非典型川崎氏症的診斷 Incomplete Kawasaki disease

Evaluation of suspected incomplete Kawasaki disease.

上圖的為非典型川崎氏症的診斷流程

典型川崎症診斷為燒五天+以下五項至少四項
1是一張嘴巴: 草莓舌 嘴唇乾裂紅腫
2是兩隻眼睛:結膜發紅，無分泌物
3是淋巴，單側淋巴結腫大超過1.5cm
4是四肢手腳掌紅腫
5是多型性紅斑 軀幹手腳出現大小的疹子 會融成一片

出自2017年AHA針對川崎氏症所法表最新的診斷與治療指引(Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease - A Scientific Statement for Health Professionals From the American Heart Association)

(1) In the absence of a “gold standard” for diagnosis, this algorithm cannot be evidence based but rather represents the informed opinion of the expert committee. Consultation with an expert should be sought any time assistance is needed.

診斷川崎氏症麻煩的就是並沒有所謂的 Golden Standard，就是抽血驗到某種東西或影像看到某種東西就可以診斷。無法確定時請會診兒童心臟專科醫師。

(2) Characteristics suggesting that another diagnosis should be considered include exudative conjunctivitis(化膿性結膜炎), exudative pharyngitis(化膿性咽炎), ulcerative intraoral lesions(口腔內潰瘍), bullous or vesicular rash(水泡狀的疹子), generalized adenopathy, or splenomegaly(全身淋巴結腫大或脾腫大).

看到以上這些症狀，要考慮川崎氏症以外的診斷。

(3) Infants ≤6 months of age are the most likely to develop prolonged fever without other clinical
criteria for Kawasaki disease; these infants are at particularly high risk of developing coronary artery abnormalities. 小於六個月以下幼兒診斷川崎症較困難，除了一直燒之外，缺乏其他典型的臨床症狀，故容易延遲診斷產生心臟冠狀動脈的併發症

(4) Echocardiography is considered positive for purposes of this algorithm
if any of 3 conditions are met: (心臟超音波陽性:以下三項裡面至少有符合一項)
1. Z score of LAD or RCA ≥2.5; (直接看到LAD or RCA Z score>2.5)
Z score 怎麼算 -> 連結在此
2. 超音波直接看到冠狀動脈瘤;

3. ≥3 other suggestive features exist, including decreased left ventricular function, mitral regurgitation, pericardial effusion  以下四下至少出現三項: LV收縮功能差, MR , 心包膜積液或 Z scores in  LAD or RCA of 2 to 2.5.

(5) If the echocardiogram is positive, treatment should be given within 10 days of fever onset or after the tenth day of fever in the presence of clinical and laboratory signs (C-reactive protein [CRP], erythrocyte sedimentation rate [ESR]) of ongoing inflammation.
治療是給予 IVIG 2gm/kg，最好能在開始發燒的10天內給予治療，若超過10天臨床上仍有症狀或有 ESR or CRP上升仍需給予治療。

(6) Typical peeling begins under the nail beds of fingers and toes.
通常在開始發燒後12~25天後手腳指甲床會出現脫皮的現象

Ref: Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease
From Journal: Circulation. 2017;135:e927–e999. DOI: 10.1161/CIR.0000000000000484

補充
治療藥物:
IVIG: 2gm/kg/dose for 12 hours
Aspirin(ASA):ASA has important anti-inflammatory activity (at high doses) and antiplatelet activity (at low doses) it does not appear to lower the frequency of development of coronary abnormalities.
Ref: Salicylate for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2006;(4):CD004175.