Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study)

Nasser, Mouhamad; Larrieu, Sophie; Si‐Mohamed, Salim; Kazemi, Ahmad; Boussel, Loîc; Brevet, Marie; Chalabreysse, Lara; Fabre, C.; Marque, Sébastien; Revel, D.; Thivolet‐Béjui, F.; Traclet, Julie; Zeghmar, Sabrina; Maucort‐Boulch, Delphine; Cottin, Vincent

doi:10.1183/13993003.02718-2020

Cited by 136 publications

(191 citation statements)

References 23 publications

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“…The percentage of patients with fibrosing ILDs other than IPF that developed progression was estimated at 18–32% in a survey of pulmonary, rheumatology and internal medicine physicians from Japan, the US and four European countries [ 19 ], and at 15% in a US claims database study [ 20 ]. We have previously described a single-centre clinical cohort with PF-ILD as part of the PROGRESS study [ 22 ] where, similarly, 27.2% of patients with a fibrosing ILD other than IPF had a progressive phenotype. In the current study, approximately 47% of patients with fibrosing ILD other than IPF had a progressive phenotype.…”

Section: Discussionmentioning

confidence: 99%

“…In the current study, approximately 47% of patients with fibrosing ILD other than IPF had a progressive phenotype. This higher percentage of patients with a progressive phenotype may be due to the fact that patients were tentatively identified based on high consumption of respiratory healthcare in a limited period of time, rather than just the results of sequential PFTs, chest CT and symptoms [ 22 ] or a physician’s survey [ 19 ]. In a preliminary validation study, algorithm 3 was found to have a specificity of 69% and a sensitivity of 46% (data not shown), indicating that our estimates were conservative.…”

Section: Discussionmentioning

confidence: 99%

“…Although data are available for individual types of PF-ILDs (for example, systemic sclerosis-ILD [SSc-ILD] and other connective tissue disease-ILDs [CTD-ILDs], hypersensitivity pneumonitis [HP], unclassifiable ILD, etc. ), data on PF-ILDs as a group come primarily from clinical trials [ 10 , 21 ] and one academic retrospective series [ 22 ]. Therefore, large-scale epidemiological data are limited [ 20 ] and this remains an unmet need, as emphasised by a research task force of the American Thoracic Society [ 23 ].…”

Section: Introductionmentioning

confidence: 99%

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Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

et al. 2021

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Background There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. An algorithm for extracting claims data was developed to indirectly identify and describe patients with PF-ILD in the French national administrative healthcare database. Methods The French healthcare database, the Système National des Données de Santé (SNDS), includes data related to ambulatory care, hospitalisations and death for 98.8% of the population. In this study, algorithms based on age, diagnosis and healthcare consumption were created to identify adult patients with PF-ILD other than idiopathic pulmonary fibrosis between 2010 and 2017. Incidence, prevalence, survival estimates, clinical features and healthcare resource usage and costs were described among patients with PF-ILD. Results We identified a total of 14,413 patients with PF-ILD. Almost half of them (48.1%) were female and the mean (± standard deviation) age was 68.4 (± 15.0) years. Between 2010 and 2017, the estimated incidence of PF-ILD ranged from 4.0 to 4.7/100,000 person-years and the estimated prevalence from 6.6 to 19.4/100,000 persons. The main diagnostic categories represented were exposure-related ILD other than hypersensitivity pneumonitis (n = 3486; 24.2%), idiopathic interstitial pneumonia (n = 3113; 21.6%) and rheumatoid arthritis-associated ILD (n = 2521; 17.5%). Median overall survival using Kaplan–Meier estimation was 3.7 years from the start of progression. During the study, 95.2% of patients had ≥ 1 hospitalisation for respiratory care and 34.3% were hospitalised in an intensive care unit. The median (interquartile range) total specific cost per patient during the follow-up period was €25,613 (10,622–54,287) and the median annual cost per patient was €18,362 (6856–52,026), of which €11,784 (3003–42,097) was related to hospitalisations. Limitations included the retrospective design and identification of cases through an algorithm in the absence of chest high-resolution computed tomography scans and pulmonary function tests. Conclusions This large, real-world, longitudinal study provides important insights into the characteristics, epidemiology and healthcare resource utilisation and costs associated with PF-ILD in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and healthcare services. Trial registration ClinicalTrials.gov, NCT03858842. ISRCTN, ISRCTN12345678. Registered 3 January 2019—Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03858842

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

et al. 2021

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“…Recently, Nasser et al reported the clinical characteristics of patients with PF-ILD other than IPF in a real-world setting. [ 40 ] They reported that 168 of 617 (27.2%) patients with non-IPF, ILD had progressive fibrosing phenotypes. This result was higher than our study; however, the definition of progressive phenotypes used in their study was based on INBUILD trial.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

Komatsu¹,

Yamamoto²,

Kitaguchi³

et al. 2021

Medicine

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Progressive fibrosing interstitial lung disease (PF-ILD) is a progressive phenotype of fibrosing ILDs with varying definitions and elusive clinical characteristics. We aimed to clarify the clinical features and prognosis of PF-ILD cases based on the deterioration of pulmonary function. Altogether, 91 consecutive ILD patients who underwent at least 2 pulmonary function tests (PFTs) with an interval of at least 24 months, as the screening period, between January 2009 and December 2015 were retrospectively reviewed. The deterioration of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLco) was calculated based on PFT data and screening period. The definition of PF-ILD was relative decline of 10% or more in FVC per 24 months or relative decline in FVC of 5% or more with decline in DLco of 15% or more per 24 months. Medical records of 34 patients with idiopathic pulmonary fibrosis (IPF), 11 patients with non-IPF, PF-ILD, and 46 patients with non-IPF, non-PF-ILD were retrospectively analyzed. Patient characteristics, pharmacologic or non-pharmacologic treatment status, and prognosis were compared between the IPF and non-IPF groups and between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Eleven patients (19.3%) showed a progressive phenotype in the non-IPF group. The pulmonary function data at the first PFT were worse in non-IPF, PF-ILD patients than in non-IPF, non-PF-ILD patients. There were no differences in the proportion of patients who were observed without pharmacologic treatment or of those receiving pharmacologic treatment between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Low %FVC at the first PFT and the usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography were risk factors for PF-ILD in the non-IPF group. The mortality in the non-IPF, PF-ILD group was significantly worse than that of the non-IPF, non-PF-ILD group and was as poor as that of the IPF group. Multivariate logistic analysis showed that aging and low %DLco at the first PFT were risk factors for mortality within the non-IPF group. The prognosis of non-IPF, PF-ILD patients was as poor as that of IPF patients. Non-IPF, PF-ILD patients require more intensive treatment before disease progression.

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“…Other non-IPF ILD subtypes that may be associated with a progressive fibrosing phenotype include connective tissue disease-associated ILD (CTD-ILD), idiopathic non-specific interstitial pneumonia (iNSIP), sarcoidosis, hypersensitivity pneumonitis (HP), occupational ILD and unclassifiable ILD (uILD) [ 1 , 2 , 3 , 4 ]. Based on survey data and cohort studies, it is estimated that 30% of non-IPF ILD patients will develop a progressive fibrosing phenotype [ 5 , 6 , 7 ]. In this review, we summarize the current pharmacological management and highlight the unmet needs in patients with non-IPF ILD.…”

Section: Introductionmentioning

confidence: 99%

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

Goos

Sadeleer

Yserbyt

et al. 2021

JCM

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A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.

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Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study)

Cited by 136 publications

References 23 publications

Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

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