KEGG   DISEASE: 若年性特発性関節炎
エントリ  
H01672                                                             
名称    
若年性特発性関節炎
  下位グループ
若年性関節リウマチ
スチル病
若年性関節炎 (JUVAR)
  上位グループ
関節リウマチ [DS:H00630]
概要    
Juvenile idiopathic arthritis (JIA) is one of the most common childhood rheumatic diseases. Clinically, it is defined as arthritis of unknown origin that starts before the age of 16, and persists for at least 6 weeks. Next to a certain genetic predisposition, environmental factors play a role leading to a chronic inflammatory response. JIA is not a single disorder but consists of a heterogeneous group of auto-immune inflammatory diseases. It has variable rates in course and activity of disease. Based on 6 months of clinical symptoms and global prognostic factors, the following clinical subtypes of JIA are recognized: systemic JIA, oligoarthritis, RF-negative polyarthritis, RF-positive polyarthritis, psoriatic JIA, enthesitis- related arthritis, and undifferentiated arthritis. Systemic JIA, also known as Still's disease, is a subtype with strong systemic clinical symptoms. Patients with systemic JIA have, in addition to arthritis, prominent symptoms of systemic inflammation such as spiking fever, rash, pericarditis, peritonitis, lymphadenopathy and organomegaly. A severe and often life-threatening complication occurring in 10-30% of patients with systemic JIA is macrophage activation syndrome. Polymorphisms in the IL6 and in the MIF gene have been found to be associated with susceptibility to the disorder. Based on the known relevance of IL6 in JIA pathophysiology, tocilizumab has been investigated and approved for use in the treatment of systemic and polyarticular JIA.
カテゴリ  
免疫系疾患
階層分類  
ヒト疾患 [BR:jp08402]
 免疫系疾患
  アレルギーと自己免疫疾患
   H01672  若年性特発性関節炎
ICD-11 による疾患分類 [BR:jp08403]
 15 筋骨格系・結合組織の疾患
  関節症
   炎症性関節症
    FA24  若年性特発性関節炎
     H01672  若年性特発性関節炎
特定疾患 (難病) [jp08407.html]
 H01672
パスウェイ 
hsa05323  関節リウマチ
関連パスウェイ
hsa04060  Cytokine-cytokine receptor interaction
hsa04630  Jak-STAT signaling pathway
病因遺伝子 
IL6 [HSA:3569] [KO:K05405]
MIF [HSA:4282] [KO:K07253]
(JUVAR) LACC1 [HSA:144811] [KO:K05810]
治療薬   
デキサメタゾン [DR:D00292]
デキサメタゾンリン酸エステルナトリウム [DR:D00975]
ベタメタゾンリン酸エステルナトリウム [DR:D00972]
トリアムシノロンアセトニド [DR:D00983]
ヒドロコルチゾン [DR:D00088]
ヒドロコルチゾンコハク酸エステルナトリウム [DR:D00978]
コルチゾン酢酸エステル [DR:D00973]
トリアムシノロン [DR:D00385]
ベタメタゾン [DR:D00244]
プレドニゾロン [DR:D00472]
プレドニゾロンコハク酸エステルナトリウム [DR:D01239]
メチルプレドニゾロン [DR:D00407]
メチルプレドニゾロン酢酸エステル [DR:D00979]
メトトレキサート [DR:D00142]
アバタセプト [DR:D03203]
エタネルセプト [DR:D00742]
アダリムマブ [DR:D02597]
カナキヌマブ [DR:D09315]
トシリズマブ [DR:D02596]
コメント  
See also H01516 Adult onset Still's disease.
リンク   
ICD-11: FA24
ICD-10: M08
MeSH: D001171
OMIM: 604302 618795
文献    
  著者
Swart JF, de Roock S, Prakken BJ
  タイトル
Understanding inflammation in juvenile idiopathic arthritis: How immune biomarkers guide clinical strategies in the systemic onset subtype.
  雑誌
Eur J Immunol 46:2068-77 (2016)
DOI:10.1002/eji.201546092
文献    
PMID:9769329 (IL6)
  著者
Fishman D, Faulds G, Jeffery R, Mohamed-Ali V, Yudkin JS, Humphries S, Woo P
  タイトル
The effect of novel polymorphisms in the interleukin-6 (IL-6) gene on IL-6 transcription and plasma IL-6 levels, and an association with systemic-onset juvenile chronic arthritis.
  雑誌
J Clin Invest 102:1369-76 (1998)
DOI:10.1172/JCI2629
文献    
PMID:11508429 (MIF)
  著者
Donn RP, Shelley E, Ollier WE, Thomson W
  タイトル
A novel 5'-flanking region polymorphism of macrophage migration inhibitory factor is associated with systemic-onset juvenile idiopathic arthritis.
  雑誌
文献    
PMID:27881174 (LACC1)
  著者
Kallinich T, Thorwarth A, von Stuckrad SL, Rosen-Wolff A, Luksch H, Hundsdoerfer P, Minden K, Krawitz P
  タイトル
Juvenile arthritis caused by a novel FAMIN (LACC1) mutation in two children with systemic and extended oligoarticular course.
  雑誌
Pediatr Rheumatol Online J 14:63 (2016)
DOI:10.1186/s12969-016-0124-2
LinkDB    

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