CARDIAC AND SKELETAL-MUSCLE DISEASE IN SYSTEMIC-SCLEROSIS (SCLERODERMA) - A HIGH-RISK ASSOCIATION
CARDIAC AND SKELETAL-MUSCLE DISEASE IN SYSTEMIC-SCLEROSIS (SCLERODERMA) - A HIGH-RISK ASSOCIATION
复制标题
DOI：
10.1016/0002-8703(93)90075-k
复制
发表时间：
1993-01-01
影响因子：
4.8
通讯作者：
MEDSGER, TA
中科院分区：
文献类型：
--
作者：
FOLLANSBEE, WP;ZERBE, TR;MEDSGER, TA
为了研究心脏和骨骼肌疾病在系统性硬化症中的可能关系，我们回顾了1095例连续系统性硬化症患者的计算机记录。183例（17%）患有骨骼肌病。183例患者中有39例（21%）符合心肌疾病标准，而912例无肌病患者中有90例（10%）符合（p< 0.0001）。183例患者中有19例（10%）有临床CHF，其余38例（4%）有临床CHF （p< 0.002）。912例无肌病患者中有27例（3%）死于心脏原因（p< 0.002），而有肌病患者中有15例（8%）死于心脏原因。在没有其他可识别的原因的情况下，确定了25例肌病和心肌疾病并存的患者。该组的特点是心脏传导异常发生率高（60%），心肌功能障碍和心律失常的严重程度，包括心房和室性心律失常。25名患者中有18人死亡；12例（6%）突然死亡。18人中有8人（44%）患有顽固性心衰，这直接导致了他们的死亡。心肌纤维化是尸检中主要的组织学异常。然而，一名死于急性“心肌炎”的患者尸检显示严重的肌细胞溶解伴收缩带坏死，但无炎症或纤维化；这与可能的缺血性损伤相一致。我们的结论是，系统性硬化症患者的骨骼和心肌疾病是相关的。肌病患者发生慢性心力衰竭、持续症状性心律失常和心源性死亡（尤其是猝死）的风险增加。
To examine the possible relationship between cardiac and skeletal muscle disease in systemic sclerosis, we reviewed computerized records of 1095 consecutive patients with systemic sclerosis. One hundred eighty three (17%) had skeletal myopathy. Thirtynine (21%) of the 183 fulfilled criteria for myocardial disease, compared with 90 (10%) of the 912 without myopathy (p< 0.0001.) Nineteen (10%) of the 183 had clinical CHF compared with 38 (4%) of the remainder (p< 0.002.) Fifteen (8%) of the patients with myopathy died of cardiac causes compared with 27 (3%) of the 912 without myopathy (p< 0.002.) Twenty-five patients with coexistent myopathy and myocardial disease, in the absence of other identifiable contributing causes, were identified. This group was characterized by a high incidence of cardiac conduction abnormalities (60%) and by the severity of the myocardial dysfunction and arrhythmias, both atrial and ventricular that they experienced. Eighteen of these 25 patients died; 12 (6%) died suddenly. Eight of the 18 (44%) had intractable CHF, which directly contributed to their deaths. Myocardial fibrosis was the predominant histologic abnormality at autopsy. However, autopsy of a patient who died in the context of acute “myocarditis” showed severe myocytolysis with contraction band necrosis but without inflammation or fibrosis; this is consistent with possible ischemically mediated injury. We conclude that skeletal and cardiac muscle disease in systemic sclerosis are associated. Patients with myopathy are at increased risk for CHF, sustained symptomatic arrhythmias, and cardiac death, particularly sudden death.