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

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

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

Prescribing Patterns and Tolerability of Mycophenolate and Azathioprine in Patients with Nonidiopathic Pulmonary Fibrosis Fibrotic Interstitial Lung Disease

Non

Rugby meets economics

In professional rugby, the right mix of centralization and decentralization must be found, with both coordination from the rugby union and autonomy for the provincial teams. Too much central control could undermine the credibility of the playing-field competition; too little could allow competitive imbalances to arise. For competitive balance, there must be some rules on the movement of players among teams; however, these rules should not put an undue burden on the players.