Jühl U, Strauer B E, Schultheiss H P
Med. Klinik für Kardiologie, Pulmonologie und Angiologie, Heinrich-Heine Universität Düsseldorf, Germany.
Postgrad Med J. 1994;70 Suppl 1:S35-42.
The aetiology of dilated cardiomyopathy is unknown. Recent clinical and experimental data have suggested a temporal relationship with viral myocarditis. The clinical diagnosis, however, is non-specific. The evaluation of endomyocardial biopsies by light microscopy and their histological classification according to the Dallas criteria is limited by the difficulty in differentiating and quantifying infiltrating inflammatory lymphocytes from non-inflammatory interstitial cells. Using immunohistological methods that allow better identification and quantification of infiltrating lymphocytes and that also provide evidence for an activated immunological process within the myocardium, myocarditis was diagnosed on endomyocardial biopsy in 48 of 130 patients (37%). On the basis of both haemodynamic and immunohistological findings, 31 of these patients were selected for immunosuppressive treatment. After a 6-month treatment period with 6-methylprednisolone, 23 patients showed an improvement according to the NYHA classification. Lymphocytic infiltrates were abolished in 24 patients. Left ventricular systolic function was improved in 20 patients (64%) as indicated by an increased ejection fraction and stroke volume with a concomitant decrease of left ventricular end diastolic pressure. The three patients with severely impaired left ventricular function did not improve either clinically or histologically, whilst in four patients the clinical findings remained unchanged in spite of histological resolution of the inflammatory infiltrate in the myocardium. Our study suggests that immunosuppressive treatment in a subgroup of patients with dilated cardiomyopathy who have a continuing active immunohistologically proven inflammatory process results in a clinical haemodynamic and immunohistological improvement in 60-70% of the patients.
扩张型心肌病的病因尚不清楚。最近的临床和实验数据表明它与病毒性心肌炎存在时间上的关联。然而,临床诊断并不具有特异性。根据达拉斯标准,通过光学显微镜对心内膜活检组织进行评估及其组织学分类,受到区分和量化浸润性炎性淋巴细胞与非炎性间质细胞的困难所限。使用免疫组织学方法能够更好地识别和量化浸润淋巴细胞,并为心肌内激活的免疫过程提供证据,在130例患者中的48例(37%)心内膜活检中诊断出心肌炎。基于血流动力学和免疫组织学结果,其中31例患者被选作免疫抑制治疗。在用6-甲基泼尼松龙治疗6个月后,根据纽约心脏协会(NYHA)分级,23例患者病情有所改善。24例患者的淋巴细胞浸润消失。20例患者(64%)左心室收缩功能改善,表现为射血分数和每搏输出量增加,同时左心室舒张末期压力降低。3例左心室功能严重受损的患者在临床和组织学上均未改善,而4例患者尽管心肌炎性浸润在组织学上消退,但临床症状仍未改变。我们的研究表明,对一组经免疫组织学证实有持续活跃炎症过程的扩张型心肌病患者进行免疫抑制治疗,60 - 70%的患者在临床、血流动力学和免疫组织学方面有改善。