title: Rationale for and design of the Idiopathic Pulmonary Fibrosis-PRospective Outcomes (IPF-PRO) registry.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by progressive loss of lung function. Its clinical course is variable but ultimately fatal. There is a need for a multicentre patient registry incorporating longitudinal clinical data and biological samples to improve understanding of the natural history of IPF and contemporary practice patterns.
  
  
  METHODS
  The Idiopathic Pulmonary Fibrosis-PRospective Outcomes (IPF-PRO) registry is a national IPF registry in the USA. This registry will enrol approximately 300 patients with newly diagnosed IPF over 2 years at approximately 14 tertiary pulmonary care sites. Participants will be followed for 3-5 years and will receive usual care, as defined by their physician. Clinical data from the year prior to diagnosis will be collected from medical record review on enrolment. Subsequently, data on diagnostic evaluations, pulmonary function tests, physical examinations, laboratory data and clinical events will be collected at routine clinical visits and via a call centre. Participants will complete patient-reported outcome questionnaires at enrolment and then at approximately 6-month intervals. Blood samples for cellular, genetic and transcriptomic analyses will be collected at the same intervals.
  
  
  RESULTS
  The first results from the IPF-PRO registry will be presented in 2015.
  
  
  CONCLUSIONS
  The IPF-PRO registry will improve understanding of the natural history of IPF, its impact on patients and current practice in the diagnosis and care of patients with IPF. The registry will establish a repository of biological samples from a well-characterised patient population for future research.
  
  
  BACKGROUND
  NCT01915511.
authors.affiliation: Department of Medicine , Duke University, Duke Clinical Research Institute , Durham, North Carolina , USA.
authors.forename: Emily C
authors.initials: EC
authors.lastname: O'Brien
authors.affiliation: Department of Medicine , Duke University, Duke Clinical Research Institute , Durham, North Carolina , USA.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Durheim
authors.affiliation: Biometrics and Data Management , Boehringer Ingelheim Pharmaceuticals Inc. , Ridgefield, Connecticut , USA.
authors.forename: Victoria
authors.initials: V
authors.lastname: Gamerman
authors.affiliation: Clinical Operations , Boehringer Ingelheim Pharmaceuticals Inc. , Ridgefield, Connecticut , USA.
authors.forename: Sandy
authors.initials: S
authors.lastname: Garfinkel
authors.affiliation: Department of Biostatistics and Bioinformatics , Duke Clinical Research Institute , Durham, North Carolina , USA.
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Anstrom
authors.affiliation: Department of Medicine , Duke University, Duke Clinical Research Institute , Durham, North Carolina , USA.
authors.forename: Scott M
authors.initials: SM
authors.lastname: Palmer
authors.affiliation: Clinical Development and Medical Affairs , Boehringer Ingelheim Pharmaceuticals Inc. , Ridgefield, Connecticut , USA.
authors.forename: Craig S
authors.initials: CS
authors.lastname: Conoscenti
keywords: Interstitial Fibrosis; Rare lung diseases
references.citation: N Engl J Med. 2014 May 29;370(22):2083-92
references.pmid: 24836312
references.citation: Respirology. 2014 Oct;19(7):1088-91
references.pmid: 25123691
references.citation: Respir Med. 2008 Nov;102(11):1545-55
references.pmid: 18662868
references.citation: Am J Respir Crit Care Med. 2007 Oct 1;176(7):636-43
references.pmid: 17585107
references.citation: Chest. 2011 Jul;140(1):221-229
references.pmid: 21729893
references.citation: Glob J Health Sci. 2013 Sep 16;5(6):131-41
references.pmid: 24171881
references.citation: Am J Respir Crit Care Med. 2011 Jul 1;184(1):92-9
references.pmid: 21474646
references.citation: Intern Med. 2007;46(18):1533-42
references.pmid: 17878639
references.citation: Lancet Respir Med. 2014 Jul;2(7):566-72
references.pmid: 24875841
references.citation: Am J Respir Crit Care Med. 2014 Apr 1;189(7):825-31
references.pmid: 24476390
references.citation: Chest. 2012 Aug;142(2):291-297
references.pmid: 22871754
references.citation: Clin Ther. 2000 Sep;22(9):1121-45
references.pmid: 11048909
references.citation: Med Care. 1996 Mar;34(3):220-33
references.pmid: 8628042
references.citation: Can Respir J. 2015 Nov 2;:
references.pmid: 26523546
references.citation: Transl Res. 2012 Apr;159(4):218-27
references.pmid: 22424426
references.citation: Eur Respir J. 2011 Feb;37(2):356-63
references.pmid: 20595144
references.citation: Eur Respir J. 2011 Apr;37(4):747-8
references.pmid: 21454892
references.citation: Health Qual Life Outcomes. 2014 Aug 20;12:124
references.pmid: 25138056
references.citation: Nat Genet. 2013 Jun;45(6):613-20
references.pmid: 23583980
references.citation: Am J Respir Crit Care Med. 2011 Aug 15;184(4):459-66
references.pmid: 21616999
references.citation: Am J Respir Crit Care Med. 2011 Feb 15;183(4):431-40
references.pmid: 20935110
references.citation: Ann Med. 2001 Jul;33(5):337-43
references.pmid: 11491192
references.citation: Respir Med. 2007 Jun;101(6):1350-4
references.pmid: 17107778
references.citation: N Engl J Med. 2011 Sep 22;365(12):1079-87
references.pmid: 21992121
references.citation: COPD. 2005 Mar;2(1):75-9
references.pmid: 17136966
references.citation: Respir Res. 2013 Jul 13;14:73
references.pmid: 23848435
references.citation: N Engl J Med. 2014 May 29;370(22):2071-82
references.pmid: 24836310
references.citation: Eur Respir J. 2012 Jul;40(1):101-9
references.pmid: 22241745
references.citation: Eur Respir Rev. 2012 Dec 1;21(126):355-61
references.pmid: 23204124
references.citation: Respir Med. 2010 Feb;104(2):296-304
references.pmid: 19815403
references.citation: Eur Respir J. 2013 Dec;42(6):1622-32
references.pmid: 23682110
references.citation: Ann Intern Med. 2012 May 15;156(10):684-91
references.pmid: 22586007
references.citation: Multivariate Behav Res. 2011 May;46(3):399-424
references.pmid: 21818162
references.citation: Chron Respir Dis. 2011;8(4):225-31
references.pmid: 21856780
references.citation: Eur Respir J. 2008 Jul;32(1):170-4
references.pmid: 18256070
references.citation: Am J Respir Crit Care Med. 2011 Oct 1;184(7):842-7
references.pmid: 21719755
references.citation: Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824
references.pmid: 21471066
references.citation: N Engl J Med. 2014 May 29;370(22):2093-101
references.pmid: 24836309
references.citation: BMJ Open Respir Res. 2014 Jan 03;1(1):e000010
references.pmid: 25478169
references.citation: Health Qual Life Outcomes. 2005 Oct 07;3:61
references.pmid: 16212668
references.citation: Am J Physiol Lung Cell Mol Physiol. 2014 Nov 1;307(9):L681-91
references.pmid: 25260757
references.citation: Expert Rev Respir Med. 2014 Apr;8(2):173-8
references.pmid: 24450476
references.citation: JAMA. 2013 Jun 5;309(21):2232-9
references.pmid: 23695349
references.citation: N Engl J Med. 2011 Apr 21;364(16):1503-12
references.pmid: 21506741
references.citation: Ann Am Thorac Soc. 2014 Oct;11(8):1171-5
references.pmid: 25167209
references.citation: Lancet Respir Med. 2013 Jun;1(4):309-317
references.pmid: 24429156
references.citation: Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7
references.pmid: 15968010