Pulmonary Fibrosis Foundation Patient Registry (PFFR)

The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosis (IPF). The aim of the Registry is to create a cohort of well-characterized patients with interstitial lung disease (ILD) for participation in retrospective and prospective research

Study Overview

• Status: Completed
• Conditions: Interstitial Lung Disease (ILD); Idiopathic Pulmonary Fibrosis (IPF)

Detailed Description

The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosis (IPF). The aim of the Registry is to create a cohort of well-characterized patients with interstitial lung disease (ILD) for participation in retrospective and prospective research.

Patients who meet inclusion and exclusion criteria and are being treated at a Registry site can be asked to participate. Patients will be required to read and sign an Institutional review board(IRB)-approved informed consent document prior to any Registry activity taking place.

At the time of informed consent, participants will be asked to indicate if they are interested in being contacted by Registry site personnel for potential participation in future clinical trials and/or studies. Participants who opt out will not be contacted for future studies.

No clinical procedures, testing, or diagnostics will be required by virtue of Registry participation. Participants will permit Registry staff to abstract clinical data obtained as part of routine clinical care in the diagnosis and treatment of ILD. These data will be entered into a web-based, electronic data capture (EDC) by the Registry staff to at regular intervals. Some of these data will be retrospective, having been collected prior to consenting for the Registry.

Computed tomography (CT) images collected for diagnosis and / or treatment will be de-identified at the Registry site and uploaded to a secure server that is a 21 Code of Federal Regulations (CFR) Part 11, Good Clinical Practice (GCP), and HIPAA compliant online imaging repository.

Participants will be asked to complete patient reported outcome (PRO) surveys related to ILD symptoms and quality of life at the time of enrollment and during clinical follow-up visits.Participants who are not seen for clinical follow-up within 12 months will be contacted by telephone or mail by Registry site personnel to complete the PRO measures.

The University of Michigan Statistical Analysis of Biomedical and Educational Research (SABER) unit will serve as the Registry Data Coordinating Center and will manage data entered into a web based, CFR 21 Part 11 compliant electronic data capture (EDC) system by the Registry sites.

• Study Type: Observational
• Enrollment (Actual): 2004

Contacts and Locations

Study Locations

• United States
  • Alabama
    • Birmingham, Alabama, United States, 35294
      • University fo Alabama at Birmingham
  • Arizona
    • Phoenix, Arizona, United States, 85013
      • Dignity Health St. Joseph's Hospital and Medical Center
    • Tucson, Arizona, United States, 85724-0001
      • The University of Arizona
  • California
    • Los Angeles, California, United States, 90095
      • UCLA, David Geffen School of Medicine at UCLA
    • San Francisco, California, United States, 94143-2204
      • University of California at San Francisco
    • Stanford, California, United States, 94305
      • Stanford University
  • Colorado
    • Denver, Colorado, United States, 80206
      • National Jewish Health
  • Connecticut
    • New Haven, Connecticut, United States, 06510-3206
      • Yale School of Medicine
  • Florida
    • Miami, Florida, United States, 33138
      • University of Miami
  • Georgia
    • Austell, Georgia, United States, 30106
      • Piedmont Healthcare
  • Illinois
    • Chicago, Illinois, United States, 60611
      • Northwestern University
    • Chicago, Illinois, United States, 60637
      • University of Chicago
  • Kansas
    • Kansas City, Kansas, United States, 66160
      • The University of Kansas Medical Center
  • Kentucky
    • Louisville, Kentucky, United States, 40202
      • University of Louisville
  • Louisiana
    • New Orleans, Louisiana, United States, 70112
      • Tulane University
  • Maryland
    • Baltimore, Maryland, United States, 21201
      • University of Maryland Medical Center
    • Baltimore, Maryland, United States, 21205
      • Johns Hopkins University - School of Medicine
  • Massachusetts
    • Boston, Massachusetts, United States, 02114
      • Massachusetts General Hospital
  • Michigan
    • Ann Arbor, Michigan, United States, 48109
      • University of Michigan
  • Minnesota
    • Minneapolis, Minnesota, United States, 55455
      • Regents of the University of Minnesota Twin Cities
    • Rochester, Minnesota, United States, 55905
      • Mayo Clinic
  • Missouri
    • Saint Louis, Missouri, United States, 63110-1010
      • Washington University School of Medicine
  • New York
    • New York, New York, United States, 10065
      • Weill Cornell Medical College
    • New York, New York, United States, 10030
      • Columbia University Medical Center
    • Rochester, New York, United States, 14642
      • University of Rochester
    • Stony Brook, New York, United States, 11794-8172
      • Stony Brook University Hospital
  • North Carolina
    • Durham, North Carolina, United States, 27710
      • Duke University Medical Center
  • Ohio
    • Cincinnati, Ohio, United States, 45267
      • University of Cincinnati
    • Columbus, Ohio, United States, 43221
      • The Ohio State University
  • Pennsylvania
    • Hershey, Pennsylvania, United States, 17033
      • Pennsylvania State University
    • Philadelphia, Pennsylvania, United States, 19128
      • University of Pennsylvania
    • Philadelphia, Pennsylvania, United States, 19129
      • Temple University Health System
    • Pittsburgh, Pennsylvania, United States, 15213
      • University of Pittsburgh
  • South Carolina
    • Charleston, South Carolina, United States, 29425
      • Medical University of South Carolina
  • Tennessee
    • Nashville, Tennessee, United States, 37232
      • Vanderbilt University
  • Texas
    • Dallas, Texas, United States, 75390
      • University of Texas Southwestern Medical Center
    • Houston, Texas, United States, 77030
      • The University of Texas Health Science Center at Houston
    • San Antonio, Texas, United States, 78253
      • UTHSC - San Antonio
  • Utah
    • Salt Lake City, Utah, United States, 84108
      • University of Utah
  • Virginia
    • Charlottesville, Virginia, United States, 22908
      • University of Virginia Interstitial Lung Disease Clinic
    • Falls Church, Virginia, United States, 22046
      • Inova Fairfax Hospital
  • Washington
    • Seattle, Washington, United States, 98195-0001
      • University of Washington

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 99 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

At least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosos (IPF).

Description

Inclusion Criteria:

1. 18 years old or older
2. Understand and sign the informed consent document

3. ILD Diagnosis must be made / confirmed at a participating Registry center.

   1. The diagnostic evaluation must include, at a minimum, a medical history, physical examination, pulmonary function testing and a computerized tomography (CT) scan of the chest.
   2. If patients exhibit another pulmonary disease (such as emphysema or asthma), the primary disease must be ILD.
4. Anticipated additional follow up at the Registry center within one year.

Exclusion Criteria:

1. Diagnosed with:

1. Sarcoid
2. Lymphangioleiomyomatosis (LAM)
3. Pulmonary alveolar proteinosis (PAP)
4. Cystic fibrosis (CF)
5. Amyloidosis

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in % predicted FVC	Analysis of registry data will lead to aggregated reports summarizing the pulmonary function	Up to 55 months
Change in % predicted DLCO	Analysis of registry data will lead to aggregated reports summarizing the pulmonary function	Up to 55 months

Collaborators and Investigators

• Sponsor: Pulmonary Fibrosis Foundation
• Collaborators: University of Michigan
• Investigators: Study Chair: Kevin R Flaherty, MD, University of Michigan

Study record dates

Study Major Dates

• Study Start (Actual): March 29, 2016
• Primary Completion (Actual): December 16, 2022
• Study Completion (Actual): December 16, 2022

Study Registration Dates

• First Submitted: April 14, 2016
• First Submitted That Met QC Criteria: April 27, 2016
• First Posted (Estimated): May 3, 2016

Study Record Updates

• Last Update Posted (Actual): July 7, 2023
• Last Update Submitted That Met QC Criteria: July 5, 2023
• Last Verified: July 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Fibrosis; Lung Diseases; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis; Lung Diseases, Interstitial
• Other Study ID Numbers: PFF01

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO