An Open-Label, Phase 2 Study of the Safety of Oral Pirfenidone in Patients With Pulmonary Fibrosis/Idiopathic Pulmonary Fibrosis

Fecha de inicio: 31 de agosto de 2003

General Inclusion Criteria: * Able to understand and sign an informed consent form * Understand the importance of adherence to study treatment and the study protocol, including concomitant medication restrictions, throughout the study period * Patients must be willing to travel to an approved regional center for all study-related visits Roll-Over Criteria: * Entry into study through rollover has been completed Criteria for Early Access Program patients: * Clinical symptoms consistent with IPF ≥3 months duration * Age 40 - 85, inclusive * At the time of registration with National Organization for Rare Disorders (NORD), patients with IPF must have a percent predicted forced vital capacity (FVC) of ≥50%, and percent predicted carbon monoxide diffusing capacity (DLCO) of ≥35% * At the time of enrollment in PIPF-002, (screening/baseline visit) percent predicted FVC must be ≥45%, and percent predicted DLCO must be ≥30% * High-resolution computed tomographic scan (HRCT) showing definite IPF. For patients with surgical lung biopsy showing definite or probable usual interstitial pneumonia (UIP), the HRCT criterion of probable IPF is sufficient * For patients aged \<50 years: open or video-assisted thoracoscopic (VATS) lung biopsy showing definite or probable UIP. In addition, no features supporting an alternative diagnosis on transbronchial biopsy or bronchoalveolar lavage if performed * For patients aged ≥50 years: at least one of the following diagnostic findings as well as the absence of any features on specimens resulting from any of these procedures that support an alternative diagnosis: 1) Open or VATS lung biopsy showing definite or probable UIP; 2) Transbronchial biopsy showing no features to support an alternative diagnosis; 3) Bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis