Afebrile Kawasaki Disease Presenting as Intestinal Pseudo-Obstruction

Thank you for providing us an opportunity to report a case of rare atypical Kawasaki disease (KD). As we know, KD is an acute febrile illness of childhood associated with vasculitis of medium-sized arteries, especially the coronary arteries. Through this letter, we would like to share our experience managing the case of a 7-month-old boy suffering from a rare atypical KD, which presented as intestinal pseudo-obstruction without fever. To our knowledge, no such case has ever been reported before. We believe that the awareness about such clinical presentation is critically important as it may otherwise lead to delayed diagnosis and therapy, subsequently may worsen the prognosis. Kawasaki disease is an acute, self-limited systemic type of vasculitis that occurs predominantly in young children and results in 20 - 25% of coronary artery abnormalities (CAA) in untreated children (1). Treatment with intravenous immunoglobulin (IVIG) in combination with acetyl salicylic acid (ASA) before the 10th day of illness reduces the prevalence of CAA to 3 - 5% (2). Therefore, timely diagnosis and treatment are essential to prevent CAA. The diagnosis is based on clinical criteria and non-specific laboratory findings. Fever is the most critical symptom in KD. Fever prolongation (≥ 5 days) is a necessary diagnostic criterion established by the American heart association (AHA) (1), that is different from the Japanese guidelines (3) in which fever prolongation isjust an optional criterion. To increase the awareness of afebrile KD, we report a 7-monthold boy who presented with intestinal pseudo-obstruction without fever and was subsequently diagnosed with KD.