Kawasaki disease

Abstract

Kawasaki disease is an acute febrile, systemic vasculitic syndrome of an unknown etiology that primarily occurs in children younger than five years of age. The principal presentations of Kawasaki disease include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develops in 15% to 25% of untreated children with the disease, which may later lead to myocardial infarction, sudden death, or ischemic heart disease. Treatment with intravenous gamma globulin (IVIG) is effective, but the mode of action is still unclear. The development of a diagnostic test, a more specific therapy, and ultimately the prevention of this potentially fatal illness in children are all dependent upon the continued advances in determining the etiopathogenesis of this fascinating disorder.

Fig. 1

(A) Bilateral, non-exudative conjunctival injection with perilimbal sparing. (B) Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding. (C) Erythematous rash involving perineum. (D) Erythema of the palms, which is often accompanied by painful, brawny edema of the dorsa of the hands. (E) Erythema of the soles, and swelling dorsa of the feet. (F) Desquamation of the fingers. (G) Erythema and induration at the site of a previous vaccination with Bacille Calmette-Gurin (BCG). (H) Perianal erythematous desquamation.

Fig. 2

On the ninth day of Kawasaki disease, acute acalculous distention of the gallbladder (hydrops) was identified by an abdominal ultrasound in a one-year-old boy.

Fig. 3

Schematic diagram of the pathogenesis of Kawasaki disease. ADCC indicates antibody-dependent cell-mediated cytotoxicity; AECA, anti-endothelial cell antibodies; ANCA, anti-neutrophil cytoplasmic antibodies; APC, antigen-presenting cell; CD40L, CD40 ligand; CTL, cytolytic T lymphocyte; ELAM-1, endothelial-leukocyte adhesion molecule-1; G-CSF, granulocyte colony stimulating factor; ICAM-1, intercellular adhesion molecule-1; IFN, interferon; IL, interleukin; HSP, heat shock protein; TNF, tumor necrosis factor.

Fig. 4

Body temperature in a 7-year-old boy with refractory Kawasaki disease. After using low-dose oral methotrexate (10 mg/BSA, once weekly), body temperature has returned to normal rapidly.

Fig. 5

(A) On the day 35 of Kawasaki disease, a 2D-echocardiogram demonstrates an aneurysm of the LCA in a six-year-old boy. (B) Coronary angiogram reveals diffuse dilatation in the left anterior descending (LAD) branch of the left coronary artery (LCA) in the same child.

Fig. 6

Abdomial aortogram (A) and a 3D-image of the computed tomogram (B) showing the aneurysm of the distal abdominal aorta, just below the origin of inferior mesenteric artery (IMA) with an involving bifurcation.