Kawasaki’s disease (KD) is an acute febrile illness of infants and children, characterized by cutaneous and mucosal erythema and edema with subsequent desquamation, cervical lymphadenitis, and complicated by coronary artery aneurysms (20%).
Causes of Kawasaki’s Disease
Generalized vasculitis. Endarteritis of vasavasorum involves adventitia/ intima of proximal coronary arteries with ectasia, aneurysm formation, vessel obstruction, and distal embolization with subsequent myocardial infarction. Other vessels: brachiocephalic, celiac, renal, iliofemoral arteries. Increased activated helper T cells and monocytes, elevated serum soluble interleukin (IL)-2 receptor levels, elevated levels of spontaneous IL-1 production by peripheral blood mononuclear cells, antiendothelial antibodies, and increased cytokine-inducible activation antigens on the vascular endothelium occur in KD. T cell response is driven by a conventional antigen.
Symptoms of Kawasaki’s Disease
Kawasaki disease has a typical set of symptoms that occur in 90% of individuals and include:
- High fever for at least 5 days
- Eye irritation (conjunctivitis) without pus
- Dry, red lips that crack and bleed and/or bumpy red tongue (“strawberry tongue”)
- Palms of hands and soles of feet are red; skin starts to peel off fingers and toes about 2 weeks after illness starts
- Red rash on body
Other symptoms may include enlarged lymph nodes in the neck (50-75% of individuals), extreme irritability, joint pain or swelling, and poor appetite.
Diagnosis
Diagnostic criteria: fever spiking to >39.4°C, lasting ≥5 days without other cause, associated with four of five criteria:
- bilateral conjunctival injection;
- at last one of following mucous membrane changes: injected/ fissured lips, injected pharynx, “strawberry” tongue;
- at least one of the following extremity changes: erythema of palms/soles, edema of hands/feet, generalized/periungual desquamation;
- diffuse scarlatiniform erythroderma, deeply erythematous maculopapular rash, iris lesions;
- cervical lymphadenopathy (at least one lymph node ≥ 1.5 cm in diameter).
Treatment
Diagnosis should be made early and attention directed at prevention of the cardiovascular complications.
Hospitalization Recommended during the phase I illness, monitoring for cardiac and vascular complications.
Systemic Therapy
Intravenous Immunoglobulin 2 g/kg as a single infusion over 10 h together with aspirin.
Aspirin 100 mg/kg/d until fever resolves or until day 14 of illness, followed by 5 to 10 mg/kg/d until ESR and platelet count have returned to normal.
Glucocorticoids Contraindicated. Associated with a higher rate of coronary aneurysms.
References