KEGG   DISEASE: Adult onset Still diseaseHelp
Entry
H01516                      Disease                                

Name
Adult onset Still disease;
Adult Still disease
Description
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.
Category
Immune system disease
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H01516  Adult onset Still disease
Human diseases in ICD-11 classification [BR:br08403]
 15 Diseases of the musculoskeletal system or connective tissue
  Arthropathies
   Inflammatory arthropathies
    FA23  Adult-onset Still disease
     H01516  Adult onset Still disease
BRITE hierarchy
Gene
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
IL-18 (polymorphism) [HSA:3606] [KO:K05482]
MEFV (polymorphism) [HSA:4210] [KO:K12803]
Other DBs
ICD-11: FA23
ICD-10: M06.1
MeSH: D016706
Reference
  Authors
Kadavath S, Efthimiou P
  Title
Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options.
  Journal
Ann Med 47:6-14 (2015)
DOI:10.3109/07853890.2014.971052
Reference
  Authors
Woo JH, Seong SS, Yoo DH
  Title
Association of interleukin 18 polymorphisms with adult onset Still's disease in Korea.
  Journal
J Rheumatol 34:1637-9 (2007)
Reference
  Authors
Nonaka F, Migita K, Jiuchi Y, Shimizu T, Umeda M, Iwamoto N, Fujikawa K, Izumi Y, Mizokami A, Nakashima M, Ueki Y, Yasunami M, Kawakami A, Eguchi K
  Title
Increased prevalence of MEFV exon 10 variants in Japanese patients with adult-onset Still's disease.
  Journal
Clin Exp Immunol 179:392-7 (2015)
DOI:10.1111/cei.12463

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