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

NCT07234032 | Recruiting Phase 3 DMC FDA Drug

• 2 other identifiers: INS1009-3122025-521769-29-00
• Study Type: interventional
• Target: 344
• Locations: 5 countries
• Sites: 9

Brief Summary

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).

Conditions

Pulmonary Hypertension; Interstitial Lung Disease

Keywords

pulmonary hypertension, interstitial lung disease

Outcome Measures

Primary Outcomes (1)

• Number of Participants Who Experienced at Least One Treatment-Emergent Adverse Events (TEAEs)

  Up to 108 weeks

Secondary Outcomes (19)

• 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

Study Arms (1)

Treprostinil Palmitil Inhalation Powder (TPIP)

EXPERIMENTAL

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; Drug: Placebo

Interventions

Treprostinil Palmitil Inhalation Powder DRUG

Oral inhalation using a capsule-based dry powder.

Also known as: INS1009

Treprostinil Palmitil Inhalation Powder (TPIP)

Placebo DRUG

Oral inhalation in initial double-dummy titration period.

Treprostinil Palmitil Inhalation Powder (TPIP)

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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.

You may not qualify if:

• 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).

Sponsors & Collaborators

• Insmed Incorporated: lead

Study Sites (9)

USA006

Naples, Florida, 34102, United States

RECRUITING

USA003

Bend, Oregon, 97701, United States

RECRUITING

JPN004

Narita, Chiba, 286-8520, Japan

NOT YET RECRUITING

KOR001

Seoul, 3080, South Korea

NOT YET RECRUITING

KOR006

Seoul, 6351, South Korea

NOT YET RECRUITING

ESP006

Madrid, 28041, Spain

NOT YET RECRUITING

ESP003

Madrid, 28046, Spain

NOT YET RECRUITING

ESP001

Seville, 41009, Spain

NOT YET RECRUITING

TWN003

Hsinchu, 30059, Taiwan

NOT YET RECRUITING

MeSH Terms

Conditions

Hypertension, Pulmonary; Lung Diseases, Interstitial

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Study Director

  Insmed Incorporated

  STUDY DIRECTOR

Central Study Contacts

Insmed Incorporated

CONTACT

18444467633; medicalinformation@insmed.com

Study Design

• Study Type: interventional
• Phase: phase 3
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR
• Expanded Access: Yes

Study Record Dates

• First Submitted: November 14, 2025
• First Posted: November 18, 2025
• Study Start: July 20, 2026
• Primary Completion (Estimated): January 22, 2031
• Study Completion (Estimated): January 22, 2031
• Last Updated: August 13, 2026

Record last verified: 2026-08

Data Sharing

• IPD Sharing: Will not share

Locations

ES (3); JP (1); KR (2); TW (1); US (2)