Combination Therapy of Cyclosporin A and Plasma Exchange for Infants with Immunoglobulin-resistant Kawasaki Disease

抄録

Kawasaki disease (KD) is an acute childhood febrile illness that is classified as a systemic vasculitis syndrome. Combination intravenous immunoglobulin (IVIG) and acetylsalicylic acid is the standard initial and second-line therapy for KD to resolve inflammation and reduce the occurrence of coronary artery lesions (CALs). The goal of treatment in the acute phase of KD is to decrease inflammation and arterial damage to prevent CALs. For IVIG-resistant KD, however, additional treatments should be administered promptly before CAL formation, such as steroids, infliximab, cyclosporin A (CsA), and plasma exchange (PE). Although the effectiveness of PE on refractory KD is well known, it is highly invasive, especially in infants. Therefore, safer treatments are needed to avoid or minimize the number of PE courses in severe infantile cases. We herein report the clinical outcomes of 2 infants with IVIG-resistant KD who were treated successfully with a combination therapy of CsA and PE as third-line treatment, thereby possibly reducing the number of PE sessions.