Clinical trial NCT01915511: Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry
Official title: Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) and Interstitial Lung Disease Prospective Outcomes (IPF-PRO/ILD-PRO) Registry
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease
Keywords: Idiopathic pulmonary fibrosis
Pulmonary fibrosis
IPF
Registry
1199.174
Interstitial Lung Disease
ILD
Interstitial Lung Disease with Progressive Phenotype
Lead sponsor: Duke University
Sponsor class: OTHER
Collaborators: name: Boehringer Ingelheim
First posted: 2013-08-05
Enrollment: 3000
Sex: ALL
Minimum age (years): 30
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
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.

Brief summary: The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry started recruiting in 2014 with the objective of studying Idiopathic Pulmonary Fibrosis. In 2018, the registry expanded to include recruitment of participants with other chronic fibrosing interstitial lung diseases (ILDs) with progressive phenotype also referred to as progressive fibrosing interstitial lung diseases in the Chronic Fibrosis Interstitial Lung Disease with Progressive Phenotype (ILD-PRO) Registry. When the third phase of the registry begins, the IPF-PRO registry will enroll additional patients with idiopathic pulmonary fibrosis. This IPF-PRO registry is a prospective registry that will collect information regarding the natural history, health care interactions, participant reported questionnaire data to assess quality of life, and the methods of treatment of participants with a diagnosis of idiopathic pulmonary fibrosis (IPF) or of another chronic fibrosing interstitial lung disease (ILD) with progressive phenotype established at the enrolling centers. In addition, blood samples and chest image studies will be collected and banked for future research projects.

Detailed description: This registry originally enrolled a total of 1002 participants newly diagnosed with IPF and continues to enroll patients with other chronic fibrosing ILDs with newly identified progressive phenotype to reach an enrollment of 1000 patients. Participants will be enrolled in three phases, (IPF-PRO and ILD-PRO) over a span of 8 years at approximately 50 sites experienced in the diagnosis and treatment of ILD in the United States. Enrollment for the original IPF cohort started in 2014 and ended in October 2018, with 1002 total participants enrolled. In the third phase of the registry new enrollment for patients with IPF will restart in 2023-2024 with the plan to enroll up to 1000 new IPF patients, for a total IPF enrollment of 2000. Enrollment for other chronic fibrosing ILDs with newly identified progressive phenotype cohort was initiated in February 2019 and will end when enrollment reaches 1000 participants with the potential of enrolling another 1000 participants with other chronic fibrosing ILDs with newly identified without a progressive phenotype. Data and samples will be collected from participants for approximately 5 years for the IPF cohort. For the chronic fibrosing ILD with progressive phenotype cohort, data and samples will be collected for a minimum of 3 years, up to approximately 5 years. Participant management and treatment decisions will be determined by participants and their health care professionals.

Eligibility criteria: Inclusion Criteria:

* Willing and able to provide informed consent
* Established a new diagnosis (within 12 months) of IPF by the enrolling center.
* Age 21 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 during the last 24 months by the enrolling center that meets the following criteria:

  * Chronic fibrosing ILD as defined by reticular abnormality with traction bronchiectasis with or without honeycombing confirmed by chest HRCT scan and/or lung biopsy.
  * Progressive phenotype as defined by fulfilling at least one of the criteria below of fibrotic changes (progression set point) within the last 24 months regardless of treatment considered appropriate in individual ILDs (8):

    * decline in FVC % predicted (% pred) based on ≥10% relative decline
    * decline in FVC % pred based on ≥5 - \<10% relative decline in FVC combined with worsening of respiratory symptoms as assessed by the site investigator
    * decline in FVC % pred based on ≥5 - \<10% relative decline in FVC combined with increasing extent of fibrotic changes on chest imaging (HRCT scan) as assessed by the site investigator
    * decline in DLCO % pred based on≥ 10% relative decline
    * worsening of respiratory symptoms as well as increasing extent of fibrotic changes on chest imaging (HRCT scan) as assessed by the site investigator independent of FVC change.

The relative decline for FVC % predicted is calculated using the formula:

Relative Decline= (FVC % Pred (Reference)-FVC % Pred (Screening))/(FVC % Pred (Reference))×100%, where FVC % Pred (Reference) is the greatest measurement of FVC % predicted in the 24 months prior to screening and FVC % Pred (Screening) is the measurement of FVC % predicted at screening.

The relative decline for DLCO % predicted is calculated using the formula:

Relative Decline= (DLCO % Pred (Reference)-DLCO % Pred (Screening))/(DLCO % Pred (Reference))×100%, Where DLCO % Pred (Reference) is the greatest measurement of DLCO % Pred in the 24 months prior to screening and DLCO % Pred (Screening) is the measurement of DLCO % Pred at screening

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 an interventional clinical trial at the time of enrollment in this registry
* For the additional IPF cohort of 1000 individuals, previous enrollment in this registry.

Primary outcomes:
measure: Data on natural history of IPF & non-IPF chronic fibrosing ILD
timeFrame: End of Study (3 years after last patient will be enrolled)
measure: Data on current practice patterns for diagnosis of IPF & non-IPF chronic fibrosing ILD
timeFrame: End of Study (3 years after last patient will be enrolled)
measure: Data on impact of IPF & non- IPF chronic fibrosing ILD on patient quality of life.
timeFrame: End of Study (3 years after last patient will be enrolled)
measure: Blood samples for future research.
timeFrame: End of Study (3 years after last patient will be enrolled)
measure: HRCT images for future research (for non-IPF chronic fibrosing ILD, and new IPF patients cohort)
timeFrame: End of Study (3 years after last patient will be enrolled)
Secondary outcomes:
measure: Data on management practices compared to existing guidelines.
timeFrame: End of Study (3 years after last patient will be enrolled)
measure: Data on center-specific practices on outcomes.
timeFrame: End of Study (3 years after last patient will be enrolled)
Other outcomes:
Locations:
city: Birmingham
country: United States
facility: University of Alabama - Birmingham
state: Alabama
city: Los Angeles
country: United States
facility: University of California - Los Angeles
state: California
city: Los Angeles
country: United States
facility: University of Southern California
state: California
city: Stanford
country: United States
facility: Stanford University
state: California
city: New Haven
country: United States
facility: Yale University
state: Connecticut
city: Gainesville
country: United States
facility: University of Florida
state: Florida
city: Tampa
country: United States
facility: University of South Florida
state: Florida
city: Atlanta
country: United States
facility: Emory University
state: Georgia
city: Austell
country: United States
facility: Piedmont Healthcare
state: Georgia
city: Chicago
country: United States
facility: University of Chicago
state: Illinois
city: Evanston
country: United States
facility: Northwestern University
state: Illinois
city: Maywood
country: United States
facility: Loyola University Health System
state: Illinois
city: Kansas City
country: United States
facility: University of Kansas
state: Kansas
city: New Orleans
country: United States
facility: Tulane University
state: Louisiana
city: Baltimore
country: United States
facility: University from Virginia
state: Maryland
city: Ann Arbor
country: United States
facility: University of Michigan
state: Michigan
city: Minneapolis
country: United States
facility: University of Minnesota
state: Minnesota
city: Jackson
country: United States
facility: University of Mississippi Medical Center
state: Mississippi
city: Saint Louis
country: United States
facility: Washington University
state: Missouri
city: New York
country: United States
facility: NYU Medical Center
state: New York
city: New York
country: United States
facility: Weill Medical College of Cornell University
state: New York
city: Chapel Hill
country: United States
facility: UNC Chapel Hill
state: North Carolina
city: Durham
country: United States
facility: Duke University
state: North Carolina
city: Greensboro
country: United States
facility: Pulmonix LLC
state: North Carolina
city: Wilmington
country: United States
facility: PMG Research
state: North Carolina
city: Winston-Salem
country: United States
facility: Wake Forest Baptist Health
state: North Carolina
city: Cincinnati
country: United States
facility: University of Cincinnati Medical Center
state: Ohio
city: Cleveland
country: United States
facility: Cleveland Clinic
state: Ohio
city: Columbus
country: United States
facility: The Ohio State University
state: Ohio
city: Oklahoma City
country: United States
facility: University of Oklahoma
state: Oklahoma
city: Portland
country: United States
facility: Oregon Clinic
state: Oregon
city: Philadelphia
country: United States
facility: Thomas Jefferson University
state: Pennsylvania
city: Charleston
country: United States
facility: Medical University of South Carolina
state: South Carolina
city: Nashville
country: United States
facility: Vanderbilt University
state: Tennessee
city: Dallas
country: United States
facility: University of Texas Southwestern
state: Texas
city: Dallas
country: United States
facility: Baylor University Medical Center at Dallas
state: Texas
city: Houston
country: United States
facility: Baylor College of Medicine
state: Texas
city: Houston
country: United States
facility: Houston Methodist Lung Center
state: Texas
city: Falls Church
country: United States
facility: Inova
state: Virginia
city: Milwaukee
country: United States
facility: The Medical College of Wisconsin
state: Wisconsin
Study officials:
affiliation: Duke Clinical Research Institute, Duke University
name: Scott Palmer, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.