• Arata Azuma.
• Fourth Department of Internal Medicine, Nippon Medical School.
• Nihon Ronen Igakkai Zasshi. 2005 Jan 1;42(1):27-30.

AbstractIdiopathic pulmonary fibrosis (IPF), a chronic form of idiopathic interstitial pneumonia, has a poor prognosis with an average life expectancy of 3-4 years from the time of diagnosis. Although patients with IPF have been treated with steroids or immunosuppressants to control the inflammation that occurs earlier in the course of disease, these drugs have not improved the survival of patients with IPF. Recently, several clinical studies of antifibrotic drugs have been conducted in patients with IPF. In Japan, we demonstrated that pirfenidone prevents deterioration of pulmonary function and significantly decreases the incidence of acute exacerbation of IPF in a well-designed, placebo-controlled, double-blind, randomized study. We have also adopted a new system for evaluation of dynamic pulmonary function, which involves measuring the lowest SpO2 level during a 6-minute walk at a constant speed and assessing the pulmonary capacity of patients with IPF. In a study of patients with pulmonary fibrosis associated with Hermansky-Pudlak syndrome in the United States, pirfenidone slowed the decrease in %FVC in patients with a %FVC of >60%, but had no significant effects on patients with a %FVC of < or =60%. Large-scale clinical studies of INF-gamma in patients with IPF in North America and Europe have reported decreases in the mortality of patients with mild IPF with a FVC of >60%, although percentages of patients with disease status rated as 'exacerbated', 'unchanged' and 'improved' after treatment did not differ between the INF-gamma and placebo groups. This presentation reported important future strategies for the treatment of IPF.

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