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首页> 外文期刊>Journal of Clinical Medicine Research >Clinical Manifestation and Incidence of Cardiopulmonary Complications in Early Systemic Sclerosis Patients with Different Antibody Profiles
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Clinical Manifestation and Incidence of Cardiopulmonary Complications in Early Systemic Sclerosis Patients with Different Antibody Profiles

机译：不同抗体谱的早期系统性硬化症患者的临床表现和心肺并发症的发生率

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Background: There has been no prior inception cohort study comparing clinical manifestations and incidence rate (IR) of cardiopulmonary involvement among early systemic sclerosis (SSc) patients by difference in autoantibody profiles. We compared the differences in the clinical presentation at study entry and cumulative organ complications at last visit, as well as the IR of cardiopulmonary complications between anti-topoisomerase I antibody-positive SSc patients (pATA), ATA-negative (nATA), and the positive anti-centromere antibody patients (pACA). Methods: An inception cohort of early diagnosis SSc patients (disease duration ≤ 3 years) seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was studied. SSc patients who had follow-up duration as less than 1 year and those diagnosed with an overlap syndrome were excluded. All participants underwent electrocardiography (ECG), echocardiography, and high-resolution computed tomography (HRCT) at the study entry and then annually. Results: A total of 114 patients (90 diffuse cutaneous SSc (dcSSc), 69 women) with mean (standard deviation, SD) disease duration of 11.7 (8.8) months at cohort entry and an observational period of 3.8 (1.6) years, were recruited. There were 89 patients (78.1%) with pATA, 18 (15.8%) with nATA, and 7 (6.1%) with pACA. At enrollment, both pATA and nATA groups had a higher prevalence of dcSSc subtype, and interstitial lung disease (ILD) when compared with the pACA group. At the last visit, the pATA group had a higher cumulative prevalence of digital ulcers, joint contracture and tendon friction rub than the other groups. Both the pATA and nATA groups had a significantly higher IR of ILD compared to the pACA group (54.9 and 57.8 vs. 6.3 per 100 person-years). During the study period, no suspected myositis, systolic pulmonary artery pressure (sPAP) ≥ 50 mm Hg or cardiac complications was observed in the pACA group. Conclusions: In our study cohort, the majority of which were dcSSc subtype with pATA, it was found that the presence of SSc-specific autoantibodies was associated with a distinctive clinical presentation and cumulative internal organ involvement, even in the early phase of the disease. Cardiopulmonary complications were rarely seen in the pACA group; whereas ILD complications were very common in both the pATA and nATA groups. A further study into the association of autoantibodies in nATA patients with ILD complications is needed.
机译：背景：尚无先期队列研究通过自身抗体谱的差异来比较早期系统性硬化症（SSc）患者的临床表现和心肺受累发生率（IR）。我们比较了研究进入时的临床表现和上次就诊时累积的器官并发症以及抗拓扑异构酶I抗体阳性SSc患者（pATA），ATA阴性（nATA）和抗着丝粒抗体阳性患者（pACA）。方法：研究了2010年1月至2016年6月在Maharaj Nakorn清迈医院风湿病诊所就诊的早期诊断SSc患者（病程≤3年）的始创队列。随访时间少于一年的SSc患者和诊断为重叠综合征的患者被排除在外。所有参与者在研究开始时均接受心电图（ECG），超声心动图和高分辨率计算机断层扫描（HRCT），然后每年进行一次。结果：总共114例患者（90例弥漫性皮肤SSc（dcSSc），69名女性）入组时平均（标准差，SD）病程为11.7（8.8）个月，观察期为3.8（1.6）年。被招募。 pATA患者89例（78.1％），nATA患者18例（15.8％），pACA患者7例（6.1％）。入组时，与pACA组相比，pATA和nATA组的dcSSc亚型和间质性肺病（ILD）的患病率更高。在最后一次访视时，pATA组的数字溃疡，关节挛缩和腱摩擦摩擦的累积患病率高于其他组。与pACA组相比，pATA和nATA组的ILD IR明显更高（每100人年56.3和57.8，而6.3）。在研究期间，pACA组未发现可疑的肌炎，收缩期肺动脉压（sPAP）≥50 mm Hg或心脏并发症。结论：在我们的研究队列中，大多数是pATA的dcSSc亚型，发现SSc特异性自身抗体的存在与独特的临床表现和累积的内脏器官受累有关，即使在疾病早期也是如此。在pACA组中很少见到心肺并发症。而IATA并发症在pATA和nATA组中都很常见。需要进一步研究在患有ILD并发症的nATA患者中自身抗体的相关性。

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