P523 Efficacy of cyclosporine therapy in intestinal Behçet's disease
T. Kawaguchi*, R. Tanaka, M. Sako, N. Yoshimura, M. Takazoe
Tokyo Yamate Medical Center, Department of IBD, Tokyo, Japan
Behçet's disease (BD) is a systemic inflammatory disease caused by abnormal responses of both innate and acquired immunity. Intestinal ulcers of BD tend to perforate easily and be refractory to immunosuppressive therapies including biologics. Cyclosporine A (CsA) is a calcineurin inhibitor which inhibits IL-2 expression in Th1. Although the efficacy of CsA therapy to BD uveitis has been proven, the efficacy to intestinal BD remains unclear. This study is aim to evaluate the efficacy of induction and maintenance therapy by CsA in the patients with active intestinal BD.
Ten patients with moderately to severely active intestinal BD were treated by peroral or intravenous CsA for 2 weeks. The dose of CsA was adjusted to achieve the CsA trough plasma concentration of 100-150ng/ml in peroral group and of 500ng/ml in intravenous group. The disease activity was assessed by Disease Activity Index for Intestinal Behçet's Disease (DAIBD) and endoscopic findings. A clinical response was defined as a decrease at least 20 points of DAIBD from the baseline. After induction therapy, patients continued to take oral CsA as maintenance therapy. The endpoint of maintenance therapy was defined as disease recurrence, addition of other treatment or withdrawal of CsA by adverse events. Non-recurrence rate was analyzed by Kaplan-Meier estimate.
In induction therapy, mean dosage of CsA was 4.2mg/kg body weight/day in oral group and 2.9mg/kg body weight/day in intravenous group. Two weeks after CsA induction therapy, mean DAIBD score was decreased from 92.5 to 43.0 and a response rate was 70.0% (60.0% in oral CsA group and 80.0% in intravenous CsA group). Even in patients with failure to biologics therapies, a response rate was high (80.0%). The endoscopic findings of all of 6 patients, who could examine in both baseline and week 2, showed partially to significantly improvement. In maintenance therapy, mean dosage of peroral CsA was 4.1 mg/kg body weight/day. Non-recurrence rate of CsA maintenance therapy was 67.5% in 26 weeks, 33.8% in 52 weeks, and 22.5% in 78 weeks. Adverse events associated with CsA therapy were pyomyositis, nephropathy, hyperkalemia, tremor, and hypertrichosis.
CsA induction therapy is effective to active intestinal BD which is refractory to the conventional therapies including biologics. Yet, the efficacy and the safety of CsA maintenance therapy remain controversial. Further controlled study is needed.