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

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

Makulova Natalya

Insmed Incorporated

700 US Highway 202/206 Bridgewater, NJ 08807-1704

medicalinformation@insmed.com

Medical Information Center

Insmed Godo Kaisha

13th Floor, 2-10-3 Nagata-cho, Chiyoda-ku Tokyo

81-120-118808

medicalinformation@insmed.com

Recruiting

July. 20, 2026

Interventional

single arm study

open(masking not used)

no treatment control/standard of care control

single assignment

treatment purpose

- 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 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 (NCT07234032) study.

- 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).

Both

Pulmonary hypertension, interstitial lung disease

Experimental: Treprostinil Palmitil Inhalation Powder
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.

Number of Participants Who Experienced at Least One Treatment-Emergent Adverse Events (TEAEs) up to 108 weeks

- 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 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 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

81-474731281

May. 01, 2026

No

United States