The purpose of this research registry is to better understand the natural history of Idiopathic Pulmonary Fibrosis and current practice patterns. The IPF-PRO registry will be used to collect data and biological samples that will support future research studies by identifying disease biomarkers for IPF. Through these studies, researchers hope to find new ways to detect, treat, and maybe prevent or cure health problems. Some of these studies may be about how genes affect health and disease, or how a person's genes affect their response to a treatment. Some of these studies may lead to new products, such as drugs or tests for diseases. We are asking you to let us collect and store some of your blood and health information so they might be used in these kinds of future studies.

If you are newly diagnosed with IPF and are eligible for participation in IPF-PRO, you will be asked to sign a consent form to become enrolled if you agree to be in this registry. At enrollment a member of MUSC research staff will collect information from you and about your medical history and medical care, as well as information about the types of health insurance (public or private) that you have. As part of your participation in this registry, you will be required to sign a medical release form giving permission for your medical records to be reviewed for the purposes of data collection for the registry. This is an observational registry which means you will not receive any investigational treatments or investigational drugs, and only minimally invasive procedures will be performed (blood draws) at scheduled clinic visits. In addition to the face to face visits for self-administered participant reported questionnaires and blood collection, at roughly 6-month intervals, sites will review the participant's medical records. Your disease management and treatment decisions will be determined by you and your health care professional. Subjects will be followed until the last enrolled subject has been followed for 3 years up to a maximum of 5 years.

This is a multi-center, randomized, double-blind, placebo-controlled trial of 200 participants to receive N-acetyl cysteine (NAC) or placebo for a 24 month duration. The purpose of this study is to see if n-acetylcysteine (NAC) can help people with idiopathic pulmonary fibrosis (IPF) who have a specific gene type and to see if NAC is safe to take without causing too many side effects.

This is a Phase 3, randomized, double-blind, placebo-controlled, efficacy and safety study of subjects with IPF treated with inhaled treprostinil over a 52-week period. This study is investigating whether a drug called inhaled treprostinil (brand name Tyvaso®) works to help people with IPF improve their lung function tests. The purpose of this research study is to see how well inhaled treprostinil works in participants with IPF and to gather information on how safe it is. This study will look at changes in your breathing tests, also called lung function tests. About 396 people will participate in this study from about 100 medical centers. Your participation in this study is voluntary and will last approximately 58 weeks. This time includes a Screening Period that could last up to approximately 6 weeks plus a 52-week Treatment Period.

The purpose of this study is to test whether a drug called PRA023/MK7240 (the study drug) is a good treatment for patients with Systemic Sclerosis associated with Interstitial Lung Disease (SSc-ILD). The study drug PRA023/7240 is an investigational drug that is given by infusion every 4 weeks. An investigational drug is not approved by The US Food and Drug Administration. It can only be used in a research study like this one. In this study, PRA023/MK7240 will be compared with a placebo (dummy drug). The placebo will be a saline solution that does not have any study drug in it. The comparison with the placebo helps to determine whether the effects seen in your body is because of the PRA023/MK7240 or not. This is a randomized study meaning that you will be assigned by chance (like flipping a coin) to receive either the study drug or placebo. This will be done with the help of a computer-based program and you will have 50% chance of receiving either the study drug or placebo. The study is double-blinded study and 50 weeks long, meaning you and your study doctor will not know what you are receiving, the study drug or placebo.

The study is sponsored by Prometheus Biosciences, Inc., a subsidiary of Merck & Co., Inc. The study is being done at approximately 25 sites across the United States. The main portion of the study will require 15 visits to the MUSC main campus and will have the following procedures completed over the course of your participation: blood draw, physician-led assessments of your disease (for example physical exam and skin thickness testing), tests to assess your pulmonary function and health (Pulmonary Function Test (PFT) and High Resolution Computed Tomography (HRCT)), electrocardiogram, as well as asked to complete surveys. If you complete the initial blinded treatment period of 50 weeks, the study doctor will discuss whether you are eligible to enter the open label period of the study, meaning no placebo. If you are eligible and agree, you will receive 500 mg of study drug once every 4 weeks for an additional 52 weeks. Compensation is available for participation.

The study will be a multi-center, double-blind, randomised, placebo-controlled 2-period cross-over study in subjects with chronic cough due to idiopathic pulmonary fibrosis (IPF).
Subjects will participate in one of two cohorts (Cohort 1 and Cohort 2). Cohort 1 will evaluate a 30 mg orvepitant dose and Cohort 2 the 10 mg dose. Within each cohort, subjects will be randomised to receive either orvepitant or placebo in the first treatment period (Treatment Period A) followed by the alternate treatment in Treatment Period B. There will be a wash-out period of 3 weeks between the two treatment periods. Subjects will be randomised 1:1 to each of the two treatment orders and 1:1 to each cohort. Subjects will enter a screening period of between 14 and 28 days to determine eligibility. Eligible subjects will be randomised at the Baseline visit and will participate in two identical 28 day treatment periods with the wash-out period between them. There will be a total of 8 visits including the Screening, Baseline and final Follow-up visits.

Following the study Entry Visit, subjects will return at Week 4,
Week 12, and every 12 weeks thereafter. Study visits will continue
for up to 6 years or until the subject prematurely discontinues study
treatment due to an adverse event or other reason, inhaled treprostinil
becomes commercially available for IPF in the region in which the
study is conducted, or the study is discontinued by the Sponsor
(whichever is sooner). Subjects will also be contacted by telephone or
email at least weekly until Week 12 and monthly thereafter in
between study visits to discuss study drug titration, assess study drug
tolerability, monitor adverse event, document changes to concomitant
medications, and remind subjects to bring in all study drug and a
device to their next clinic visit.

The study will be an open-label, randomized, parallel arm study that will include a
treatment arm and control arm. Participants will have clinic visits at screening, randomization (day 1) and weeks 4, 12, 18, and 24. After week 24, participants will have clinic visits at weeks 32, 40, and 48. Participants will also have a telehealth visit on day 2 and phone calls to assess adverse events (AEs), serious adverse events (SAEs), and review patient education will occur during weeks 5, 8, 36, and 44. The phone calls may be converted to telehealth visits or clinic visits and / or followed by clinic visits, if the study team deems it necessary. Pulmonary function testing, quality of life survey (St. George's Respiratory Questionnaire (SGRQ)), and blood draw will occur at each clinic visit.

The Program will allow access to brensocatib for patients who have completed the INS1007-301 ASPEN Clinical Trial. Patients will receive brensocatib 10 mg orally once daily. Eligible, compliant patients may receive brensocatib in this program until the drug is commercially available or until Insmed terminates the program.

This is a Phase 2, multicenter, randomized, double-blind, placebo-controlled, study to evaluate the efficacy, safety, and tolerability of 200 mg twice daily (BID) of BBT-877 in patients with IPF, with or without AF approved background therapies (pirfenidone or nintedanib). For both the placebo and BBT-877 groups, patients will be selected so that 40% to 60% of the patients in each group will be treated with pirfenidone or nintedanib in order to investigate if BBT-877 could be used as either monotherapy or add-on therapy to AFs, to further reduce the decline in lung function and/or improve symptoms as compared to those drugs alone. As a result, 60% to 40% of the patients will receive BBT-877 or placebo, alone.

The purpose of this study is to learn more about interstitial lung disease through collection of information and blood samples to be used in future research projects. Basic information and a blood sample will be collected in conjunction with clinical care for this study, approximately every 3 months.