RT Journal Article
SR Electronic
T1 Idiopathic pulmonary fibrosis: state of the art for 2023
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 2200957
DO 10.1183/13993003.00957-2022
VO 61
IS 4
A1 Anna J. Podolanczuk
A1 Carey C. Thomson
A1 Martine Remy-Jardin
A1 Luca Richeldi
A1 Fernando J. Martinez
A1 Martin Kolb
A1 Ganesh Raghu
YR 2023
UL http://erj.ersjournals.com/content/61/4/2200957.abstract
AB 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.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