MCID: ADL030
MIFTS: 68

Adult-Onset Still's Disease malady

Nephrological diseases, Bone diseases categories

Summaries for Adult-Onset Still's Disease

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8Disease Ontology, 42NIH Rare Diseases, 32MalaCards
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NIH Rare Diseases:42 Adult-onset still's disease is an inflammatory condition characterized by high fevers, rash, sore throat, and joint pain. as it progresses, adult-onset still's disease may lead to chronic arthritis and other complications. still's disease was named after an english doctor named george still, who described the condition in children in 1896. still's disease which occurs in children (those under the age of 16) is now known as systemic onset juvenile rheumatoid arthritis (jra). in 1971, the term "adult still's disease" was used to describe adults who had a condition similar to systemic onset jra. the cause of adult-onset still's disease is unknown. no risk factors for the disease have been identified. there's no cure for adult-onset still's disease; however, treatment may offer symptom relief and help prevent complications.  last updated: 8/12/2009

MalaCards: Adult-Onset Still's Disease, also known as adult still's disease, is related to hepatitis and skin disease, and has symptoms including hyperlipidemia/hypercholesterolemia/hypertriglyceridemia, bone marrow failure/pancytopenia and cartilage destruction/chondrolysis. An important gene associated with Adult-Onset Still's Disease is IL18 (interleukin 18 (interferon-gamma-inducing factor)), and among its related pathways are Rheumatoid arthritis and Regulation by c-FLIP. The compounds indomethacin and dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and t cells, and related mouse phenotypes are skeleton and integument.

Disease Ontology:8 An arthritis that is a rare from of inflammatory arthritis characterized by fevers, rash, and joint pain.

Aliases & Classifications for Adult-Onset Still's Disease

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Sources:
48Orphanet, 8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH, 56SNOMED-CT, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 35MESH via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Nephrological diseases, Bone diseases


Characteristics (Orphanet epidemiological data):

48
adult still's disease:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

adult-onset still's disease 8
adult still's disease 42 48
still's disease adult onset 42
adult onset still's disease 8
stills disease adult-onset 44
adult-onset stills disease 10
adult onset still disease 42
adult-onset still disease 60
wissler-fanconi syndrome 48


External Ids:

Disease Ontology8 DOID:14256
MeSH34 D016706
ICD10 via Orphanet26 M06.1
SNOMED-CT via Orphanet57 239920006, 68190001
UMLS via Orphanet61 C0043195, C0085253
MESH via Orphanet35 D014924, D016706
ICD1025 M06.1

Related Diseases for Adult-Onset Still's Disease

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17GeneCards, 18GeneDecks
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Diseases related to Adult-Onset Still's Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 205)
idRelated DiseaseScoreTop Affiliating Genes
1hepatitis30.4IL18, FASLG, TFRC
2skin disease30.2IL2RA
3hemophagocytic lymphohistiocytosis30.2IL18, IL2RA
4arthritis30.2CSF1, TNFRSF1A, CRP, MIF, IL18, IL1RN
5fanconi syndrome10.3
6wissler's syndrome10.3
7cellulitis10.1
8adult syndrome10.1
9hepatitis a10.1
10dermatitis10.0IL18
11peritonitis10.0CRP
12crohn's disease10.0CRP
13cervical cancer10.0IL1R1
14aortic aneurysm10.0CRP
15pancreatitis10.0CRP
16myocardial infarction10.0CRP
17lung cancer10.0NKX2-1
18ankylosing spondylitis10.0CRP
19tonsillitis10.0CRP
20thyroiditis10.0FASLG
21histiocytosis10.0S100A9
22pneumonia10.0CRP
23myasthenia gravis10.0FCGR2A
24eye disease10.0CRP
25hemolytic-uremic syndrome10.0THBD
26measles10.0CSF1
27allergic rhinitis10.0IL18
28malignant histiocytosis10.0CSF1
29eclampsia10.0THBD
30celiac disease10.0TFRC
31ischemia10.0MMP3
32hepatitis c10.0IL1R1, IL18
33aspergillosis10.0CSF1
34hypothyroidism10.0NKX2-1
35gout10.0S100A9
36leishmaniasis10.0TNFRSF1A
37rheumatic fever10.0CRP
38cystic fibrosis10.0S100A9, IL1R1
39encephalitis10.0CX3CL1
40primary hyperoxaluria10.0THBD
41hyper-igd syndrome10.0IL1RN
42hyperglycemia10.0CRP
43systemic lupus erythematosus10.0FCGR2A, CRP
44pleurisy10.0IL2RA
45schizophrenia10.0TNFRSF1A
46pulmonary disease, chronic obstructive10.0CRP
47sarcoma10.0IL1R1, CSF1
48paracoccidioidomycosis10.0TNFRSF1A
49lupus erythematosus10.0THBD, IL18
50hepatocellular carcinoma10.0NKX2-1, FASLG

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



Diseases related to adult-onset still's disease

Clinical Features for Adult-Onset Still's Disease

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48Orphanet
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Symptoms:

48 (show all 26)
  • hyperlipidemia/hypercholesterolemia/hypertriglyceridemia
  • bone marrow failure/pancytopenia
  • cartilage destruction/chondrolysis
  • meningitis/meningeal syndrome
  • myocardium anomalies/myocarditis
  • abnormal hepatic enzymes/transaminases
  • hepatitis/icterus/cholestasis
  • arthritis/synovitis/synovial proliferation
  • hydrarthrosis/articular/joint effusion
  • articular/joint pain/arthralgia
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • splenomegaly
  • hepatomegaly/liver enlargement (excluding storage disease)
  • pruritus/itching
  • urticaria
  • hyperleukocytosis/leukocytosis
  • fever/chilling
  • pharyngitis
  • myalgia/muscular pain
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • mediastinal/hilar adenopathies
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • acute abdominal pain/colic
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • asthenia/fatigue/weakness
  • cutaneous rash

Drugs & Therapeutics for Adult-Onset Still's Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Adult-Onset Still's Disease

Drug clinical trials:

Search ClinicalTrials for Adult-Onset Still's Disease

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

Search CenterWatch for Adult-Onset Still's Disease

Genetic Tests for Adult-Onset Still's Disease

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Anatomical Context for Adult-Onset Still's Disease

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32MalaCards
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MalaCards organs/tissues related to Adult-Onset Still's Disease:

32
Liver, Bone, T cells, Lymph node, Bone marrow, Skin, Thyroid, Lung, Spleen, Eye, Endothelial, Heart, Small intestine, Nk cells

Animal Models for Adult-Onset Still's Disease or affiliated genes

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36MGI
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Publications for Adult-Onset Still's Disease

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50PubMed
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Articles related to Adult-Onset Still's Disease:

(show top 50)    (show all 489)
idTitleAuthorsYear
1
MEFV gene mutations and their clinical significance in Korean patients with adult-onset Still's disease. (24064016)
2013
2
Successful treatment of glucocorticoid and cyclosporine refractory adult-onset Still's disease complicated with hemophagocytic syndrome with plasma exchange therapy and tocilizumab : a case report. (24390109)
2013
3
Efficacy of Tocilizumab in refractory Adult-onset Still's Disease; Report of 2 Cases. (24076178)
2013
4
Complement alternative pathway activation in the course of thrombotic microangiopathy associated with adult-onset Still's disease. (23562215)
2013
5
A case of refractory adult-onset Still's disease with high serum interleukin-18 levels treated with monitoring of serum levels of cyclosporine. (22423500)
2012
6
Adult-Onset Still's Disease Masquerading as Sepsis in an Asplenic Active Duty Soldier. (23251171)
2012
7
Adult-onset Still's disease with dermatomyositis-like eruption. (22512689)
2012
8
Serum S100A8/A9, but not follistatin-like protein 1 and interleukin 18, may be a useful biomarker of disease activity in adult-onset Still's disease. (22660800)
2012
9
Anti-Interleukin-1 Agents in Adult Onset Still's Disease. (22611515)
2012
10
Hemophagocytic syndrome secondary to adult-onset Still's disease but very similar to lymphoma. (22670185)
2012
11
A series of 22 patients with adult-onset Still's disease presenting with fever of unknown origin. A difficult diagnosis? (21603876)
2012
12
Adult-onset Still's disease: how to make a diagnosis in an atypical case. (21877245)
2012
13
Successful treatment of adult-onset Still's disease with tocilizumab monotherapy: two case reports and literature review. (22215118)
2012
14
Adult-onset Still's disease misdiagnosed as pneumonia: two case reports. (22472935)
2011
15
Interstitial inflammation in visceral organs is a pathologic feature of adult-onset Still's disease. (19882158)
2011
16
Adult-onset Still's disease complicated with hemophagocytic syndrome. (20123591)
2010
17
A case report of successful treatment with plasma exchange for adult-onset Still's disease with autoimmune hepatitis. (20101675)
2010
18
IL1-receptor antagonist anakinra provides long-lasting efficacy in the treatment of refractory adult-onset Still's disease. (20107032)
2010
19
Cyclosporine A-induced neck fibrosis in a patient with adult-onset Still's disease. (19763667)
2010
20
Maternal exposure to leflunomide and methotrexate in a patient with adult-onset Still's disease. (20435649)
2010
21
Coexistence of adult-onset Still's disease and autoimmune hyperthyroidism in a patient who responded to corticosteroids and I^-blocker. (20201625)
2010
22
Potential benefit of paracetamol administration in adult-onset Still's disease. (19776706)
2009
23
Potential role of macrophage migration inhibitory factor in adult-onset Still's disease. (18686192)
2009
24
A case of adult onset Still's disease showing marked accumulation in the liver and spleen, on positron emission tomography-CT images. (18443796)
2008
25
Adult-onset Still's disease complicated by multiple cranial nerve palsy and hypothyroidism. (18419687)
2008
26
Nocardiosis in adult-onset Still's disease and vasculitis syndrome. (18626243)
2008
27
Adult-onset Still's disease: a report of 28 cases and review of the literature. (18754076)
2008
28
Anakinra in patients with treatment-resistant adult-onset Still's disease: four case reports with serial cytokine measurements and a review of the literature. (17583775)
2007
29
Leflunomide and methotrexate combination in refractory adult-onset Still's disease. (17535840)
2007
30
Adult-onset Still's disease with prurigo pigmentosa-like skin eruption. (16469087)
2006
31
Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still's disease: the first case report. (16173267)
2005
32
Nasal septal perforation: a novel clinical manifestation of systemic juvenile idiopathic arthritis/adult onset Still's disease. (16331776)
2005
33
Clarithromycin in adult-onset Still's disease. Case report with a 1-year follow-up. (15144141)
2004
34
A case of adult-onset Still's disease presenting with angioedema. (14749997)
2004
35
Persistent generalized erythema in adult-onset Still's disease. (14521701)
2003
36
Association between HLA-DR B1 and clinical features of adult onset Still's disease in Korea. (12942703)
2003
37
Interleukin 18 and hepatocyte growth factor in fulminant hepatic failure of adult onset Still's disease. (12734913)
2003
38
"Adolescent-onset Still's disease": characteristics and outcome in comparison with adult-onset Still's disease. (12102485)
2002
39
Prolonged remission state of refractory adult onset Still's disease following CD34-selected autologous peripheral blood stem cell transplantation. (10871738)
2000
40
Bone scintigraphy and magnetic resonance imaging in adult-onset Still's disease. (10503565)
1999
41
TNFalpha and response of treatment-resistant adult-onset Still's disease to thalidomide. (9716064)
1998
42
Fulminant hepatic failure in adult onset Still's disease. (8730149)
1996
43
Inappropriate secretion of antidiuretic hormone (SIADH) in a patient with pleurisy related to adult onset Still's disease. (8719553)
1995
44
T cell lymphoma masquerading as adult onset Still's disease. (7554577)
1995
45
Adult-onset Still's disease. An underdiagnosed condition?]. (8539715)
1995
46
Osseous tuberculosis traced by magnetic resonance imaging in a patient with adult-onset Still's disease. (8508290)
1993
47
Adult onset Still's disease: clinical features and course. (1486743)
1992
48
Oral low-dose methotrexate (MTX) to be efficient in refractory rheumatoid arthritis and adult-onset Still's disease. (2741938)
1989
49
Characterization of lymph node histology in adult onset Still's disease. (2724252)
1989
50
Hepatic abnormalities in adult onset Still's disease. (529250)
1979

Genetic Variations for Adult-Onset Still's Disease

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Expression for genes affiliated with Adult-Onset Still's Disease

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Adult-Onset Still's Disease

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

Pathways for genes affiliated with Adult-Onset Still's Disease

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Sources:
29KEGG, 53Reactome, 37NCBI BioSystems Database, 52R&D Systems, 12EMD Millipore, 51QIAGEN
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Pathways related to Adult-Onset Still's Disease according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.4IL18
2
Hide members
10.2FASLG, TNFRSF1A
3
Immune response Bacterial infections in normal airways
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10.2FASLG, IL1RN, IL1R1
4
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10.1IL2RA, FASLG, CABIN1
5
Hide members
10.1IL2RA, FASLG, TNFRSF1A
6
Hide members
10.1IL1R1, IL18, IL2RA, FASLG
7
Hide members
10.1IL2RA, IL18, IL1RN, IL1R1
8
Hide members
10.1IL1R1, IL1RN, IL18, IL2RA
9
Hide members
10.1TNFRSF1A, IL18, FCGR2A, FCGR3A
1010.1TNFRSF1A, IL18, FCGR2A, FCGR3A
11
Hide members
10.0IL18, IL2RA, FASLG, TNFRSF1A
1210.0CSF1, TFRC, IL2RA, IL1R1
139.9CSF1, TNFRSF1A, IL1R1, FCGR2A, FCGR3A
149.9CSF1, TNFRSF1A, CX3CL1, MMP3
15
Hide members
9.9CSF1, TNFRSF1A, CX3CL1, IL2RA, IL1R1
16
Hide members
9.9IL2RA, CX3CL1, FASLG, TNFRSF1A, CSF1
17
Hide members
9.8CSF1, TNFRSF1A, CX3CL1, IL2RA, IL18, IL1R1

Compounds for genes affiliated with Adult-Onset Still's Disease

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Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 49PharmGKB, 24HMDB, 2BitterDB
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Compounds related to Adult-Onset Still's Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 191)
idCompoundScoreTop Affiliating Genes
1indomethacin44 59 28 1113.7IL1R1, CSF1
2dexamethasone44 49 28 1113.6FASLG, NKX2-1, IL1R1
3p0034410.6FASLG, CSF1
4cycloheximide4410.5FASLG, IL18, TFRC
5cyclosporin a44 28 5912.4THBD, IL1R1, IL2RA, CABIN1
6aspartate4410.3G6PD, TFRC, FASLG
7rantes4410.3TNFRSF1A, CSF1, IL2RA, IL1R1, FASLG
8adalimumab44 49 1112.2IL1RN, FCGR3A, FCGR2A, MMP3, CRP, IL1R1
9endotoxin4410.2TNFRSF1A, FASLG, IL18BP, THBD, S100A9
10prednisolone44 28 1112.2CSF1, CRP, IL2RA, MMP3, FCGR2A, THBD
11rituximab44 49 1112.1FCGR3A, CSF1, IL2RA, IL1R1, FCGR2A, CRP
12polysulfone4410.1IL1R1, IL1RN, CRP, FASLG, IL2RA
13cyclophosphamide44 49 1112.1G6PD, THBD, IL1R1, IL2RA, MIF, CRP
14sulfasalazine44 28 49 1113.1G6PD, CRP, IL2RA, IL1RN, IL1R1, MMP3
15infliximab44 49 1112.1TNFRSF1A, CRP, IL2RA, IL18, IL1RN, IL1R1
16oxygen44 2411.1FCGR3A, TNFRSF1A, S100A9
17creatinine4410.0CSF1, TNFRSF1A, CRP, IL1RN, IL18BP, MMP3
18chloroquine44 2 49 28 1114.0IL2RA, IL18, IL1R1, TFRC, G6PD, TNFRSF1A
19etanercept44 49 1112.0FCGR3A, FCGR2A, MMP3, IL1R1, IL1RN, IL18
20polyethylene glycol4410.0TNFRSF1A, TFRC, CRP, IL1R1, THBD, G6PD
21thalidomide44 49 59 1113.0FASLG, IL1R1, IL18, IL2RA, MIF, S100A9
22ivig449.9FCGR3A, FCGR2A, IL1R1, IL1RN, IL2RA, CRP
23simvastatin44 49 59 28 11 2414.9IL18, IL1RN, THBD, FASLG, CRP, MMP3
24glutamate449.9CX3CL1, TFRC, THBD, TNFRSF1A
25aspirin44 49 28 2412.9IL1RN, IL1R1, THBD, CSF1, IL18, G6PD
26epinephrine44 11 2411.9FASLG, CSF1, FCGR2A, G6PD, IL2RA, IL18
27neopterin449.9TFRC, IL2RA, IL18, IL1RN, IL1R1, THBD
28sb 20358044 5910.9TNFRSF1A, CRP, MIF, IL18, IL1RN, IL1R1
29heparin44 28 11 2412.8MMP3, TNFRSF1A, IL1RN, CSF1, IL2RA, FASLG
30rapamycin449.8CSF1, IL18, IL1R1, G6PD, FASLG, TFRC
31methotrexate44 49 1111.8FASLG, FCGR3A, MMP3, IL1R1, IL1RN, IL18
32histamine44 28 2411.8FCGR2A, MMP3, IL18, IL1RN, CSF1, IL2RA
33n acetylcysteine449.7IL1R1, MMP3, TNFRSF1A, FASLG, IL18, IL2RA
34ascorbic acid44 2410.7FASLG, THBD, MMP3, IL1R1, IL1RN, IL2RA
35serine449.7IL18, IL1RN, NKX2-1, MMP3, IL1R1, MIF
36paraffin449.7FASLG, THBD, CSF1, TFRC, NKX2-1, MMP3
37dmso449.6CSF1, TNFRSF1A, THBD, MMP3, TFRC, G6PD
38testosterone44 59 11 2412.6G6PD, IL1R1, IL1RN, IL18, TNFRSF1A, CRP
39lactate449.6IL18, THBD, MMP3, CRP, IL1R1, TFRC
40h2o2449.6IL2RA, IL18, IL1RN, IL1R1, FCGR2A, THBD
41cholesterol44 28 11 2412.6MMP3, FCGR2A, FCGR3A, CRP, IL1R1, IL1RN
42doxorubicin44 49 1111.6MIF, IL2RA, FASLG, G6PD, TFRC, IL18
43vegf449.5FASLG, MIF, THBD, MMP3, IL1RN, IL1R1
44zinc44 2410.5FASLG, NKX2-1, MIF, IL2RA, IL18, IL1RN
45alanine449.4IL1RN, IL1R1, IL18BP, TFRC, MMP3, THBD
46actinomycin d449.4CSF1, TNFRSF1A, FASLG, G6PD, THBD, FCGR2A
47retinoic acid44 2410.4S100A9, CSF1, TNFRSF1A, THBD, FASLG, TFRC
48cysteine449.4THBD, FASLG, TFRC, MIF, MMP3, CX3CL1
49tyrosine449.2MIF, TFRC, FASLG, IL1RN, IL1R1, MMP3
50lipid448.9THBD, FCGR2A, S100A9, TNFRSF1A, G6PD, CRP

GO Terms for genes affiliated with Adult-Onset Still's Disease

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Sources:
16Gene Ontology
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Cellular components related to Adult-Onset Still's Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:00989710.0FCGR3A, IL2RA, TFRC, FASLG
2cell surfaceGO:0099869.5TNFRSF1A, MIF, CX3CL1, IL1R1, THBD
3extracellular spaceGO:0056159.2MMP3, S100A9, CSF1, TNFRSF1A, FASLG, CRP
4extracellular regionGO:0055769.0CRP, TFRC, FASLG, TNFRSF1A, S100A9, MIF
5plasma membraneGO:0058868.9CRP, TFRC, TNFRSF1A, CSF1, S100A9, CX3CL1

Biological processes related to Adult-Onset Still's Disease according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1T-helper 1 type immune responseGO:04208810.2IL18BP, IL18
2positive regulation of inflammatory responseGO:05072910.0S100A9, TNFRSF1A, CX3CL1, IL18
3response to ethanolGO:0454719.9CRP, G6PD, TNFRSF1A, S100A9
4immune responseGO:0069559.9FCGR3A, IL1R1, IL1RN, IL18, IL2RA, CX3CL1
5inflammatory responseGO:0069549.8S100A9, CSF1, TNFRSF1A, CRP, MIF, IL18
6response to lipopolysaccharideGO:0324969.8S100A9, TNFRSF1A, FASLG, IL18BP
7cell surface receptor signaling pathwayGO:0071669.7IL1R1, IL2RA, MIF, CABIN1

Molecular functions related to Adult-Onset Still's Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1IgG bindingGO:01986410.3FCGR3A, FCGR2A
2cytokine activityGO:0051259.8IL18, MIF, FASLG, CSF1

Products for genes affiliated with Adult-Onset Still's Disease

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  • Antibodies
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Sources for Adult-Onset Still's Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet