Category
Lung

A new drug is now available for patients with idiopathic pulmonary fibrosis, to slow the deterioration in lung function.

A big step forward for IPF patients

In April 2013, the National Institute for Health and Care Excellence (NICE) approved the use of the drug, pirfenidone, for the management of IPF. As part of the European Named Patient Programme (NPP), clinicians at Royal Brompton Hospital have been able to prescribe this drug since 2011 for patients with mild to moderate IPF. NICE approval means that all patients with mild to moderate IPF in England can now be prescribed pirfenidone.

Professor Toby Maher, consultant respiratory physician explains:

"Royal Brompton is the largest prescriber of pirfenidone in the UK. As a much-needed treatment for this terrible disease, the feedback on the drug that we were able to give to NICE was very positive."

"This is a great step forward for patients, as pirfenidone is the first drug which has been shown to slow deterioration in lung function for patients with IPF." 

Royal Brompton hospital has the largest number of IPF and interstitial lung disease patients in Europe, and receives around 700 new referrals every year. Since pirfenidone has only been approved for use in some countries, consultants have seen a surge in demand from overseas patients, particularly those from the US, Middle East and Australia.

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease that causes scarring of the lungs. The cause of IPF is unclear but the condition appears to be connected with injury occurring to specific cells inside the lungs known as alveolar epithelial cells (AECs).

It is thought that these cells become damaged and then begin to die. The body tries to repair the damage by stimulating the growth of another type of cell known as a fibroblast. In individuals with IPF, the production of fibroblasts is excessive and results in scarring and hardening (fibrosis) of the delicate tissues of the lungs.

Levels of fibrosis increase as the IPF progresses, leading to a gradual decline in lung function which ultimately results in patients requiring oxygen to undertake even simple day-to-day activities. Due to the progressive nature of the disease, the median life expectancy after diagnosis of IPF is two to three years. However, some patients can live for much longer.

Did you know?

  • Pirfenidone is the first and only licensed treatment for adult patients with mild to moderate IPF.
  • Clinical trials have shown that pirfenidone can reduce the risk of disease progression by 30 per cent.

Consultant

Professor Toby Maher

Consultant respiratory physician

For more information, or to book an appointment with Professor Toby Maher, email: privatepatients@rbht.nhs.uk or call +44(0) 20 3131 5141.