The natural history of progressive fibrosing interstitial lung diseases

The European respiratory journal :

article

Kevin K. Brown¹  Vincent Cottin²  Athol U. Wells³  Fernando J. Martinez⁵  Simon L.F. Walsh³  Victor J. Thannickal⁶  Antje Prasse⁷  Rozsa Schlenker-Herceg⁸  Rainer-Georg Goeldner⁹  Emmanuelle Clerisme-Beaty^1,10  Kay Tetzlaff^1,11 

[1] Dept of Medicine, National Jewish Health;National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Claude Bernard University Lyon 1;National Heart and Lung Institute, Imperial College;National Institute for Health Research Respiratory Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust;Dept of Medicine, Weill Cornell Medicine;Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama at Birmingham;Dept of Respiratory Medicine, MHH Hannover Medical School and Biomedical Research in Endstage and Obstructive Lung Disease (BREATH);Inc.;Boehringer Ingelheim Pharma GmbH & Co. KG;Boehringer Ingelheim International GmbH;Boehringer Ingelheim International GmbH, Ingelheim am Rhein;Dept of Sports Medicine, University of Tübingen

DOI  :  10.1183/13993003.00085-2020

学科分类：呼吸医学

来源: European Respiratory Society

【 摘 要 】

We used data from the INBUILD and INPULSIS trials to investigate the natural history of progressive fibrosing interstitial lung diseases (ILDs). Subjects in the two INPULSIS trials had a clinical diagnosis of idiopathic pulmonary fibrosis (IPF) while subjects in the INBUILD trial had a progressive fibrosing ILD other than IPF and met protocol-defined criteria for ILD progression despite management. Using data from the placebo groups, we compared the rate of decline in forced vital capacity (FVC) (mL·year −1 ) and mortality over 52 weeks in the INBUILD trial with pooled data from the INPULSIS trials. The adjusted mean annual rate of decline in FVC in the INBUILD trial (n=331) was similar to that observed in the INPULSIS trials (n=423) (−192.9 mL·year −1 and −221.0 mL·year −1 , respectively; nominal p-value=0.19). The proportion of subjects who had a relative decline in FVC >10% predicted at Week 52 was 48.9% in the INBUILD trial and 48.7% in the INPULSIS trials, and the proportion who died over 52 weeks was 5.1% in the INBUILD trial and 7.8% in the INPULSIS trials. A relative decline in FVC >10% predicted was associated with an increased risk of death in the INBUILD trial (hazard ratio 3.64) and the INPULSIS trials (hazard ratio 3.95). These findings indicate that patients with fibrosing ILDs other than IPF, who are progressing despite management, have a subsequent clinical course similar to patients with untreated IPF, with a high risk of further ILD progression and early mortality.

【 授权许可】

CC BY