Idiopathic pulmonary fibrosis is a chronic, progressive fibrotic interstitial lung disease of unknown origin. It is a difficult disease to diagnose and often requires the collaborative expertise of a consultant respiratory physician, radiologist and histopathologist to reach a consensus diagnosis. Most people with idiopathic pulmonary fibrosis experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum, is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death.
National Institute for Health and Care Excellence (NICE) has updated the guidelines on Idiopathic pulmonary fibrosis in adults: diagnosis and management in May 2017. These are the major recommendations
1.1 Awareness of clinical features of idiopathic pulmonary fibrosis
1.1.1 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.
1.2.1 Assess everyone with suspected idiopathic pulmonary fibrosis by:
- taking a detailed history, carrying out a clinical examination (see recommendation 1.1.1 for clinical features) and performing blood tests to help exclude alternative diagnoses, including lung diseases associated with environmental and occupational exposure, with connective tissue diseases and with drugs and
- performing lung function testing (spirometry and gas transfer) and
- reviewing results of chest X‑ray and
- performing CT of the thorax (including high-resolution images).
1.2.2 Diagnose idiopathic pulmonary fibrosis only with the consensus of the multidisciplinary team (listed in table 1), based on:
- the clinical features, lung function and radiological findings (see recommendation 1.2.1)
- pathology when indicated (see recommendation 1.2.4).
1.2.3 At each stage of the diagnostic care pathway the multidisciplinary team should consist of a minimum of the healthcare professionals listed in table 1, all of whom should have expertise in interstitial lung disease.
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 physicianConsultant radiologistInterstitial lung disease specialist nurseMultidisciplinary team coordinator|
|When considering performing bronchoalveolar lavage, and/or transbronchial biopsy or surgical lung biopsyOnly some patients will have bronchoalveolar lavage or transbronchial biopsy but they may be being considered for surgical lung biopsy||Consultant respiratory physicianConsultant radiologistConsultant histopathologistThoracic surgeon as appropriateInterstitial lung disease specialist nurseMultidisciplinary team coordinator|
|When considering results of bronchoalveolar lavage, transbronchial biopsy or surgical lung biopsy||Consultant respiratory physicianConsultant radiologistConsultant histopathologistInterstitial lung disease specialist nurseMultidisciplinary team coordinator|
|See chapter 6.5 (Multidisciplinary Team) in full guideline for more information on the expertise of the multidisciplinary team.|
If a confident diagnosis cannot be made
1.2.4 If the multidisciplinary team cannot make a confident diagnosis from clinical features, lung function and radiological findings, consider:
- bronchoalveolar lavage or transbronchial biopsy and/or
- surgical lung biopsy, with the agreement of the thoracic surgeon.
1.2.5 Discuss with the person who may have idiopathic pulmonary fibrosis:
- the potential benefits of having a confident diagnosis compared with the uncertainty of not having a confident diagnosis and
- the increased likelihood of obtaining a confident diagnosis with surgical biopsy compared with bronchoalveolar lavage or transbronchial biopsy and
- the increased risks of surgical biopsy compared with bronchoalveolar lavage or transbronchial biopsy.
1.2.6 When considering bronchoalveolar lavage, transbronchial biopsy or surgical lung biopsy take into account:
- the likely differential diagnoses and
- the person’s clinical condition, including any comorbidities.
1.2.7 If a confident diagnosis cannot be made continue to review the person under specialist care.
1.3 Information and support
1.3.1 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.1.3.2 NICE has produced guidance on the components of good patient experience in adult NHS services. Follow the recommendations in Patient experience in adult NHS services (NICE clinical guideline 138).1.3.3 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.1.3.4 Offer advice, support and treatment to aid smoking cessation to all people with idiopathic pulmonary fibrosis who also smoke, in line with Smoking cessation services (NICE public health guidance 10).
1.4.1 Measure the initial rate of decline in the person’s condition, which may predict subsequent prognosis, by using lung function test results (spirometry and gas transfer) at:
- diagnosis and
- 6 months and 12 months after diagnosis. Repeat the lung function tests at shorter intervals if there is concern that the person’s condition is deteriorating rapidly.
1.4.2 Discuss prognosis with people with idiopathic pulmonary fibrosis in a sensitive manner and include information on:
- the severity of the person’s disease and average life expectancy
- the varying courses of disease and range of survival
- management options available.
1.4.3 Do not use the 6‑minute walk distance at diagnosis to estimate prognosis. (The 6‑minute walk test may be useful for other purposes, see recommendation 1.5.1.)
1.5.1 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.1.5.2 Repeat the assessment for pulmonary rehabilitation for people with idiopathic pulmonary fibrosis at 6‑month or 12‑month intervals.1.5.3 If appropriate after each assessment, offer pulmonary rehabilitation including exercise and educational components tailored to the needs of people with idiopathic pulmonary fibrosis in general.1.5.4 Pulmonary rehabilitation should be tailored to the individual needs of each person with idiopathic pulmonary fibrosis. Sessions should be held somewhere that is easy for people with idiopathic pulmonary fibrosis to get to and has good access for people with disabilities.
Best supportive care
1.5.5 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.
1.5.6 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.
1.5.7 If the person is breathless at rest consider:
- assessment for the causes of breathlessness and degree of hypoxia and
- assessment for additional ambulatory oxygen therapy and long-term oxygen therapy and
- the person’s psychosocial needs and offering referral to relevant services such as palliative care services and
- pharmacological symptom relief with benzodiazepines and/or opioids.
1.5.8 Assess the oxygen needs of people who have been hospitalised with idiopathic pulmonary fibrosis before they are discharged.1.5.9 If the person has a cough consider:
- treatment for causes other than idiopathic pulmonary fibrosis (such as gastro-oesophageal reflux disease, post-nasal drip)
- treating with opioids if the cough is debilitating
- discussing treatment with thalidomide with a consultant respiratory physician with expertise in interstitial lung disease if the cough is intractable.
1.5.10 Ensure people with idiopathic pulmonary fibrosis, and their families and carers, have access to the full range of services offered by palliative care teams. Ensure there is collaboration between the healthcare professionals involved in the person’s care, community services and the palliative care team.
Disease-modifying pharmacological interventions
There is no conclusive evidence to support the use of any drugs to increase the survival of people with idiopathic pulmonary fibrosis.1.5.11 For guidance on pirfenidone, see the NICE technology appraisal on pirfenidone for the treatment of idiopathic pulmonary fibrosis. For guidance on nintedanib, see the NICE technology appraisal on nintedanib for the treatment of idiopathic pulmonary fibrosis.1.5.12 Do not use any of the drugs below, either alone or in combination, to modify disease progression in idiopathic pulmonary fibrosis:
- mycophenolate mofetil
1.5.13 Advise the person that oral N‑acetylcysteine is used for managing idiopathic pulmonary fibrosis, but its benefits are uncertain.1.5.14 If people with idiopathic pulmonary fibrosis are already using prednisolone or azathioprine, discuss the potential risks and benefits of discontinuing, continuing or altering therapy.1.5.15 Manage any comorbidities according to best practice.
1.5.16 Discuss lung transplantation as a treatment option for people with idiopathic pulmonary fibrosis who do not have absolute contraindications. Discussions should:
- take place between 3 and 6 months after diagnosis or sooner if clinically indicated
- be supported by an interstitial lung disease specialist nurse
- include the risks and benefits of lung transplantation
- involve the person’s family and carers with the person’s consent.(See recommendations 1.5.5 – 1.5.10 about best supportive care.)
1.5.17 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.
1.5.18 A respiratory physician or specialist nurse with an interest in interstitial lung disease should discuss the poor outcomes associated with mechanical ventilation (including non-invasive mechanical ventilation) for respiratory failure with people with idiopathic pulmonary fibrosis. These discussions should ideally take place between 3 to 6 months after diagnosis or sooner if clinically indicated. (See recommendations 1.5.5 – 1.5.10 about best supportive care.)1.5.19 Do not routinely offer mechanical ventilation (including non-invasive mechanical ventilation) to people with idiopathic pulmonary fibrosis who develop life-threatening respiratory failure.
1.6 Review and follow-up
1.6.1 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).
1.6.2 Consider follow-up of people with idiopathic pulmonary fibrosis:
- every 3 months or sooner if they are showing rapid disease progression or rapid deterioration of symptoms or
- every 6 months or sooner if they have steadily progressing disease or
- initially every 6 months if they have stable disease and then annually if they have stable disease after 1 year.
To read the full guidelines click on the link given below.