KEGG   DISEASE: 川崎病
エントリ  
H01718                                                             
名称    
川崎病
概要    
Kawasaki disease (KD) is an acute systemic vasculitis of childhood that does not have a known cause or aetiology. KD is a self-limited illness that is not associated with the production of autoantibodies or the deposition of immune complexes, and it rarely recurs. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, accompanied by four out of five findings: bilateral conjunctival injection, oral changes such as cracked and erythematous lips and strawberry tongue, cervical lymphadenopathy, extremity changes such as erythema or palm and sole desquamation, and polymorphous rash. Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings. The standard treatment of acute KD is intravenous immunoglobulin (IVIG) infusion and aspirin. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. If there is no response to treatment, patients are given a second dose of IVIG with or without corticosteroids or other adjunctive treatment.
カテゴリ  
免疫系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  非器官特異的全身性自己免疫疾患
   4A44  血管炎
    H01718  川崎病
治療薬   
アスピリン [DR:D00109]
シクロスポリン [DR:D00184]
インフリキシマブ [DR:D02598]
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コハク酸エステルナトリウム [DR:D00751]
ポリエチレングリコール処理人免疫グロブリン [DR:D08783]
乾燥スルホ化人免疫グロブリン [DR:D08786]
乾燥ポリエチレングリコール処理人免疫グロブリン [DR:D08788]
リンク   
ICD-11: 4A44.5
ICD-10: M30.3
MeSH: D009080
文献    
  著者
Hara T, Nakashima Y, Sakai Y, Nishio H, Motomura Y, Yamasaki S
  タイトル
Kawasaki disease: a matter of innate immunity.
  雑誌
Clin Exp Immunol 186:134-143 (2016)
DOI:10.1111/cei.12832
文献    
  著者
Galeotti C, Kaveri SV, Cimaz R, Kone-Paut I, Bayry J
  タイトル
Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease.
  雑誌
Drug Discov Today 21:1850-1857 (2016)
DOI:10.1016/j.drudis.2016.08.004
文献    
  著者
Saguil A, Fargo M, Grogan S
  タイトル
Diagnosis and management of kawasaki disease.
  雑誌
Am Fam Physician 91:365-71 (2015)
文献    
  著者
Rodriguez-Gonzalez M, Matamala-Morillo MA, Segado-Arenas A
  タイトル
Infliximab as rescue therapy in refractory Kawasaki disease.
  雑誌
Ann Pediatr Cardiol 7:74-5 (2014)
DOI:10.4103/0974-2069.126578
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