An Open-Label Extension Study of Treprostinil Palmitil Inhalation Powder (TPIP) in Participants With Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

• Treprostinil Palmitil Inhalation Powder (Drug), also known as INS1009: Oral inhalation using a capsule-based dry powder.
• Placebo (Drug): Oral inhalation in initial double-dummy titration period.

Primary outcomes

• Number of Participants Who Experienced at Least One Treatment-Emergent Adverse Events (TEAEs) [Up to 108 weeks]

Secondary outcomes

• Change From Baseline in 6-Minute Walk Distance (6MWD) Measured Post-Dose [Up to 104 weeks]
• Absolute Change From Baseline in Forced Vital Capacity (FVC) [Up to 104 weeks]
• Percent Change From Baseline in Forced Vital Capacity (FVC) [Up to 104 weeks]
• Absolute Change From Baseline in Percent Predicted FVC (FVC% pred) [Up to 104 weeks]
• Percent Change From Baseline in Percent Predicted FVC (FVC% pred) [Up to 104 weeks]
• Absolute Change From Baseline in Forced Expiratory Volume in 1 Second (FEV1) [Up to 104 weeks]
• Percent Change From Baseline in Forced Expiratory Volume in 1 Second (FEV1) [Up to 104 weeks]
• Absolute Change From Baseline in Percent Predicted FEV1 (FEV1%) [Up to 104 weeks]
• Percent Change From Baseline in Percent Predicted FEV1 (FEV1%) [Up to 104 weeks]
• Change From Baseline in the Plasma Concentration of N-Terminal Pro B-Type Natriuretic Peptide (NT-proBNP) [Up to 104 weeks]
• Annualized Rate of Occurrence of Exacerbations of Interstitial Lung Disease (ILD) [Up to 104 weeks]
• Percentage of Participants With a Clinical Worsening Events [Up to 104 weeks]
• Mean Change From Baseline in Living With Pulmonary Fibrosis (L-PF) Domain Score [Up to 104 weeks]
• Mean Change From Baseline in L-PF Cough Domain Score [Up to 104 weeks]
• Mean Change From Baseline in L-PF Dyspnea Domain Score [Up to 104 weeks]
• Mean Change From Baseline in L-PF Impact Domain Score [Up to 104 weeks]
• Mean Change From Baseline in the EuroQoL- 5 Dimensions (EQ-5D-5L) Index Score [Up to 104 weeks]
• Mean Change From Baseline in the EQ-5D-5L Visual Analog Scale (VAS) [Up to 104 weeks]
• Percentage of Participants With Major Morbidity or Mortality Events [Up to 104 weeks]

Sex: All, ages: 18 Years to any

Inclusion Criteria

* Participants who have completed the lead-in PH-ILD TPIP Study INS1009-311 (NCT07179380).
* Capable of giving signed informed consent that includes compliance with the requirements and restrictions listed in the informed consent form (ICF) and in this protocol.
* Agree not to participate in any other interventional trials or use investigational drugs or devices while participating in the INS1009-312 study.

Exclusion Criteria

* Participants who experienced any adverse events (AEs) evaluated as causally related to TPIP by the Investigator in a lead-in study, which in the opinion of the Investigator, could pose an unreasonable risk of continued treatments for the participant.
* Current use or expected need for pulmonary arterial hypertension (PAH)-approved therapy, including prostacyclin, prostacyclin analogues or other prostacyclin receptor agonists, endothelin receptor antagonists, and/or soluble guanylate cyclase stimulator, or any PH-ILD approved treprostinil therapy. Use of phosphodiesterase 5 inhibitors in line with applicable guidelines is allowed.
* Diagnosis of Pulmonary Hypertension World Health Organisation (WHO) Groups 1, 2, 4, or 5, or subtypes of PH WHO Group 3 other than interstitial lung disease (including combined pulmonary fibrosis and emphysema).
* Evidence of left ventricular failure, heart failure with preserved ejection fraction (HFpEF) or postcapillary PH.
* Known hypersensitivity or contraindication to treprostinil or TPIP or TPIP formulation excipients (eg, mannitol, leucine).

Note: Other protocol-defined inclusion/exclusion criteria may apply.

• Study Director, Study director, Insmed Incorporated

NIH grants: 2025