NIPH Clinical Trials Search

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

Basic Information

Recruitment status	Pending
Health condition(s) or Problem(s) studied	Pulmonary hypertension, interstitial lung disease
Date of first enrollment	05/06/2026
Target sample size	344
Countries of recruitment
Study type	Interventional
Intervention(s)	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.

Outcome(s)

Primary Outcome

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

Secondary Outcome

- 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

Key inclusion & exclusion criteria

Age minimum	>= 18age old
Age maximum	Not applicable
Gender	Both
Include 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 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.
Exclude 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).