Efficacy and safety of plasma exchange for Kawasaki disease with coronary artery dilatation

Abstract

Abstract Background The treatment of Kawasaki disease is controversial when intravenous immunoglobulin therapy fails, although it typically relies on combinations of prednisolone, infliximab, cyclosporine, and plasma exchange therapy. The goal of the treatment is no longer merely to reduce mortality but also to decrease the sequelae of coronary artery lesions, which are the most common and potentially life-threatening complications. Recently, plasma exchange therapy has been used to treat intravenous immunoglobulin-unresponsive Kawasaki disease with coronary artery lesions. When performed before coronary artery dilatation, the outcomes for plasma exchange are known to be excellent; however, when dilatation is already present, sequelae persist. Methods Between December 2006 and April 2015, we treated ten patients with Kawasaki disease complicated by coronary artery lesions that received plasma exchange because intravenous immunoglobulin therapy had proven to be ineffective. Here, we retrospectively review the efficacy and safety of plasma exchange therapy in such unresponsive cases against coronary artery lesions in patients with Kawasaki disease when plasma exchange performed after coronary artery dilatation. Results In nine of the ten patients (90.0%), the body temperature was confirmed to be < 37.5 °C at an average of 2.7 ± 1.4 days after starting plasma exchange. Serum C-reactive protein levels decreased significantly from 9.9 ± 4.9 mg/dL before exchange to 1.9 ± 2.9 mg/dL after exchange (P < 0.05). One year after plasma exchange treatment, the coronary artery lesions had regressed to within normal limits in six of the ten patients. Although lesions remained in three patients, all three of these patients were asymptomatic. In addition, there were no stenosis of the coronary artery in nine of the ten patients. One patient died due to a ruptured giant coronary aneurysm 1 day after starting plasma exchange. Conclusions In conclusion, plasma exchange may be effective in not only regressing coronary artery lesions but also preventing sequelae in patients with Kawasaki disease when plasma exchange is performed after coronary artery dilatation.

Keywords

• Kawasaki disease
• Plasma exchange
• Coronary artery