Pulmonary Fibrosis Treated with Inhaled Interferon-gamma (IFN-γ)

Journal of Aerosol Medicine and Pulmonary Drug Delivery
Timothy FusiakRany Condos

Abstract

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.

References

Jan 9, 2004·The New England Journal of Medicine·Ganesh RaghuUNKNOWN Idiopathic Pulmonary Fibrosis Study Group
Mar 27, 2004·American Journal of Respiratory and Critical Care Medicine·Robert M StrieterUNKNOWN Idiopathic Pulmonary Fibrosis Biomarkers Study Group
Feb 16, 2007·The European Respiratory Journal·N I ChaudharyJ E Park
Mar 1, 2012·Journal of Aerosol Medicine and Pulmonary Drug Delivery·Keith T DiazRany Condos
May 20, 2014·The New England Journal of Medicine·Luca RicheldiUNKNOWN INPULSIS Trial Investigators
May 20, 2014·The New England Journal of Medicine·Talmadge E KingUNKNOWN ASCEND Study Group

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Citations

Feb 8, 2018·Respiratory Research·Omar S UsmaniToby M Maher
May 14, 2020·Journal of Personalized Medicine·Sean D McCarthyBrendan D Higgins
Apr 5, 2019·Respiratory Research·Louise SweeneyRonan MacLoughlin
Aug 31, 2020·Chinese Medicine·Hong XiangDeshi Dong

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Methods Mentioned

BETA
biopsy
bronchoalveolar lavage

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