A Pilot Study of Aerosol Interferon-Gamma Treatment in Pulmonary Fibrosis

Inclusion Criteria:

• Patients diagnosed with IPF based on accepted criteria within 30 months prior to screening
• Age 20-70
• A failed trial of prednisone with or without cyclophosphamide/ azathioprine
• Patient taking 0-15mg prednisone or the equivalent for 28 days prior to study enrollment and willing to remain on that same dose of corticosteroid
• FVC >= 50% and <= 90% of predicted baseline value at screening
• PaO2 > 60 mm Hg at rest on room air
• Patient able to understand and willing to sign a written informed consent and willing to comply with all requirements of the study protocol including:
• Patient fits criteria for research bronchoscopy and is willing to undergo procedure
• Patient able to have medication administered three times per week at the GCRC unit at Bellevue Hospital

Exclusion Criteria:

• No adequate treatment trial with prednisone, cyclophosphamide, or azathioprine
• patient unwilling or unable to undergo research bronchoscopy
• Patient with known asthma or severe COPD
• Patient requiring oxygen therapy for maintenance of adequate arterial oxygenation
• Patient with hypersensitivity to study medication or other component medication
• Patient with known severe cardiac disease, severe peripheral vascular disease or seizure disorder which may be exacerbated by study drug administration (contraindications to drug administration as per package insert).
• Pregnant or lactating. Females of child-bearing age will be required to have negative pregnancy test and be required to use accepted form of birth control (abstinence for study duration is the preferred method).
• Evidence of active infection within one week prior to treatment
• Any condition, other than IPF, which is likely to result in the death of the patient within one year from study enrollment

• Abnormal serum laboratory values including:

  1. Liver function above specified limits: total bilirubin > 1.5 X upper limits of normal, alanine amino transferase > 3X upper limit of normal, alkaline phosphatase < 3X upper limit of normal, albumin < 3.0 at screening.
  2. CBC outside specified limits: WBC < 2,500/mm3, hematocrit <30 or >59, platelets < 100,000/mm3.
  3. Creatinine > 1.5X upper limits normal at screening
• Drugs for therapy for pulmonary fibrosis, excluding corticosteroids/cyclophosphamide/azathioprine, within the previous six months
• Prior therapy with any class of interferon medication
• Investigational therapy for any indication within the last 28 days