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

Goos, Tinne; De Sadeleer, Laurens J.; Yserbyt, Jonas; Verleden, Geert M.; Vermant, Marie; Verleden, Stijn E.; Wuyts, Wim A.

doi:10.3390/jcm10061330

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

Tinne Goos

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Laurens J. De Sadeleer

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Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department CHROMETA, KU Leuven, B-3000 Leuven, Belgium

Department of Respiratory Diseases, University Hospitals Leuven, B-3000 Leuven, Belgium

Author to whom correspondence should be addressed.

Academic Editors: Carlos Barrero and Antje Prasse

J. Clin. Med. 2021, 10(6), 1330; https://doi.org/10.3390/jcm10061330

Received: 22 January 2021 / Revised: 5 March 2021 / Accepted: 15 March 2021 / Published: 23 March 2021

(This article belongs to the Special Issue The New Perspective in Pulmonary Fibrosis)

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Abstract

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. View Full-Text

Keywords: progressive fibrosing interstitial lung disease; pulmonary fibrosis; interstitial lung disease; management

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

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MDPI and ACS Style

Goos, T.; De Sadeleer, L.J.; Yserbyt, J.; Verleden, G.M.; Vermant, M.; Verleden, S.E.; Wuyts, W.A. Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be. J. Clin. Med. 2021, 10, 1330. https://doi.org/10.3390/jcm10061330

AMA Style

Goos T, De Sadeleer LJ, Yserbyt J, Verleden GM, Vermant M, Verleden SE, Wuyts WA. Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be. Journal of Clinical Medicine. 2021; 10(6):1330. https://doi.org/10.3390/jcm10061330

Chicago/Turabian Style

Goos, Tinne, Laurens J. De Sadeleer, Jonas Yserbyt, Geert M. Verleden, Marie Vermant, Stijn E. Verleden, and Wim A. Wuyts. 2021. "Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be" Journal of Clinical Medicine 10, no. 6: 1330. https://doi.org/10.3390/jcm10061330

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