系统性硬皮病相关肌病：一项与临床和免疫学特征相关的病例对照研究。

Myopathies related to systemic sclerosis: a case-control study of associated clinical and immunological features.

机构信息

Department of Internal Medicine, French National Reference Center for Systemic Sclerosis and Necrotizing Vasculitides, Cochin Hospital, Faculty of Medicine, Paris Descartes University, the Public Assistance Hospitals of Paris (AH-HP), France.

出版信息

Scand J Rheumatol. 2010 Nov;39(6):498-505. doi: 10.3109/03009741003774626. Epub 2010 Aug 20.

DOI:10.3109/03009741003774626

PMID:20726682

Abstract

OBJECTIVE

Little is known about systemic sclerosis (SSc)-related myopathy. We aimed to compare the clinical and immunological features of SSc patients with or without associated myopathy.

METHODS

Forty SSc patients with myopathy, defined by myalgia or muscle weakness associated with creatine kinase (CK) more than five times the upper limit range or myopathic electromyography (EMG) or abnormal myopathology, were identified from the records of four French hospital centres. For each patient, we selected two SSc controls matched for cutaneous SSc form, sex, age at SSc onset, and disease duration. We performed a case-control study testing clinical and immunological SSc-related features for association with myopathy by conditional logistic regression.

RESULTS

Muscle and SSc features of patients with myopathy did not differ significantly among the four centres of origin. Only four (10%) patients with SSc-associated myopathy had anti-polymyositis-scleroderma (PM-Scl) antibodies. Case-control univariate analysis revealed that reduced forced vital capacity (FVC) [odds ratio (OR) 3.0, 95% confidence interval (CI) 1.3-34.9], heart involvement, defined as clinical congestive heart failure, left ventricular ejection fraction (LVEF) < 60%, arrhythmia or conductive abnormalities (OR 2.9, 95% CI 1.3-6.5), and scleroderma renal crisis (OR 3.0, 95% CI 1.3-34.9) were significantly more frequent in patients with myopathy than in controls. Two autoantibodies were more frequent in patients with myopathy: anti-PM-Scl (OR 5.0, 95% CI 1.1-23.9) and anti-RNP (OR 6.9, 95% CI 1.1-64.4). Multivariate analysis retained two variables associated positively with myopathy [reduced FVC (OR 3.1, 95% CI 1.3-9.8) and heart involvement (OR 2.5, 95% CI 1.1-7.1)], while anti-centromere antibodies were associated negatively (OR 0.11, 95% CI 0.03-0.53).

CONCLUSION

Heart monitoring of SSc patients with myopathy should be undertaken regularly because of the association of myocardial and skeletal myopathies in such patients.

摘要

目的

系统性硬化症（SSc）相关肌病的相关信息知之甚少。本研究旨在比较伴有或不伴有相关肌病的 SSc 患者的临床和免疫学特征。

方法

从法国四家医院的病历中，我们确定了 40 例肌病 SSc 患者，其肌病定义为肌痛或肌无力，伴肌酸激酶（CK）升高超过正常值上限 5 倍，或肌电图（EMG）呈肌病表现，或肌活检异常。对于每位患者，我们选择了两名 SSc 对照者，其皮肤 SSc 类型、性别、SSc 发病年龄和疾病持续时间与患者匹配。我们通过条件逻辑回归对肌病与临床和免疫学 SSc 相关特征的相关性进行了病例对照研究。

结果

不同起源中心的肌病 SSc 患者的肌肉和 SSc 特征无显著差异。仅有 4 例（10%）肌病 SSc 患者存在抗多发性肌炎-硬皮病（PM-Scl）抗体。病例对照单变量分析显示，用力肺活量（FVC）降低（比值比[OR]3.0，95%置信区间[CI]1.3-34.9）、定义为充血性心力衰竭的心脏受累、左心室射血分数（LVEF）<60%、心律失常或传导异常（OR 2.9，95% CI 1.3-6.5）和硬皮病肾危象（OR 3.0，95% CI 1.3-34.9）在肌病患者中更常见。两种自身抗体在肌病患者中更为常见：抗 PM-Scl（OR 5.0，95% CI 1.1-23.9）和抗 RNP（OR 6.9，95% CI 1.1-64.4）。多变量分析保留了两个与肌病呈正相关的变量[FVC 降低（OR 3.1，95% CI 1.3-9.8）和心脏受累（OR 2.5，95% CI 1.1-7.1）]，而抗着丝点抗体则呈负相关（OR 0.11，95% CI 0.03-0.53）。