Guidelines for Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease (JCS 2013)
Year: 2014
Type: article
Abstract: adolescence or later, and the occurrence of acute coronary syndrome in adults with a history of Kawasaki disease in whom coronary artery lesions were considered regressed after the acute phase of the disease.We also have to address the problem of susceptibility to atherosclerosis in patients with a history of vasculitis due to Kawasaki disease.Because the pathophysiology of cardiovascular sequelae in Kawasaki disease changes over time during childhood, adolescence and adulthood, guidance on the diagnosis, treatment and management of cardiovascular sequelae in different ages Forty-five years have passed since 1967, 1 when the first case series of Kawasaki disease was reported.Currently, more than half of the patients diagnosed with Kawasaki disease are 20 years of age or older.As this timeline suggests, it is expected that more than 10,000 patients with cardiovascular sequelae in Kawasaki disease have reached adulthood.However, since Kawasaki disease develops most frequently by around 1 year of age, many internists are still not familiar with it (See Table 3).Recent issues on Kawasaki disease include the high percentage of patients who stop visiting their clinic in early 2524JCS Joint Working Group the number of patients have tended to increase annually.There is a seasonable pattern in the number of new cases.In the recent two years the number of new cases was low in fall (September and October) while high in spring and summer.Patients under three years of age accounted for 66.8%.The incidence rate by age shows a monomodal distribution and is highest in boys 6~8 months of age and girls 9~11 months of age.Kawasaki disease was especially prevalent in Kanagawa, Nagano and Wakayama Prefectures, and there have been sporadic increases in cases in specific areas.Patients with a family history of Kawasaki disease accounted for 1.6% of all patients.Among the reported cases, 163 patients (0.7% of the reported cases; 0.6% in males and 0.8% in females) had a parent who has suffered from Kawasaki disease (74 fathers and 69 mothers had the disease).Recurrent cases accounted for 3.6% (3.9% in males and 3.1% in females).In the latest 2 years, one patient died (mortality: 0.004%).The patient had typical Kawasaki disease that occurred at 3 months of age, and died within 2 months after the onset due to cerebral infarction.Table 3 summarizes guidance for the diagnosis of Kawasaki disease. 5 Cardiovascular Complications (Figure 2)Among the patients assessed in the 21st nationwide survey, 9.3% (11.0% in males and 7.1% in females) experienced acutephase cardiovascular complications, and 3.0% (3.6% in males and 2.1% in females) experienced cardiovascular sequelae.Acute-phase complications included coronary dilatation in 7.26%, valvular lesions in 1.19%, aneurysms in 1.04%, giant aneurysms in 0.24%, coronary stenosis in 0.03%, and myocardial infarction in 0.01%, which were less prevalent as compared with the previous survey. 4ardiovascular sequelae included coronary dilatation in 1.90%, aneurysms in 0.78%, valvular lesions in 0.29%, giant aneurysms in 0.22%, coronary stenosis in 0.03%, and myocardial infarction in 0.02%.The incidence rate of giant aneurysms was about three-fold higher in males than in females. 4ble 3. Diagnostic Guidelines of Kawasaki Disease (MCLS: Infantile Acute Febrile Mucocutaneous Lymph Node Syndrome) This is a disease of unknown etiology affecting most frequently infants and young children under 5 years of age.The symptoms can be classified into two categories, principal symptoms and other significant symptoms or findings. A. Principal symptoms1. Fever persisting for 5 days or more (inclusive of those cases in whom the fever has subsided before the 5th day in response to therapy) 2. Bilateral conjunctival congestion 3. Changes of lips and oral cavity: Redding of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa 4. Polymorphous exanthema 5. Changes of peripheral extremities:(Acute phase): Redding of palms and soles, indurative edema (Convalescent phase): Membranous desquamation from fingertips 6. Acute nonpurulent cervical lymphadenopathy At least five items of 1∼6 should be satisfied for diagnosis of Kawasaki disease.However, patients with four items of the principal symptoms can be diagnosed as Kawasaki disease when coronary aneurysm or dilatation is recognized by two-dimensional (2D) echocardiography or coronary angiography. B. Other significant symptoms or findingsThe following symptoms and findings should be considered in the clinical evaluation of suspected patients.1. Cardiovascular: Auscultation (heart murmur, gallop rhythm, distant heart sounds), ECG changes (prolonged PR/QT intervals, abnormal Q wave, low-voltage QRS complexes, ST-T changes, arrhythmias), chest X-ray findings (cardiomegaly), 2D echo findings (pericardial effusion, coronary aneurysms), aneurysm of peripheral arteries other than coronary (e.g., axillary), angina pectoris or myocardial infarction 2. Gastrointestinal (GI) tract: Diarrhea, vomiting, abdominal pain, hydrops of gallbladder, paralytic ileus, mild jaundice, slight increase of serum transaminase 3. Blood: Leukocytosis with shift to the left, thrombocytosis, increased erythrocyte sedimentation rate (ESR), positive C reactive protein (CRP), hypoalbuminemia, increased α2-globulin, slight decrease in erythrocyte and hemoglobin levels 4. Urine: Proteinuria, increase of leukocytes in urine sediment 5. Skin: Redness and crust at the site of BCG inoculation, small pustules, transverse furrows of the finger nails 6. Respiratory: Cough, rhinorrhea, abnormal shadow on chest X-ray 7. Joint: Pain, swelling 8. Neurological: Cerebrospinal fluid (CSF) pleocytosis
Source: Circulation Journal
Author: JCS Joint Working Group
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4244865467