Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease

Abstract Interstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolutio...

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Autores principales: Yun Jae Kim, Jooae Choe, Ho Jeong Kim, Jin Woo Song
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Lenguaje:EN
Publicado: Nature Portfolio 2021
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Acceso en línea:https://doaj.org/article/ba4f33c0695a495788d04471b2377d4a
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spelling oai:doaj.org-article:ba4f33c0695a495788d04471b2377d4a2021-12-02T17:24:00ZLong-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease10.1038/s41598-021-92024-22045-2322https://doaj.org/article/ba4f33c0695a495788d04471b2377d4a2021-06-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-92024-2https://doaj.org/toc/2045-2322Abstract Interstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolution computed tomography (HRCT) images of 62 patients with primary SJS-ILD were retrospectively analyzed (biopsy-proven cases, n = 16). The mean patient age was 59.8 years; 83.9% of the patients were females, and 38.7% showed a usual interstitial pneumonia (UIP) pattern on HRCT. The median follow-up period was 61.5 months. During follow-up, 15 patients (24.2%) died, 7 (11.3%) experienced acute exacerbation (AE), and 27 (43.5%) progressed. The 1-, 3- and 5-year survival rates were 93.5%, 85.8%, and 81.1%, respectively. Age (hazard ratio [HR]: 1.158, P = 0.003), C-reactive protein (CRP) level (HR: 1.212, P = 0.045), FVC (HR: 0.902, P = 0.005), and a UIP pattern on HRCT (HR: 4.580, P = 0.029) were significant prognostic factors in multivariable Cox analysis. In conclusion, death, AE, and ILD progression occurred in 25%, 10%, and 50% of the patients with SJS-ILD, respectively. Older age, higher CRP level, lower FVC, and a UIP pattern on HRCT indicated poor prognosis.Yun Jae KimJooae ChoeHo Jeong KimJin Woo SongNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-8 (2021)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Yun Jae Kim
Jooae Choe
Ho Jeong Kim
Jin Woo Song
Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
description Abstract Interstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolution computed tomography (HRCT) images of 62 patients with primary SJS-ILD were retrospectively analyzed (biopsy-proven cases, n = 16). The mean patient age was 59.8 years; 83.9% of the patients were females, and 38.7% showed a usual interstitial pneumonia (UIP) pattern on HRCT. The median follow-up period was 61.5 months. During follow-up, 15 patients (24.2%) died, 7 (11.3%) experienced acute exacerbation (AE), and 27 (43.5%) progressed. The 1-, 3- and 5-year survival rates were 93.5%, 85.8%, and 81.1%, respectively. Age (hazard ratio [HR]: 1.158, P = 0.003), C-reactive protein (CRP) level (HR: 1.212, P = 0.045), FVC (HR: 0.902, P = 0.005), and a UIP pattern on HRCT (HR: 4.580, P = 0.029) were significant prognostic factors in multivariable Cox analysis. In conclusion, death, AE, and ILD progression occurred in 25%, 10%, and 50% of the patients with SJS-ILD, respectively. Older age, higher CRP level, lower FVC, and a UIP pattern on HRCT indicated poor prognosis.
format article
author Yun Jae Kim
Jooae Choe
Ho Jeong Kim
Jin Woo Song
author_facet Yun Jae Kim
Jooae Choe
Ho Jeong Kim
Jin Woo Song
author_sort Yun Jae Kim
title Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
title_short Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
title_full Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
title_fullStr Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
title_full_unstemmed Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease
title_sort long-term clinical course and outcome in patients with primary sjögren syndrome-associated interstitial lung disease
publisher Nature Portfolio
publishDate 2021
url https://doaj.org/article/ba4f33c0695a495788d04471b2377d4a
work_keys_str_mv AT yunjaekim longtermclinicalcourseandoutcomeinpatientswithprimarysjogrensyndromeassociatedinterstitiallungdisease
AT jooaechoe longtermclinicalcourseandoutcomeinpatientswithprimarysjogrensyndromeassociatedinterstitiallungdisease
AT hojeongkim longtermclinicalcourseandoutcomeinpatientswithprimarysjogrensyndromeassociatedinterstitiallungdisease
AT jinwoosong longtermclinicalcourseandoutcomeinpatientswithprimarysjogrensyndromeassociatedinterstitiallungdisease
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