When coronaries speak last; atypical Kawasaki disease beyond the classical criteria - A four-case series

Coronary artery aneurysms (CAAs) are the most serious complication of Kawasaki disease (KD). Delayed diagnosis is associated with an increased risk of CAA, particularly in atypical presentations lacking classic mucocutaneous features. We report four children diagnosed with atypical KD only after coronary abnormalities were detected on echocardiography. Initial presentations included acalculous cholecystitis with marked transaminase elevation, aseptic meningitis, and an inflammatory arthritis. None of the cases had fulfilled complete diagnostic criteria at admission. Coronary imaging showed single- or multi-segment aneurysms ranging from small to giant (maximum Z-score +11.7). All received intravenous immunoglobulin and high-dose acetylsalicylic acid; corticosteroids, anticoagulation, or biologic therapy were added as treatment in cases with giant CAA. CAA persisted in patients with giant aneurysms. Early echocardiography is essential to establish the diagnosis in atypical KD and it may prevent irreversible coronary complications.