Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis
BMJ 2022; 377 doi: https://doi.org/10.1136/bmj-2021-066354 (Published 29 June 2022) Cite this as: BMJ 2022;377:e066354
- Gabrielle Y Liu, pulmonary and critical care fellow,
- G R Scott Budinger, professor of medicine, chief of pulmonary and critical care in the Department of Medicine,
- Jane E Dematte, professor of medicine
- Division of Pulmonary and Critical Care Medicine, Department of Medicine, Northwestern University, Chicago, IL, USA
- Correspondence to: J E Dematte j-dematte{at}northwestern.edu
ABSTRACT
Similarly to idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases can develop progressive pulmonary fibrosis (PPF) characterized by declining lung function, a poor response to immunomodulatory therapies, and early mortality. The pathophysiology of disordered lung repair involves common downstream pathways that lead to pulmonary fibrosis in both IPF and PPF. The antifibrotic drugs, such as nintedanib, are indicated for the treatment of IPF and PPF, and new therapies are being evaluated in clinical trials. Clinical, radiographic, and molecular biomarkers are needed to identify patients with PPF and subgroups of patients likely to respond to specific therapies. This article reviews the evidence supporting the use of specific therapies in patients with IPF and PPF, discusses agents being considered in clinical trials, and considers potential biomarkers based on disease pathogenesis that might be used to provide a personalized approach to care.
Footnotes
Series explanation: State of the Art Reviews are commissioned on the basis of their relevance to academics and specialists in the US and internationally. For this reason they are written predominantly by US authors
Contributors: All authors contributed to the intellectual content, did the literature search, and participated in the preparation, editing, and critical review of the manuscript.
Funding: GYL is supported by NIH grant F32-HL162318 and North Western University’s Lung Sciences Training Program 5T32HL076139-17. GRSB is supported by supported by NIH grants ES013995, HL071643, and AG049665 and the Veterans Administration grant BX000201.
Competing interests: We have read and understood the BMJ policy on declaration of interests and declare: none.
Patient involvement: No patients or members of the public were involved in the design, conduct, reporting, or dissemination plans of this manuscript.
Provenance and peer review: Commissioned; externally peer reviewed.
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