| H01718 | |
| H番号 | 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 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 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] pH4処理酸性人免疫グロブリン [DR:D08779] 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] |
| コメント | - |
| リンク | ICD-11: 4A44.5 ICD-10: M30.3 MeSH: D009080 |
| 文献 | PMID:27342882 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 PMID:27506874 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 PMID:25822554 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) PMID:24701096 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 |