Kärkkäinen, M., Kettunen, H., Nurmi, H. et al. Comparison of disease progression subgroups in idiopathic pulmonary fibrosis. BMC Pulm Med 19, 228 (2019). https://doi.org/10.1186/s12890-019-0996-2
Comparison of disease progression subgroups in idiopathic pulmonary fibrosis
|Author:||Kärkkäinen, Miia1,2,3; Kettunen, Hannu-Pekka4; Nurmi, Hanna1,5;|
1Division of Respiratory Medicine, Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences, University of Eastern Finland, P.O. Box 1627, 70211, Kuopio, Finland
2Kuopio City Home Care, Rehabilitation and Medical Services for Elderly, Tulliportinkatu 37E, 70100, Kuopio, Finland
3Kuopio University Hospital, P.O. Box 100, Puijonlaaksontie 2, 70210, Kuopio, Finland
4Department of Clinical Radiology, Kuopio University Hospital, P.O. Box 100, 70029 KYS, Kuopio, Finland
5Center of Medicine and Clinical Research, Division of Respiratory Medicine, Kuopio University Hospital, P.O. Box 100, 70029 KYS, Kuopio, Finland
6Science Services Center, Kuopio University Hospital, P.O. Box 100, 70029 KYS, Kuopio, Finland
7Respiratory Medicine, Research Unit of Internal Medicine, University of Oulu and Medical Research Center Oulu, Oulu University Hospital, P.O. Box 20, 90029, Oulu, Finland
|Online Access:||PDF Full Text (PDF, 0.6 MB)|
|Persistent link:|| http://urn.fi/urn:nbn:fi-fe202003309591
|Publish Date:|| 2020-03-30
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial pneumonia with an unpredictable course. The aims of this study were to retrospectively re-evaluate a cohort of patients with IPF according to the 2011 international IPF guidelines and 1) to characterize the subgroups of patients when classified according to their observed survival times and 2) to evaluate whether Composite Physiologic Index (CPI), Gender-Age-Physiology (GAP) Index or clinical variables could predict mortality.
Methods: Retrospective data was collected and patients were classified into subgroups according to their observed lifespans. Differences in clinical variables, CPI and GAP stages as well as in comorbidities were investigated between the subgroups. Predictors of mortality were identified by COX proportional hazard analyses.
Results: A total of 132 patients were included in this study. The disease course was rapid (≤ 2 years) in 30.0%, moderate (2—5 years) in 28.0% and slow (≥ 5 years) in 29.0% of the patients. Pulmonary function tests (PFT) and CPI at baseline differentiated significantly between the rapid disease course group and those patients with longer survival times. However, the predictive accuracy of the investigated clinical variables was mainly less than 0.80. The proportions of patients with comorbidities did not differ between the subgroups, but more patients with a rapid disease course were diagnosed with heart failure after the diagnosis of IPF. Most patients with a rapid disease course were categorized in GAP stages I and II, but all patients in GAP stage III had a rapid disease course. The best predictive multivariable model included age, gender and CPI. GAP staging had slightly better accuracy (0.67) than CPI (0.64) in predicting 2-year mortality.
Conclusions: Although the patients with a rapid disease course could be differentiated at baseline in terms of PFT and CPI, the predictive accuracy of any single clinical variable as well as CPI and GAP remained low. GAP staging was unable to identify the majority of patients with a rapid disease progression. It is challenging to predict disease progression and mortality in IPF even with risk prediction models.
BMC pulmonary medicine
|Type of Publication:||
A1 Journal article – refereed
|Field of Science:||
3121 General medicine, internal medicine and other clinical medicine
The funding sources did not have any role in the design of the study or collection, analysis, and interpretation of data or in writing the manuscript. M. K. has received grants for scientific work from The Foundation of The Finnish Anti-Tuberculosis Association, The Organization for Respiratory Health in Finland, Väinö and Laina Kivi Foundation, The Kuopio Region Respiratory Foundation, Jalmari and Rauha Ahokas Foundation and from the Center of Medicine and Clinical Research in Kuopio University Hospital; H.N. has received grants for scientific work from The Foundation of The Finnish Anti-Tuberculosis Association, The Organization for Respiratory Health in Finland, Väinö and Laina Kivi Foundation, the North Savo Regional Fund of the Finnish Cultural Foundation, The Kuopio Region Respiratory Foundation, Jalmari and Rauha Ahokas Foundation and the state subsidy of the Kuopio University Hospital; M.P. received grants for scientific work from Research Director of Kuopio University Hospital, Foundation of the Finnish Anti-Tuberculosis Association, The Kuopio Region Respiratory Foundation and the Jalmari and Rauha Ahokas Foundation; R. K. has received grants for scientific work and for the research team from the state subsidy of Kuopio University Hospital, The Foundation of The Finnish Anti-Tuberculosis Association and The Kuopio Region Respiratory Foundation.
© The Authors 2019. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.