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Clinical and Laboratory Characteristics and Mortality in Korean Patients with Systemic Sclerosis: A Nationwide Multicenter Retrospective Cohort Study

Authors
Moon, Ki WonLee, Shin-SeokLee, Yun JongJun, Jae-BumYoo, Su-JinJu, Ji HyeonChang, Sung HaeChoi, In AhKang, Tae YoungLee, Eun BongLee, Seung-Geun
Issue Date
1-Aug-2018
Publisher
Journal of Rheumatology Publishing Co., Ltd.
Keywords
SYSTEMIC SCLEROSIS; EPIDEMIOLOGY; NEOPLASMS; REPUBLIC OF KOREA; MORTALITY
Citation
Journal of Rheumatology, v.45, no.9, pp 1281 - 1288
Pages
8
Journal Title
Journal of Rheumatology
Volume
45
Number
9
Start Page
1281
End Page
1288
URI
https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/5723
DOI
10.3899/jrheum.171443
ISSN
0315-162X
1499-2752
Abstract
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 (deSSc) 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 deSSc (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-Sc1-70 antibody is higher in Korean SSc patients compared with whites, while the prevalence of anticentromere antibody is lower.
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