Patient Registries in Idiopathic Pulmonary Fibrosis

Overview

Journal Am J Respir Crit Care Med

Specialty Critical Care

Date 2019 Apr 30

PMID 31034241

Citations 30

Authors

Daniel A Culver

Jurgen Behr

John A Belperio

Tamera J Corte

Joao A de Andrade

Kevin R Flaherty

Mridu Gulati

Tristan J Huie

Lisa H Lancaster

Jesse Roman

Christopher J Ryerson

Hyun J Kim

Affiliations

Soon will be listed here.

Abstract

Over the past decade, several large registries of patients with idiopathic pulmonary fibrosis (IPF) have been established. These registries are collecting a wealth of longitudinal data on thousands of patients with this rare disease. The data collected in these registries will be complementary to data collected in clinical trials because the patient populations studied in registries have a broader spectrum of disease severity and comorbidities and can be followed for a longer period of time. Maintaining the quality and completeness of registry databases presents administrative and resourcing challenges, but it is important to ensuring the robustness of the analyses. Data from patient registries have already helped improve understanding of the clinical characteristics of patients with IPF, the impact that the disease has on their quality of life and survival, and current practices in diagnosis and management. In the future, analyses of biospecimens linked to detailed patient profiles will provide the opportunity to identify biomarkers linked to disease progression, facilitating the development of precision medicine approaches for prognosis and therapy in patients with IPF.

Citing Articles

Idiopathic pulmonary fibrosis in the UK: findings from the British Thoracic Society UK Idiopathic Pulmonary Fibrosis Registry.

Fahim A, Loughenbury M, Stewart I, Agnew S, Almond H, Casimo L BMJ Open Respir Res. 2025; 12(1).

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Design and baseline characteristics of the ILD-PRO registry in patients with progressive pulmonary fibrosis.

Lobo L, Liu Y, Li P, Ramaswamy M, Swaminathan A, Veeraraghavan S BMC Pulm Med. 2024; 24(1):468.

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A data set for the design and implementation of the upper limb disability registry.

Moulaei K, Sheikhtaheri A, Haghdoost A, Nezhadd M, Bahaadinbeigy K J Educ Health Promot. 2023; 12:130.

PMID: 37397108 PMC: 10312779. DOI: 10.4103/jehp.jehp_721_22.

Economic Burden of Idiopathic Pulmonary Fibrosis in Spain: A Prospective Real-World Data Study (OASIS Study).

Rodriguez-Nieto M, Cano-Jimenez E, Romero Ortiz A, Villar A, Morros M, Ramon A Pharmacoeconomics. 2023; 41(8):999-1010.

PMID: 37249823 PMC: 10322761. DOI: 10.1007/s40273-023-01278-3.

Russian Registry of Idiopathic Pulmonary Fibrosis: Clinical Features, Treatment Management, and Outcomes.

Chikina S, Cherniak A, Merzhoeva Z, Tyurin I, Trushenko N, Proshkina A Life (Basel). 2023; 13(2).

PMID: 36836792 PMC: 9964580. DOI: 10.3390/life13020435.

References

Collard H, Tino G, Noble P, Shreve M, Michaels M, Carlson B . Patient experiences with pulmonary fibrosis. Respir Med. 2006; 101(6):1350-4. DOI: 10.1016/j.rmed.2006.10.002. View

Wuyts W, Dahlqvist C, Slabbynck H, Schlesser M, Gusbin N, Compere C . Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry. BMJ Open Respir Res. 2018; 5(1):e000331. PMC: 6267319. DOI: 10.1136/bmjresp-2018-000331. View

Topol E . High-performance medicine: the convergence of human and artificial intelligence. Nat Med. 2019; 25(1):44-56. DOI: 10.1038/s41591-018-0300-7. View

Widdows H, Cordell S . The ethics of biobanking: key issues and controversies. Health Care Anal. 2011; 19(3):207-19. DOI: 10.1007/s10728-011-0184-x. View

Zurkova M, Kriegova E, Kolek V, Lostakova V, Sterclova M, Bartos V . Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. Respir Res. 2019; 20(1):16. PMC: 6341650. DOI: 10.1186/s12931-019-0977-2. View

Raghu G . Idiopathic pulmonary fibrosis: lessons from clinical trials over the past 25 years. Eur Respir J. 2017; 50(4). DOI: 10.1183/13993003.01209-2017. View

Collard H, Chen S, Yeh W, Li Q, Lee Y, Wang A . Health care utilization and costs of idiopathic pulmonary fibrosis in U.S. Medicare beneficiaries aged 65 years and older. Ann Am Thorac Soc. 2015; 12(7):981-7. DOI: 10.1513/AnnalsATS.201412-553OC. View

Bouros D, Daniil Z, Papakosta D, Antoniou K, Markopoulou K, Kolilekas L . Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF). Respiration. 2018; 96(1):41-47. DOI: 10.1159/000487244. View

Jo H, Glaspole I, Grainge C, Goh N, Hopkins P, Moodley Y . Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017; 49(2). DOI: 10.1183/13993003.01592-2016. View

10.

King Jr T, Bradford W, Castro-Bernardini S, Fagan E, Glaspole I, Glassberg M . A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370(22):2083-92. DOI: 10.1056/NEJMoa1402582. View

11.

Graham J . Missing data analysis: making it work in the real world. Annu Rev Psychol. 2008; 60:549-76. DOI: 10.1146/annurev.psych.58.110405.085530. View

12.

Cottin V . Current approaches to the diagnosis and treatment of idiopathic pulmonary fibrosis in Europe: the AIR survey. Eur Respir Rev. 2014; 23(132):225-30. PMC: 9487571. DOI: 10.1183/09059180.00001914. View

13.

Tarca A, Carey V, Chen X, Romero R, Draghici S . Machine learning and its applications to biology. PLoS Comput Biol. 2007; 3(6):e116. PMC: 1904382. DOI: 10.1371/journal.pcbi.0030116. View

14.

Rajala K, Lehto J, Sutinen E, Kautiainen H, Myllarniemi M, Saarto T . Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life. BMC Pulm Med. 2018; 18(1):172. PMC: 6247520. DOI: 10.1186/s12890-018-0738-x. View

15.

Spitzer M, Nolan G . Mass Cytometry: Single Cells, Many Features. Cell. 2016; 165(4):780-91. PMC: 4860251. DOI: 10.1016/j.cell.2016.04.019. View

16.

Wang R, Maron B, Loscalzo J . Systems medicine: evolution of systems biology from bench to bedside. Wiley Interdiscip Rev Syst Biol Med. 2015; 7(4):141-61. PMC: 4457580. DOI: 10.1002/wsbm.1297. View

17.

Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S . The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018; 19(1):141. PMC: 6064050. DOI: 10.1186/s12931-018-0845-5. View

18.

Karsdal M, Krarup H, Sand J, Christensen P, Gerstoft J, Leeming D . Review article: the efficacy of biomarkers in chronic fibroproliferative diseases - early diagnosis and prognosis, with liver fibrosis as an exemplar. Aliment Pharmacol Ther. 2014; 40(3):233-49. DOI: 10.1111/apt.12820. View

19.

Yu G, Ibarra G, Kaminski N . Fibrosis: Lessons from OMICS analyses of the human lung. Matrix Biol. 2018; 68-69:422-434. PMC: 6015529. DOI: 10.1016/j.matbio.2018.03.014. View

20.

Singh S, Collins B, Sharma B, Joshi J, Talwar D, Katiyar S . Interstitial Lung Disease in India. Results of a Prospective Registry. Am J Respir Crit Care Med. 2016; 195(6):801-813. DOI: 10.1164/rccm.201607-1484OC. View