IPF

September 19, 2009
IPF or Idiopathic Pulmonary Fibrosis is a chronic interstitial pneumonia of unknown cause and poor prognosis. The life expectancy of IPF patients has been estimated as 2 to 3 years after diagnosis. IPF is characterized by clinical, radiologic and histologic criteria. The frequently used therapy of steroids (prednisolone) and immunosuppressants (azothioprine) has not been shown to be effective. No drugs for the therapy of IPF are approved in the USA. Bosentan, pirfenidone and N-acetyl cysteine are currently in clinical trials with preliminary results suggesting they may prolong the life expectancy of patients with IPF. New approaches to the treatment of IPF have been proposed. They include endothelial and cytokine antagonists, and antioxidants. Japanese health authorities approved pirfenidone (Shionogi) for the tretment of IPF earlier this year. Additional clinical studies are neeed for its approval by Food and Drug Administration in the USA. Pirfenidone is and old drug and its possible usefulness in the therapy of IPF has been known for over 15 years. Why does it take so long to make pirfenidone available to IPF patients in this country? My blogs will try to answer this question and explore other possible approaches to the therapy of IPF.
Alex Scriabine, MD pharmacologist and an IPF patient.