title: Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis.
abstract: 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.
  
  
  METHODS
  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. The plasma concentration of the drug was serially followed. Radiographic scores, pulmonary functions, and arterial blood oxygen pressure were compared at three time points: at one-year before treatment, at the time of entry, and at one-year after entry.
  
  
  RESULTS
  While pirfenidone did not show a definite therapeutic effect on overall survival (2 years after entry). During one-year treatment, there was no significant deterioration in terms of chest radiographic scores and arterial oxygen pressure, and the drug was well tolerated with minimal adverse effects within the ranges observed in this study, plasma pirfenidone concentrations did not seem to relate to the appearance of adverse effects or differences in therapeutic effects.
  
  
  CONCLUSIONS
  The feasibility of Pirfenidone as a therapeutic drug was confirmed for patients with advanced pulmonary fibrosis.
authors.affiliation: Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto.
authors.forename: Sonoko
authors.initials: S
authors.lastname: Nagai
authors.forename: Kunio
authors.initials: K
authors.lastname: Hamada
authors.forename: Michio
authors.initials: M
authors.lastname: Shigematsu
authors.forename: Masayosi
authors.initials: M
authors.lastname: Taniyama
authors.forename: Shitotomo
authors.initials: S
authors.lastname: Yamauchi
authors.forename: Takateru
authors.initials: T
authors.lastname: Izumi
mesh_terms: D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000097:blood / Q000627:therapeutic use*; D001784:Blood Gas Analysis; D002908:Chronic Disease; D005240:Feasibility Studies; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy*; D011728:Pyridones / Q000009:adverse effects / Q000097:blood / Q000627:therapeutic use*; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone