Pulmonary and Critical Care Clinical Trials

The purpose of this registry is to collect clinical information about people with interstitial lung disease (ILD) to help us better understand the causes of ILD and determine if particular types of treatment work better for some causes of ILD than others. The Registry will also be used to determine who might be eligible for and interested in participating in future studies related to ILD.

Participants agree to allow the collection of information from the medical record for research purposes and agree to allow us to contact them about future studies. Participants may also complete questionnaires during their standard of care visits, participate in an optional frailty study by taking a brief physical ability test at the time of standard of care visits, give optional blood for research, and optionally allow us to contact them about their health on an annual basis.

We are conducting the INSIGHT COPD study because symptoms of chronic obstructive pulmonary disease (COPD) and high body mass index (BMI) overlap. There are many medications for patients with COPD, but there is little mention of weight loss as a possible treatment in current research. We are trying to find out if a lifestyle program that promotes modest weight loss and increased physical activity will improve COPD symptoms for those with a high BMI. We hope that the program will lead to weight loss and better exercise tolerance. We are also looking at the effects on shortness of breath, quality-of-life, and cardiovascular disease risk factors.

1 year, 2 visits.

The purpose of this study is to better understand the development of scleroderma-related lung fibrosis (ILD). We would like to investigate the role of macrophages and other important cells in the development of ILD. Macrophages are types of white blood cells important in the immune system. In healthy people, macrophages produce proteins that activate the immune system to assist in tissue repair. However, there is evidence that in people with scleroderma, the macrophages behave abnormally and contribute to scarring in the lungs.

Patients who participate in this study will:

• donate fluid collected from their lungs during a bronchoscopy (taken at the time they are already scheduled to have a bronchoscopy or during their scheduled endoscopy)
• give a blood sample
• give esophageal biopsies (taken during their scheduled endoscopy)
• as an option, give skin biopsies

Patients with COVID-19 disease are at risk of blood clots, which may affect many parts of the body. It is recommended that patients hospitalized with COVID-19 disease be placed on a blood thinner called heparin to prevent clots. The purpose of this study is to determine if adding a platelet inhibitor, a different type of blood thinner, will be even better at reducing the risk of blood clots. All of these blood thinner medications are already approved by the United States Food and Drug Administration (FDA) for preventing clots in different situations. We do not currently know the best combination of these drugs in people hospitalized with COVID-19.

Participants in this study will receive an assigned study treatment that will last up to 14 days or until discharge from the hospital, whichever comes first. The study team will also contact participants about 90 days after hospital discharge and about 1 year after hospital discharge to check on the participant's health.

Currently, participants will be randomly assigned to either blood thinner alone or blood thinner with platelet inhibitor.

Participants may also optionally give blood samples at four different times during their hospital stay. The blood will be stored for future genetic research.

The purpose of this trial is to see if macitentan 75 mg versus placebo is useful and safe for treating patients with chronic thromboembolic pulmonary hypertension (CTEPH). Macitentan 10 mg once-daily is approved in many countries around the world for the treatment of pulmonary arterial hypertension (PAH). Macitentan at a dose of 75 mg is not approved for use by any Regulatory Authority in any country. This means that macitentan is an investigational drug, since it is not approved for use in patients with CTEPH, and can only be used in a research study such as this one.

Participants will be in this study for up to 3.5 years. Participants who choose to join the open label part of the study will be in the study between 2.5 and 6 years.

Participants will:

• Randomly assigned to macitentan 75 mg or placebo
• Return for study visits at least 10 times for the first 7 months and then every 12 weeks until the end of the double-blind period.
• Receive a phone call at week 5 and week 9.
• If continuing in the open-label study, return for 7 visits for the first 6 months and then every 6 months until the end of the open-label period.
• Study visits may include health questionnaires, physical exam, blood draw, EKG, 6 minute walk test, urine pregnancy test.
• Monitor body weight at least once per week at home until week 12.
• Wear a device to monitor sleep and activity until week 28.
• Complete questionnaires at home on a mobile device provide to you during the double-blind period.
• Optionally undergo a right heart catheterization at week 28.

Participants will be paid a stipend after completion of each study visit.

The purpose of this study is to investigate the antibody and immune responses to the COVID-19 vaccine in patients with sarcoidosis, both treated and not treated with immunosuppressive medications, compared to healthy controls. This study will address a critical gap in our understanding of vaccine responses in people with sarcoidosis.

Participants will:

• Complete a brief screening/health history questionnaire
• Give 5-7 tubes of blood