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Endotyping idiopathic pulmonary fibrosis should improve outcomes for all patients with progressive fibrotic lung disease
  1. Gisli Jenkins
  1. Correspondence to Dr Gisli Jenkins, Respiratory Research Unit, Clinical Sciences Building, City Hospital Campus, Nottingham University Hospitals, Hucknall Road, Nottingham NG5 1PB, UK; gisli.jenkins{at}

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The first few years of the current millennium has seen a paradigm shift in the way people view idiopathic pulmonary fibrosis (IPF). In 2004, Raghu et al1published the first multicentre, double blind, randomised, placebo-controlled trial in IPF. Although a negative study, it taught the field much about the natural history and conduct of clinical trials in IPF. Ultimately, this study paved the way for the Clinical Studies Assessing Pirfenidone in idiopathic pulmonary fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY) studies2 that lead to the approval of Pirfenidone in Europe and eventually the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND)3 and Prospective Observation of Fibrosis in the Lung: Clinical Endpoints (IMPULSIS)4 studies, which might lead to approval for the use of two disease-modifying drugs for the treatment of IPF in both the USA and Europe. Any thought at the turn of the century that progressive fibrotic lung disease, in the absence of inflammation, might be modifiable would have been regarded with a fair degree of scepticism. The field has come far.

However, the journey has only just begun. Extrapolating data from recent epidemiological studies would suggest there has been approximately a doubling of the incidence of IPF and associated healthcare requirements since the turn of the century.5 ,6 Furthermore, patients enrolled into clinical trials represent a unique subpopulation of patients with IPF, itself a subpopulation of patients …

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  • Competing interests GJ received research grants from GlaxoSmithKline, Novartis and BiogenIdec and have provided consultancy for GlaxoSmithKline, Boehringer Ingleheim, Intermune, MedImmune and BiogenIdec.

  • Provenance and peer review Not commissioned; internally peer reviewed.

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