 IL1RN

Biological processes related to Adult-Onset Still's Disease according to GeneCards Suite gene sharing:

(show top 50) (show all 51)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.2 TNFRSF1A TLR7 NLRP3 IL6 IL1RN IL1RAPL2
2 positive regulation of gene expression GO:0010628 10.08 TNF IL6 IL37 IL1B IL18 CRP
3 MAPK cascade GO:0000165 10 TNF MAP4K3 IL1B IL18
4 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.97 TNFRSF1A TNF IL37 IL1B HLA-DRB1
5 positive regulation of protein phosphorylation GO:0001934 9.95 TNF MIF IL1B HLA-DRB1
6 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.95 TNF NLRP3 IL6 IL1B IL18
7 defense response GO:0006952 9.94 TNFRSF1A TNF NLRP3 CXCL8
8 defense response to Gram-positive bacterium GO:0050830 9.91 TNF NLRP3 IL6 CRP
9 regulation of inflammatory response GO:0050727 9.9 TNF NLRP3 IL37 IL1R1
10 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.88 TNF MIF IL6
11 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.88 TNFRSF1A TNF IL6 IL18
12 cellular response to mechanical stimulus GO:0071260 9.87 TNFRSF1A TLR7 IL1B
13 cellular response to organic cyclic compound GO:0071407 9.86 TNF IL1B IL18
14 I-kappaB kinase/NF-kappaB signaling GO:0007249 9.86 TNFRSF1A TNF TLR7
15 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.86 TNF TLR7 IL1B IL18
16 response to glucocorticoid GO:0051384 9.85 TNF IL6 IL1RN
17 immune response GO:0006955 9.85 TNF TLR7 IL6 IL37 IL1RN IL1R1
18 positive regulation of interleukin-6 production GO:0032755 9.84 TNF TLR7 IL6 IL1B
19 positive regulation of smooth muscle cell proliferation GO:0048661 9.83 TNF IL6 IL18
20 lipopolysaccharide-mediated signaling pathway GO:0031663 9.8 TNF IL1B IL18
21 positive regulation of interleukin-8 production GO:0032757 9.8 TNF TLR7 IL1B
22 positive regulation of interferon-gamma production GO:0032729 9.8 TNF IL1R1 IL1B IL18
23 positive regulation of inflammatory response GO:0050729 9.8 TNFRSF1A TNF TLR7 IL1B IL18
24 cellular response to lipopolysaccharide GO:0071222 9.8 TNF NLRP3 IL6 IL37 IL1B CXCL8
25 negative regulation of neurogenesis GO:0050768 9.79 TNF IL6 IL1B
26 positive regulation of chemokine production GO:0032722 9.77 TNF TLR7 IL6
27 positive regulation of glial cell proliferation GO:0060252 9.76 TNF IL6 IL1B
28 positive regulation of chemokine biosynthetic process GO:0045080 9.71 TNF IL1B
29 positive regulation of chemokine (C-X-C motif) ligand 2 production GO:2000343 9.71 TNF MIF
30 positive regulation of ceramide biosynthetic process GO:2000304 9.7 TNFRSF1A TNF
31 neutrophil activation GO:0042119 9.7 IL18 CXCL8
32 activation of MAPKKK activity GO:0000185 9.7 TNF MAP4K3
33 T-helper 1 type immune response GO:0042088 9.7 IL18BP IL18 HLA-DRB1
34 positive regulation of granulocyte macrophage colony-stimulating factor production GO:0032725 9.69 IL1B IL18
35 interleukin-6 production GO:0032635 9.69 IL1B IL18
36 negative regulation of lipid storage GO:0010888 9.69 TNF IL6 CRP
37 death-inducing signaling complex assembly GO:0071550 9.68 TNFRSF1A TNF
38 negative regulation of interleukin-1-mediated signaling pathway GO:2000660 9.67 IL6 IL1RN
39 positive regulation of T-helper 2 cell cytokine production GO:2000553 9.67 NLRP3 IL6
40 positive regulation of T-helper 2 cell differentiation GO:0045630 9.67 NLRP3 IL18
41 inflammatory response to antigenic stimulus GO:0002437 9.67 IL37 IL1RN IL1B HLA-DRB1
42 interleukin-18-mediated signaling pathway GO:0035655 9.66 IL18BP IL18
43 interleukin-1 beta production GO:0032611 9.66 NLRP3 IL1B
44 positive regulation of fever generation GO:0031622 9.65 TNF IL1B
45 regulation of establishment of endothelial barrier GO:1903140 9.65 TNFRSF1A TNF IL1B
46 cytokine-mediated signaling pathway GO:0019221 9.65 TNFRSF1A TNF IL6 IL37 IL1RN IL1RAPL2
47 sequestering of triglyceride GO:0030730 9.64 TNF IL1B
48 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.63 TNF IL1B
49 positive regulation of T-helper 1 cell cytokine production GO:2000556 9.61 IL1R1 IL1B IL18
50 positive regulation of neuroinflammatory response GO:0150078 9.56 TNF IL6 IL1B IL18

Molecular functions related to Adult-Onset Still's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.23 TNF MIF IL6 IL37 IL1RN IL1B
2 interleukin-1 receptor activity GO:0004908 9.16 IL1RAPL2 IL1R1
3 interleukin-1 receptor binding GO:0005149 9.13 IL37 IL1RN IL1B

Sources for Adult-Onset Still's Disease

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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