Clinical Research Details Clinical Research An Open-label Extension Study of Inhaled Treprostinil in Subjects with Fibrotic Lung Disease (20212063) Study Description This study is investigating long-term treatment with a drug called inhaled treprostinil (brand name Tyvaso®) in people with Idiopathic Pulmonary Fibrosis (IPF). In the study, you will receive inhaled treprostinil, which you may have also received in the RIN-PF-301 or RIN-PF-303 study (unless you received placebo). There is no placebo use in this study, so you are guaranteed to receive the active study drug. In this consent form, inhaled treprostinil will be referred to as “study drug.” The study drug is given by inhaling (breathing in) the medicine into your lungs using a machine called a nebulizer (inhaling device). The nebulizer changes the medicine into a mist for you to breathe into your lungs. Inhaled treprostinil works by keeping the pulmonary arteries open to allow more blood to flow into your lungs and may reduce lung scarring. Inhaled treprostinil is approved in the United States, Argentina, and Israel for the treatment of certain types of pulmonary hypertension (i.e. high blood pressure in the lungs). Inhaled treprostinil is considered investigational in this study and has not been approved for the treatment of IPF. The purpose of this research study is to investigate the long-term safety of inhaled treprostinil in people with IPF. The study will also provide or continue to provide inhaled treprostinil to eligible people who participated in the RIN-PF-301 or RIN-PF-303 study. All participants in this study will receive treatment with inhaled treprostinil. Inclusion/Exclusion Criteria A subject is eligible to particpate in this study if all the following criteria apply: 1. Subject gives voluntary informed consent to participate in the study. 2. The subject participated in RIN-PF-301 or RIN-PF-303 and had 1 of the following applied: a. Remained on study drug and completed all scheduled study visits b. Was enrolled in RIN-PF-301 or RIN-PF-303 at the time that the study or study subject was discontinued by the Sponsor. 3. Women of childbearing potential must be non-pregnant (as confirmed by a urine pregnancy test at OLE Entry Visit and Baseline) and non-lactating, and will do 1 of the following: a. Abstain from intercourse (when it is in line with their preferred and usual lifestyle) b. Use 2 medically acceptable, highly effective forms of contraception for the duration of the study, and at least 30 days after discontinuing study drug. Medically acceptable, highly effective forms of contraception can include approved hormonal contraceptives (oral, injectable, and implantable) and barrier methods (such as a condom or diaphragm) when used with a spermicide. Women who are successfully sterilized or postmenopausal are not considered to be of reproductive potential. 4. Males with a partner of childbearing potential must use a condom for the duration of treatment and for at least 48 hours after discontinuing study drug. 5. In the opinion of the Investigator, the subject is able to communicate effectively with study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements, including attending all study visits. A subject is eligible for inclusion in this study if all the following criteria apply: 1. Subject gives voluntary informed consent to participate in the study. 2. The subject participated in RIN-PF-301 or RIN-PF-303 and had 1 of the following applied: a. Remained on study drug and completed all scheduled study visits b. Was enrolled in RIN-PF-301 or RIN-PF-303 at the time that the study or study subject was discontinued by the Sponsor. 3. Women of childbearing potential must be non-pregnant (as confirmed by a urine pregnancy test at OLE Entry Visit and Baseline) and non-lactating, and will do 1 of the following: a. Abstain from intercourse (when it is in line with their preferred and usual lifestyle) b. Use 2 medically acceptable, highly effective forms of contraception for the duration of the study, and at least 30 days after discontinuing study drug. Medically acceptable, highly effective forms of contraception can include approved hormonal contraceptives (oral, injectable, and implantable) and barrier methods (such as a condom or diaphragm) when used with a spermicide. Women who are successfully sterilized or postmenopausal are not considered to be of reproductive potential. 4. Males with a partner of childbearing potential must use a condom for the duration of treatment and for at least 48 hours after discontinuing study drug. 5. In the opinion of the Investigator, the subject is able to communicate effectively with study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements, including attending all study visits. A subject is not eligible to participate in this study if any of the following criteria apply: 1. Subject is pregnant or lactating. 2. In the opinion of the Investigator, enrollment in RIN-PF-302 would represent a risk to the subject's overall health Open Enrollment Contact Name: Kathleen KileyContact Phone: (904) 244-9632Contact Email: kathleen.kiley@jax.ufl.edu Investigators Vandana K. Seeram, M.B.B.S. (M.D.) Medicine