Dissecting Kawasaki disease: a state-of-the-art review

Area of Science:

• Pediatric rheumatology
• Immunology
• Cardiology

Background:

• Kawasaki disease (KD) is a leading cause of acquired pediatric heart disease.
• It presents with persistent fever and systemic inflammation, primarily affecting coronary arteries.
• Coronary artery aneurysms (CAA) are the main complication, potentially leading to myocardial infarction.

Purpose of the Study:

• To review the current understanding of KD etiology, diagnosis, and management.
• To highlight the challenges in treating non-responsive KD cases.
• To emphasize the importance of long-term cardiovascular risk assessment in KD survivors.

Main Methods:

• Review of existing literature on Kawasaki disease.
• Analysis of diagnostic criteria and imaging techniques (echocardiography).
• Discussion of treatment protocols, including intravenous immunoglobulin (IVIG) and alternative therapies.

Main Results:

• KD diagnosis relies on fever and specific clinical signs.
• Echocardiography is crucial for detecting and monitoring CAA using diameter and z-scores.
• 10-20% of patients exhibit resistance to standard IVIG treatment, necessitating further interventions.

Conclusions:

• Despite advances, KD etiology remains incompletely understood, with genetic factors like the ITPKC pathway gaining attention.
• Long-term follow-up is crucial as KD patients transition to adulthood.
• Understanding the long-term cardiovascular risks associated with KD and CAA is an evolving area of research.