Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition)

Abstract

Diagnostic guidelines for Kawasaki Disease was revised to meet the present situation in 2002. This issue intends to explain new guidelines and their backgrounds. Major alterations are interpretation of cases with 4 or fewer febrile days shortened by early intravenous immunoglobulin treatment, and the clinical importance of atypical (incomplete, or suspected) cases.

Immunoglobulin Resistance in Kawasaki Disease

“…30 Steroid therapy has been recommended, more so in the immunoglobulin-resistant phenotype, to reduce the incidence of coronary artery aneurysms. [38][39][40][41][42] Infliximab, a monoclonal antibody against tumor necrosis factor a associated with signaling pathways of inflammation in Kawasaki disease, 43 has been reported to control fever in immunoglobulin-resistant children. 44 The immunoglobulin-resistant phenotype in the patients in the current study was similar to that described in previously published literature with a similar prevalence, and there were associations with a significantly longer duration of fever and a higher coronary artery involvement, signifying a hyperinflammatory status in resistant children.…”

ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms

“…The ethical committee of RIKEN approved the study, and all the parents of the patients gave written informed consent. All Japanese individuals with Kawasaki disease (male/female ratio = 384:253) were diagnosed by pediatricians based on the Japanese criteria for the disease 35 . Mean age of disease onset was 29.3 months (range 2-127 months).…”

Kawasaki Disease as a Systemic Vasculitis in Childhood

“…4) The principal symptoms include: 1) fever persisting for 5 days or more; 2) bilateral conjunctival congestion; 3) changes in the lips and oral cavity: reddening of the lips, strawberry tongue, and diffuse infection of the oral and pharyngeal mucosa; 4) polymorphous exanthema; 5) changes in the peripheral extremities: initial stage = reddening of the palms and soles, and indurative edema, convalescent stage = membranous desquamation from the fingertips; and 6) acute nonpurulent cervical lymphadenopathy. At least five of the above six items must be satisfied for a diagnosis of Kawasaki disease.…”