Safety Study of Oral Pirfenidone in Patients With Pulmonary Fibrosis/Idiopathic Pulmonary Fibrosis

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

To assess the safety of treatment with pirfenidone (up to 3600 mg/d) in patients with pulmonary fibrosis/idiopathic pulmonary fibrosis (PF/IPF).

This study has been designed as a rollover study to collectively include safety data from various previous studies.

In addition, InterMune has also initiated an Early Access Program to make pirfenidone available to a limited number of patients with idiopathic pulmonary fibrosis in the United States. This program is also being conducted under this protocol. Registration of patients with documented IPF has been closed as of October 2005.

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