McNamara D M, Rosenblum W D, Janosko K M, Trost M K, Villaneuva F S, Demetris A J, Murali S, Feldman A M
Division of Cardiology, University of Pittsburgh (Pa) Medical Center 15213, USA.
Circulation. 1997 Jun 3;95(11):2476-8. doi: 10.1161/01.cir.95.11.2476.
Although an autoimmune pathogenesis has been postulated for dilated cardiomyopathy, immunosuppressive therapy has not been shown to be effective in clinical trials. Immune modulatory therapy with immune globulin is an effective therapy for Kawasaki disease in children, and recent data suggest that it improves ventricular function in children with new-onset dilated cardiomyopathies. The role of immune globulin therapy in adults with this disorder has not previously been evaluated.
Ten patients were treated with high-dose intravenous immune globulin infusions (2 g/kg). All were hospitalized with NYHA class III to IV heart failure, left ventricular ejection fraction (LVEF) < 0.40, and symptoms for < 6 months at the time of presentation. One patient died before the completion of therapy. The remaining 9 were discharged, and LVEF was reassessed 12 months after therapy. LVEF improved from 0.24 +/- 0.02 (mean +/- SEM) at baseline to 0.41 +/- 0.04 at follow-up (P = .003). All 9 patients improved functionally to NYHA class I to II, and there have been no subsequent hospitalizations for heart failure during the course of follow-up.
In this series of patients with new-onset dilated cardiomyopathy treated with high-dose immune globulin, LVEF improved 17 EF units. The effectiveness of intravenous immune globulin therapy in this disorder should be evaluated in a randomized, multicenter trial.
尽管已推测扩张型心肌病存在自身免疫发病机制,但免疫抑制疗法在临床试验中尚未显示出有效性。免疫球蛋白免疫调节疗法对儿童川崎病是一种有效疗法,最近的数据表明它可改善新发扩张型心肌病患儿的心室功能。此前尚未评估免疫球蛋白疗法在患有这种疾病的成人中的作用。
10例患者接受了大剂量静脉注射免疫球蛋白输注(2 g/kg)。所有患者入院时均为纽约心脏协会(NYHA)心功能III至IV级心力衰竭,左心室射血分数(LVEF)<0.40,且症状出现时间<6个月。1例患者在治疗完成前死亡。其余9例出院,治疗12个月后重新评估LVEF。LVEF从基线时的0.24±0.02(平均值±标准误)提高到随访时的0.41±0.04(P = .003)。所有9例患者心功能均改善至NYHA I至II级,随访期间无因心力衰竭再次住院情况。
在这组接受大剂量免疫球蛋白治疗的新发扩张型心肌病患者中,LVEF提高了17个EF单位。静脉注射免疫球蛋白疗法在这种疾病中的有效性应在一项随机、多中心试验中进行评估。