Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease characterised by worsening respiratory symptoms and physiological impairment. Increasing awareness of the clinical manifestations of IPF, more widespread use of computed tomography scans and other potential factors have contributed to a rising prevalence of IPF over the last two decades, especially among people over the age of 65 years. Significant advances in the understanding of the pathobiology of IPF have emerged, and multiple genetic and nongenetic contributors have been identified. The individual patient course and the rate of disease progression in IPF are often unpredictable and heterogeneous. The rate of lung function decline is further modified by treatment with antifibrotic therapies, which have been shown to slow down disease progression. The presence of comorbid conditions may increase symptom burden and impact survival. Clinical monitoring at regular intervals to assess for disease progression by worsening symptoms, physiological parameters and/or radiological features is essential to assess the natural disease course and to guide further management, including prompt detection of complications and comorbid conditions that warrant additional treatment considerations, and timely consideration of referral to palliative care and lung transplantation for the appropriate patient. More studies are needed to determine whether early detection of IPF might improve patient outcomes. The purpose of this concise clinical review is to provide an update on IPF diagnosis, epidemiology, natural history and treatment in the context of new knowledge and latest clinical practice guidelines.
Abstract
This concise clinical review provides an update on IPF diagnosis, epidemiology, natural history and treatment in the context of new knowledge and the latest clinical practice guidelines https://bit.ly/3vDrRLR
Footnotes
Conflict of interest: A.J. Podolanczuk reports grants from the American Lung Association and NHLBI, consulting fees from Regeneron, Roche and Imvaria, lecture honoraria from National Association for Continuing Education and EBSCO/DynaMed, and advisory board participation with Boehringer Ingelheim, outside the submitted work. L. Richeldi reports grants from Roche, Boehringer Ingelheim and the Italian Drug Agency, consulting fees from Biogen, Celgene, Nitto, Pliant Therapeutics, Toray, BMS, RespiVant and CSL Behring, lecture honoraria from Boehringer Ingelheim, Zambon and Cipla, travel support from Boehringer Ingelheim and Roche, advisory board participation with Roche, Boehringer Ingelheim, FibroGen and Promedior, and steering committee membership with Boehringer Ingelheim and Roche, outside the submitted work. F.J. Martinez reports steering committee membership with Afferent/Merck, Bayer, Biogen, Nitto, Novartis, Patara/Respivant, Promedior/Roche and Veracyte, consulting fees from Abvie, Boehringer Ingelheim, BMS, Bridge Biotherapeutics, CSL Behring, DevPro, Genentech, IQVIA, Sanofi, Shionogi, twoXAR and Veracyte, travel support from Boehringer Ingelheim, CSL Behring and Patara/Respivant, and advisory board membership with Biogen and Boehringer Ingelheim, outside the submitted work. M. Kolb reports grants from Boehringer Ingelheim, Pieris and Roche, consulting fees from Boehringer Ingelheim, Roche, Horizon, Cipla, AbbVie, Belerophon, Algernon, CSL Behring and United Therapeutics, lecture honoraria from Roche, Novartis and Boehringer Ingelheim, payment for expert testimony from Roche, advisory board membership with United Therapeutics and LabCorp, and has been remunerated for Chief Editorship of the European Respiratory Journal. G. Raghu reports personal fees from Boehringer Ingelheim, BMS, United Therapeutics and Veracyte, consulting fees and/or advisory board membership from Boehringer Ingelheim, Biogen, Bellerophan, Fibrogen, Nitto, Roche Genentech, Novartis, Zambon, Avalyn and Blade Therapeutics, and research grants from the National Institutes of Health, outside the submitted work. All other authors have nothing to disclose.
- Received May 9, 2022.
- Accepted December 22, 2022.
- Copyright ©The authors 2023. For reproduction rights and permissions contact permissions{at}ersnet.org
LIBRARY USERS
Log in through your institution
Purchase access
Jump To
- Article
- Abstract
- Abstract
- Introduction
- Diagnosis of IPF
- Incidence and prevalence
- Risk factors for IPF
- Early detection and screening
- Natural history of IPF
- Acute exacerbation of IPF
- Comorbidities
- Treatment of IPF
- Monitoring for disease progression
- Biomarkers
- Morbidity and mortality
- Summary and future directions
- Supplementary material
- Shareable PDF
- Footnotes
- References
- Figures & Data
- Info & Metrics