Mucocutaneous lymph node syndrome Ma Lian Kawasaki Disease Mucocutaneous lymph node syndrome Ma Lian

Introduction The leading cause of acquired heart disease in children Kawasaki disease (KD) is a common vasculitic disorder usually seen in children below 5 years of age The leading cause of acquired heart disease in children

Kawaski T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children: clinical observations of 50 cases. Jpn J Allergol.1967; 16 :178 –222

China kawasaki disease Epidemiologic study of admitted children with Kawasaki disease in Beijing from 1995 to 1999 :The incidence of KD in Beijing is lower than that reported in Japan, similar to the incidence in the United States and higher than in those other Western countries.

Etiology No one knows what causes Kawasaki disease. But it is thought to start from an infection or from exposure to some toxin. There is no firm evidence that the disease can spread from one person to another. Superantigen-producing bacteria.

Histroy Kawasaki disease has 3 stages, as follows Acute stage (1-11 d) Subacute stage (11-30 d) Convalescent/chronic phase (>30 d)

Acute stage (1-11 d) 1. High fever (temperature >39℃) 2. Nonexudative bilateral conjunctivitis (90%) 3. Polymorphous erythematous rash 4. Acral erythema and edema that impede ambulation 5. Strawberry tongue and lip fissures 6. Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm

Acute stage Nonexudative bilateral conjunctivitis (90%)

Acute stage Polymorphous erythematous rash

Acute stage Acral erythema and edema that impede ambulation

Acute stage Strawberry tongue and lip fissures

Acute stage Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm

Acute stage (1-11 d) 7. Hepatic, renal, and gastrointestinal dysfunction 8. Myocarditis and pericarditis 9. Irritability 10. Anterior uveitis (70%) 11. Perianal erythema (70%)

Irritability

Subacute stage (11-30 d) Persistent irritability, anorexia, and conjunctival injection Decreased temperature Thrombocytosis Acral desquamation Aneurysm forms

Aneurysm forms

Convalescent/chronic phase (>30 d) Expansion of aneurysm Possible MI A tendency for smaller aneurysms to resolve on their own (60% of cases)

Diagnostic criteria 1.Fever(> 5 days) and refractory to appropriate antibiotic therapy 2.Polymorphous erythematous rash 3.Oropharyngeal changes, including diffuse hyperemia, strawberry tongue, and lip changes (eg, swelling, fissuring, erythema, bleeding)

Diagnostic criteria 4.Peripheral extremity changes, including erythema, edema, induration, and desquamation 5.Nonpurulent cervical lymphadenopathy 6.Nonexudative bilateral conjunctivitis

Diagnostic criteria Patients with classic Kawasaki disease must have 5 of the former symptoms, with fever an absolute criterion.

Differentials Staphylococcal infection (such as scalded skin syndrome, toxic shock syndrome) Streptococcal infection (such as scarlet fever, toxic shock-like syndrome). Throat carriage of group A streptococcus does not exclude the possibility of Kawasaki disease

Differentials Measles and other viral exanthems Leptospirosis Rickettsial disease Stevens-Johnson syndrome Drug reaction Juvenile rheumatoid arthritis

Lab Studies Mild-to-moderate normochromic anemia moderate-to-high WBC count ESR ↑, C-reactive protein ↑, and serum a-1-antitrypsin ↑. Culture results are all negative

Lab Studies ANA, RF,ASO normal Platelets Thrombocytosis （2-3w） associated with severe coronary artery disease and MI. Liver enzymes AST, ALT↑ bilirubin ↑

Lab Studies Cardiac enzymes ↑ （ CK，CK-MB, cardiac troponin, LDH） Radiography: rule out cardiomegaly or subclinical pneumonitis.

Imaging Studies Echocardiography: rule out CAAs and myocarditis, valvulitis, or pericardial effusion. Diffuse dilatation of coronary lumina can be observed in 50% of patients by the 10th day of illness. Echocardiography should be repeated in the second or third week of illness and 1 month after all other laboratory results have normalized.

Imaging Studies Ultrasonography: Gall bladder ultrasonography （liver or gall bladder dysfunction ） scrotal ultrasound to evaluate for epididymitis.

Imaging Studies MRA: defines CAA in patients with Kawasaki disease. noninvasive.

Other Tests ECG acute infarction. Tachycardia, a prolonged PR interval, ST-T wave changes, decreased voltage of R waves may indicate myocarditis. Q waves or ST-T wave changes may indicate an MI.

Medical Care The main goal of treatment is to prevent coronary artery disease and relieve symptoms.: Full doses of salicylates (aspirin); intravenous gammaglobulin are the mainstays of treatment.

Drug Category IVIG (first line but not the sole therapy) Neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, blocks Fc receptors on macrophages suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination

IVIG 400 mg/kg/d IV as a single daily infusion for 4 d-5d Alternatively,; 2 g/kg IV infused over 12 h once as single dose

Drug Category Aspirin decrease inflammation, inhibit platelet aggregation improve complications of venous stases and thrombosis. Irreversibly inactivates cyclooxygenase, ultimately preventing thromboxane A2 production in platelets.

Drug Category Aspirin 80-100 mg/kg/d PO divided qid for 2 wk initially; then 5-10 mg/kg PO qd for 6-8 wk until sedimentation rate and platelet count are within the reference range, typically used for 6-12 wk

Drug Category Corticosteroid Not recommended to use only. Prescript only when the therapeutic effect of IVIG is not satisfied. 2mg/kg, 2—4 weeks

Complications Cardiovascular Significant heart failure or myocardial dysfunction (unlikely to occur once fever is resolved) Diffuse coronary artery ectasia and aneurysm formation, giant aneurysm (internal luminal diameter >8 mm) MI

Complications Cardiovascular Myocarditis (common but rarely causes CHF) Valvulitis, usually mitral (only occurs in 1% of patients and rarely requires valve replacement) Pericarditis with small pericardial effusions (occurs in 25% of patients with acute illness) Systemic artery aneurysms Rupture of CAA with hemopericardium

Other complications Extreme irritability, especially in younger infants Aseptic meningitis Arthritis Mild hepatic dysfunction, rarely jaundice 巨嗜细胞活化过度综合症：死亡原因之一

Other complications Gallbladder hydrops (diagnosed by means of ultrasonography but usually resolves without surgical intervention) Diarrhea Pneumonitis Otitis media

Other complications Erythema and induration at the site of BCG inoculation (reported in Japan) Peripheral extremity gangrene (extremely rare) Bowel ischemia and necrosis

Prognosis With prompt treatment, the prognosis is good. The current mortality rate is 0.1-2%. 2% to 4%% of treated children still develop coronary artery disease

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