25 research outputs found
Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry
Rationale
Progressive fibrosing interstitial lung disease (PF-ILD) is characterized by progressive
physiologic, symptomatic, and/or radiographic worsening. The real-world prevalence and
characteristics of PF-ILD remain uncertain.
Methods
Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015-2020.
PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung
transplantation, or any 2 of: relative FVC decline ≥5 and <10%, worsening respiratory
symptoms, or worsening fibrosis on computed tomography of the chest, all within 24 months of
diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups.
Characteristics associated with progression were determined by multivariable regression.
Results
Of 2,746 patients with fibrotic ILD (mean age 65±12 years, 51% female), 1,376 (50%) met PFILD criteria in the first 24 months of follow-up. PF-ILD occurred in 427 (59%) patients with
idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP),
281 (51%) with unclassifiable ILD (U-ILD), and 402 (45%) with connective tissue diseaseassociated ILD (CTD-ILD). Compared to IPF, time to progression was similar in patients with
HP (hazard ratio [HR] 0.96, 95% confidence interval, CI 0.79-1.17), but was delayed in patients
with U-ILD (HR 0.82, 95% CI 0.71-0.96) and CTD-ILD (HR 0.65, 95% CI 0.56-0.74).
Background treatment varied across diagnostic subtypes with 66% of IPF patients receiving
antifibrotic therapy, while immunomodulatory therapy was utilized in 49%, 61%, and 37% of
patients with CHP, CTD-ILD, and U-ILD respectively. Increasing age, male sex,
gastroesophageal reflux disease, and lower baseline pulmonary function were independently
associated with progression.
Interpretation
Progression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF.
Routinely collected variables help identify patients at risk for progression and may guide
therapeutic strategie
The clinical frailty scale for risk stratification in patients with fibrotic interstitial lung disease.
BACKGROUND
Previous studies have shown the importance of frailty in patients with fibrotic interstitial lung disease (ILD).
RESEARCH QUESTION
Is the Clinical Frailty Scale (CFS) a valid tool to improve risk stratification in patients with fibrotic ILD?
STUDY DESIGN AND METHODS
Patients with fibrotic ILD were included from the prospective multicenter Canadian Registry for Pulmonary Fibrosis. The CFS was assessed using available information from initial ILD clinic visits. Patients were stratified into fit (CFS 1-3), vulnerable (CFS 4), and frail (CFS 5-9) subgroups. Cox proportional hazards and logistic regression models with mixed effects were used to estimate time to death or lung transplantation. A derivation and validation cohort were used to establish prognostic performance. Trajectories of functional tests were compared using joint models.
RESULTS
Of the 1587 patients with fibrotic ILD, 858 (54%) were fit, 400 (25%) vulnerable and 329 (21%) frail. Frailty was a risk factor for early mortality (HR 5.58, 95%CI 3.64-5.76, p<0.001) in the entire cohort, in individual ILD diagnoses, and after adjustment for potential confounders. Adding frailty to established risk prediction parameters improved the prognostic performance in derivation and validation cohorts. Frail patients had larger annual declines in forced vital capacity (FVC) %-predicted compared to fit patients (-2.32 (95%CI -3.39 to -1.17) vs. -1.55 (95%CI -2.04 to -1.15); p=0.02, respectively).
INTERPRETATION
The simple and practical CFS is associated with pulmonary and physical function decline in patients with fibrotic ILD and provides additional prognostic accuracy in clinical practice
Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
Background:
The CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) is a multi-center, prospective registry designed to study the natural history of fibrotic interstitial lung disease (ILD) in adults. The aim of this cross-sectional sub-study was to describe the baseline characteristics, risk factors, and comorbidities of patients enrolled in CARE-PF to date.
Methods:
Patients completed study questionnaires and clinical measurements at enrollment and each follow-up visit. Environmental exposures were assessed by patient self-report and comorbidities by the Charlson Comorbidity Index (CCI). Baseline characteristics, exposures, and comorbidities were described for the overall study population and for incident cases, and were compared across ILD subtypes.
Results:
The full cohort included 1285 patients with ILD (961 incident cases (74.8%)). Diagnoses included connective tissue disease-associated ILD (33.3%), idiopathic pulmonary fibrosis (IPF) (24.7%), unclassifiable ILD (22.3%), chronic hypersensitivity pneumonitis (HP) (7.5%), sarcoidosis (3.2%), non-IPF idiopathic interstitial pneumonias (3.0%, including idiopathic nonspecific interstitial pneumonia (NSIP) in 0.9%), and other ILDs (6.0%). Patient-reported exposures were most frequent amongst chronic HP, but common across all ILD subtypes. The CCI was ≤2 in 81% of patients, with a narrow distribution and range of values.
Conclusions:
CTD-ILD, IPF, and unclassifiable ILD made up 80% of ILD diagnoses at ILD referral centers in Canada, while idiopathic NSIP was rare when adhering to recommended diagnostic criteria. CCI had a very narrow distribution across our cohort suggesting it may be a poor discriminator in assessing the impact of comorbidities on patients with ILD.Medicine, Faculty ofPharmaceutical Sciences, Faculty ofOther UBCNon UBCMedicine, Department ofReviewedFacult
Costs of Workplace Productivity Loss in Patients with Connective Tissue Disease Associated Interstitial Lung Disease.
RATIONALE
Interstitial lung disease (ILD) develops in a large percentage of patients with connective tissue disease (CTD) and is associated with increased morbidity and mortality. Patients with CTD-associated ILD (CTD-ILD) often present at a young age, suggesting that ILD likely impacts workplace productivity.
OBJECTIVES
We aimed to determine the employment rate and workplace productivity loss, its associated factors, and its estimated costs in patients with fibrotic CTD-ILD.
METHODS
Patients with fibrotic CTD-ILD from the six centres of the CAnadian REgistry for Pulmonary Fibrosis were eligible. Health-related productivity loss was assessed using the Work Productivity and Activity Impairment questionnaire. Proposed factors associated with low workplace productivity were forced into a multivariable regression model. Average productivity loss in hours/week were used to calculate the costs of productivity loss based on hourly wage obtained from national census data matched for age and sex. Workplace productivity loss outcomes in CTD-ILD patients were compared to patients with a non-CTD fibrotic ILD.
RESULTS
Of 375 eligible patients with fibrotic CTD-ILD, 113 (30%) were employed. Productivity loss was reported by 59% of employed patients with mean loss of 9.4±1.2 hours/week, including 3.9±0.9 hours/week from absenteeism and 5.5±0.7 hours/week from presenteeism. Employment among patients with fibrotic CTD-ILD 25-54 years old was 27% lower than the matched general Canadian population (56% vs. 83%, p<0.001). Employment in CTD-ILD patients ≥55 years old was 17% lower than the matched population (19% vs. 36%, p<0.001). Workplace productivity loss was not associated with respiratory symptoms or lung physiology. Annual costs of productivity loss were calculated at 13,593 Canadian Dollars per employee with fibrotic CTD-ILD. Workplace productivity loss was similar in patients with fibrotic CTD-ILD and non-CTD fibrotic ILD.
CONCLUSIONS
Patients with fibrotic CTD-ILD frequently report workplace productivity loss, which is unexplained by respiratory symptoms or lung physiology and is associated with significant costs
A cluster-based analysis evaluating the impact of comorbidities in fibrotic interstitial lung disease
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.Medicine, Faculty ofPharmaceutical Sciences, Faculty ofNon UBCMedicine, Department ofReviewedFacult
The Canadian Registry for Pulmonary Fibrosis: Design and Rationale of a National Pulmonary Fibrosis Registry
Background. The relative rarity and diversity of fibrotic interstitial lung disease (ILD) have made it challenging to study these diseases in single-centre cohorts. Here we describe formation of a multicentre Canadian registry that is needed to describe the outcomes of fibrotic ILD and to enable detailed healthcare utilization analyses that will be the cornerstone for future healthcare planning. Methods. The Canadian Registry for Pulmonary Fibrosis (CARE-PF) is a prospective cohort anticipated to consist of at least 2,800 patients with fibrotic ILD. CARE-PF will be used to (1) describe the natural history of fibrotic ILD, specifically determining the incidence and outcomes of acute exacerbations of ILD subtypes and (2) determine the impact of ILD and acute exacerbations of ILD on health services use and healthcare costs in the Canadian population. Consecutive patients with fibrotic ILD will be recruited from five Canadian ILD centres over a period of five years. Patients will be followed up as clinically indicated and will complete standardized questionnaires at each clinic visit. Prespecified outcomes and health services use will be measured based on self-report and linkage to provincial health administrative databases. Conclusion. CARE-PF will be among the largest prospective multicentre ILD registries in the world, providing detailed data on the natural history of fibrotic ILD and the healthcare resources used by these patients. As the largest and most comprehensive cohort of Canadian ILD patients, CARE-PF establishes a network for future clinical research and early phase clinical trials and provides a platform for translational and basic science research.Peer Reviewe
Validation and minimum important difference of the UCSD Shortness of Breath Questionnaire in fibrotic interstitial lung disease
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.Medicine, Faculty ofPharmaceutical Sciences, Faculty ofNon UBCMedicine, Department ofRespiratory Medicine, Division ofReviewedFacultyResearcherOthe