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

A Long-term Open-Label Extension Study of Treprostinil Palmitil Inhalation Powder for Treatment of Pulmonary Hypertension Associated With Interstitial Lung Disease

The primary objective of this study is to evaluate the safety and tolerability of the long-term use of TPIP in participants with PH-ILD from Study INS1009-311 (NCT07179380).

Study Overview

• Status: Not yet recruiting
• Conditions: Pulmonary Hypertension; Interstitial Lung Disease
• Intervention / Treatment: Drug: Treprostinil Palmitil Inhalation Powder; Drug: Placebo

• Study Type: Interventional
• Enrollment (Estimated): 344
• Phase: Phase 3

Expanded Access

Available outside the clinical trial. See expanded access record.

Contacts and Locations

• Study Contact: Name: Insmed Incorporated; Phone Number: 18444467633; Email: medicalinformation@insmed.com

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

Description

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 Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

Experimental: Treprostinil Palmitil Inhalation Powder (TPIP); Participants transitioning from study INS1009-311 (NCT07179380) will undergo an initial double-dummy titration with the stable dose from the lead-in study and either TPIP or placebo for 4 weeks. They will then receive open-label TPIP at a stable maintenance dose with optional escalation (80-1280 micrograms once daily) for the remainder of the 104-week treatment period.

Drug: Treprostinil Palmitil Inhalation Powder; Oral inhalation using a capsule-based dry powder.; Other Names: INS1009; Drug: Placebo; Oral inhalation in initial double-dummy titration period.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Number of Participants Who Experienced at Least One Treatment-Emergent Adverse Events (TEAEs)	Up to 108 weeks

Secondary Outcome Measures

Outcome Measure	Time Frame
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

Collaborators and Investigators

• Sponsor: Insmed Incorporated

Study record dates

Study Major Dates

• Study Start (Estimated): June 5, 2026
• Primary Completion (Estimated): January 22, 2031
• Study Completion (Estimated): January 22, 2031

Study Registration Dates

• First Submitted: November 14, 2025
• First Submitted That Met QC Criteria: November 14, 2025
• First Posted (Actual): November 18, 2025

Study Record Updates

• Last Update Posted (Actual): May 26, 2026
• Last Update Submitted That Met QC Criteria: May 21, 2026
• Last Verified: May 1, 2026

More Information

Terms related to this study

• Keywords: pulmonary hypertension, interstitial lung disease
• Additional Relevant MeSH Terms: Vascular Diseases; Cardiovascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Lung Diseases, Interstitial; Hypertension, Pulmonary
• Other Study ID Numbers: INS1009-312; 2025-521769-29-00 (Other Identifier: EU CT Number)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: Yes
• Studies a U.S. FDA-regulated device product: No