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

  • Diagnose idiopathic pulmonary fibrosis only with the consensus of the multidisciplinary team (listed in table 1), based on:

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 chapter 6.5 (Multidisciplinary Team) in 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.

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 (see recommendation 1.3.1)

    • 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:

    • 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 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 (see recommendations 1.5.16 and 1.5.17 )

    • 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)