Background: Kawasaki disease (KD) is an acute, self-limited vasculitis that predominantly affects children under five years of age. While most patients respond well to first-line treatment with intravenous immunoglobulin (IVIG), a subset may develop refractory KD, leading to severe cardiovascular complications, including giant coronary artery aneurysms (CAAs) and thrombosis. The management of these cases is complex and requires a multidisciplinary approach.
Case Presentation: Our case report outlines a complex and serious complication of Kawasaki disease (KD) in an 11-month-old male. He was diagnosed with KD after initially presenting with classic symptoms of fever, conjunctivitis, rash, cracked lips, and cervical lymphadenopathy. Despite timely administration of two doses of intravenous immunoglobulin (IVIG), aspirin, and corticosteroids, he developed refractory KD characterized by persistent inflammation and the formation of multiple giant coronary aneurysms, notably a 16 mm aneurysm in the left main coronary artery (LMCA) with a Z-score of +37. His condition worsened despite aggressive treatment, including infliximab and anakinra, and he subsequently developed bilateral coronary artery thrombosis, necessitating thrombolytic therapy with tissue plasminogen activator, anticoagulation with heparin and warfarin, and dual antiplatelet therapy. Although he achieved temporary stabilization, he suffered a fatal cardiac arrest 17 days post-discharge.
Conclusion: Refractory Kawasaki disease can have an aggressive course, leading to giant coronary artery aneurysms (CAAs) and coronary thrombosis, and represent significant therapeutic challenges. Our case highlights the critical importance of early identification, aggressive immunomodulatory therapy, and close cardiac monitoring. Despite intensive and multidisciplinary management, the prognosis in such severe cases remains poor, underscoring the need for ongoing research and the development of optimized, evidence-based treatment protocols.
Keywords: Kawasaki disease; Refractory KD; Coronary artery aneurysm; Coronary thrombosis; IVIG resistance; Anakinra; Infliximab; Pediatric vasculitis; tPA; Anticoagulation
