Kawasaki disease: characteristics, diagnosis, and unusual presentations

ABSTRACT

Introduction: Kawasaki disease (KD) is one of the commonest pediatric vasculitides and is associated with a significant risk of development of coronary artery abnormalities if left untreated.

Areas covered: In this review, we have highlighted the incomplete and unusual presentations of KD and also emphasize the controversies pertaining to 2D echocardiography in KD. A PubMed search was performed regarding diagnosis and unusual presentations of KD.

Expert opinion: Diagnosis of KD is essentially clinical and based on recognition of typical clinical features that may appear sequentially and all signs and symptoms may not be present at one point of time. There is no confirmatory laboratory test for diagnosis of this condition. Further complicating the picture is the fact that incomplete and atypical forms KD may be seen in up to 50% patients. Although 2D echocardiography continues to be the preferred imaging modality for cardiac assessment in patients with KD, it has its limitations.

KEYWORDS:

• Kawasaki disease
• diagnosis
• unusual presentations
• echocardiography
• coronary artery abnormalities

Article highlights

• Diagnosis of KD is essentially clinical and based on guidelines that have been provided by American Heart Association and Japanese Ministry of Health. These guidelines are based on clinical signs and symptoms and there are, as yet, no confirmatory laboratory tests for diagnosis of this condition.

• In the absence of typical clinical features, the diagnosis of KD is often challenging even for clinicians with experience in this field.

• It has been reported that as many as half of all cases of KD diagnosed at any center may have incomplete presentations. Risk of coronary artery abnormalities in children with incomplete forms of KD is no less than the risk in children with complete KD.

• Severest form of KD is usually seen in infants and is associated with more severe coronary artery involvement as compared to older children.

• KD in adolescents and adults is often associated with delays in diagnosis and management, as this condition is rarely considered in differential diagnosis of febrile illnesses at this age.

• KD shock syndrome (KDSS) is an uncommon, under-recognized, and potentially life-threatening manifestation of KD.

• KD-associated macrophage activation syndrome is one of the most severe manifestations of KD.

• Echocardiography remains an important imaging modality for assessment of coronary arteries in children with KD but has several limitations.

• Assessment of coronary artery diameters in patients with KD should be based on body surface area-adjusted Z scores. However, one should be aware of the problems associated with obtaining accurate Z scores.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.