Kawasaki disease/ Mucocutaneous lymph node Syndrome/ Infantile Polyarteritis nodosa

It’s a acute febrile vasculitis of childhood. Affecting six months to four years of age and the peak incidence towards the end of the first year. Vasculitis mainly affecting medium and large arteries with a predilection to coronary arteries. Coronary artery involvement is the most serious complication and will leas to aneurysm formation. Etiology remains unknown but similarity to toxic shock syndrome suggest an infective origin with a super antigen activity.

Diagnosis of Kawasaki disease is based on demonstration of characteristic clinical signs.

It requires fever at least for five days and presence of four of the five clinical signs

1. Bilateral bulbar conjuntival injection, generally non purulent

2. Changes in the mucosa of the oropharynx- injected pharynx, dry fissured lips, strawberry tongue.

3. Changes of the peripheral extremities edema and/or redness of hands or feet in acute phase and periungal desquamation in the sub acute phase.

4. Rash primarily truncal polymorphous but non vesicular.

5. Cervical lymphadenopathy- usual unilateral >/ 1.5 cm.

Disease has three phases

Acute phase – first two weeks of the illness. Fever and the other acute signs are present.

Sub acute phase- Until the fourth week. Thrombocytosis, desquremation and coronary aneuryrsm formation occur.

Convalescent phase- begins when the patent is asymptomatic and until the ESR returns to normal. Approximately, it takes about six to eight weeks after the illness.

Management

Immediate management

1. Control fever with Paracetamol 15 mg/kg/dose six hourly

2. QHT

3. Cannula – take blood for investigations

4. Start empirical antibiotics – take blood for culture before starting antibiotics.

Specific management

Patients with acute Kawasaki disease should treat with intravenous immune globulins (IVIG) and Asprin as soon as possible. IVIG preferably should give within ten days of illness to prevent coronary aneurysm formation.

Acute stage

IVIG 2mg/kg over 10-12 hours

- 9kgs * 2mg/kg

18 mgs

Child was given 17.5 mg of IVIG on the tenth day of the illness. (Three and a half vials of IVIG)

Asprin 80-100 mgs /kg/day divided six hourly until the fourteenth day of the illness. Initially treated is with anti-inflammatory high doses.

Some patients occasionally will not respond to an initial IVIG or may have a partial response. Must strongly consider to retreat with IVIG in these patients. But the efficacy of such treatment is not proven.

Convalescent stage

Asprin 3-5 mg/kg 9 (Antithrombotic dose) once daily for six to eight weeks( Until ESR become normal)- starting after fourteen days of Asprin treatment or patient is fever free for three to four days within the initial fourteen days.

Long-term therapy for those with aneurysms

Small aneurysms – only Asprin antiplatelet dose.

Patients with giant aneurysms will need additional drugs.

Dipyridamole 4-6mg/kg/day divided doses.

Some times, add Warfarin too for those who are at high risk of thrombosis.

Acute coronary thrombosis – prompt fibrinolytic therapy- streptokinase or tPA.

Complications

Recovery is complete without any long-term effects for patients who don’t have coronary aneurysms.

Coronary artery aneurysms are the dreaded complication of Kawasaki disease and usually occur in the second week of the illness. It leads to thrombosis, rupture or stenosis, which will lead to acute myocardial infarction or sudden cardiac death.

(Small aneurysms will resolves ecocardiographically by one or two years. But giant aneurysms are unlikely to resolve and may need CABG with a arterial graft which grows with the child and prevent any aneurysm formation.)

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