Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: An international case-cohort study

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (\u3baw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the Cindex. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (\u3baw=0.65, IQR 0.53-0.72, p20 years of experience (C-index=0.72, IQR 0.0-0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70-0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72-0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts

Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial

Individuals with IPF who follow a MUFA diet report a lower incidence of pirfenidone gastrointestinal adverse events than those that follow a SFA diet, which could explain the different prevalence in GI pirfenidone AEs reported by countries in IPF cohorts https://bit.ly/3LuzAUJ Idiopathic pulmonary fibrosis (IPF) is a chronic and lethal interstitial lung disease (ILD) [1, 2]. Antifibrotic medications such as pirfenidone have been a turning point in the management of IPF, slowing of disease progression and improving survival [1–5]. eng research projects or scientific advice from Esteve-Teijin, Roche, Boehringer Ingelheim and Chiesi. V. Vicens-Zygmunt received fees for scientific advice from Boehringer Ingelheim. P. Rivera-Ortega declares speaker and consultation fees from Boehringer Ingelheim and Hoffmann-La Roche, and fees received for research projects from Boehringer Ingelheim, Hoffmann-La Roche, CSL Behring, FibroGen, Vicore Pharma AB, Gilead Sciences and Galecto; all research fees were paid to her institution. F. Bonella declares speaker and consultation fees from Boehringer Ingelheim, Hoffman La Roche and Fibrogene, outside the submitted work. E. Renzoni reports grants, lecture fees and advisory board fees from Boehringer Ingelheim, lecture fees from Roche and Chiesi, research grants from Raynaud's and Scleroderma, and support for attending meetings from Boehringer Ingelheim, outside the submitted work; all grants and fees were paid to her institution. A-M. Russell declares speaker and consultation fees from Boehringer Ingelheim and Hoffman-La Roche. T.M. Maher reports consultancy fees from AstraZeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, Galapagos, Galecto, GlaxoSmithKline, IQVIA, Pliant, Respivant Sciences, Roche/Genentech, Theravance Biopharma and Veracyte, and fees for presentations from Boehringer Ingelheim and Roche/Genentech. G. Suarez-Cuartin has received grants from Grifols, travel grants from Chiesi, Menarini and Boehringer Ingelheim, a speaker fee from Insmed, and advisory board fees from Insmed Inc. and Zambon. M. Wijsenbeek has received grants from Boehringer Ingelheim, The Netherlands Organisation for Health Research and Development, The Dutch Lung Foundation, Sarcoidose.nl and The Dutch Pulmonary Society, consulting fees from Boehringer Ingelheim, Galapagos, Bristol Myers Squibb, Galecto, Respivant, NeRRe Therapeutics, Horizon Therapeutics, PureTech health, Kinevant Sciences, Molecure and CLS Behring, speaker fees from Boehringer Ingelheim, Hoffman-La Roche, Novartis and CLS Behring, support for attending meetings from Boehringer Ingelheim, Galapagos and Hoffman-La Roche, and has participated in advisory boards of different patient associations (unpaid); all grants and fees were paid to her institution. C. Vancheri served on advisory committees of InterMune, Roche, AstraZeneca, Sanofi, Insmed, Zambon and Boehringer Ingelheim, and received lecture fees and nongovernmental research support from InterMune, Roche, Boehringer Ingelheim, Novartis, Chiesi, Menarini, AstraZeneca, GSK, Sanofi and Insmed. The rest of the authors have no relevant relationships to disclose.Published version, accepted version (12 month embargo), submitted versionThe article is available via Open Access. Click on the 'Additional link' above to access the full-text

Acute exacerbation of idiopathic pulmonary fibrosis: International survey and call for harmonisation

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. 509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%). Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed. Copyright © ERS 202

Acute exacerbation of idiopathic pulmonary fibrosis

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. 509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%). Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed