Recurrent Intravenous-Immunoglobulin-Resistant Kawasaki Disease Within 14 Weeks Despite High-Risk Intensification: A Case Report

Kawasaki disease (KD) is an acute vasculitis of childhood and the leading cause of acquired heart disease in children. Recurrence occurs in approximately four percent of cases after a median of 18 months and a first episode is typically managed with intravenous immunoglobulin (IVIG) and aspirin as initial therapy. Adjunctive infliximab is generally reserved for IVIG-resistant cases or for patients categorized as high risk for developing coronary artery aneurysms. We describe a 15-month-old male with recurrent IVIG-resistant KD only 14 weeks after an initial IVIG-resistant episode that was complicated by small coronary aneurysms of the right coronary, left coronary, and left anterior descending arteries (z scores 3.6, 4.2, 3.8). Both episodes were preceded by prolonged gastrointestinal (GI) symptoms. During his second episode, he initially received IVIG, aspirin, and infliximab in accordance with the American Heart Association (AHA) high-risk guidelines. However, fever persisted after 36 hours and he was subsequently started on intravenous methylprednisolone, after which he demonstrated clinical improvement and normalization of coronary artery measurement. This case highlights the unusual presentation of recurrent KD that was also resistant to IVIG and preceded by GI symptoms in both episodes. The short interval to recurrence underscores the importance of early surveillance after a high-risk first episode, and the need for more explicit escalation strategies in recurrent resistant KD.