Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases

Respiration. 2014;88(3):199-207. doi: 10.1159/000363064. Epub 2014 Aug 9.


Background: Pirfenidone is a novel antifibrotic drug for the treatment of mild-to-moderate idiopathic pulmonary fibrosis (IPF). However, adverse events may offset treatment benefits and compliance.

Objectives: To assess recent course of disease, adverse events and compliance in patients who started pirfenidone.

Methods: In an observational cohort study, 63 patients with mild-to-moderate IPF who started pirfenidone between May 2011 and June 2013 were reviewed. Pulmonary function, adverse events and treatment compliance were recorded at each clinic visit. Disease progression was defined as a reduction of vital capacity ≥10% and/or diffusion capacity (DLCO) ≥15%.

Results: Follow-up time on pirfenidone treatment was 11 (±7) months. Sixty-six percent of the patients continued with pirfenidone monotherapy and 34% of the patients received pirfenidone combined with corticosteroids (CCS) and/or N-acetylcysteine (NAC). There was a nonsignificant reduction in mean decline of percent predicted forced vital capacity after treatment start (0.7 ± 10.9%) compared to the pretreatment period (6.6 ± 6.7%, p = 0.098). Sixty-two percent of the patients had stable disease on pirfenidone treatment. Adverse events affected 85% of the patients, leading to discontinuation of pirfenidone in 20%. Adverse events and treatment discontinuation were seen more frequently in patients with concomitant CCS and/or NAC treatment.

Conclusions: Adverse events affect the majority of patients treated with pirfenidone, but are mostly manageable with supportive measures. In this heterogeneous patient group, a nonsignificant effect of pirfenidone treatment on pulmonary function was seen, underlining the need for more data on patient selection criteria and efficacy of pirfenidone, particularly in patients with coexistent emphysema and concomitant NAC/CCS treatment.

Publication types

  • Observational Study

MeSH terms

  • Acetylcysteine / therapeutic use
  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
  • Cohort Studies
  • Disease Progression
  • Drug Eruptions / etiology
  • Drug Therapy, Combination
  • Expectorants / therapeutic use
  • Fatigue / chemically induced
  • Female
  • Gastrointestinal Diseases / chemically induced
  • Germany
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Male
  • Medication Adherence
  • Middle Aged
  • Pyridones / therapeutic use*
  • Retrospective Studies
  • Tertiary Care Centers
  • Treatment Outcome
  • Vital Capacity
  • Weight Loss


  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents, Non-Steroidal
  • Expectorants
  • Pyridones
  • pirfenidone
  • Acetylcysteine