Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort

Objectives: Interstitial lung disease (ILD) is associated with impaired survival among patients with connective tissue diseases (CTDs), but population-based data on the frequency of ILD and pulmonary hypertension (PH) in different CTD subtypes and the impact on survival are sparse. Methods: We inclu...

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Autores principales:	Charlotte Hyldgaard, Elisabeth Bendstrup, Alma Becic Pedersen, Lars Pedersen, Torkell Ellingsen
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	interstitial lung disease connective tissue disease pulmonary hypertension clinical epidemiology rare lung disease Medicine R
Acceso en línea:	https://doaj.org/article/a81c880954d0427ba2bc0f5735a020e4
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spelling	oai:doaj.org-article:a81c880954d0427ba2bc0f5735a020e42021-11-11T17:29:57ZInterstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort10.3390/jcm102148302077-0383https://doaj.org/article/a81c880954d0427ba2bc0f5735a020e42021-10-01T00:00:00Zhttps://www.mdpi.com/2077-0383/10/21/4830https://doaj.org/toc/2077-0383Objectives: Interstitial lung disease (ILD) is associated with impaired survival among patients with connective tissue diseases (CTDs), but population-based data on the frequency of ILD and pulmonary hypertension (PH) in different CTD subtypes and the impact on survival are sparse. Methods: We included patients with a first-time ICD-10 diagnosis of systemic sclerosis (SSc), mixed connective tissue disease (MCTD), myositis, systemic lupus erythematosus (SLE), or Sjögren’s disease registered in the Danish National Patient Registry between 2000 and 2015. Among these, we identified patients with ILD and PH. Using Kaplan–Meier analysis, we assessed survival for the five subtypes of CTD ± ILD and compared survival among CTD patients overall ± ILD with survival in the general population ± ILD. Results: We identified 11,731 patients with a diagnosis of CTD; 637 (5.4%) had a diagnosis of ILD. The proportion of patients with ILD was higher in SSc (13.4%) and MCTD (9.1%) than in myositis (6.0%), SLE (4.1%), and Sjögren (2.8%). Fifty-one percent were diagnosed with ILD in their fifties and sixties. PH was more frequent in SSc (7.5%) and MCTD (4.1%). Five-year survival was 73.3% (66.7–80.6) in SSc-ILD, 81.0% (69.0–95.1) in MCTD-ILD, 84.7% (77.3–92.9) in myositis-ILD, 83.5% (76.2–91.5) in SLE-ILD, and 84.7 (78.4–91.6) in Sjögren-associated ILD. Survival in CTD-ILD overall was impaired for all age groups compared with CTD alone. Age-stratified survival was comparable between CTD-ILD and ILD in the general population. The survival gap between ILD and non-ILD increased with age. Conclusion: Survival was comparable between different CTD-ILD subtypes and comparable to survival in non-CTD-ILD.Charlotte HyldgaardElisabeth BendstrupAlma Becic PedersenLars PedersenTorkell EllingsenMDPI AGarticleinterstitial lung diseaseconnective tissue diseasepulmonary hypertensionclinical epidemiologyrare lung diseaseMedicineRENJournal of Clinical Medicine, Vol 10, Iss 4830, p 4830 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	interstitial lung disease connective tissue disease pulmonary hypertension clinical epidemiology rare lung disease Medicine R
spellingShingle	interstitial lung disease connective tissue disease pulmonary hypertension clinical epidemiology rare lung disease Medicine R Charlotte Hyldgaard Elisabeth Bendstrup Alma Becic Pedersen Lars Pedersen Torkell Ellingsen Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
description	Objectives: Interstitial lung disease (ILD) is associated with impaired survival among patients with connective tissue diseases (CTDs), but population-based data on the frequency of ILD and pulmonary hypertension (PH) in different CTD subtypes and the impact on survival are sparse. Methods: We included patients with a first-time ICD-10 diagnosis of systemic sclerosis (SSc), mixed connective tissue disease (MCTD), myositis, systemic lupus erythematosus (SLE), or Sjögren’s disease registered in the Danish National Patient Registry between 2000 and 2015. Among these, we identified patients with ILD and PH. Using Kaplan–Meier analysis, we assessed survival for the five subtypes of CTD ± ILD and compared survival among CTD patients overall ± ILD with survival in the general population ± ILD. Results: We identified 11,731 patients with a diagnosis of CTD; 637 (5.4%) had a diagnosis of ILD. The proportion of patients with ILD was higher in SSc (13.4%) and MCTD (9.1%) than in myositis (6.0%), SLE (4.1%), and Sjögren (2.8%). Fifty-one percent were diagnosed with ILD in their fifties and sixties. PH was more frequent in SSc (7.5%) and MCTD (4.1%). Five-year survival was 73.3% (66.7–80.6) in SSc-ILD, 81.0% (69.0–95.1) in MCTD-ILD, 84.7% (77.3–92.9) in myositis-ILD, 83.5% (76.2–91.5) in SLE-ILD, and 84.7 (78.4–91.6) in Sjögren-associated ILD. Survival in CTD-ILD overall was impaired for all age groups compared with CTD alone. Age-stratified survival was comparable between CTD-ILD and ILD in the general population. The survival gap between ILD and non-ILD increased with age. Conclusion: Survival was comparable between different CTD-ILD subtypes and comparable to survival in non-CTD-ILD.
format	article
author	Charlotte Hyldgaard Elisabeth Bendstrup Alma Becic Pedersen Lars Pedersen Torkell Ellingsen
author_facet	Charlotte Hyldgaard Elisabeth Bendstrup Alma Becic Pedersen Lars Pedersen Torkell Ellingsen
author_sort	Charlotte Hyldgaard
title	Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
title_short	Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
title_full	Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
title_fullStr	Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
title_full_unstemmed	Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort
title_sort	interstitial lung disease in connective tissue diseases: survival patterns in a population-based cohort
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/a81c880954d0427ba2bc0f5735a020e4
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Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort

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