Abstract
BackgroundConnective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD. The objective of this study was to identify baseline demographic and clinical characteristics that are associated with progression and mortality in CTD-ILD.MethodsPatients were retrospectively identified from an adult CTD-ILD clinic. The predictive significance of baseline variables on serial forced vital capacity (FVC), diffusion capacity (DLCO), and six-minute walk distance (6MWD) was assessed using linear mixed effects models, and Cox regression analysis was performed to assess impact on mortality.Results359 patients were included in the study. Median follow-up time was 4.0 (IQR 1.5–7.6) years. On both unadjusted and multivariable analysis, male sex and South Asian ethnicity were associated with decline in FVC. Male sex, positive smoking history, and diagnosis of systemic sclerosis (SSc) vs. other CTD were associated with decline in DLCO. Male sex and usual interstitial pneumonia (UIP) pattern predicted decline in 6MWD. There were 85 (23.7%) deaths. Male sex, older age, First Nations ethnicity, and a diagnosis of systemic sclerosis vs. rheumatoid arthritis were predictors of mortality on unadjusted and multivariable analysis.ConclusionMale sex, older age, smoking, South Asian or First Nations ethnicity, and UIP pattern predicted decline in lung function and/or mortality in CTD-ILD. Further longitudinal studies may add to current clinical prediction models for prognostication in CTD-ILD.
Highlights
Connective tissue disease-associated interstitial lung disease (CTD-Interstitial lung disease (ILD)) is associated with reduced quality of life and poor prognosis
The prevalence of ILD varies with disease subtype; ILD is reported in up to 90% of patients with systemic sclerosis (SSc), whereas it is less prevalent in rheumatoid arthritis (RA, 4–68%), mixed connective tissue disease (MCTD, 20–85%), and the inflammatory myopathies polymyositis and dermatomyositis (PM/DM, 15–70%), reported numbers vary [1,2,3,4,5]
Older age, baseline severity of lung function impairment, and decline in physiologic parameters over time are associated with disease progression and mortality in studies of individual Connective tissue disease-associated interstitial lung disease (CTD-ILD) subtypes [4, 6, 10,11,12,13]; these studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD
Summary
Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Older age, baseline severity of lung function impairment, and decline in physiologic parameters over time are associated with disease progression and mortality in studies of individual CTD-ILD subtypes [4, 6, 10,11,12,13]; these studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD. Identification of such variables could have a substantial impact on patient care by identifying patients who might warrant more aggressive therapy or earlier referral for lung transplantation assessment
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.