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1. Males and females at least 18 years of age who give voluntary written informed consent to participate in the study.
2. Patients with a diagnosis of ILD based on computed tomography imaging, including:
   • Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis
   • Connective tissue disease-associated ILD with FVC <70%
   • Hypersensitivity pneumonitis
   • Scleroderma-related ILD
   • Autoimmune ILD
   • Nonspecific interstitial pneumonia
   • Occupational lung disease
   • Combined pulmonary fibrosis and emphysema
3. Patients must meet a total of 2 or more criteria from at least 2 distinct categories below (ie, Category 1, Category 2, Category 3) based on the Investigator’s clinical judgment, within 180 days of Screening.

Category 1: Clinical tests

a. PFTs with at least 1 of the following results:

• DLCO <40%
• DLCO decline of ≥15% based on 2 most recent assessments
• Worsening DLCO (decline >10%) with stable FVC (decline <5%) (ie, worsening FVC/DLCO ratio) based on 2 most recent assessments

b. Historical HRCT with any of the following findings:

• Right ventricle enlargement: RV:LV ratio >1
• Pulmonary artery enlargement
• Pulmonary artery/aorta ratio >1.0
• Ventricular septal flattening
• Enlarged pulmonary arteries in the lung periphery

Category 2: Symptoms, oxygenation, exercise capacity, and BNP/NT-proBNP

• Symptoms disproportionate to ILD severity or changes in symptoms not explained by ILD progression
• Desaturation on 6MWT disproportionate to ILD severity in the opinion of the Investigator
• 6-Minute Walk Distance decline of ≥15% based on 2 more recent assessments
• Worsening desaturation requiring more supplemental oxygen
• Recent worsening desaturation
• BNP >200 pg/mL or NT-proBNP >395 pg/mL
• Physical exam findings (at least 1 of the following): syncope, jugular venous distension, ankle swelling/peripheral edema, ascites, loud P2 or S2 heart sound, or hepatomegaly

Category 3: Echocardiography

• RV systolic pressure >35 mmHg
• Any RV dilation or enlargement
• Other RV abnormalities in the clinician’s judgment
• Tricuspid annular plane systolic excursion <2 cm

Patients are excluded from the study if any of the following criteria apply:

1. Prior RHC with mPAP >20 mmHg
2. Currently on a Food and Drug Administration (FDA)-approved pulmonary arterial hypertension medication
3. Diagnosed with chronic obstructive pulmonary disease
4. Uncontrolled or untreated sleep apnea
5. Pulmonary embolism within the past 3 months
6. History of ischemic heart disease or left-sided myocardial dysfunction within 12 months of Screening, defined as LV ejection fraction <40% or pulmonary capillary wedge pressure>15 mmHg
7. Any other clinical features that, in the opinion of the Investigator, might adversely affect interpretation of study data or study safety, or make the patient unsuitable for RHC

• Age: ≥ 40 years old
• FVC ≥ 45% and ≤ 80% predicted
• If on background pirfenidone or nintedanib for IPF, stable dose for ≥ 30 days prior to Baseline
• Concomitant use of both pirfenidone and nintedanib is not permitted
• Diagnosis of IPF based on the 2018 ATS/ERS/JRS/JRS/ALAT Clinical Practice Guidelines (Raghu 2018)
• HRCT within the last 12 months “consistent with UIP” (confirmed by central reader)
• HCRT may be performed during screening window as study assessment if historic scan within 12 months not available
• Females of child-bearing potential: must have confirmed negative pregnancy test at Screening and Baseline and willing to practice abstinence or use 2 forms of birth control for duration of study and 30 days after discontinuing study drug
• Hormonal/IUD and barrier with spermicide
• Males with a partner of childbearing potential: must use a condom for the duration of treatment and for at least 48 hours after discontinuing study drug
• Able to communicate effectively with study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements

• Pregnant or lactating
• Primary obstructive disease: pre-bronchodilator FEV1/FVC <0.70
• Diagnosis of connective tissue disease-ILD or combined pulmonary fibrosis and emphysema
• >10L/min of supplemental oxygen at rest at Baseline
• Receipt of any prostacyclin therapy, IP receptor agonist, ERA, or PDE5-I or sGC stimulator within 60 days prior to Baseline (washout permitted)
• Intermittent use of PDE5-1 for ED allowed
• Shown intolerance or significant lack of efficacy to a prostacyclin or prostacyclin analogue that resulted in discontinuation or inability to effectively titrate therapy
• Myocardial infarction within 6 months prior to Baseline or unstable angina within 30 days prior to Baseline
• Use of any of the following medications:
  • Azathioprine (AZA), cyclosporine, mycophenolate mofetil, tacroliumus, oral corticosteroids (OCS) >20 mg/day or the combination of OCS+AZA+N-acetylcysteine within 30 days prior to Baseline
  • Cyclophosphamide within 60 days prior to Baseline
  • Rituximab within 6 months prior to Baseline
• Exacerbation of IPF or active pulmonary or upper respiratory infection within 30 days prior to Baseline
• Antibiotic or steroid regimens for infection or acute exacerbation must be completed more than 30 days prior to Baseline
• Must have been discharged more than 90 days prior to Baseline if hospitalized for an acute exacerbation of IPF or a pulmonary or upper respiratory infection
• Any condition that would interfere with the interpretation of study assessments or would impair study participation or cooperation

• Age 18 - 75 y, BMI 19.0 - 32.0 kg/m2 (inclusive)
• WHO Group 1 PH (PAH):
• Right heart catheterization (RHC) at screening or within 30 days of screening with the following hemodynamics:
  • PAP ≥ 25 mmHg, PCWP ≤ 15 mmHg, & PVR of ≥ 5 WU
• NYHA/WHO Functional capacity Class II-III
• No evidence of thromboembolic disease
• No more than 2 medications from the following classes: Endothelin receptor antagonists (eg, ambrisentan, bosentan, macitentan), phosphoesterase type 5 inhibitors (eg, sildenafil, tadalafil), guanylate cyclase stimulator (eg, riociguat)
• Stable PH medications for 60 days
• FVC ≥70% within 1 year of Screening

At least two 6MWTs at Screening between 150 - 550 m, both values are w/i 15% of each other

• PH Group 1 as described in IC
• Hypersensitivity / contraindication to TPIP or TRE or mannitol
• Previous intolerance to prostacyclin analogs or receptor agonists (eg, selexipag)
• QTcF interval > 480 ms
• Known ventricular or supraventricular tachyarrhythmia (except for paroxysmal atrial fibrillation), or any symptomatic bradycardia
• LVEF ≤ 40%
• SBP < 90 mmHg
• Renal or liver disease
• HIV or Hep B or C
• Triple therapy of endothelin receptor agonists, phosphoesterase type 5 inhibitors, and guanylate cyclase stimulators

MDI Use

• Proper administration technique - spacers not permitted

Reproduction

• Pregnancy test to be performed on all women of childbearing potential.
• Acceptable contraceptive measures

Permitted Asthma Meds During Screening

• Run-in BFF MDI BID and albuterol prn only;
• SCS or ICS for the treatment of an asthma exacerbation (defined use)

eDiary Compliance

• 14 day compliance ≥70% during screening

Respiratory Infection During Screening:

• Completed treatment more than 4 weeks prior to randomization (V5)

Asthma Exacerbation During Screening

• Completed treatment with SCS and/or additional ICS more than 4 weeks prior to V5

Asthma History

• Life- threatening asthma
• Hospitalization for asthma w/in 2 months of V1

Nebulizer Usage

• Regular use of a nebulizer or a home nebulizer for receiving asthma medications

Oral B2- agonist Usage

• Use of oral 2-agonist within 3 months of V1

SCS Usage

• Oral and IV CS use (any dose) except for exacerbation within 4 weeks of V1

Immuno-modulators/ immuno-suppressive Usage

• Use of any immunomodulators or immunosuppressive meds within 3 months or 5 half-lives, whichever is longer, and not permitted during study duration

Medical conditions

• Historical or current evidence of a clinically significant disease; clinically relevant abnormal findings on P.E., clinical labs, or ECG

Eye Disorders

• Narrow angle glaucoma not adequately treated and/or change in vision that may be

relevant, in the opinion of the Investigator, w/in 3 months of V1

Smoking history

• Current smokers, former smokers with >10 pack-years hx or who stopped smoking <6 months prior to V1 (includes marijuana, e-cigs & vaping devices)