NICE is creating new clinical guidelines on the management of idiopathic pulmonary fibrosis, and is asking for views on its recommendations.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease associated with scarring of the lungs. 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 ultimately death. Each year, around 4,000 adults in the UK are diagnosed with idiopathic pulmonary fibrosis.

IPF is relatively rare and can be mistaken for other diseases – this new draft clinical guidance recommends that healthcare professionals, particularly GPs, should be aware of the clinical features of the condition to ensure patients have access to chest X-rays and specialist referrals.

NICE also says health professionals should look out for key signs and symptoms, such as, breathlessness, persistent cough, and respiratory crackles when listening to the chest, and clubbing of the fingers.

Once IPF is suspected, the NICE draft guideline recommends a diagnosis should only be made with the consensus of a multidisciplinary team, including always a chest physician, a radiologist and a specialist nurse, and that diagnosis should be based on a patient’s clinical features, lung function and radiological findings, with the addition of pathology when indicated.

NICE also provisionally recommends that at the time of diagnosis, people are assessed for pulmonary rehabilitation. It goes on to say that pulmonary rehabilitation should be tailored to each individual’s needs and can improve the wellbeing and quality of life for people with chronic breathing problems.

Professor Mark Baker, director of the centre for clinical practice at NICE, said: “Idiopathic pulmonary fibrosis is a chronic condition that significantly affects a person’s life. After initial symptoms, a person’s condition can decline rapidly, so it is vital that people have access to a timely diagnosis and access to the correct treatments and therapeutic interventions. There are no known causes of this chronic progressive condition, so healthcare professionals must be aware of the signs to be able to provide supportive care that can improve a person’s quality of life.

“We would urge all those registered as stakeholders in the development of this guideline to have their say on our draft recommendations, and submit their comments via the NICE website.” The draft consultation runs from Friday 11 January 2013 to Friday 22 February 2013, and can be found here.

In November, NICE launched a public consultation on its preliminary recommendation that InterMune’s Esbriet (pirfenidone) should not be recommended for the treatment of mild-to-moderate idiopathic pulmonary fibrosis.

The pill, the first of its kind for the disease, was deemed too expensive by the watchdog, and earned NICE a reprimand from the ABPI, which bemoaned its decision not to recommend the medicine.

A decision as to whether NICE will recommend the drug for NHS funding is expected later this year.