難治型川崎病治療的回顧

Review of Treatment for Refractory Kawasaki Disease

郭瓊文(Chiung-Wen Kuo) ； 李忠成(Chung-Chen Li) ； 謝正芳(Cheng-Fang Hsieh)

《南臺灣醫學雜誌》 12卷1期 (2016/07) Pp. 1-10

https://doi.org/10.6726/MJST.201607_12(1).0001

摘要

川崎病(Kawasaki disease)致病機轉是急性全身性血管炎， 主要侵犯中型大小的血管， 特別是冠狀動脈。病程初期未獲得良好處理，可能會有20到25%的機會導致嚴重的心血管損傷， 死亡主要原因為心血管併發症與延誤診治。急性期治療主要針對減輕發炎症狀和預防血栓形成，一般是給予高劑量aspirin合併單劑靜脈注射免疫球蛋白(intravenous immunoglobulin[IVIG])來降低發炎、發燒症狀及減少冠狀動脈瘤的發生。有些病人在給予IVIG治療36到48小時後仍然有發燒情形，則被認為是難治型川崎病，約有9.4到23%，其心臟異常風險會增加，須要進一步治療。對於難治型川崎病可考慮的療法有：(1)投予第二劑IVIG、(2)類固醇治療、(3)腫瘤壞死因子-α ( tumornecrosis factor-alpha[TNF-α])抑制劑，如單株抗體(infliximab)治療、(4)免疫抑制劑，如cyclosporine、cyclophosphamide或methotrexate治療或(5)其他治療，如血漿置換術(plasma exchange)、抗介白質-1和抗CD-20抑制劑等。本篇文章主要是針對難治型川崎病的治療進行文獻回顧， 讓大家面對這樣的病患時可以有更清楚的治療選擇。

關鍵字

川崎病 ； 難治型 ； 類固醇 ； 藥物不良反應

並列摘要

The pathogenesis of Kawasaki disease (KD) is acute systemic vasculitis, involving medium-sized vessels especially coronary arteries. If untreated, 20–25% of children develop severe cardiovascular injury causing death. Main goals in acute stage are to alleviate inflammation and prevent from thrombosis. Conventionally, large-dose plus single dose intravenous immunoglobulin (IVIG) may reduce inflammation, resolve fever and lower the incidence of coronary artery aneurysm. Some patients have fever at 36 to 48 hours later after initial IVIG, so-called refractory KD, accounting for 9.4 to 23% of KD. The risk of cardiac abnormalities in refractory KD is increased and additional therapy is needed. The options for refractory KD include: (1) second dose of IVIG, (2) corticosteroids, ( 3 ) TNF-α inhibitors (infliximab), (4) immunosuppressants (cyclosporine, cyclophosphamide, or methotrexate), (5) other therapies (plasma exchange, anti-interleukin-1 or anti-CDaspirin 20). This article is focused on literature review for refractory KD, making clinicians more familiar with treatment options for such patients.

並列關鍵字

Kawasaki disease ； refractory ； IVIG ； steroid ； adverse events