Idiopathic pulmonary fibrosis in adults: diagnosis and management – NICE guideline

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This National Institute for Health and Care Excellence (NICE) guideline covers diagnosing and managing idiopathic pulmonary fibrosis in people aged 18 and over. It aims to improve the quality of life for people with idiopathic pulmonary fibrosis by helping healthcare professionals to diagnose the condition and provide effective symptom management.

Key recommendations

Awareness of clinical features of idiopathic pulmonary fibrosis

  • 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
    • 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.

Diagnosis

  • Diagnose idiopathic pulmonary fibrosis only with the consensus of the multidisciplinary team, based on:
    • the clinical features, lung function and radiological findings
    • pathology when indicated.

Information and support

  • The consultant respiratory physician or interstitial lung disease specialist nurse should provide accurate and clear information (verbal and written) to people with idiopathic pulmonary fibrosis, and their families and carers with the person's consent. This should include information about investigations, diagnosis and management.
  • An interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with idiopathic pulmonary fibrosis and their families and carers with the person's consent.

Pulmonary rehabilitation

  • Assess people with idiopathic pulmonary fibrosis for pulmonary rehabilitation at the time of diagnosis. Assessment may include a 6‑minute walk test (distance walked and oxygen saturation measured by pulse oximetry) and a quality-of-life assessment.

Best supportive care

  • Offer best supportive care to people with idiopathic pulmonary fibrosis from the point of diagnosis. Best supportive care should be tailored to disease severity, rate of progression, and the person's preference, and should include if appropriate:
    • information and support
    • symptom relief
    • management of comorbidities
    • withdrawal of therapies suspected to be ineffective or causing harm
    • end of life care.
  • If the person is breathless on exertion consider assessment for:
    • causes of breathlessness and degree of hypoxia and
    • ambulatory oxygen therapy and long-term oxygen therapy and/or
    • pulmonary rehabilitation.

Disease-modifying pharmacological interventions

  • For guidance on pirfenidone for the management of idiopathic pulmonary fibrosis, refer to Pirfenidone for the treatment of idiopathic pulmonary fibrosis (NICE technology appraisal guidance 282).
  • Do not use any of the drugs below, either alone or in combination, to modify disease progression in idiopathic pulmonary fibrosis:
    • ambrisentan
    • azathioprine
    • bosentan
    • co-trimoxazole
    • mycophenolate mofetil
    • prednisolone
    • sildenafil
    • warfarin.

Lung transplantation

  • Refer people with idiopathic pulmonary fibrosis for lung transplantation assessment if they wish to explore lung transplantation and if there are no absolute contraindications. Ask the transplant centre for an initial response within 4 weeks.

Review and follow-up

  • In follow-up appointments for people with idiopathic pulmonary fibrosis:
    • assess lung function
    • assess for oxygen therapy
    • assess for pulmonary rehabilitation
    • offer smoking cessation advice, in line with Smoking cessation services (NICE public health guidance 10)
    • identify exacerbations and previous respiratory hospital admissions
    • consider referral for assessment for lung transplantation in people who do not have absolute contraindications
    • consider psychosocial needs and referral to relevant services as appropriate
    • consider referral to palliative care services
    • assess for comorbidities, which may include:
      • anxiety
      • bronchiectasis
      • depression
      • diabetes
      • dyspepsia
      • ischaemic heart disease
      • lung cancer
      • pulmonary hypertension.

The median survival for people with idiopathic pulmonary fibrosis in the UK is approximately 3 years from the time of diagnosis. However, about 20% of people with the disease survive for more than 5 years. The rate of disease progression can vary greatly. A person's prognosis is difficult to estimate at the time of diagnosis and may only become apparent after a period of careful follow-up.

This guideline contains recommendations on the diagnosis of idiopathic pulmonary fibrosis and delivery of care to people with idiopathic pulmonary fibrosis, from initial suspicion of the disease and referral to a consultant respiratory physician, to best supportive care and disease-modifying treatments.

You can read the guideline on NICE's website.