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.

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.

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.

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.