GKT137831 in IPF Patients With Idiopathic Pulmonary Fibrosis

Prior treatment

• The occurrence of any acute infection requiring systemic antibiotic therapy within 2 weeks prior to Screening (Visit 1).
• Treatment with any investigational agent within 4 weeks of Screening (Visit 1) or 5 half-lives of the investigational medicinal product (whichever is longer).
• Undergoing transplantation evaluation, or listed with the United Network for Organ Sharing (UNOS) as a lung transplantation candidate at the time of enrollment in this trial.

Other illness

• History of malignancy, excluding basal or squamous cell skin cancer and low-risk prostate cancer, the latter defined as stage T1 or T2a, with prostate specific antigen \<10 ng/dl. NOX inhibition is not known to promote cancer, and these criteria are within current guidelines.
• Treatment for \>14 days within the preceding month with \>20 mg. prednisone (or equivalent) or any treatment during the last month with a cellular immunosuppressant (e.g., cyclophosphamide, methotrexate, calcineurin inhibitors, etc.), given increased risks of opportunistic infections.
• Fertile women who do not agree to contraception or abstinence, or who are breast feeding. IPF is a disease of older adults, and male predominant, so this will not be a frequent consideration.
• Subjects with known hypersensitivity to GKT137831 or its excipients (e.g. capsule "bulking" agents).
• Severe cardiovascular disease, defined as any of the following within the preceding 12 weeks: acute myocardial infarction or unstable angina, a coronary revascularization procedure, congestive heart failure (NYHA Class III or IV), or stroke, including a transient ischemic attack.
• End-stage renal disease requiring dialysis.

Lab values

• A history of bone marrow disorder including aplastic anemia, or marked anemia defined as hemoglobin \< 10.0 g/dL (or 6.2 mmol/L).
• Evidence of cardiac conducting abnormalities, defined as second or third degree atrial-ventricular (AV) block not successfully treated with a pacemaker, or a personal or family history of long QT syndrome (QTc interval \>450 msec for males or 470 msec for females).
• Liver function tests (transaminases, alkaline phosphatase, direct and total bilirubin) \>3x upper limit of normal values

Other

• Diagnosis of major comorbidities expected to interfere with study participation

• Age between 40-85 years old.
• A diagnosis of IPF that fulfills current American Thoracic Society (ATS) Consensus Criteria.
• IPF duration \<5 years, based on the date of definitive diagnosis.
• Ability and willingness to give informed consent and adhere to study requirements.
• Ratio of forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) \>70% of predicted values