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Characteristics and risk factors of mortality in patients with systemic sclerosis-associated interstitial lung disease

 Hye Jin Jang  ;  Ala Woo  ;  Song Yee Kim  ;  Seung Hyun Yong  ;  Youngmok Park  ;  Kyungsoo Chung  ;  Su Hwan Lee  ;  Ah Young Leem  ;  Sang Hoon Lee  ;  Eun Young Kim  ;  Ji Ye Jung  ;  Young Ae Kang  ;  Young Sam Kim  ;  Moo Suk Park 
 ANNALS OF MEDICINE, Vol.55(1) : 663-671, 2023-12 
Journal Title
Issue Date
Humans ; Lung / diagnostic imaging ; Lung Diseases, Interstitial* / etiology ; Retrospective Studies ; Risk Factors ; Scleroderma, Systemic* / complications ; Vital Capacity
Systemic sclerosis ; autoimmune disease ; fibroblast ; interstitial lung disease
Background: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by dysregulation of fibroblast function, which often involves the lungs. Interstitial lung disease (ILD) associated with SSc (SSc-ILD) is a major cause of death among patients with SSc. Our study aimed to identify risk factors for mortality and compare the clinical characteristics of patients with SSc-ILD. Patients and methods: Patients were retrospectively enrolled between 2010 and 2018 in a tertiary hospital in Korea. Patients with SSc-ILD were classified depending on the first pulmonary function test or radiologic findings: extensive (n = 46, >20% disease extent on computed tomography (CT) or forced vital capacity [FVC] < 70% in indeterminate cases) and limited (n = 60, <20% disease extent on CT or FVC ≥70% in indeterminate cases). Results: Patients in the extensive group were younger (mean age ± SD 49.3 ± 11.5) than those in the limited group (53.9 ± 12.5, p =.067) at diagnosis. The extensive group showed frequent pulmonary hypertension (43.5% vs. 16.7%, p =.009) and higher erythrocyte sedimentation rate (61.3 ± 33.7 vs. 42.1 ± 26.0, p =.003) and mortality (32.6%, mean duration of follow-up, 100.0 ± 44.7 months vs. 10.0%, 86.0 ± 53.4 months, p =.011). ILD was detected within five years from the first visit (median years 3.5 (1.0, 6.0) vs. 4.5 (0.6, 9.0), survivors vs. non-survivors), and mortality occurred in 19.8% of all patients during a 15-year follow-up. Older age, lower FVC, and initial disease stage (limited or extensive) were associated with mortality, but FVC decline was similar in the limited and extensive groups, such as 15–20% in the first year and 8–10% in the next year, regardless of the initial extent of the disease. Conclusions: Approximately 10% of patients with SSc-ILD in the limited and extensive group showed progression. ILD was detected at a median of less than five years from the first visit; therefore, it is necessary to carefully monitor patients’ symptoms and signs from an early stage. Long-term surveillance is also required.Key messages Patients with systemic sclerosis-interstitial lung disease manifested a heterogeneous disease course. Approximately 10% of the patients in the limited group showed progression, which was similar to the proportion of patients in the extensive group. Interstitial lung disease was detected at a median of less than five years from the first visit. © 2023 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Young Ae(강영애) ORCID logo https://orcid.org/0000-0002-7783-5271
Kim, Song Yee(김송이) ORCID logo https://orcid.org/0000-0001-8627-486X
Kim, Young Sam(김영삼) ORCID logo https://orcid.org/0000-0001-9656-8482
Kim, Eun Young(김은영) ORCID logo https://orcid.org/0000-0002-3281-5744
Park, Moo Suk(박무석) ORCID logo https://orcid.org/0000-0003-0820-7615
Park, Youngmok(박영목) ORCID logo https://orcid.org/0000-0002-5669-1491
Yong, Seung Hyun(용승현)
Woo, Ala(우아라)
Lee, Sang Hoon(이상훈) ORCID logo https://orcid.org/0000-0002-7706-5318
Lee, Su Hwan(이수환) ORCID logo https://orcid.org/0000-0002-3487-2574
Leem, Ah Young(임아영) ORCID logo https://orcid.org/0000-0001-5165-3704
Jang, Hye Jin(장혜진)
Jung, Kyung Soo(정경수) ORCID logo https://orcid.org/0000-0003-1604-8730
Jung, Ji Ye(정지예) ORCID logo https://orcid.org/0000-0003-1589-4142
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