While all patients with IPF have progressive pulmonary fibrosis, there exists a variety of other ILDs that may also develop a
progressive fibrosing ILD.2,3
Progression of pulmonary fibrosis
Worsening respiratory symptoms
Decline of pulmonary function
In progressive fibrosing ILD, pulmonary fibrosis continues to develop independent
of the originating disease or trigger, along common pathogenic pathways2-5
*Data based on online surveys with 486 physicians.1
In addition to the functional impact, patients with progressive fibrosing ILD may suffer emotional distress, including feelings of depression, loss, and powerlessness.1,6
See the impact of progressive fibrosing ILDs across a wide range of ILDs
Up to 3 in 10 patients with CTD-ILD including:
See the impact of progressive fibrosing ILDs across a range of autoimmune diseases
Slowing disease progression is of critical importance in patients with a progressive fibrosing
ILD as the risk of early death is heightened2
Dr Nazia Chaudhuri explains the progressive fibrosing ILD phenotype
See what Professor Toby Maher has to say
Refer patients with a progressive fibrosing ILD for pulmonary rehabilitation as soon as
possible to help keep them active and preserve their quality of life6
Wijsenbeek M, Kreuter M, Fischer A, et al. Non-IPF progressive fibrosing interstitial lung disease (PF-ILD): the patient journey. Am J Respir Crit Care Med. 2018;197:1-2.
Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of ... in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076.
Selman M, King TE Jr, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med. 2001;134(2):136-151.
Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. Eur Respir Rev. 2015;24(135):102-114.
Morisset J, Dubè B-P, Garvey C, et al The unmet educational needs of patients with interstitial lung disease, Setting the stage for tailored pulmonary rehabilitation. Ann Am Thorac Soc.2016;13(7):1026-1033.
Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;https://doi.org/10.1007/s12325-019-00992-9.
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