**4. Diagnosis and acute therapy for KD**

The recent diagnostic guidelines in Japan are shown in **Table 1** [13]. The typical clinical symptoms are shown in **Figure 1**. Acute treatment should be initiated immediately after diagnosis to prevent cardiovascular complications. Although the incidence of CAL was reported in 23–43% of patients treated only with aspirin, treatment with IVIG and aspirin for four consecutive days reduced the incidence of CAL to 8–15% [14, 15]. Moreover, a single infusion of 2 g/kg IVIG, which is the current standard regimen, reduces the incidence of CAL to 4.6% [16]. Therefore, IVIG is currently the standard therapy for acute KD. A systematic review by the Cochrane Collaboration revealed that the development of CAL can be reduced by a single dose of 2 g/kg IVIG administered before the tenth day of illness [17].

The risk of developing CAL is closely related to responsiveness to treatment. KD patients with IVIG resistance are at an increased risk of developing CALs compared

Principal clinical features


Other significant demographic, clinical, echocardiographic, and laboratory features

	- Elevation of hepatic transaminases early in the course of the disease.
	- Increased leukocytes in the urine sediment of an infant.
	- Thrombocytosis in the convalescent phase
	- Elevation of BNP or NT-pro BNP
	- Mitral valve regurgitation or pericardial effusion by echocardiography
	- Enlargement of the gallbladder (hydrops of gallbladder)
	- Hypoalbuminemia or hyponatremia
	- Hemodynamically significant myocarditis
	- Hypotention (shock)
	- Paralytic ileus
	- Decreased level of consciousness
	- Leukocytosis with left shift
	- thrombocytopenia
	- hypoalbuminemia
	- hyponatremia
	- hyperbilirubinemia (jaundice)
	- elevation of CRP
	- Age <1 year
	- Irritability
	- Cardiovascular: abnormal extra heart sounds, electrocardiogram changes, aneurysm of peripheral arteries other than coronary (axillary etc.),
	- Gastrointestinal: abdominal pain, vomiting, diarrhea
	- Hematologic: increased erythrocyte sedimentation rate, anemia
	- Dermatologic: micropustular rash, transverse grooves across the finger nails.
	- Respiratory: cough, rhinorrhea, retropharyngeal edema, infiltrate on chest radiograph.
	- Rheumatologic: pain, swelling.
	- Neurologic: cerebrospinal fluid pleocytosis, seizures, facial nerve palsy, paralysis of the extremities.

#### **Table 1.**

 *Diagnostic guideline for Kawasaki disease.* 

#### **Figure 1.**

 *Typical clinical symptoms of Kawasaki disease. (a) Bulbar conjunctival injection, (b) Reddening of lips, (c) Redness at the site of Bacille Calmette-Guerin inoculation.* 

*Cardiovascular Health in Kawasaki Disease DOI: http://dx.doi.org/10.5772/intechopen.108679*

to IVIG responders; therefore, various additional treatments such as prednisolone, infliximab, cyclosporine, urinary trypsin inhibitors, and plasma exchange have been established to prevent CAL.

In addition, to improve the prognosis of CALs, several risk-scoring systems to predict IVIG non-responders before initial treatment have been established and are widely used in clinical practice in Japan [18–20]. For patients with a high-risk score, two randomized control trials revealed the efficacy of a first-line combined treatment strategy, IVIG and prednisolone or IVIG and cyclosporin A [21, 22]. As several reports from other countries revealed that these risk-scoring systems are inadaptable to the prediction of IVIG non-responders in regions other than Japan [23, 24], it may be desirable to develop risk-scoring systems that can be used globally or an original scoring system to be adapted to each region for the suppression of KD vasculitis. Although these strategies have improved the prognosis of coronary arteries, the occurrence of giant coronary aneurysms is still observed, and further treatment is desirable.
