Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis
Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis
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DOI：
10.1007/s10067-014-2730-z
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发表时间：
2014-08-01
影响因子：
3.4
通讯作者：
Shinjo, Samuel Katsuyuki
中科院分区：
文献类型：
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作者：
Aguila, Lisbeth Aranbicia;Ugolini Lopes, Michelle Remiao;Shinjo, Samuel Katsuyuki
由于重叠综合征 (OS) 很少被描述，我们回顾性分析了巴西系列 31 例与系统性红斑狼疮 (SLE)、系统性硬化症 (SSc) 或类风湿性关节炎 (RA) 相关的皮肌炎 (DM)/多发性肌炎 (PM) 患者在转诊单一中心就诊的频率和相关性。研究了肌炎特异性自身抗体（MSAs：抗 Jo-1、抗 PL-7、抗 PL-12、抗 EJ、抗 OJ、抗 SRP、抗 Mi-2）和肌炎相关自身抗体（MAA：抗 PM-Scl75、抗 PM-Scl100、抗 Ku）以及与 SLE、SSc 和 RA 相关的特异性自身抗体。 OS 患者的平均年龄（9 DM 和 22 PM）为 44.6 +/- 15.4 岁，其中以女性 (83.9 %) 和白人 (58.1 %) 为主。 PM 是最常见的炎症性肌病，DM/PM 的临床表现在 OS 组之间存在显着差异。与 SSc (48.4 %)、SLE (29.0 %) 和 RA (22.6 %) 存在重叠。大多数病例中 DM/PM 的临床表现与 SSc 和 RA 同时被识别，这与 SLE 组的识别相反 (p < 0.05)。所有患者的抗核抗体均为阳性，所有 OS 组中 MSA 和 MAA 的患病率为 38.8%，相互排斥，且在 SSc 组中更为常见。比较临床和实验室特征，SSc 组血管（皮肤溃疡、雷诺现象）和肺部（间质性肺病）受累的频率较高（p < 0.05）。此外，各组之间在疾病复发和死亡方面没有差异。总之，这是第一项显示巴西异质人群中一系列结缔组织病 (CTD)-OS 患者不同特征的研究。
Because overlap syndromes (OSs) are rarely described, we analyzed retrospectively their frequencies and correlations in Brazilian series of 31 patients with dermatomyositis (DM)/polymyositis (PM) associated with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or rheumatoid arthritis (RA) attended at a referral single center. Myositis-specific autoantibodies (MSAs: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-SRP, anti-Mi-2) and myositis-associated autoantibodies (MAAs: anti-PM-Scl75, anti-PM-Scl100, anti-Ku) as well as specific autoantibodies related to SLE, SSc, and RA were investigated. The mean age of the OS patients (9 DM and 22 PM) was 44.6 +/- 15.4 years, with a predominance of women (83.9 %) and white ethnicity (58.1 %). PM was the most frequent inflammatory myopathy, and the clinical presentation of DM/PM was significantly different among the OS groups. Overlap was found with SSc (48.4 %), SLE (29.0 %), and RA (22.6 %). The clinical manifestations of DM/PM were identified simultaneously with SSc and RA in the majority of cases, in contrast to identification in the SLE group (p < 0.05). All patients were positive for antinuclear antibodies, and the prevalence of MSA and MAA was 38.8 % in all OS groups, mutually exclusive, and more frequent in the SSc group. Comparing the clinical and laboratory features, there was a higher frequency of vascular (skin ulcers, Raynaud's phenomenon) and pulmonary (interstitial lung disease) involvement in the SSc group (p < 0.05). Moreover, there were no differences among the groups in relation to disease relapse and deaths. Concluding, this is the first study to show the different characteristics of a series of patients with connective tissue disease (CTD)-OS in the heterogeneous Brazilian population.