Idiopathic pulmonary fibrosis (IPF) – isn’t it time we sat up and paid attention?

This is how Dr Hilary Jones ends his foreword in Inspiration, a landmark report published on February 29th 2016 to mark Rare Disease Day and designed to raise awareness of the fatal lung disease Idiopathic Pulmonary Fibrosis.1

In the UK alone, more than 15,000 people have IPF,2 characterised by permanent scarring of the lungs and loss of lung function over time.3 Yet despite these relatively large numbers, the average time to diagnosis is one to two years following the onset of symptoms – partly because the symptoms of IPF are similar to those of other, more common, lung conditions like asthma and COPD.Half of patients with the condition will not live more than three years from diagnosis.4

What might make IPF stand out though is the distinctive crackling sound that a doctor might hear when they put a stethoscope to the chest of a presenting patient.4

Although classed as a rare disease, the scale of the condition is such that it is forecast there will be 20,000 hospital admissions related to IPF in 2020, costing the NHS £20 million,5 and patient groups Action for Pulmonary Fibrosis and the British Lung Foundation – which participated in the report alongside patients and leading clinicians – believe much more could be done to raise awareness of the need for early and accurate diagnosis of IPF.

Why is it important to sit up and pay attention?

The relief often felt by patients on receiving a diagnosis of IPF can be short-lived when they realise the prognosis may be as bad as, or even worse than, it is for lung cancer.6 There are limited treatment options and no cure. Worse still, no-one knows the cause. On top of this the number of people with IPF and other interstitial lung diseases is increasing every year.4 But awareness among GPs, and difficulties in diagnosis, mean this is likely to be just the tip of the iceberg.

This was the driving force behind the production of Inspiration.

Inspiration, developed by Boehringer Ingelheim and pharmaphorum media, is the result of a collaboration between patients, patient organisations and leading clinicians, and can be found here:


  1. Inspiration, Boehringer Ingelheim. Available here: [last accessed March 2016]
  2. The British Lung Foundation, and the British Lung Foundation report, Lost in the system – IPF: the patient experience in England ( ) [Last accessed March 2016]
  3. NHS Choices Pulmonary Fibrosis (Idiopathic) [Last accessed March 2016]
  4. NHS Choices Pulmonary Fibrosis (Idiopathic) [Last accessed March 2016]
  5. Navaratnam V et al “The Increasing Secondary Care Burden of Idiopathic Pulmonary Fibrosis: Hospital Admission Trends in England From 1998 to 2010,” Chest Journal, vol. 143, no. 4, 2013.
  6. Richeldi L (2015) Idiopathic pulmonary fibrosis: moving forward. BMC Medicine 13:231 [Last accessed February 2016)