Patient Registries in Idiopathic Pulmonary Fibrosis

Daniel A Culver, Jürgen 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

Research output: Contribution to journalReview articlepeer-review

37 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)160-167
Number of pages8
JournalAmerican journal of respiratory and critical care medicine
Issue number2
StatePublished - Jul 15 2019

Bibliographical note

Funding Information:
Definition of abbreviations: AIPFR = Australian IPF Registry; BTS = British Thoracic Society; CARE-PF = Canadian Registry for Pulmonary Fibrosis; EMPIRE = European MultiPartner IPF Registry; eurIPFreg = European IPF Registry and Biobank; EXCITING = Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases; FIBRONET=IPF Italian observational study; HRCT=high-resolution computed tomography; ILD=interstitial lung disease; INDULGE IPF = Investigating Idiopathic Pulmonary Fibrosis in Greece; INSIGHTS-IPF = Investigating Significant Health Trends in Idiopathic Pulmonary Fibrosis; IPF = idiopathic pulmonary fibrosis; IPF-PRO/ILD-PRO=Idiopathic Pulmonary Fibrosis/Interstitial Lung Disease Prospective Outcomes; JIPS=Japanese Idiopathic Interstitial Pneumonias; PFF-PR=Pulmonary Fibrosis Foundation Patient Registry; PORTRAY=Idiopathic Pulmonary Fibrosis Registry China Study; PROOF=Prospective Observational Registry to Describe the Disease Course and Outcomes in Idiopathic Pulmonary Fibrosis; PROOF-NEXT=Prospective Observational Registry to Describe the Disease Course and Outcomes in Idiopathic Pulmonary Fibrosis Patients in a Real-World Clinical Setting: New and Extended Belgium-Luxembourg; REGIS = Romanian Registry for Interstitial Lung Diseases; TURK-UIP = Turkish Thoracic Society Usual Interstitial Pneumonia registry study; UIP = usual interstitial pneumonia.

Funding Information:
Acknowledgment: Writing support was provided by Elizabeth Ng, B.Sc., and Wendy Morris, M.Sc., of FleishmanHillard Fishburn, London, United Kingdom, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. Boehringer Ingelheim was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations.

Publisher Copyright:
Copyright © 2019 by the American Thoracic Society


  • Biomarkers
  • Interstitial lung diseases
  • Observational study
  • Pulmonary fibrosis

PubMed: MeSH publication types

  • Journal Article


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