Guidance
Key priorities for implementation
Key priorities for implementation
The following recommendations have been identified as priorities for implementation.
Awareness of clinical features of idiopathic pulmonary fibrosis
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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:
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age over 45 years
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persistent breathlessness on exertion
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persistent cough
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bilateral inspiratory crackles when listening to the chest
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clubbing of the fingers
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normal spirometry or impaired spirometry usually with a restrictive pattern but sometimes with an obstructive pattern.
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Diagnosis
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Diagnose idiopathic pulmonary fibrosis only with the consensus of the multidisciplinary team (listed in table 1), based on:
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the clinical features, lung function and radiological findings (see recommendation 1.2.1 in the section on diagnosis)
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pathology when indicated (see recommendation 1.2.4 in the section on if a confident diagnosis cannot be made).
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Stage of diagnostic care pathway |
Multidisciplinary team composition (all healthcare professionals should have expertise in interstitial lung disease) |
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After clinical evaluation, baseline lung function and CT |
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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 |
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When considering results of bronchoalveolar lavage, transbronchial biopsy or surgical lung biopsy |
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See the full guideline for more information on the expertise of the multidisciplinary team.
Information and support
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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.
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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
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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
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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:
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information and support (see recommendation 1.3.1 in the section on information and support)
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symptom relief
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management of comorbidities
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withdrawal of therapies suspected to be ineffective or causing harm
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end of life care.
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If the person is breathless on exertion consider assessment for:
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the causes of breathlessness and degree of hypoxia and
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ambulatory oxygen therapy and long-term oxygen therapy and/or
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pulmonary rehabilitation.
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Disease-modifying pharmacological interventions
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For recommendations on pirfenidone, see NICE's technology appraisal guidance on pirfenidone for the treatment of idiopathic pulmonary fibrosis. For recommendations on nintedanib, see NICE's technology appraisal guidance on nintedanib for the treatment of idiopathic pulmonary fibrosis.
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Do not use any of the drugs below, either alone or in combination, to modify disease progression in idiopathic pulmonary fibrosis:
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ambrisentan
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azathioprine
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bosentan
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co-trimoxazole
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mycophenolate mofetil
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prednisolone
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sildenafil
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warfarin.
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Lung transplantation
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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
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In follow-up appointments for people with idiopathic pulmonary fibrosis:
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assess lung function
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assess for oxygen therapy
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assess for pulmonary rehabilitation
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offer smoking cessation advice, in line with NICE's guideline on tobacco
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identify exacerbations and previous respiratory hospital admissions
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consider referral for assessment for lung transplantation in people who do not have absolute contraindications (see the recommendations in the section on lung transplantation)
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consider psychosocial needs and referral to relevant services as appropriate
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consider referral to palliative care services
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assess for comorbidities (which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension).
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