Idiopathic pulmonary fibrosis cluster analysis highlights diagnostic delay and cardiovascular comorbidity association with outcome.

Idiopathic pulmonary fibrosis (IPF) prognosis is heterogeneous despite antifibrotic treatment. Cluster analysis has proven to be a useful tool in identifying interstitial lung disease phenotypes, which has yet to be performed in IPF. The aim of this study is to identify phenotypes of IPF with different prognoses and requirements. Observational retrospective study including 136 IPF patients receiving antifibrotic treatment between 2012 and 2018. Six patients were excluded due to follow-up in other centres. Cluster analysis of 30 variables was performed using approximate singular value-based tensor decomposition method and comparative statistical analysis. The cluster analysis identified three different groups of patients according to disease behaviour and clinical features, including mortality, lung transplant and progression-free survival time after 3-year follow-up. Cluster 1 (n=60) was significantly associated (p=0.02) with higher mortality. Diagnostic delay was the most relevant characteristic of this cluster, as 48% of patients had ≥2 years from first respiratory symptoms to antifibrotic treatment initiation. Cluster 2 (n=22) had the longest progression-free survival time and was correlated to subclinical patients evaluated in the context of incidental findings or familial screening. Cluster 3 (n=48) showed the highest percentage of disease progression without cluster 1 mortality, with metabolic syndrome and cardiovascular comorbidities as the main characteristics. This cluster analysis of IPF patients suggests that diagnostic and treatment delay are the most significant factors associated with mortality, while IPF progression was more related to metabolic syndrome and cardiovascular comorbidities.

Informations de copyright

Copyright ©The authors 2021.

Déclaration de conflit d'intérêts

Conflict of interest: J. Bordas-Martínez reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: R. Gavaldà reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: J.G. Shull reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: V. Vicens-Zygmunt reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: L. Planas-Cerezales reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: G. Bermudo-Peloche reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: S. Santos reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: N. Salord reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: C. Monasterio reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: M. Molina-Molina reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study. Conflict of interest: G. Suarez-Cuartin reports FIS-ISCIII grant PI18/00367 (cofunded by the European Regional Development Fund (ERDF)), Spanish Society of Pneumology and Thoracic Surgery (SEPAR) grants 631/2018 and 685/2018, Emerging ILD Group of SEPAR grant 005 (Boehringer–Roche), Pneumology Foundation of Catalonia grant 2019, Spanish Sleep Society grant 2019, institutional support of the CERCA Programme/Generalitat de Catalunya, and research support BRN-Fundació Ramon Pla Armengol from ISCIII grant CM20/00093 (cofunded by European Regional Development Fund), during the conduct of the study.