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Possible new standard of care for progressive fibrotic interstitial lung diseases (ILDs)

Study in The Lancet Respiratory Medicine shows efficacy and safety of pirfenidone, a drug that slows the disease progression in IPF patients.

ILBD/CPC-scientists around study coordinator Marion Frankenberger (ILBD/CPC-M) and author Jürgen Behr (University Hospital Munich, LMU) investigated four non-IPF ILDs. So far, for these progressive fibrotic interstitial lung diseases there are few treatment options:


  • Fibrotic non-specific interstitial pneumonia
  • Connective tissue disease associated ILD
  • Chronic hypersensitivity pneumonitis
  • Asbestos-induced lung fibrosis

Pirfenidone however has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF). In view of the pathomechanistic and clinical similarities between IPF and the other progressive fibrotic ILDs, the scientists investigated the efficacy and safety of pirfenidone versus placebo in patients aged 18-80 years. As a result of an interim analysis triggered by unanticipated slow recruitment, the study was terminated early for futility after enrolment of 127 patients. Despite this limitation, the team observed encouraging signals in primary and secondary endpoints: pirfenidone treatment seemed to be associated with benefits in lung function compared with placebo after 48 weeks of treatment. In addition, mortality and other serious adverse events, and pulmonary infections leading to adverse events, were numerically lower in the pirfenidone group.

The study adds further support for the concept that patients with progressive, non-idiopathic pulmonary fibrosis (IPF) fibrotic ILD seem to be similarly responsive to antifibrotic therapy as those with IPF. The team therefore suggests that antifibrotic therapy represents a new standard of care in treatment of progressive fibrotic ILDs.

Original publication:
Jürgen Behr et al., Lancet Respir Med, 2021: 
Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial