Pulmonary Fibrosis Foundation Patient Registry

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.

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