MCID: ADL030
MIFTS: 61

Adult-Onset Still's Disease malady

Nephrological, Bone categories

Summaries for Adult-Onset Still's Disease

Sources:
8Disease Ontology, 43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 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 c and hemophagocytic lymphohistiocytosis, and has symptoms including hepatomegaly/liver enlargement (excluding storage disease), splenomegaly and arthritis/synovitis/synovial proliferation. 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 skin, liver and lymph node, 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

Sources:
49Orphanet, 8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 61UMLS, 35MeSH, 57SNOMED-CT, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 36MESH via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
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 43 49
still's disease adult onset 43
adult onset still's disease 8
stills disease adult-onset 45
adult-onset stills disease 10
adult onset still disease 43
adult-onset still disease 61
wissler-fanconi syndrome 49


External Ids:

Disease Ontology8 DOID:14256
MeSH35 D016706
ICD10 via Orphanet26 M06.1
SNOMED-CT via Orphanet58 239920006, 68190001
UMLS via Orphanet62 C0043195, C0085253
MESH via Orphanet36 D014924, D016706
ICD1025 M06.1

Related Diseases for Adult-Onset Still's Disease

Sources:
17GeneCards, 18GeneDecks
See all sources

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

Sources:
49Orphanet
See all sources


Symptoms:

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

Drugs & Therapeutics for Adult-Onset Still's Disease

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

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

Anatomical Context for Adult-Onset Still's Disease

Sources:
33MalaCards
See all sources

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

33
Skin, Liver, Lymph node, Lung, Spleen, Heart, Small intestine, Thyroid, Nk cells, T cells, B cells, Endothelial

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

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Adult-Onset Still's Disease

Sources:
51PubMed
See all sources

Articles related to Adult-Onset Still's Disease:

(show top 50)    (show all 491)
idTitleAuthorsYear
1
Biologic treatments for adult-onset Still's disease. (23864171)
2014
2
N2010 adult-onset Still's disease complicated by hemophagocytic syndrome and catastrophic antiphospholipid syndrome resulting in four limb amputation. (23781758)
2013
3
Anakinra in adult-onset Still's disease: long-term treatment in patients resistant to conventional therapy. (23225779)
2013
4
Very low density lipoprotein, chylomicron, and lipoprotein (a) are more useful to detect the development of macrophage activation syndrome in adult-onset still's disease as compared with cytokines and triglyceride. (23760887)
2013
5
Clinical features and prognosis in 82 patients with adult-onset Still's disease. (24050706)
2013
6
Reversal of severe hepatitis with infliximab in adult-onset Still's disease. (23331455)
2013
7
Mycoplasma pneumoniae infection associated with urticarial vasculitis mimicking adult-onset Still's disease. (21918897)
2012
8
Transient impairment of NK cell function in an infant born to a mother with adult-onset Still's disease: perinatal effect of maternal IL-18. (22572478)
2012
9
Natural killer T cell deficiency in active adult-onset Still's Disease: correlation of deficiency of natural killer T cells with dysfunction of natural killer cells. (22605480)
2012
10
Use of glycosylated ferritin assay to aid the diagnosis of adult-onset Still's disease: a local laboratory experience in Hong Kong. (21785960)
2012
11
Adult-onset Still's disease in pregnancy. (21755417)
2012
12
Adult-onset Still's disease with macrophage activation syndrome successfully treated with a combination of methotrexate and etanercept. (21670967)
2012
13
Adult onset Still's disease: experience from a tertiary care rheumatology unit. (23253241)
2012
14
Successful outcome of two pregnancies in patients with adult-onset Still's disease treated with IL-1 receptor antagonist (anakinra). (22153586)
2011
15
A case of adult onset Still's disease complicated with cryptogenic organizing pneumonia. (21297329)
2011
16
A case of Crohn's disease complicated by adult onset Still's disease. (21122546)
2010
17
Adult-onset Still's disease. (20020138)
2010
18
A case of adult-onset Still's disease complicated with diffuse alveolar hemorrhage. (19270830)
2009
19
Adult-onset Still's disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood]. (19838717)
2009
20
Continuation of anakinra despite the development of a pruritic rash in an otherwise refractory case of adult-onset Still's disease. (19213754)
2009
21
Richter's syndrome developing in a patient with adult onset Still's disease. (18607590)
2009
22
Adult-onset Still's disease: a review. (19584494)
2009
23
Etanercept as a rescue agent in patient with adult onset Still's disease complicated with congestive heart failure. (18500457)
2008
24
Effective treatment of steroid refractory adult-onset Still's disease with anakinra. (18464320)
2008
25
Pure red cell aplasia in a patient with adult-onset Still's disease. (18512764)
2008
26
How can we improve the management of adult-onset Still's disease? (17321778)
2007
27
Cardiac death in a patient with adult-onset Still's disease treated with the interleukin 1 receptor inhibitor anakinra. (17179171)
2007
28
Macrophage activation syndrome associated with adult-onset Still's disease]. (18174671)
2007
29
Urticaria as a cutaneous sign of adult-onset Still's disease. (17241583)
2006
30
Current concepts on the physiopathology of adult-onset Still's disease]. (15996569)
2005
31
Usefulness of glycosylated ferritin in atypical presentations of adult onset Still's disease. (15082500)
2004
32
Pseudo-angiocholitis revealing adult-onset Still's disease. (15185868)
2004
33
Crohn's disease complicated by adult-onset Still's disease. (14564635)
2003
34
A shock associated with adult-onset Still's disease. (11235787)
2001
35
Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still's disease. (11465725)
2001
36
Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still's disease. (11263769)
2001
37
Isotretinoin-induced adult onset Still's disease. (11072605)
2000
38
Adult-onset Still's disease. Differential diagnosis in recurrent pharyngitis, fever of unknown original and excessive hyperferritinemia]. (10432943)
1999
39
Clinical characteristics, treatment, and outcome of adult onset Still's disease in southern Chinese. (9858428)
1998
40
Adult onset Still's disease associated hemophagocytosis. (9263166)
1997
41
Serological findings in patients with acute syndromes fulfilling the proposed criteria of adult onset Still's disease. (9385343)
1997
42
Severe sore throat as a presenting symptom of adult onset Still's disease: a case series and review of the literature. (9058672)
1997
43
Cerebral haemorrhage complicating adult-onset Still's disease: a case report. (8959535)
1996
44
Interleukin 6: a possible marker of disease activity in adult onset Still's disease. (8978961)
1996
45
Aseptic meningitis and focal central nervous system involvement in a case of adult onset Still's disease. (8496886)
1993
46
Adult-onset Still's disease in India. (1596706)
1992
47
Adult-onset Still's disease revealed by a pleuropericarditis. (2289554)
1990
48
Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases. (3659248)
1986
49
Adult-onset Still's disease. (3857480)
1985
50
Case report: Adult-onset Still's disease, variant of rheumatoid arthritis. (1161667)
1975

Genetic Variations for Adult-Onset Still's Disease

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

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
30KEGG, 54Reactome, 38NCBI BioSystems Database, 53R&D Systems, 12EMD Millipore, 52QIAGEN
See all sources

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
Hide members
10.2FASLG, IL1RN, IL1R1
4
Hide members
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

Sources:
45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 50PharmGKB, 24HMDB, 2BitterDB
See all sources

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

(show top 50)    (show all 191)
idCompoundScoreTop Affiliating Genes
1indomethacin45 60 29 1113.7IL1R1, CSF1
2dexamethasone45 50 29 1113.6FASLG, NKX2-1, IL1R1
3p0034510.6FASLG, CSF1
4cycloheximide4510.5FASLG, IL18, TFRC
5cyclosporin a45 29 6012.4THBD, IL1R1, IL2RA, CABIN1
6aspartate4510.3G6PD, TFRC, FASLG
7rantes4510.3TNFRSF1A, CSF1, IL2RA, IL1R1, FASLG
8adalimumab45 50 1112.2IL1RN, FCGR3A, FCGR2A, MMP3, CRP, IL1R1
9endotoxin4510.2TNFRSF1A, FASLG, IL18BP, THBD, S100A9
10prednisolone45 29 1112.2CSF1, CRP, IL2RA, MMP3, FCGR2A, THBD
11rituximab45 50 1112.1FCGR3A, CSF1, IL2RA, IL1R1, FCGR2A, CRP
12polysulfone4510.1IL1R1, IL1RN, CRP, FASLG, IL2RA
13cyclophosphamide45 50 1112.1G6PD, THBD, IL1R1, IL2RA, MIF, CRP
14sulfasalazine45 29 50 1113.1G6PD, CRP, IL2RA, IL1RN, IL1R1, MMP3
15infliximab45 50 1112.1TNFRSF1A, CRP, IL2RA, IL18, IL1RN, IL1R1
16oxygen45 2411.1FCGR3A, TNFRSF1A, S100A9
17creatinine4510.0CSF1, TNFRSF1A, CRP, IL1RN, IL18BP, MMP3
18chloroquine45 2 50 29 1114.0IL2RA, IL18, IL1R1, TFRC, G6PD, TNFRSF1A
19etanercept45 50 1112.0FCGR3A, FCGR2A, MMP3, IL1R1, IL1RN, IL18
20polyethylene glycol4510.0TNFRSF1A, TFRC, CRP, IL1R1, THBD, G6PD
21thalidomide45 50 60 1113.0FASLG, IL1R1, IL18, IL2RA, MIF, S100A9
22ivig459.9FCGR3A, FCGR2A, IL1R1, IL1RN, IL2RA, CRP
23simvastatin45 50 60 29 11 2414.9IL18, IL1RN, THBD, FASLG, CRP, MMP3
24glutamate459.9CX3CL1, TFRC, THBD, TNFRSF1A
25aspirin45 50 29 2412.9IL1RN, IL1R1, THBD, CSF1, IL18, G6PD
26epinephrine45 11 2411.9FASLG, CSF1, FCGR2A, G6PD, IL2RA, IL18
27neopterin459.9TFRC, IL2RA, IL18, IL1RN, IL1R1, THBD
28sb 20358045 6010.9TNFRSF1A, CRP, MIF, IL18, IL1RN, IL1R1
29heparin45 29 11 2412.8MMP3, TNFRSF1A, IL1RN, CSF1, IL2RA, FASLG
30rapamycin459.8CSF1, IL18, IL1R1, G6PD, FASLG, TFRC
31methotrexate45 50 1111.8FASLG, FCGR3A, MMP3, IL1R1, IL1RN, IL18
32histamine45 29 2411.8FCGR2A, MMP3, IL18, IL1RN, CSF1, IL2RA
33n acetylcysteine459.7IL1R1, MMP3, TNFRSF1A, FASLG, IL18, IL2RA
34ascorbic acid45 2410.7FASLG, THBD, MMP3, IL1R1, IL1RN, IL2RA
35serine459.7IL18, IL1RN, NKX2-1, MMP3, IL1R1, MIF
36paraffin459.7FASLG, THBD, CSF1, TFRC, NKX2-1, MMP3
37dmso459.6CSF1, TNFRSF1A, THBD, MMP3, TFRC, G6PD
38testosterone45 60 11 2412.6G6PD, IL1R1, IL1RN, IL18, TNFRSF1A, CRP
39lactate459.6IL18, THBD, MMP3, CRP, IL1R1, TFRC
40h2o2459.6IL2RA, IL18, IL1RN, IL1R1, FCGR2A, THBD
41cholesterol45 29 11 2412.6MMP3, FCGR2A, FCGR3A, CRP, IL1R1, IL1RN
42doxorubicin45 50 1111.6MIF, IL2RA, FASLG, G6PD, TFRC, IL18
43vegf459.5FASLG, MIF, THBD, MMP3, IL1RN, IL1R1
44zinc45 2410.5FASLG, NKX2-1, MIF, IL2RA, IL18, IL1RN
45alanine459.4IL1RN, IL1R1, IL18BP, TFRC, MMP3, THBD
46actinomycin d459.4CSF1, TNFRSF1A, FASLG, G6PD, THBD, FCGR2A
47retinoic acid45 2410.4S100A9, CSF1, TNFRSF1A, THBD, FASLG, TFRC
48cysteine459.4THBD, FASLG, TFRC, MIF, MMP3, CX3CL1
49tyrosine459.2MIF, TFRC, FASLG, IL1RN, IL1R1, MMP3
50lipid458.9THBD, FCGR2A, S100A9, TNFRSF1A, G6PD, CRP

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

Sources:
16Gene Ontology
See all sources

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Adult-Onset Still's Disease

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet