Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry.

Progressive fibrosing interstitial lung disease (PF-ILD) is characterised by progressive physiological, symptomatic and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain. Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015 and 2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation or any two 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. Of 2746 patients with fibrotic ILD (mean±sd age 65±12 years; 51% female), 1376 (50%) met PF-ILD 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 disease-associated ILD (CTD-ILD). Compared with IPF, time to progression was similar in patients with HP (hazard ratio (HR) 0.96, 95% 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 utilised in 49%, 61% and 37% of patients with CHP, CTD-ILD and U-ILD, respectively. Increasing age, male sex, gastro-oesophageal reflux disease and lower baseline pulmonary function were independently associated with progression. 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 strategies.

Hambly N ，Farooqi MM ，Dvorkin-Gheva A ，Donohoe K ，Garlick K ，Scallan C ，Chong SG ，MacIsaac S ，Assayag D ，Johannson KA ，Fell CD ，Marcoux V ，Manganas H ，Morisset J ，Comes A ，Fisher JH ，Shapera S ，Gershon AS ，To T ，Wong AW ，Sadatsafavi M ，Wilcox PG ，Halayko AJ ，Khalil N ，Cox G ，Richeldi L ，Ryerson CJ ，Kolb M ... -  《-》

A cluster-based analysis evaluating the impact of comorbidities in fibrotic interstitial lung disease.

Wong AW ，Lee TY ，Johannson KA ，Assayag D ，Morisset J ，Fell CD ，Fisher JH ，Shapera S ，Gershon AS ，Cox G ，Halayko AJ ，Hambly N ，Manganas H ，Sadatsafavi M ，Wilcox PG ，To T ，Marcoux V ，Khalil N ，Kolb M ，Ryerson CJ ... -  《RESPIRATORY RESEARCH》

Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis.

Fisher JH ，Kolb M ，Algamdi M ，Morisset J ，Johannson KA ，Shapera S ，Wilcox P ，To T ，Sadatsafavi M ，Manganas H ，Khalil N ，Hambly N ，Halayko AJ ，Gershon AS ，Fell CD ，Cox G ，Ryerson CJ ... -  《BMC Pulmonary Medicine》

Hospitalization Rates in Interstitial Lung Disease: An Analysis of the Pulmonary Fibrosis Foundation Registry.

King CS ，Ignacio RV ，Khangoora V ，Nyquist A ，Singhal A ，Thomas C ，Cantres OF ，Aryal S ，Shlobin OA ，Flaherty K ，Lasky J ，Nathan SD ... -  《-》

Real-life prevalence of progressive fibrosing interstitial lung diseases.

The concept of progressive fibrosing interstitial lung disease (PF-ILD) has recently emerged. However, real-life proportion of PF-ILDs outside IPF is still hard to evaluate. Therefore, we sought to estimate the proportion of PF-ILD in our ILD cohort. We also determined the proportion of ILD subtypes within PF-ILD and investigated factors associated with PF-ILDs. Finally, we quantified interobserver agreement between radiologists for the assessment of fibrosis. We reviewed the files of ILD patients discussed in multidisciplinary discussion between January 1st 2017 and December 31st 2019. Clinical data, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCTs) were centrally reviewed. Fibrosis was defined as the presence of traction bronchiectasis, reticulations with/out honeycombing. Progression was defined as a relative forced vital capacity (FVC) decline of ≥ 10% in ≤ 24 months or 5% < FVC decline < 10% and progression of fibrosis on HRCT in ≤ 24 months. 464 consecutive ILD patients were included. 105 had a diagnosis of IPF (23%). Most frequent non-IPF ILD were connective tissue disease (CTD)-associated ILD (22%), hypersensitivity pneumonitis (13%), unclassifiable ILD (10%) and sarcoidosis (8%). Features of fibrosis were common (82% of CTD-ILD, 81% of HP, 95% of uILD). After review of HRCTs and PFTs, 68 patients (19% of non-IPF ILD) had a PF-ILD according to our criteria. Interobserver agreement for fibrosis between radiologists was excellent (Cohen's kappa 0.86). The main diagnosis among PF-ILD were CTD-ILD (36%), HP (22%) and uILD (20%). PF-ILD patients were significantly older than non-F-ILD (P = 0.0005). PF-ILDs represent about 20% of ILDs outside IPF. This provides an estimation of the proportion of patients who might benefit from antifibrotics. Interobserver agreement between radiologists for the diagnosis of fibrotic ILD is excellent.

Gagliardi M ，Berg DV ，Heylen CE ，Koenig S ，Hoton D ，Tamirou F ，Pieters T ，Ghaye B ，Froidure A ... -  《Scientific Reports》