Browsing by Author "Cox, Gerard"

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    ItemOpen Access
    A cluster-based analysis evaluating the impact of comorbidities in fibrotic interstitial lung disease
    (2020-12-07) Wong, Alyson W; Lee, Tae Y; Johannson, Kerri A; Assayag, Deborah; Morisset, Julie; Fell, Charlene D; Fisher, Jolene H; Shapera, Shane; Gershon, Andrea S; Cox, Gerard; Halayko, Andrew J; Hambly, Nathan; Manganas, Helene; Sadatsafavi, Mohsen; Wilcox, Pearce G; To, Teresa; Marcoux, Veronica; Khalil, Nasreen; Kolb, Martin; Ryerson, Christopher J
    Abstract Background Comorbidities are frequent and have been associated with poor quality of life, increased hospitalizations, and mortality in patients with interstitial lung disease (ILD). However, it is unclear how comorbidities lead to these negative outcomes and whether they could influence ILD disease progression. The goal of this study was to identify clusters of patients based on similar comorbidity profiles and to determine whether these clusters were associated with rate of lung function decline and/or mortality. Methods Patients with a major fibrotic ILD (idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, connective tissue disease-associated ILD, and unclassifiable ILD) from the CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) were included. Hierarchical agglomerative clustering of comorbidities, age, sex, and smoking pack-years was conducted for each ILD subtype to identify combinations of these features that frequently occurred together in patients. The association between clusters and change in lung function over time was determined using linear mixed effects modeling, with adjustment for age, sex, and smoking pack-years. Kaplan Meier curves were used to assess differences in survival between the clusters. Results Discrete clusters were identified within each fibrotic ILD. In IPF, males with obstructive sleep apnea (OSA) had more rapid decline in FVC %-predicted (− 11.9% per year [95% CI − 15.3, − 8.5]) compared to females without any comorbidities (− 8.1% per year [95% CI − 13.6, − 2.7]; p = 0.03). Females without comorbidities also had significantly longer survival compared to all other IPF clusters. There were no significant differences in rate of lung function decline or survival between clusters in the other fibrotic ILD subtypes. Conclusions The combination of male sex and OSA may portend worse outcomes in IPF. Further research is required to elucidate the interplay between sex and comorbidities in ILD, as well as the role of OSA in ILD disease progression.
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    ItemOpen Access
    Letter to the Editor
    (2010-01-01) Kelly, Margaret M; Hargreave, Frederick E; Cox, Gerard
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    Validation and minimum important difference of the UCSD Shortness of Breath Questionnaire in fibrotic interstitial lung disease
    (2021-07-08) Chen, Tao; Tsai, Amy P. Y.; Hur, Seo A.; Wong, Alyson W.; Sadatsafavi, Mohsen; Fisher, Jolene H.; Johannson, Kerri A.; Assayag, Deborah; Morisset, Julie; Shapera, Shane; Khalil, Nasreen; Fell, Charlene D.; Manganas, Helene; Cox, Gerard; To, Teresa; Gershon, Andrea S.; Hambly, Nathan; Halayko, Andrew J.; Wilcox, Pearce G.; Kolb, Martin; Ryerson, Christopher J.
    Abstract Rationale The University of California, San Diego Shortness of Breath Questionnaire (UCSDSOBQ) is a frequently used domain-specific dyspnea questionnaire; however, there is little information available regarding its use and minimum important difference (MID) in fibrotic interstitial lung disease (ILD). We aimed to describe the key performance characteristics of the UCSDSOBQ in this population. Methods UCSDSOBQ scores and selected anchors were measured in 1933 patients from the prospective multi-center Canadian Registry for Pulmonary Fibrosis. Anchors included the St. George’s Respiratory Questionnaire (SGRQ), European Quality of Life 5 Dimensions 5 Levels questionnaire (EQ-5D-5L) and EQ visual analogue scale (EQ-VAS), percent-predicted forced vital capacity (FVC%), diffusing capacity of the lung for carbon monoxide (DLCO%), and 6-min walk distance (6MWD). Concurrent validity, internal consistency, ceiling and floor effects, and responsiveness were assessed, followed by estimation of the MID by anchor-based (linear regression) and distribution-based methods (standard error of measurement). Results The UCSDSOBQ had a high level of internal consistency (Cronbach’s alpha = 0.97), no obvious floor or ceiling effect, strong correlations with SGRQ, EQ-5D-5L, and EQ-VAS (|r| > 0.5), and moderate correlations with FVC%, DLCO%, and 6MWD (0.3 < |r| < 0.5). The MID estimate for UCSDSOBQ was 5 points (1–8) for the anchor-based method, and 4.5 points for the distribution-based method. Conclusion This study demonstrates the validity of UCSDSOBQ in a large and heterogeneous population of patients with fibrotic ILD, and provides a robust MID estimate of 5–8 points.