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GPs urged to suspect pulmonary fibrosis in older patients with persistent cough

NICE is urging GPs to be alert to the clinical features of idiopathic pulmonary fibrosis (IPF) and refer patients who may have the condition promptly, in new guidance aimed at speeding up diagnosis and treatment for the condition.

In the first NICE guidance for IPF, says GPs should suspect the disease in patients aged 45 or older who present with a persistent cough, breathlessness and bilateral inspiratory crackles and ensure that patients are referred on for chest X-rays and specialist care.

IPF is relatively rare and difficult to diagnose, often requiring a consultant respiratory physician, radiologist and histopathologist to reach a consensus diagnosis. But NICE emphasises that early treatment and initiation of pulmonary rehabilitation for patients diagnosed with IPF is vital.

Around 4,000 people are diagnosed with IPF each year in the UK. The median survival is just three years from the time of diagnosis, with a fifth of patients living longer than five years.

Dr Nik Hirani, chair of the IPF guideline development group and consultant in respiratory medicine at the University of Edinburgh, said: ‘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.’


NICE recommendations for GPs

Be aware of idiopathic pulmonary fibrosis when assessing a patient with the clinical features listed below and when considering requesting a chest X-ray or referring to a specialist:

·       age over 45 years

·       persistent breathlessness on exertion

·       persistent cough – with or without sputum

·       bilateral inspiratory crackles when listening to the chest

·       clubbing of the fingers

·       normal spirometry or impaired spirometry usually with a restrictive 
pattern but sometimes with an obstructive pattern.

Source: NICE – Diagnosis and management of suspected idiopathic pulmonary fibrosis, June 2013