Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry (IPF/ILD-PRO)

• ClinicalTrials.gov Identifier: NCT01915511
• Recruitment Status: Recruiting
• First Posted: August 5, 2013
• Last Update Posted: April 10, 2024
• Sponsor: Duke University
• Collaborator: Boehringer Ingelheim
• Information provided by (Responsible Party): Duke University

Tracking Information

• First Submitted Date: July 31, 2013
• First Posted Date: August 5, 2013
• Last Update Posted Date: April 10, 2024
• Study Start Date: June 2014
• Estimated Primary Completion Date: January 2028 (Final data collection date for primary outcome measure)

Current Primary Outcome Measures (submitted: February 6, 2019)

• Data on natural history of IPF & non-IPF chronic fibrosing ILD [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Characterize and describe the natural history of patients with a recent confirmed diagnosis of IPF, with emphasis on demographics, co-morbidities, medications, and risks for disease progression or death.
• Data on current practice patterns for diagnosis of IPF & non-IPF chronic fibrosing ILD [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Understand the current practice patterns for diagnosis of IPF & non-IPF chronic fibrosing ILD
• Data on impact of IPF & non- IPF chronic fibrosing ILD on patient quality of life. [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Describe the impact of IPF & non- IPF chronic fibrosing ILD on patient quality-of-life (QOL).
• Blood samples for future research. [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Collect longitudinal bio-samples for future research on disease presentation, progression, and subject response to clinical interventions.
• HRCT images for future research (for non-IPF chronic fibrosing ILD) [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Collect longitudinal HRCT images for future research

Original Primary Outcome Measures (submitted: August 1, 2013)

• Data on natural history of IPF. [ Time Frame: Up to 5 years ]
  Characterize and describe the natural history of patients with a recent confirmed diagnosis of IPF, with emphasis on demographics, co-morbidities, medications, and risks for disease progression or death.
• Data on current practice patterns for diagnosis of IPF. [ Time Frame: Up to 5 years. ]
  Understand the current practice patterns for diagnosis of IPF.
• Data on impact of IPF on patient quality of life. [ Time Frame: Up to 5 years. ]
  Describe the impact of IPF on patient quality-of-life (QOL).
• Blood samples for future research. [ Time Frame: Up to 5 years. ]
  Collect longitudinal bio-samples for future research on disease presentation, progression, and subject response to clinical interventions.

Current Secondary Outcome Measures (submitted: April 28, 2016)

• Data on management practices compared to existing guidelines. [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Compare disease-specific management practices with existing guidelines.
• Data on center-specific practices on outcomes. [ Time Frame: End of Study (3 years after last patient will be enrolled) ]
  Determine the influence of center-specific practices on patient outcomes.

Original Secondary Outcome Measures (submitted: August 1, 2013)

• Data on clinical trial participation and management practices. [ Time Frame: Up to 5 years ]
  Identify factors associated with participation in interventional clinical trials, as part of a patient's care plan.
• Data on management practices compared to existing guidelines. [ Time Frame: Up to 5 years. ]
  Compare disease-specific management practices with existing guidelines.
• Data on center-specific practices on outcomes. [ Time Frame: Up to 5 years. ]
  Determine the influence of center-specific practices on patient outcomes.

• Current Other Pre-specified Outcome Measures: Not Provided
• Original Other Pre-specified Outcome Measures: Not Provided

Descriptive Information

• Brief Title: Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry
• Official Title: Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) and Chronic Fibrosing Interstitial Lung Disease With Progressive Phenotype Prospective Outcomes (IPF-PRO/ILD-PRO) Registry
• Brief Summary: This registry will collect data on the strategies used to achieve a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) and Chronic Fibrosing Interstitial Lung Disease with Progressive Phenotype (ILD) and the treatment and management efforts applied throughout study follow-up, clinical outcome events and patient reported outcome data. Blood samples will be collected periodically throughout the study for use in future research efforts. For participants with non-IPF, chronic fibrosing ILD with progressive phenotype, HRCT images will be collected throughout the study for use in future research efforts.
• Detailed Description: Not Provided
• Study Type: Observational [Patient Registry]
• Study Design: Observational Model: Cohort; Time Perspective: Prospective
• Target Follow-Up Duration: 3 Years

Biospecimen

Retention:   Samples With DNA

Description:

Whole blood for DNA collected at enrollment. Plasma, serum, and RNA samples collected at enrollment and approximate 6-month intervals throughout study follow-up.

• Sampling Method: Non-Probability Sample
• Study Population: Subjects with a new diagnosis of IPF or a non- IPF chronic fibrosing ILD established at the time of enrollment in the registry are eligible for participation in the IPF-PRO/ILD-PRO registry if the participant meets the selection criteria.
• Condition: Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease
• Intervention: Not Provided

Study Groups/Cohorts

• Subjects with a new IPF diagnosis
  Subjects with a new diagnosis of IPF established at the time of enrollment in the registry
• Subjects with a non-IPF ILD diagnosis
  Subjects with a diagnosis of a non-IPF ILD of any duration, including, but not limited to Idiopathic Non-Specific Interstitial Pneumonia (iNSIP), Unclassifiable Idiopathic Interstitial Pneumonias (IIPs), Interstitial Pneumonia with Autoimmune Features (IPAF), Autoimmune ILDs such as Rheumatoid Arthritis (RA-ILD) and Systemic Sclerosis (SSc-ILD), Chronic Hypersensitivity Pneumonitis (HP), Sarcoidosis or Exposure-related ILDs such as asbestosis with progressive phenotype

Publications *

• Swaminathan AC, Hellkamp AS, Neely ML, Bender S, Paoletti L, White ES, Palmer SM, Whelan TPM, Dilling DF; Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry investigators. Disparities in Lung Transplant among Patients with Idiopathic Pulmonary Fibrosis: An Analysis of the IPF-PRO Registry. Ann Am Thorac Soc. 2022 Jun;19(6):981-990. doi: 10.1513/AnnalsATS.202105-589OC.
• Kim HJ, Snyder LD, Neely ML, Hellkamp AS, Hotchkin DL, Morrison LD, Bender S, Leonard TB, Culver DA; IPF-PRO Registry investigators. Clinical Outcomes of Patients with Combined Idiopathic Pulmonary Fibrosis and Emphysema in the IPF-PRO Registry. Lung. 2022 Feb;200(1):21-29. doi: 10.1007/s00408-021-00506-x. Epub 2022 Jan 7.
• de Andrade JA, Kulkarni T, Neely ML, Hellkamp AS, Case AH, Culver DA, Guntupalli K, Bender S, Conoscenti CS, Snyder LD; IPF-PRO Registry Investigators. Associations between resources and practices of ILD centers and outcomes in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO Registry. Respir Res. 2022 Jan 7;23(1):3. doi: 10.1186/s12931-021-01921-7.
• Kim HJ, Snyder LD, Adegunsoye A, Neely ML, Bender S, White ES, Conoscenti CS, Strek ME; IPF-PRO Registry Investigators. Hospitalizations in patients with idiopathic pulmonary fibrosis. Respir Res. 2021 Sep 30;22(1):257. doi: 10.1186/s12931-021-01851-4.
• Salisbury ML, Conoscenti CS, Culver DA, Yow E, Neely ML, Bender S, Hartmann N, Palmer SM, Leonard TB; IPF-PRO Registry principal investigators as follows. Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry. Ann Am Thorac Soc. 2020 Nov;17(11):1413-1423. doi: 10.1513/AnnalsATS.201912-880OC.
• Fan Y, Bender SD, Conoscenti CS, Davidson-Ray L, Cowper PA, Palmer SM, de Andrade JA; IPF-PRO Registry Investigators. Hospital-Based Resource Use and Costs Among Patients With Idiopathic Pulmonary Fibrosis Enrolled in the Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry. Chest. 2020 Jun;157(6):1522-1530. doi: 10.1016/j.chest.2019.12.041. Epub 2020 Jan 29.
• O'Brien EC, Hellkamp AS, Neely ML, Swaminathan A, Bender S, Snyder LD, Culver DA, Conoscenti CS, Todd JL, Palmer SM, Leonard TB; IPF-PRO Registry investigators. Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry. Chest. 2020 May;157(5):1188-1198. doi: 10.1016/j.chest.2019.11.042. Epub 2020 Jan 15.
• Todd JL, Neely ML, Overton R, Durham K, Gulati M, Huang H, Roman J, Newby LK, Flaherty KR, Vinisko R, Liu Y, Roy J, Schmid R, Strobel B, Hesslinger C, Leonard TB, Noth I, Belperio JA, Palmer SM; IPF-PRO Registry investigators. Peripheral blood proteomic profiling of idiopathic pulmonary fibrosis biomarkers in the multicentre IPF-PRO Registry. Respir Res. 2019 Oct 22;20(1):227. doi: 10.1186/s12931-019-1190-z.
• Snyder L, Neely ML, Hellkamp AS, O'Brien E, de Andrade J, Conoscenti CS, Leonard T, Bender S, Gulati M, Culver DA, Kaner RJ, Palmer S, Kim HJ; IPF-PRO Registry investigators. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res. 2019 May 30;20(1):105. doi: 10.1186/s12931-019-1043-9.
• O'Brien EC, Durheim MT, Gamerman V, Garfinkel S, Anstrom KJ, Palmer SM, Conoscenti CS. Rationale for and design of the Idiopathic Pulmonary Fibrosis-PRospective Outcomes (IPF-PRO) registry. BMJ Open Respir Res. 2016 Jan 11;3(1):e000108. doi: 10.1136/bmjresp-2015-000108. eCollection 2016.

Recruitment Information

• Recruitment Status: Recruiting
• Estimated Enrollment (submitted: November 2, 2018): 2000
• Original Estimated Enrollment (submitted: August 1, 2013): 300
• Estimated Study Completion Date: January 2028
• Estimated Primary Completion Date: January 2028 (Final data collection date for primary outcome measure)

Eligibility Criteria

Inclusion Criteria:

• Willing and able to provide informed consent
• Established a new diagnosis of IPF by the enrolling subspecialty center (as defined by ATS/ERS/JRS/ALAT criteria)
• Age 30 years or older, or
• Diagnosis of a non-IPF ILD of any duration, including, but not limited to Idiopathic Non-Specific Interstitial, Pneumonia (iNSIP), Unclassifiable Idiopathic Interstitial Pneumonias (IIPs), Interstitial Pneumonia with Autoimmune Features (IPAF), Autoimmune ILDs such as Rheumatoid Arthritis (RA-ILD) and Systemic Sclerosis (SSc-ILD), Chronic Hypersensitivity Pneumonitis (HP), Sarcoidosis or Exposure-related ILDs such as asbestosis with progressive phenotype

Exclusion Criteria:

• Malignancy, treated or untreated, other than skin or early stage prostate cancer, within the past 5 years
• Currently listed for lung transplantation at the time of enrollment
• Currently enrolled in a clinical trial at the time of enrollment in this registry

Sex/Gender

• Sexes Eligible for Study: All

• Ages: 30 Years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Contacts

Contact: Rosalia Blanco; 919-660-0890; rosalia.blanco@duke.edu

• Listed Location Countries: Puerto Rico, United States

Administrative Information

• NCT Number: NCT01915511
• Other Study ID Numbers: Pro00046131; 1199.174 ( Other Identifier: DCRI )
• Has Data Monitoring Committee: No
• U.S. FDA-regulated Product: Not Provided
• IPD Sharing Statement: Not Provided
• Current Responsible Party: Duke University
• Original Responsible Party: Same as current
• Current Study Sponsor: Duke University
• Original Study Sponsor: Same as current
• Collaborators: Boehringer Ingelheim

Investigators

• Principal Investigator: Scott Palmer, MD; Duke Clinical Research Institute, Duke University

• PRS Account: Duke University
• Verification Date: April 2024