Chapter 177: Kawasaki Disease

Patient Story

A 13-month-old previously healthy boy was admitted to the hospital with a 7-day history of high fever and marked irritability. Over the past 3 days, his parents noted that he had developed a red rash over his face, trunk, and extremities, as well as redness and cracking of his lips. He was also noted to have swelling in his hands and feet. He was admitted because of concern of Kawasaki disease. On physical examination, he was irritable and ill-appearing, had a diffuse pleomorphic rash on his face, trunk, and extremities (Figure 177-1), nonpurulent conjunctivitis, cracked fissured lips (Figure 177-2), a tender 2 cm-diameter lymph node in his posterior cervical area, and swelling in his extremities (Figure 177-3). He was treated with intravenous immune globulin and high-dose aspirin and recovered completely. Baseline echocardiography did not reveal any coronary artery abnormalities. Follow-up echocardiograms 2 weeks and 8 weeks after his presentation were normal.

Introduction

Kawasaki disease is an acute vasculitis that has emerged as the most common cause of acquired heart disease in children in the developed world. Based on the epidemiology and clinical features of this disorder, an infectious etiology is considered likely, although the precise etiology remains elusive. It is important to recognize the clinical manifestations of KD because the diagnosis is based on clinical criteria, and because timely treatment significantly reduces the risk of coronary artery disease, the most feared consequence of this disease.

Synonyms

Mucocutaneous Lymph Node Syndrome or Kawasaki Syndrome.

Epidemiology

• Eighty percent of children are under 5 years of age; rare in infants less than 3 months of age and children older than 8 years of age.¹

• Male: Female ratio is 3:2.

• Predominance of cases in winter-spring in temperate climates.

• Incidence highest among Asian children, even in those living in non-Asian countries.²

• Epidemics of disease can occur in all ethnic groups.

• Recurrence of disease occurs rarely.

• No person-to-person spread of disease.

Etiology and Pathophysiology

• Precise etiology is not known, but epidemiologic features (age group affected, seasonal predilection, occurrence of epidemics) point to an infectious etiology.

• Marked immunological activation in affected patients leads to inflammatory cell ...