Blétry O, De Prost Y, Scheuble C, Frank R, Godeau P
Arch Mal Coeur Vaiss. 1979 Jul;72(7):799-805.
The case of a 50 year old male with the Fiessinger-Leroy-Reiter syndrome, ankylosing spondylitis and generalised pustular psoriasis is reported. This condition wax complicated by non-obstructive cardiomyopathy, congestive cardiac failure and first-degree atrioventricular block, the site of which was localised by electrophysiological studies (nodal block with an infrahisian conduction defect). After failure of several therapeutic regimes, a spectacular improvement was obtained with Methotrexate associated with a diuretic; the signs of heart failure regressed and the cardiomyopathy stablised. A parallel improvement was seen in the skin, cardiac and articular lesions and has been maintained with an 18 months follow-up. Left ventricular performance was studied by echocardiography. The mechanism of the beneficial effect of Methotrexate is unclear; this therapeutic trial is to be extended to include other cases of primary cardiomyopathy without obstruction.
报告了一例50岁男性,患有菲辛格-勒鲁瓦-赖特综合征、强直性脊柱炎和泛发性脓疱型银屑病。病情因非梗阻性心肌病、充血性心力衰竭和一度房室传导阻滞而复杂化,通过电生理研究确定了房室传导阻滞的部位(结性阻滞伴希氏束下传导缺陷)。在几种治疗方案失败后,甲氨蝶呤联合利尿剂取得了显著改善;心力衰竭的体征消退,心肌病稳定。皮肤、心脏和关节病变也有类似改善,并在18个月的随访中得以维持。通过超声心动图研究了左心室功能。甲氨蝶呤有益作用的机制尚不清楚;该治疗试验将扩大到包括其他无梗阻性原发性心肌病病例。