Key priorities for implementation

The following recommendations have been identified as priorities for implementation.

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

Table 1 Minimum composition of multidisciplinary team involved in diagnosing idiopathic pulmonary fibrosis

Stage of diagnostic care pathway

Multidisciplinary team composition (all healthcare professionals should have expertise in interstitial lung disease)

After clinical evaluation, baseline lung function and CT

  • Consultant respiratory physician

  • Consultant radiologist

  • Interstitial lung disease specialist nurse

  • Multidisciplinary team coordinator

When considering performing bronchoalveolar lavage, and/or transbronchial biopsy or surgical lung biopsy

Only some patients will have bronchoalveolar lavage or transbronchial biopsy but they may be being considered for surgical lung biopsy

  • Consultant respiratory physician

  • Consultant radiologist

  • Consultant histopathologist

  • Thoracic surgeon as appropriate

  • Interstitial lung disease specialist nurse

  • Multidisciplinary team coordinator

When considering results of bronchoalveolar lavage, transbronchial biopsy or surgical lung biopsy

  • Consultant respiratory physician

  • Consultant radiologist

  • Consultant histopathologist

  • Interstitial lung disease specialist nurse

  • Multidisciplinary team coordinator

See the full guideline for more information on the expertise of the multidisciplinary team.

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.

    Be aware that some pulse oximeters can underestimate or overestimate oxygen saturation levels, especially if the saturation level is borderline. Overestimation has been reported in people with dark skin. See also the NHS England Patient Safety Alert on the risk of harm from inappropriate placement of pulse oximeter probes.

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:

  • If the person is breathless on exertion consider assessment for:

    • the causes of breathlessness and degree of hypoxia and

    • ambulatory oxygen therapy and long-term oxygen therapy and/or

    • pulmonary rehabilitation.

Disease-modifying pharmacological interventions

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 NICE's guideline on tobacco

    • identify exacerbations and previous respiratory hospital admissions

    • consider referral for assessment for lung transplantation in people who do not have absolute contraindications (see the recommendations in the section on lung transplantation)

    • 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 and pulmonary hypertension).

  • National Institute for Health and Care Excellence (NICE)