Vincent Cottin,1 Christopher J Ryerson,2 Kevin R Flaherty,3 Joyce S Lee,4 Tamera J Corte,5 Birgit Schinzel,6 Leticia Orsatti,6 Manuel Quaresma,6 Athol U Wells7
1National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon, France; 2Department of Medicine & Centre for Heart Lung Innovation, University of British Columbia, Vancouver, Canada; 3University of Michigan, Ann Arbor, MI, USA; 4Dept of Medicine, University of Colorado Denver, Aurora, CO, USA; 5Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia and University of Sydney, New South Wales, Australia; 6Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany; 7National Institute for Health Research Respiratory Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, and National Heart and Lung Institute, Imperial College, London, UK.
Introduction
- A greater extent of emphysema on high-resolution computed tomography (HRCT) may be associated with a smaller decline in forced vital capacity (FVC) in patients with idiopathic pulmonary fibrosis (IPF).1
- The combined pulmonary fibrosis and emphysema (CPFE) index estimates the extent of emphysema on HRCT in patients with IPF based on pulmonary function tests. The formula was developed in a cohort of 212 patients diagnosed with IPF between 1990 and 1996 and tested in 455 patients with IPF enrolled in two clinical trials and in a real-world cohort of 191 patients diagnosed with IPF between 2011 and 2014.2
- In the INPULSIS trials, nintedanib reduced the annual rate of decline in FVC (mL/year) in patients with IPF by 49% versus placebo.3