Idiopathic pulmonary fibrosis (IPF) is a rapidly progressive interstitial lung disease of unknown origin with marked impairment of quality of life and an expected survival after diagnosis of 3.2 to 5 years. It is characterized by multiple foci of fibrotic activity with minimal inflammation. A new approach to treating this disease is needed since no therapeutic regimen including steroids has been demonstrated to be effective. Although the results of a trial using large-dose, injected interferon gamma (IFNg) have been disappointing, there is a good theoretical basis for hypothesizing that interferons might be efficacious. Lung biopsy samples have shown that interferon levels are low while levels of transforming growth factor b1 (TGFb1) are high. Interferon alpha (IFNa) can increase production of IFNg and aquaporins and inhibit (TGFb1), fibroblast activity and collagen synthesis. We have been running a trial of low-dose, orally administered IFNa (150 IU three times daily), which has minimal to no side effects. Subjects are evaluated with pulmonary function tests every 3 months and HRCTs at yearly intervals. Of the 9 subjects who have completed at least 1 year, the forced vital capacity has remained stable in 8 and the oxygen saturation after a 6-minute walk has been stable in 7 and improved in 1. One subject showing lack of progression has been followed for over 3.5 years and another for 2 years. The 8 subjects whose pulmonary function tests were stable showed no evidence of disease progression on HRCT scans. Most subjects who entered the study with a cough noted marked improvement within the first few weeks of treatment with corresponding increases in quality of life scores. These results strongly suggest that this regimen can prevent progression according to the criteria defined in the International Consensus Statement published by the American Thoracic Society.
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