Estimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data
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REVIEW
Estimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data from a Physician Survey Amy Olson . Nadine Hartmann . Padmaja Patnaik . Laura Wallace . Rozsa Schlenker-Herceg . Mouhamad Nasser Anna-Maria Hoffmann-Vold
. Luca Richeldi .
. Vincent Cottin
Received: October 14, 2020 / Accepted: November 19, 2020 Ó The Author(s) 2020
ABSTRACT Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis exhibit a progressive clinical phenotype. These chronic progressive fibrosing ILDs have a variety of underlying diseases, and their prevalence is currently unknown. Here we carry out the first systematic review of literature on the
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s12325020-01578-6) contains supplementary material, which is available to authorized users.
prevalence of fibrosing ILDs and progressive fibrosing ILDs using data from physician surveys to estimate frequency of progression among different ILDs. We searched MEDLINE and Embase for studies assessing prevalence of ILD, individual ILDs associated with fibrosis and progressive fibrosing ILDs. These were combined with data from previously published physician surveys to obtain prevalence estimates of each chronic fibrosing ILD with a progressive phenotype and of progressive fibrosing ILDs overall. We identified 16 publications, including five reporting overall ILD prevalence, estimated at 6.3–76.0 per 100,000
A. Olson (&) Interstitial Lung Disease Program, Department of Medicine, National Jewish Health, Denver, CO, USA e-mail: [email protected]
L. Richeldi Fondazione Policlinico Universitario A. Gemelli IRCCS, Universita` Cattolica del Sacro Cuore, Rome, Italy
N. Hartmann Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
A.-M. Hoffmann-Vold Department of Rheumatology, Oslo University Hospital, Oslo, Norway
P. Patnaik L. Wallace Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, CT, USA
V. Cottin National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, UMR 754, University Claude Bernard Lyon 1, Lyon, France
R. Schlenker-Herceg Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany M. Nasser Department of Respiratory Medicine, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Lyon, France
Adv Ther
people in Europe (four studies) and 74.3 per 100,000 in the USA (one study). In total, 13–40% of ILDs were estimated to develop a progressive fibrosing phenotype, with overall prevalence estimates for progressive fibrosing ILDs of 2.2–20.0 per 100,000 in Europe and 28.0 per 100,000 in the USA. Prevalence estimates for individual progressive fibrosing ILDs varied up to 16.7 per 100,000 people. These conditions represent a sizeable fraction of chronic respiratory disorders and have a high unmet need.
Keywords: Epidemiology; Fibrosis; Interstitial lung disease; Prevalence; Progressive Key Summar
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