Long‐term efficacy of plasma exchange treatment for refractory Kawasaki disease

Abstract

Background: The treatment of Kawasaki disease patients who fail to respond to initial i.v. immunoglobulin (IVIG) therapy is controversial. The aim of the present study was to investigate the long-term efficacy of plasma exchange (PE) treatment for refractory Kawasaki disease. Methods: A total of 125 Kawasaki disease patients refractory to IVIG were treated with PE. Coronary artery lesions (CAL) before PE, in the acute period, and during the late period were examined retrospectively. Results: Residual sequelae requiring medical treatment occurred in six cases in the late period. The outcomes of treatment tended to be better when PE was begun in the early stage. Sequelae remained in 2.8% of patients in whom PE was initiated prior to day 9 after onset, and were present in 15% of patients in whom PE was started on or after day 10. The 105 patients whose coronary arteries were normal before PE had no sequelae (residual sequelae: 0%). Dilatation was present before PE in 14 patients, but remained in only two patients in the late period (residual sequelae, 14.3%). In four of the six patients in whom aneurysms had already formed before PE, the lesions had advanced into giant aneurysms, but in the other two patients they returned to the normal range (residual sequelae, 66.6%). Conclusions:The outcomes of PE for Kawasaki disease refractory to IVIG are favorable, and the effectiveness of this treatment is excellent, particularly if it is initiated before CAL arise.Key words coronary artery lesion, i.v. immunoglobulin, Kawasaki disease, plasma exchange, refractory Kawasaki disease.Through the establishment of a high-dose i.v. immunoglobulin (IVIG) protocol for Kawasaki disease, a high level of effectiveness can be achieved, 1 but between 10 and 15% of patients still fail to respond. 2Alternative treatments for patients refractory to IVIG have not yet been developed, and the approaches being tested vary greatly from institution to institution. Plasma exchange (PE) is a method with promising efficacy, but the number of facilities that perform it is limited, and few reports on outcomes have been published so far.3,4 In particular, there are no large-scale studies on the effects of PE on coronary artery lesions (CAL) in the late period.In the present study, we investigated the long-term efficacy of PE in IVIG-refractory Kawasaki disease patients. Methods DemographicsThe subjects were 125 children with Kawasaki disease (onset: between August 1994 and March 2007) who had been treated with PE because IVIG had proven ineffective. Those patients in whom steroids other than IVIG had been given before PE had been started were excluded from the study (two patients). One patient who had been pre-treated with infliximab was also excluded. One hundred and sixteen patients received their first IVIG treatment in 19 affiliated hospitals located close to each other. When a patient was judged to be refractory to IVIG, the patient was referred to one of the two hospitals (Yokohama City University Hospital and Yokohama City University Med...

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

“…Furthermore, the aneurysms did not develop into giant aneurysms even in more than 1 year after onset in all of our patients. The period of until the start of the plasma exchange after onset was 13.7 ± 5.7 days in our study, whereas 8.1 ± 1.9 days reported by Hokosaki et al [7]. Though plasma exchange was started late, coronary artery lesions regressed in almost all of our patients.…”

“…However, when dilatation had already begun before the therapy, about 30% (6/20) patients showed persistent sequelae. Further, the size of aneurysms increased in all patients in whom sequelae remained and developed into giant aneurysms in five of the six patients in the late period (≧1 year after onset) [7]. However, despite starting plasma exchange after the beginning of coronary artery expansion, we demonstrated that plasma exchange could improve the size of coronary artery lesions in almost all of the patients.…”

“…Plasma exchange was generally conducted as a vein-tovein procedure, although an artery-to-vein option was also used until 2006 [7]. For the procedure, a 6-or 7-Fr double-lumen catheter was inserted into the femoral vein.…”

“…Recent studies have reported the use of plasma exchange therapy in the treatment of IVIG-unresponsive Kawasaki disease [6][7][8]. When plasma exchange was initiated before the beginning of coronary artery dilatation, no sequelae developed in patients; however, when dilatation had already begun, sequelae developed in 30% of patients despite the plasma exchange [7].…”

“…When plasma exchange was initiated before the beginning of coronary artery dilatation, no sequelae developed in patients; however, when dilatation had already begun, sequelae developed in 30% of patients despite the plasma exchange [7]. Hokosaki et al also reported that treatment outcomes tended to be better when plasma exchange was initiated before day 9 of the disease onset [7]. In this study, we retrospectively reviewed the efficacy and safety of plasma exchange against coronary artery lesions in patients with Kawasaki disease unresponsive to IVIG therapy when coronary artery dilatation had already started.…”