Third-line therapies in patients with Kawasaki disease refractory to first- and second-line intravenous immunoglobulin therapy

Excerpt

Kawasaki disease (KD) is an acute febrile illness characterized by systemic vasculitis affecting the small- and medium-sized arteries in children [1, 2]. One of the most critical complications of KD is the development of coronary artery lesions (CALs) approximately after 12 days of illness, which may lead to myocardial infarction [3]. High-dose intravenous immunoglobulin (IVIG) and oral administration of aspirin have been established as the first-line therapy for KD [4, 5]. However, 10%–20% of patients with KD are resistant to it, necessitating the use of additional therapies. According to the Japanese guideline [4], IVIG, steroids, infliximab (IFX), cyclosporin A (CsA), or plasma exchange are recommended as third-line therapies for KD. A nationwide Japanese survey reported that IVIG, steroids, IFX, other immunosuppressive drugs, and/or plasma exchange were administered as second-line or later therapy in 21.6%, 6.3%, 2.6%, 1.5%, and/or 0.5% patients, respectively [6]. If second-line therapy is unsuccessful in treating KD, the optimal choice for the third-line therapy remains unclear. Therefore, we aimed to review patients with KD refractory to the second-line therapy and study the outcomes according to the different third-line therapies received. …