韩国系统性硬化症患者的临床和实验室特征及死亡率：一项全国多中心回顾性队列研究。
Clinical and Laboratory Characteristics and Mortality in Korean Patients with Systemic Sclerosis: A Nationwide Multicenter Retrospective Cohort Study.
机构信息
From the Division of Rheumatology, Department of Internal Medicine, Kangwon National University School of Medicine, Chuncheon; Department of Rheumatology, Chonnam National University Medical School, Gwangju; Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam; Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine; Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, Institute of Medical Science, College of Medicine, The Catholic University of Korea, Seoul; Division of Rheumatology, Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon; Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University College of Medicine Cheonan Hospital, Cheonan; Division of Rheumatology, Department of Internal Medicine, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju; Department of Rheumatology, Yonsei University Wonju College of Medicine, Wonju; Division of Rheumatology, Department of Internal Medicine, Pusan National University School of Medicine, Pusan National University Hospital, Busan, Korea.
K.W. Moon, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Kangwon National University School of Medicine; S.S. Lee, MD, PhD, Department of Rheumatology, Chonnam National University Medical School; Y.J. Lee, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital; J.B. Jun, MD, PhD, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; S.J. Yoo, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Chungnam National University School of Medicine; J.H. Ju, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, Institute of Medical Science, College of Medicine, The Catholic University of Korea; S.H. Chang, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University College of Medicine Cheonan Hospital; I.A. Choi, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Chungbuk National University Hospital, Chungbuk National University College of Medicine; T.Y. Kang, MD, PhD, Department of Rheumatology, Yonsei University Wonju College of Medicine; E.B. Lee, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine; S.G. Lee, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Pusan National University School of Medicine, Pusan National University Hospital.
出版信息
J Rheumatol. 2018 Aug;45(9):1281-1288. doi: 10.3899/jrheum.171443. Epub 2018 Jul 15.
OBJECTIVE
We aimed to investigate demographic and clinical features and predictors of mortality in Korean patients with systemic sclerosis (SSc).
METHODS
We performed a retrospective multicenter medical chart review in Korean patients diagnosed with SSc from 1986 to 2016 at 11 university hospitals representing each geographic area of Korea. SSc patients were defined according to the American College of Rheumatology preliminary classification criteria and subtyped as limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) SSc.
RESULTS
We enrolled 751 patients (female, 86.7%; mean age at diagnosis, 48.9 yrs). The most common organ involvement was interstitial lung disease (52.7%), followed by gastroesophageal reflux disease (32.9%) and pulmonary arterial hypertension (13.6%). Patients with lcSSc were more common than those with dcSSc (64.8 vs 35.2%), whereas anti-Scl-70 and anticentromere antibody positivity were identified in 302 (42.5%) and 175 (25.5%) patients, respectively. In the 46 (6.1%) patients who developed a malignancy, lung cancer (23.9%) was the most common diagnosis, followed by gastric (13%) and breast cancer (13%). During the study period, 57 (7.6%) patients died, and the 5- and 10-year survival rates were 94% and 87%, respectively. Increased age at diagnosis, cardiovascular involvement, and anti-Scl-70 antibody positivity were significant predictors of death.
CONCLUSION
Clinical manifestations and survival rates in Korean SSc patients are similar to those of other populations. However, the prevalence of anti-Scl-70 antibody is higher in Korean SSc patients compared with whites, while the prevalence of anticentromere antibody is lower.
目的
本研究旨在探讨韩国系统性硬化症（SSc）患者的人口统计学和临床特征，以及死亡的预测因素。
方法
我们对 1986 年至 2016 年期间在韩国 11 所大学医院就诊的韩国 SSc 患者进行了回顾性多中心病历回顾。根据美国风湿病学会初步分类标准诊断 SSc 患者，并将其分为局限性皮肤型（lcSSc）或弥漫性皮肤型（dcSSc）。
结果
共纳入 751 例患者（女性占 86.7%；诊断时的平均年龄为 48.9 岁）。最常见的器官受累是间质性肺疾病（52.7%），其次是胃食管反流病（32.9%）和肺动脉高压（13.6%）。lcSSc 患者多于 dcSSc 患者（64.8% vs 35.2%），而抗 Scl-70 和抗着丝点抗体阳性分别在 302（42.5%）和 175（25.5%）例患者中检出。在发生恶性肿瘤的 46 例（6.1%）患者中，肺癌（23.9%）是最常见的诊断，其次是胃癌（13%）和乳腺癌（13%）。在研究期间，57 例（7.6%）患者死亡，5 年和 10 年生存率分别为 94%和 87%。诊断时年龄较大、心血管受累和抗 Scl-70 抗体阳性是死亡的显著预测因素。
结论
韩国 SSc 患者的临床表现和生存率与其他人群相似。然而，与白人相比，韩国 SSc 患者抗 Scl-70 抗体的阳性率较高，而抗着丝点抗体的阳性率较低。
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