Pulmonary Fibrosis Treated with Inhaled Interferon-gamma (IFN-γ).
Parenteral IFN-γ was unsuccessful as a treatment for pulmonary fibrosis. Inhaled IFN-γ targeted to the lungs may be more effective. Our patient, a 56-year-old male with biopsy proven usual interstitial pneumonia (UIP) and declining pulmonary function tests (PFTs) was initially diagnosed with idiopathic pulmonary fibrosis (IPF). He enrolled in a 2-year research protocol and was treated with inhaled IFN-γ (100 μg, Actimmune, Horizon Pharma, Deerfield, IL) 3 times per week. After completion of the protocol, he was able to secure the drug and continued therapy for a total of 7 years. He felt better, returning to work. His only complaint was transient cough during inhalation. PFTs improved (e.g., DLCO, 58% at baseline, 81% at 2 years, 69% currently). Clinical monitoring showed preserved exercise tolerance and stable CT scans. He was ultimately diagnosed (year 5) with scleroderma-like connective tissue disease after he developed sclerodactyly and a positive antinuclear antibody. Inhaled IFN-γ was well tolerated for 7 years and may stabilize fibrotic lung disease.
1 Division of Pulmonary, Critical Care and Sleep Medicine, State University of New York at Stony Brook , Stony Brook, New York.
Timothy
T
Fusiak
1 Division of Pulmonary, Critical Care and Sleep Medicine, State University of New York at Stony Brook , Stony Brook, New York.
Gerald C
GC
Smaldone
2 Division of Pulmonary and Critical Care Medicine, New York University School of Medicine , New York, New York.
Rany
R
Condos
D000280:Administration, Inhalation; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology; D007371:Interferon-gamma / Q000008:administration & dosage*; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged
D007371:Interferon-gamma
aerosols; interstitial lung disease; nebulizers; pulmonary function test