AOSD
MCID: ADL030
MIFTS: 59

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 75 53 15 17
Adult-Onset Still Disease 12 59 72 33
Still's Disease, Adult-Onset 44
Adult Onset Still's Disease 12
Still's Disease Adult Onset 53
Stills Disease Adult-Onset 55
Adult Onset Still Disease 37
Wissler-Fanconi Syndrome 59
Adult Still's Disease 53
Wissler's Syndrome 72
Aosd 59

Characteristics:

Orphanet epidemiological data:

59
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:



External Ids:

Disease Ontology 12 DOID:14256
KEGG 37 H01516
MeSH 44 D016706
ICD10 33 M06.1
MESH via Orphanet 45 D014924 D016706
ICD10 via Orphanet 34 M06.1
UMLS via Orphanet 73 C0043195 C0085253
Orphanet 59 ORPHA829
UMLS 72 C0043195 C0085253

Summaries for Adult-Onset Still's Disease

NIH Rare Diseases : 53 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 exanthem and vasculitis, 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 ERK Signaling. The drugs Interleukin 1 Receptor Antagonist Protein and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include testes, liver and skin, and related phenotypes are arthritis and splenomegaly

Disease Ontology : 12 An arthritis that is a rare from of inflammatory arthritis characterized by fevers, rash, and joint pain.

KEGG : 37
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 : 75 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 565)
# Related Disease Score Top Affiliating Genes
1 exanthem 31.6 TNF IL6 IL1RN CRP
2 vasculitis 31.3 TNF MEFV CRP
3 purpura 31.2 TNF IL6 CRP
4 autoimmune disease 31.2 TNF IL6 IL1B
5 pharyngitis 31.0 MEFV IL1B CRP
6 amyloidosis aa 30.9 MEFV CRP
7 neutrophilia, hereditary 30.8 IL6 CRP
8 pericardial effusion 30.8 TNFRSF1A IL6 CRP
9 acute respiratory distress syndrome 30.8 TNF IL6 IL1B
10 endocarditis 30.8 TNF IL6 CRP
11 pustulosis of palm and sole 30.8 TNF IL1RN
12 cardiac tamponade 30.7 TNFRSF1A IL1RN
13 polyarteritis nodosa 30.7 MEFV IL6 CRP
14 fasciitis 30.7 TNF IL6 IL18
15 drug reaction with eosinophilia and systemic symptoms 30.6 TNF CRP
16 lymphadenitis 30.6 TNF IL1B IL18
17 erysipelas 30.6 TNF MEFV
18 pleurisy 30.6 TNF MEFV CRP
19 cardiogenic shock 30.5 TNF IL6
20 subacute thyroiditis 30.5 IL6 CRP
21 meningitis 30.5 TNF IL6 IL1B CRP
22 inflammatory myopathy with abundant macrophages 30.4 TNFRSF1A MEFV
23 melioidosis 30.4 TNF IL6 IL18
24 cytomegalovirus infection 30.4 TNF IL6 IL1B
25 infective endocarditis 30.4 TNF IL6 CRP
26 necrotizing fasciitis 30.4 IL6 IL1RN IL18
27 end stage renal failure 30.4 TNF IL6 CRP
28 bronchiolitis 30.4 TNF IL6 CRP
29 systemic onset juvenile idiopathic arthritis 30.3 TNF MIF MEFV IL18
30 toxic shock syndrome 30.3 TNF IL6 IL1B
31 crohn's colitis 30.3 TNF IL1B
32 pneumonia 30.3 TNF IL6 IL1B CRP
33 interstitial lung disease 30.2 TNF IL6 IL1B
34 spondylitis 30.2 TNF IL6 IL1RN CRP
35 colitis 30.2 TNF IL6 IL1B
36 listeriosis 30.1 TNF IL6
37 sarcoidosis 1 30.1 TNF IL1B IL18
38 tonsillitis 30.1 TNF IL6 IL1B
39 toxoplasmosis 30.1 TNF IL6 IL1B
40 rheumatic disease 30.1 TNF IL1R1 IL1B IL18 CRP
41 osteoarthritis 30.0 TNF IL6 IL1B
42 bacterial meningitis 30.0 TNF IL6 IL1B CRP
43 lyme disease 29.9 TNF IL6 IL1B CRP
44 chikungunya 29.9 TNF IL6 IL1B
45 periodic fever, familial, autosomal dominant 29.9 TNFRSF1A TNF MEFV
46 keratoconjunctivitis sicca 29.9 TNF IL6 IL1B
47 bacterial infectious disease 29.8 TNF IL6 IL1B IL18 CRP
48 cryopyrin-associated periodic syndrome 29.7 IL6 IL1RN IL1R1 CRP
49 pyelonephritis 29.7 TNF IL6 IL1B CRP
50 appendicitis 29.7 TNF IL6 IL1B 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:

59 32 (show all 29)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthritis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001369
2 splenomegaly 59 32 hallmark (90%) Very frequent (99-80%) HP:0001744
3 hepatomegaly 59 32 hallmark (90%) Very frequent (99-80%) HP:0002240
4 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
5 fatigue 59 32 hallmark (90%) Very frequent (99-80%) HP:0012378
6 joint swelling 59 32 hallmark (90%) Very frequent (99-80%) HP:0001386
7 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
8 pruritus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000989
9 skin rash 59 32 hallmark (90%) Very frequent (99-80%) HP:0000988
10 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
11 restrictive ventilatory defect 59 32 hallmark (90%) Very frequent (99-80%) HP:0002091
12 leukocytosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001974
13 elevated c-reactive protein level 59 32 hallmark (90%) Very frequent (99-80%) HP:0011227
14 elevated erythrocyte sedimentation rate 59 32 hallmark (90%) Very frequent (99-80%) HP:0003565
15 neutrophilia 59 32 hallmark (90%) Very frequent (99-80%) HP:0011897
16 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
17 pericarditis 59 32 frequent (33%) Frequent (79-30%) HP:0001701
18 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
19 pleuritis 59 32 frequent (33%) Frequent (79-30%) HP:0002102
20 generalized lymphadenopathy 59 32 frequent (33%) Frequent (79-30%) HP:0008940
21 hepatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012115
22 bone marrow hypocellularity 59 32 occasional (7.5%) Occasional (29-5%) HP:0005528
23 elevated hepatic transaminase 59 32 occasional (7.5%) Occasional (29-5%) HP:0002910
24 myocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012819
25 recurrent pharyngitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100776
26 meningitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001287
27 cartilage destruction 59 32 occasional (7.5%) Occasional (29-5%) HP:0100773
28 abnormal circulating lipid concentration 32 occasional (7.5%) HP:0003119
29 abnormality of lipid metabolism 59 Occasional (29-5%)

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


exanthema

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

46 (show all 15)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.22 CRP CSF1 IL18 IL1B IL1R1 IL1RN
2 immune system MP:0005387 10.2 CRP CSF1 IL18 IL1B IL1R1 IL1RN
3 cardiovascular system MP:0005385 10.19 CRP CSF1 IL18 IL1B IL1R1 IL1RN
4 hematopoietic system MP:0005397 10.19 CSF1 IL18 IL1B IL1R1 IL1RN IL6
5 cellular MP:0005384 10.18 CSF1 IL18 IL1R1 IL1RN IL6 MEFV
6 growth/size/body region MP:0005378 10.16 CSF1 IL18 IL1B IL1R1 IL1RN IL6
7 integument MP:0010771 10.09 CSF1 IL18 IL1B IL1R1 IL1RN IL6
8 mortality/aging MP:0010768 10.02 CSF1 IL18 IL1B IL1R1 IL1RN IL6
9 adipose tissue MP:0005375 9.95 IL18 IL1R1 IL6 TNF TNFRSF1A
10 liver/biliary system MP:0005370 9.88 CSF1 IL1R1 IL6 MEFV TNF TNFRSF1A
11 muscle MP:0005369 9.8 CSF1 IL18 IL1R1 IL6 TNF TNFRSF1A
12 nervous system MP:0003631 9.76 CSF1 IL18 IL1B IL1R1 IL6 MIF
13 neoplasm MP:0002006 9.73 IL1B IL1R1 IL6 MIF TNF TNFRSF1A
14 skeleton MP:0005390 9.56 CSF1 IL1B IL1R1 IL1RN IL6 MEFV
15 vision/eye MP:0005391 9.1 CSF1 IL18 IL1R1 IL6 TNF TNFRSF1A

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 40)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Interleukin 1 Receptor Antagonist Protein Phase 3
2 Antirheumatic Agents Phase 3
3
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
4
leucovorin Approved Phase 2 58-05-9 143 6006
5
Azathioprine Approved Phase 2 446-86-6 2265
6
Leflunomide Approved, Investigational Phase 2 75706-12-6 3899
7
Rilonacept Approved, Investigational Phase 2 501081-76-1 104924
8
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9 Folic Acid Antagonists Phase 2
10 Nucleic Acid Synthesis Inhibitors Phase 2
11 Vitamin B9 Phase 2
12 Immunosuppressive Agents Phase 2
13 Folate Phase 2
14 Vitamin B Complex Phase 2
15 Dermatologic Agents Phase 2
16 Antimetabolites Phase 2
17 Antimetabolites, Antineoplastic Phase 2
18 Immunologic Factors Phase 2
19 Antibodies Phase 2
20 Antibodies, Monoclonal Phase 2
21 Immunoglobulins Phase 2
22
Prednisolone phosphate Approved, Vet_approved Phase 1 302-25-0
23
Methylprednisolone Approved, Vet_approved Phase 1 83-43-2 6741
24
Methylprednisolone hemisuccinate Approved Phase 1 2921-57-5
25
Prednisolone Approved, Vet_approved Phase 1 50-24-8 5755
26
Prednisolone hemisuccinate Experimental Phase 1 2920-86-7
27 Prednisolone acetate Phase 1
28 Methylprednisolone Acetate Phase 1
29 Anti-Inflammatory Agents Phase 1
30 Gastrointestinal Agents Phase 1
31 Antiemetics Phase 1
32 Peripheral Nervous System Agents Phase 1
33 Neuroprotective Agents Phase 1
34 Hormones Phase 1
35 glucocorticoids Phase 1
36 Protective Agents Phase 1
37 Antineoplastic Agents, Hormonal Phase 1
38 Hormone Antagonists Phase 1
39 Autonomic Agents Phase 1
40 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1

Interventional clinical trials:

(show all 13)
# 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 Recruiting NCT03046381 Phase 2 Tocilizumab
7 Canakinumab for Treatment of Adult Onset Still's Disease to Achieve Reduction of Arthritic Manifestation Terminated NCT02204293 Phase 2 Canakinumab;Placebo Injection;Canakinumab
8 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
9 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
10 Admission of Adult-onset Still Disease Patients in the ICU: a French Retrospective Multicentric Study Completed NCT03276650
11 Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes. Recruiting NCT02143986
12 Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still s Disease and Related Inflammatory Conditions Recruiting NCT03510442
13 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:

41
Testes, Liver, 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 1244)
# Title Authors PMID Year
1
[Adult-onset Still's disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood]. 9 38
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. 9 38
19684151 2009
3
Lymph node IL-18 expression in adult-onset Still's disease. 9 38
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. 9 38
18762861 2009
5
The future of the IL-1 receptor antagonist anakinra: from rheumatoid arthritis to adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis. 9 38
18321234 2008
6
Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. 9 38
17947302 2008
7
Steroid-resistant adult-onset Still's disease which showed a quick response to methotrexate. 9 38
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. 9 38
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. 9 38
18078628 2007
10
Association of interleukin 18 polymorphisms with adult onset Still's disease in Korea. 9 38
17611971 2007
11
Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? 9 38
17538564 2007
12
Interleukin 1 receptor antagonist therapy-induced thrombocytopenia in adult onset Still's disease. 9 38
17407250 2007
13
Cardiac death in a patient with adult-onset Still's disease treated with the interleukin 1 receptor inhibitor anakinra. 9 38
17179171 2007
14
[Blood cytokine levels as a clinical laboratory test]. 9 38
17441472 2007
15
Interleukin-18 in chronic joint diseases. 9 38
17317618 2007
16
A promoter haplotype of the interleukin-18 gene is associated with juvenile idiopathic arthritis in the Japanese population. 9 38
16563174 2006
17
-137 (G/C) IL-18 promoter polymorphism in patients with inflammatory bowel disease. 9 38
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. 9 38
15934126 2005
19
Pathogenesis of hemophagocytic syndrome (HPS). 9 38
15003190 2004
20
Interleukin 18 and hepatocyte growth factor in fulminant hepatic failure of adult onset Still's disease. 9 38
12734913 2003
21
Association between adult-onset Still's disease and interleukin-18 gene polymorphisms. 9 38
12424620 2002
22
Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still's disease. 9 38
11465725 2001
23
Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still's disease. 9 38
11263769 2001
24
A shock associated with adult-onset Still's disease. 9 38
11235787 2001
25
High serum level of macrophage-colony stimulating factor (M-CSF) in adult-onset Still's disease. 9 38
10371293 1999
26
Interleukin 6: a possible marker of disease activity in adult onset Still's disease. 9 38
8978961 1996
27
Acquired Amegakaryocytic Thrombocytopenia in Adult-onset Still's Disease: Successful Combination Therapy with Tocilizumab and Cyclosporine. 38
31391399 2019
28
Biologics for the treatment of adult-onset still's disease. 38
31379214 2019
29
Pulmonary arterial hypertension in adult-onset Still's disease: A case series and systematic review of the literature. 38
30580885 2019
30
Refractory adult-onset Still disease treated by tocilizumab combined with methotrexate: A STROBE-compliant article. 38
31393368 2019
31
Fever with persistent flagellate erythema in a primigravida: a rare presentation of adult-onset Still's disease. 38
31302619 2019
32
[Adult-onset Still's disease : Rare adult-onset autoinflammatory syndrome]. 38
30937478 2019
33
Adult-onset Still's disease-like manifestation accompanied by the cancer recurrence after long-term resting state. 38
27846765 2019
34
Atypical cutaneous and histological presentation of adult-onset Still's disease. 38
31384208 2019
35
Autoinflammation: Lessons from the study of familial Mediterranean fever. 38
31337526 2019
36
Refractory macrophage activation syndrome in the setting of adult-onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus. 38
30927277 2019
37
Urticarial rash, fever, and arthritis: A case of refractory Adult-onset Still's disease with good response to tocilizumab. 38
31361930 2019
38
Predictive factors and prognosis of macrophage activation syndrome associated with adult-onset Still's disease. 38
31287402 2019
39
The role of low density granulocytes and NETosis in the pathogenesis of adult-onset Still's Disease. 38
31365336 2019
40
Adult onset Still's disease and pregnancy. 38
31323360 2019
41
Biomarkers for adult-onset still's disease: up-to-date. 38
31248291 2019
42
Macrophage activation syndrome in neonates born to mothers with adult-onset Still's disease: Perinatal effect of maternal IL-18. 38
31299380 2019
43
Prescribing motivations and patients' characteristics related to the use of biologic drugs in adult-onset Still's disease: analysis of a multicentre "real-life" cohort. 38
31263993 2019
44
Macrophage activation syndrome complicating rheumatic diseases in adults: case-based review. 38
31367795 2019
45
The levels of CXCL12 and its receptor, CXCR4, as a biomarker of disease activity and cutaneous manifestation in adult-onset Still's disease. 38
31287410 2019
46
[It is time to reconcile systemic juvenile idiopathic arthritis and adult-onset Still's disease]. 38
31221454 2019
47
The role of damage-associated molecular pattern for pathogenesis and biomarkers in adult-onset Still's disease. 38
31055973 2019
48
Elevated serum levels of interleukin-10 in adult-onset Still's disease are associated with disease activity. 38
31236746 2019
49
Proposal for a simple algorithm to differentiate adult-onset Still's disease with other fever of unknown origin causes: a longitudinal prospective study. 38
30710221 2019
50
Still's Disease and Myopericarditis. 38
31423379 2019

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 79)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.83 TNFRSF1A TNF MIF MEFV IL6 IL1RN
2
Show member pathways
13.77 TNFRSF1A TNF IL6 IL1R1 IL1B IL18
3
Show member pathways
13.59 TNFRSF1A TNF MIF IL6 IL1RN IL1R1
4
Show member pathways
13.46 TNFRSF1A TNF IL6 IL1R1 IL1B IL18
5
Show member pathways
13.37 TNFRSF1A TNF IL6 IL1R1 IL1B IL18
6
Show member pathways
13.28 TNFRSF1A TNF IL6 IL1RN IL1R1 IL1B
7
Show member pathways
13.26 TNFRSF1A TNF IL6 IL1R1 IL1B IL18
8
Show member pathways
13.07 TNFRSF1A TNF MEFV IL6 IL1B IL18
9
Show member pathways
12.92 TNFRSF1A TNF IL6 IL1R1 IL1B
10
Show member pathways
12.85 TNFRSF1A TNF IL6 IL1B IL18
11 12.76 TNFRSF1A TNF IL1R1 IL1B CSF1
12
Show member pathways
12.71 TNF IL6 IL1B IL18
13
Show member pathways
12.7 TNFRSF1A TNF IL6 IL1R1 IL1B
14
Show member pathways
12.64 TNFRSF1A TNF IL1R1 IL1B
15
Show member pathways
12.6 TNF IL6 IL1R1 IL1B IL18
16
Show member pathways
12.57 TNFRSF1A TNF IL1R1 IL1B
17
Show member pathways
12.56 TNF IL6 IL1B IL18
18 12.54 TNFRSF1A TNF IL1RN IL1B
19 12.36 TNFRSF1A TNF IL6 IL1R1
20
Show member pathways
12.34 TNF IL6 IL1B CSF1 CRP
21
Show member pathways
12.32 TNF MIF IL6 IL1R1 IL1B
22
Show member pathways
12.24 TNF IL6 IL1B IL18
23 12.23 TNFRSF1A TNF IL6 IL1B IL18
24
Show member pathways
12.21 TNFRSF1A TNF IL1B
25
Show member pathways
12.21 TNF IL6 IL1R1 IL1B
26 12.2 TNF IL6 IL1RN IL1B
27
Show member pathways
12.17 TNFRSF1A TNF IL1B
28
Show member pathways
12.17 TNF IL6 IL1B
29 12.14 TNFRSF1A TNF IL6
30 12.12 TNFRSF1A TNF IL1R1 IL1B
31
Show member pathways
12.08 TNFRSF1A IL6 IL1R1 IL1B IL18
32
Show member pathways
12.05 TNF IL6 IL1R1 IL1B IL18
33 12.04 TNF MIF IL6
34 12.03 TNFRSF1A TNF IL1R1 IL1B CSF1
35
Show member pathways
12.01 TNF IL6 IL1B
36 12.01 TNF IL6 IL1B IL18
37 11.98 TNF IL6 CSF1
38
Show member pathways
11.96 IL1R1 IL1B IL18
39
Show member pathways
11.95 TNFRSF1A TNF IL6
40 11.95 TNF IL6 IL1R1 IL1B
41 11.95 TNF MIF IL6 IL1R1 IL1B
42 11.94 TNFRSF1A TNF IL1R1 IL1B
43 11.9 TNF IL6 IL1B
44 11.88 TNFRSF1A TNF IL6 IL1B CSF1
45
Show member pathways
11.88 TNF IL6 IL1RN IL1R1 IL1B IL18
46 11.82 TNF IL1R1 IL1B IL18
47 11.81 IL6 IL1B IL18
48 11.78 TNF IL6 IL1B
49 11.78 TNF IL6 IL1B CSF1
50 11.75 TNF IL6 IL1R1 IL1B CSF1

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 space GO:0005615 9.61 TNFRSF1A TNF MIF IL6 IL1RN IL1B
2 vesicle GO:0031982 9.33 MIF IL1RN IL1B
3 extracellular region GO:0005576 9.32 TNFRSF1A TNF MIF IL6 IL1RN IL1R1

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

(show top 50) (show all 53)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.99 TNF IL6 IL1RN IL1R1 IL1B IL18
2 positive regulation of gene expression GO:0010628 9.93 TNF IL6 IL1B IL18 CSF1 CRP
3 positive regulation of protein phosphorylation GO:0001934 9.88 TNF MIF IL1B
4 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.88 TNFRSF1A TNF IL1RN IL1B
5 cellular response to lipopolysaccharide GO:0071222 9.87 TNF IL6 IL1RN IL1B
6 positive regulation of DNA-binding transcription factor activity GO:0051091 9.85 TNF IL6 IL1B
7 defense response to Gram-positive bacterium GO:0050830 9.85 TNF IL6 CRP
8 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.85 TNF IL6 IL1B IL18
9 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.84 TNF MIF IL6
10 positive regulation of JNK cascade GO:0046330 9.84 TNF IL1RN IL1B
11 positive regulation of inflammatory response GO:0050729 9.83 TNFRSF1A TNF IL18
12 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.82 TNFRSF1A IL6 IL18
13 cellular response to organic cyclic compound GO:0071407 9.81 TNF IL1B IL18
14 positive regulation of smooth muscle cell proliferation GO:0048661 9.79 TNF IL6 IL18
15 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.79 TNF IL1B IL18
16 positive regulation of interleukin-6 production GO:0032755 9.78 TNF IL6 IL1RN IL1B
17 interleukin-1-mediated signaling pathway GO:0070498 9.77 IL1RN IL1R1 IL1B
18 lipopolysaccharide-mediated signaling pathway GO:0031663 9.73 TNF IL1B IL18
19 positive regulation of interleukin-8 production GO:0032757 9.71 TNF IL1B
20 positive regulation of cytokine secretion GO:0050715 9.71 TNF MIF
21 negative regulation of myoblast differentiation GO:0045662 9.71 TNF IL18
22 negative regulation of neurogenesis GO:0050768 9.71 TNF IL6 IL1B
23 positive regulation of osteoclast differentiation GO:0045672 9.7 TNF CSF1
24 positive regulation of chemokine production GO:0032722 9.7 TNF IL6
25 positive regulation of glial cell proliferation GO:0060252 9.7 TNF IL6 IL1B
26 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.69 TNF IL1B
27 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.69 IL18 CSF1
28 negative regulation of lipid catabolic process GO:0050995 9.69 TNF IL1B
29 positive regulation of membrane protein ectodomain proteolysis GO:0051044 9.68 TNF IL1B
30 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.68 TNF IL1B
31 positive regulation of granulocyte macrophage colony-stimulating factor production GO:0032725 9.68 IL1B IL18
32 positive regulation of ceramide biosynthetic process GO:2000304 9.67 TNFRSF1A TNF
33 response to glucocorticoid GO:0051384 9.67 TNF IL6 IL1RN
34 positive regulation of interferon-gamma production GO:0032729 9.67 TNF IL1R1 IL1B IL18
35 positive regulation of chemokine biosynthetic process GO:0045080 9.66 TNF IL1B
36 death-inducing signaling complex assembly GO:0071550 9.65 TNFRSF1A TNF
37 interleukin-6 production GO:0032635 9.65 IL1B IL18
38 positive regulation of fever generation GO:0031622 9.63 TNF IL1B
39 positive regulation of chemokine (C-X-C motif) ligand 2 production GO:2000343 9.63 TNF MIF
40 negative regulation of interleukin-1-mediated signaling pathway GO:2000660 9.62 IL6 IL1RN
41 sequestering of triglyceride GO:0030730 9.61 TNF IL1B
42 negative regulation of lipid storage GO:0010888 9.61 TNF IL6 CRP
43 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.59 TNF IL1B
44 regulation of establishment of endothelial barrier GO:1903140 9.58 TNFRSF1A TNF IL1B
45 cytokine-mediated signaling pathway GO:0019221 9.56 TNFRSF1A TNF IL6 IL1RN IL1R1 IL1B
46 positive regulation of T-helper 1 cell cytokine production GO:2000556 9.5 IL1R1 IL1B IL18
47 positive regulation of neuroinflammatory response GO:0150078 9.46 TNF IL6 IL1B IL18
48 carboxylic acid metabolic process GO:0019752 9.39 MIF
49 inflammatory response GO:0006954 9.36 TNFRSF1A TNF MIF MEFV IL6 IL1RN
50 positive regulation of JUN kinase activity GO:0043507 9.27 TNF

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.72 TNF MIF MEFV CSF1 CRP
2 cytokine activity GO:0005125 9.17 TNF MIF IL6 IL1RN IL1B IL18
3 interleukin-1 receptor binding GO:0005149 9.16 IL1RN IL1B
4 protease binding GO:0002020 9.13 TNF MIF IL1R1
5 protein binding GO:0005515 10 TNFRSF1A TNF MIF MEFV IL6 IL1RN

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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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