GPs should assess people complaining of a persistent cough, breathlessness and respiratory cackles for idiopathic pulmonary fibrosis to speed up treatment and improve each patient's life. A new National Institute for Health and Care Excellence (NICE) clinical guideline on the diagnosis and management of idiopathic pulmonary fibrosis (IPF) recommends that healthcare professionals, particularly family doctors, should be aware of the symptoms of this disease to make sure patients have access to chest X-rays and specialists so a prompt diagnosis can be made.

Idiopathic pulmonary fibrosis is a chronic, progressive disease in which the lungs become scarred. Most people with the condition experience symptoms of breathlessness and a cough which both lead to the decline of lung function, reduced quality of life and death. Each year around 4,000 adults in the UK are diagnosed with IPF with only 20% surviving for longer than five years.

NICE also recommends that as soon as a person is diagnosed with IPF, they should be assessed to see if their quality of life can be improved though exercise and education[1]. This should be tailored to each person's needs and can improve the well-being and quality of life for people with the condition.

Professor Mark Baker, Director of the Centre for Clinical Practice at NICE, said: “Idiopathic pulmonary fibrosis is a devastating condition that significantly affects a person's life. After initial symptoms, a patient gets sicker very quickly, so it is vital they have access to a prompt diagnosis and to the correct treatments.

“The causes of this chronic progressive condition are not known, so doctors and nurses must be aware of the signs to be able to provide the best care to improve a person's quality of life.”

Nik Hirani, Chair of the IPF Guideline Development Group and Consultant in Respiratory Medicine, said: “This is a landmark document for patients with IPF, their carers and professionals across multiple disciplines. For many years IPF was considered a rare disease for which there was no treatment. Thankfully a condition that claims over 4,000 lives a year, and greatly reduces the well-being of those affected, is now gaining the attention it deserves.

“In the last 10 years or so, we've witnessed huge efforts to find treatments for lung fibrosis. There is still much to be done, but the NICE guideline distils the most important aspects of IPF diagnosis and management. I hope the recommendations are implemented and I'm certain that if they are, we will soon see the benefits.”

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Notes to Editors

About the guidance

The diagnosis and management of suspected idiopathic pulmonary fibrosis clinical guideline will be available from Wednesday 12 June 2013.

About NICE

The National Institute for Health and Care Excellence (NICE) is the independent body responsible for driving improvement and excellence in the health and social care system. We develop guidance, standards and information on high-quality health and social care. We also advise on ways to promote healthy living and prevent ill health.

Formerly the National Institute for Health and Clinical Excellence, our name changed on 1 April 2013 to reflect our new and additional responsibility to develop guidance and set quality standards for social care, as outlined in the Health and Social Care Act (2012).

Our aim is to help practitioners deliver the best possible care and give people the most effective treatments, which are based on the most up-to-date evidence and provide value for money, in order to reduce inequalities and variation.

Our products and resources are produced for the NHS, local authorities, care providers, charities, and anyone who has a responsibility for commissioning or providing healthcare, public health or social care services.

[1] Pulmonary rehabilitation is an exercise and educational programme designed to help patients with limiting symptoms due to respiratory conditions such as COPD. The aim of pulmonary rehabilitation is to improve patients' exercise tolerance and functional independence.