STX-100 in Patients With Idiopathic Pulmonary Fibrosis (IPF)

Inclusion Criteria:

1. Consenting male or female patients, 50 to 84 years old, inclusive. Patients 18 to 49 years of age are eligible if they have a histopathologic diagnosis of usual interstitial pneumonia (UIP) based upon a surgical lung biopsy in the appropriate clinical setting, and meet all other inclusion/exclusion criteria.
2. Clinical features consistent with IPF prior to screening (based on the ATS/ERS/JRS/ALAT consensus criteria for the diagnosis of IPF).
3. FVC ≥ 50% of predicted value.
4. DLco (corrected for hemoglobin) ≥ 35% predicted value.
5. Oxygen saturation > 90% by pulse oximetry while breathing ambient air at rest.
6. Residual volume ≤ 120% predicted value.
7. Ratio of FEV1 to FVC ≥ 0.65 after the use of a bronchodilator.
8. Other known causes of interstitial lung disease have been excluded (e.g., drug toxicities, environmental exposures, connective tissue diseases).
9. HRCT image fulfills the criteria for 'UIP pattern'.
10. If the HRCT image does not fulfill the criteria for 'UIP pattern' a surgical lung biopsy is necessary for the diagnosis of IPF.
11. Adequate bone marrow and liver function.
12. Patient has a life expectancy of at least 12 months.

Exclusion Criteria:

1. Findings that are diagnostic of a condition other than UIP on surgical lung biopsy, HRCT imaging, transbronchial lung biopsy, or bronchoalveolar lavage (BAL).
2. Currently receiving high dose corticosteroid, cytotoxic (e.g., chlorambucil, azathioprine, cyclophosphamide, methotrexate), anti-fibrotic (e.g., pirfenidone), vasodilator therapy for pulmonary hypertension (e.g., bosentan), unapproved (e.g., INF-γ, penicillamine, cyclosporine, mycophenolate, Nacetylcysteine), and/or investigational therapy for IPF or administration of such therapeutics within 4 weeks of initial screening. A current dose of ≤ 15 mg/day of prednisone or its equivalent is acceptable if it is anticipated the dose will remain stable during the study.
3. History of malignancy, including carcinoma during the preceding 5 years.
4. Significant cardiac disease (e.g., New York Heart Association [NYHA] Class 3 or 4; myocardial infarction within the past 6 months; unstable angina; coronary angioplasty or coronary artery bypass graft [CABG] within the past 6 months; or uncontrolled atrial or ventricular cardiac arrhythmias).
5. Serious local infection or systemic infection within 3 months prior to screening.
6. Positive test for HBsAg, HCV antibody, or HIV antibody at screening.
7. Treatment with another investigational drug, investigational device, or approved therapy for investigational use within 4 weeks of initial screening.
8. Currently listed and/or anticipated to be listed for lung transplantation within the next 6 months.