Quality statement 1: Diagnosis of idiopathic pulmonary fibrosis

Quality statement

People are diagnosed with idiopathic pulmonary fibrosis only with the consensus of a multidisciplinary team with expertise in interstitial lung disease.

Rationale

Idiopathic pulmonary fibrosis is difficult to diagnose. The main symptom is shortness of breath that gradually becomes worse. It can only be diagnosed with confidence by a specialist multidisciplinary team with expertise in interstitial lung disease. Diagnosis is based on clinical features, lung function, radiological findings and pathology if indicated. Because of the severity of idiopathic pulmonary fibrosis and its poor prognosis, being incorrectly diagnosed with this disease can cause unnecessary distress for the person and their families or carers.

Quality measures

Structure

Evidence of local arrangements to ensure the availability of a multidisciplinary team with expertise in interstitial lung disease to diagnose idiopathic pulmonary fibrosis.

Data source: Local data collection.

Process

Proportion of people diagnosed with idiopathic pulmonary fibrosis by a multidisciplinary team with expertise in interstitial lung disease.

Numerator – the number in the denominator whose condition was diagnosed with the consensus of a multidisciplinary team with expertise in interstitial lung disease.

Denominator – the number of people diagnosed with idiopathic pulmonary fibrosis.

Data sources: British Thoracic Society's BTS Lung Disease Registry Programme for idiopathic pulmonary fibrosis and NICE guideline CG163 clinical audit tool.

What the quality statement means for service providers, healthcare professionals and commissioners

Service providers (hospitals and regional specialist centres) collaborate to ensure that a multidisciplinary team comprising healthcare professionals with expertise in interstitial lung disease is available to diagnose people who have suspected idiopathic pulmonary fibrosis.

Healthcare professionals who suspect idiopathic pulmonary fibrosis refer people to a multidisciplinary team with expertise in interstitial lung disease for confirmation of the diagnosis. Healthcare professionals within the multidisciplinary team collaborate to diagnose idiopathic pulmonary fibrosis by consensus based on clinical features, lung function, radiological findings and pathology if indicated.

Commissioners (NHS England through specialised services area teams collaborating with clinical commissioning groups) commission services from regional specialist centres that have a multidisciplinary team consisting of healthcare professionals with expertise in interstitial lung disease.

What the quality statement means for patients, service users and carers

People who might have idiopathic pulmonary fibrosis receive a diagnosis only after their symptoms and any test results have been discussed by a team of healthcare professionals who specialise in diagnosing and treating lung diseases. This will help to make sure that people are given the correct diagnosis. They do not need to attend this discussion.

Source guidance

Definitions of terms used in this quality statement

Suspected idiopathic pulmonary fibrosis

The presence of clinical features of idiopathic pulmonary fibrosis. These are:

  • age over 45 years

  • persistent breathlessness on exertion

  • persistent cough

  • bilateral inspiratory crackles in the chest

  • clubbing of the fingers

  • normal or impaired spirometry, usually with a restrictive pattern but sometimes with an obstructive pattern.

[Adapted from idiopathic pulmonary fibrosis (NICE guideline CG163), recommendation 1.1.1]

Multidisciplinary team with expertise in interstitial lung disease

A multidisciplinary team that includes a consultant respiratory physician, a consultant thoracic radiologist, an interstitial lung disease specialist nurse and a multidisciplinary team coordinator. The multidisciplinary team is based in a regional specialist centre.

[Idiopathic pulmonary fibrosis (NICE guideline CG163), recommendation 1.2.3 and expert opinion]

In addition, the following healthcare professionals join the multidisciplinary team at these stages of diagnosis and treatment.

  • When considering bronchoalveolar lavage, transbronchial biopsy or surgical lung biopsy: a consultant histopathologist and a thoracic surgeon (as appropriate).

  • When considering the results of bronchoalveolar lavage, transbronchial biopsy or surgical biopsy: a consultant histopathologist.

[Idiopathic pulmonary fibrosis (NICE guideline CG163), recommendations 1.2.2 (key priority for implementation), 1.2.3 and 1.2.4]

For more information on the expertise of the multidisciplinary team see section 6.1.4 of the full guideline on idiopathic pulmonary fibrosis.

Interstitial lung disease

A group of lung diseases of known and unknown cause that are characterised by varying degrees of inflammation and fibrosis of the lung tissue.

[Full guideline on idiopathic pulmonary fibrosis]

Equality and diversity considerations

People with suspected idiopathic pulmonary fibrosis are likely to need hospital tests and investigations. Some services may not be available in local hospitals and people may need to go to specialist centres. To ensure equality of access to care, measures should be put in place to help people attend local hospitals and specialist centres, for example by providing transport and offering appointments in centres as near to the person's home as possible.