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特发性扩张型心肌病患者的免疫球蛋白吸附

Immunoglobulin adsorption in patients with idiopathic dilated cardiomyopathy.

作者信息

Müller J, Wallukat G, Dandel M, Bieda H, Brandes K, Spiegelsberger S, Nissen E, Kunze R, Hetzer R

机构信息

German Heart Institute Berlin, Germany.

出版信息

Circulation. 2000 Feb 1;101(4):385-91. doi: 10.1161/01.cir.101.4.385.

Abstract

BACKGROUND

Idiopathic dilated cardiomyopathy (IDC) frequently is a progressive disease without causative therapy options. Following the hypothesis that in certain patients autoantibodies against cardiac structures may induce, maintain, or promote the progression of the disease, we investigated whether the elimination of these autoantibodies through immunoadsorption would improve cardiac function.

METHODS AND RESULTS

This prospective case-control study included 34 patients with IDC. Each patient presented with moderate to severe heart failure and evidence of autoantibodies directed against beta(1)-adrenoceptors (beta(1)-AABs). Seventeen patients received standard medical therapy (control group), whereas 17 were also treated with immunoadsorption (treatment group) to eliminate beta(1)-AABs. A 1-year follow-up included echocardiographic assessment of left ventricular ejection fraction and internal diameters, beta(1)-AAB levels, and clinical status every 3 months. Within 1 year, the mean+/-SD left ventricular ejection fraction rose from 22.3+/-3.3% to 37.9+/-7.9% (P=0.0001) in the treatment group, with a relative increase of 69.9%. However, in the control group, no overall increase was seen (from 23.8+/-3.0% to 25.2+/-5.9%, P=0. 3154). Left ventricular diameter in diastole decreased by 14.5% from 74.5+/-7.1 to 63.7+/-6.0 mm in the treatment group (P=0.0001) and by 3.8% (P=0.2342) in the control group. In the treatment group, the NYHA functional rating improved after immunoadsorption (P=0.0001). beta(1)-AABs did not increase anew.

CONCLUSIONS

In IDC, the use of immunoadsorption is superior to the use of standard medical therapy. It significantly improves cardiac performance and clinical status.

摘要

背景

特发性扩张型心肌病(IDC)通常是一种无病因治疗选择的进行性疾病。基于某些患者体内针对心脏结构的自身抗体可能诱导、维持或促进疾病进展这一假设,我们研究了通过免疫吸附消除这些自身抗体是否会改善心脏功能。

方法与结果

这项前瞻性病例对照研究纳入了34例IDC患者。每位患者均表现为中度至重度心力衰竭且有针对β1肾上腺素能受体的自身抗体(β1 - AABs)证据。17例患者接受标准药物治疗(对照组),而17例患者还接受免疫吸附治疗(治疗组)以消除β1 - AABs。为期1年的随访包括每3个月进行一次超声心动图评估左心室射血分数和内径、β1 - AAB水平以及临床状况。1年内,治疗组的平均±标准差左心室射血分数从22.3±3.3%升至37.9±7.9%(P = 0.0001),相对增幅为69.9%。然而,对照组未见总体升高(从23.8±3.0%至25.2±5.9%,P = 0.3154)。治疗组舒张期左心室直径从74.5±7.1降至63.7±6.0 mm,下降了14.5%(P = 0.0001),对照组下降了3.8%(P = 0.2342)。在治疗组中,免疫吸附后纽约心脏协会(NYHA)功能分级得到改善(P = 0.0001)。β1 - AABs未再次升高。

结论

在IDC中,免疫吸附的应用优于标准药物治疗。它能显著改善心脏性能和临床状况。

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