artery complications. 4 However, IVIG resistance affects approximately 10-20 % of KD patients and is associated with a higher risk of CAA. 8 While several predictive models, such as the Egami, Sano, and Kobayashi scores, have been developed to identify IVIGresistant patients, their accuracy is variable, especially outside of Japanese populations. 9 In our patient, some predictive factors were present, but not all, highlighting the known limitations of the existing tools for evaluating a broader population. 10 Gallbladder hydrops, a known complication of KD, has also been identified as a predictor of IVIG resistance. 11 Timely administration of IVIG and second-line therapies is crucial, as early treatment can mitigate the risk of CAA. 12
For IVIG-resistant disease, the 2024 American Heart Association( AHA) identifies several acceptable therapies, including repeat IVIG infusion, corticosteroids, and tumor necrosis factor-α inhibitors( e. g. infliximab). 4 The 2024 guidelines cite infliximab as an effective alternative rescue therapy, supported by evidence demonstrating faster fever resolution, shorter hospital stays, and comparable coronary artery outcomes relative to repeat IVIG. 4 In this patient, infliximab was selected in combination with IVIG redosing due to persistent systemic inflammation, IVIG resistance, and the presence of gallbladder hydrops, an indicator of heightened inflammatory response. Corticosteroids alone were not initially favored given concerns for delayed fever resolution and heterogeneous effects on coronary outcomes, while etanercept was less strongly supported by available comparative data. Thus, the combined use of infliximab and IVIG in this case aligns with AHA-endorsed treatment pathways for refractory KD and reflects a tailored approach aimed at prompt inflammation control and prevention of coronary artery complications.
CONCLUSION
This patient case highlights the diagnostic complexity and therapeutic challenges of KD, particularly in the setting of viral positivity and IVIG resistance. Although initially presenting with four days of persistent high-grade fever and viral symptoms, the patient subsequently developed classic features of KD: bilateral conjunctivitis, morbilliform rash, fissured lips, strawberry tongue, extremity swelling, and cervical lymphadenopathy, leading to the initiation of treatment with IVIG and high-dose aspirin. Despite standard therapy, the patient remained febrile. Expanded workup revealed gallbladder hydrops, a recognized manifestation of atypical KD. This finding reinforced the diagnosis to support escalation of care to additional immunomodulatory therapies. The patient’ s subsequent rapid improvement and normal repeat echocardiogram underscore the importance of early recognition and prompt management of IVIG-resistant KD. Clinicians must maintain a high index of suspicion for KD, including IVIG-resistant and atypical forms, even in the presence of positive laboratory testing for infectious pathogens. Importantly, the identification of infectious pathogens should not preclude a comprehensive evaluation for KD, as delayed recognition and treatment may increase the risk of CAA formation. Manifestations such as gallbladder hydrops may provide supportive evidence for escalation of therapy in situations where diagnostic uncertainty exists.
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