Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis.

Objective: We attempted to evaluate the feasibility (therapeutic efficacy, tolerance, and clinical courses after treatment) of pirfenidone, an anti-fibrotic drug for patients with chronic pulmonary fibroses such as idiopathic pulmonary fibrosis (IPF).

Methods: Open-label one-year treatment for compassionate-use.

Patients Or Materials: Oral pirfenidone (40 mg/kg body weight) was administered to 8 patients with advanced IPF and 2 with interstitial pneumonia associated with diffuse systemic sclerosis.