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Quick facts: Idiopathic pulmonary fibrosis (IPF)

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Causes, symptoms and clinical features

Is IPF a restrictive or obstructive lung disorder?

Restrictive. Idiopathic pulmonary fibrosis is a serious and progressive disease in which the alveoli (the tiny air sacs of the lungs) and the lung tissue next to the alveoli become damaged and scarred.

What is the cause of IPF?

The cause of the disease is unknown, although many are smokers.

Can IPF be cured?

Unless the patient can receive a lung transplant, IPF is incurable. Symptoms are associated with a decline in lung function, reduced quality of life and ultimately death.

What are the symptoms of IPF?

Most people with IPF have symptoms of breathlessness, which may initially occur only on exertion, and a cough, with or without sputum.

What is the predicted life expectancy for those with IPF?

The median survival for people with IPF in the UK is approximately 3 years from the time of diagnosis. However, about 20% of people with the disease survive for more than 5 years.

What are the clinical features you would expect to see in a patient with IPF?

  • 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.

Is oxygen therapy helpful for patients with IPF?

Many patients with IPF have exertional hypoxia. Where activity is significantly limited by breathlessness on exertion and associated with a drop in oxygen levels, ambulatory oxygen therapy may be helpful in enabling people to maintain levels of daily activity. Patients often need high flow rates to correct oxygen saturations and get symptom relief but may find the equipment cumbersome and difficult to manage.

Patients with low oxygen saturations at rest may need to use oxygen continuously for symptom relief.

Are there any other interventions that may be beneficial in IPF?

Treatment is aimed primarily at slowing the rate of decline, identifying and reducing the impact of co-morbidities and managing symptoms. In the UK, pirfenidone, an antifibrotic agent, is the only medication licensed for use in IPF and may be effective in some patients to slow the progression of lung scarring. It can cause a number of unpleasant side effects and patients require support and careful monitoring.

Pulmonary rehabilitation may offer some benefit in terms of addressing breathlessness associated deconditioning, loss of confidence and symptom management. Similarly, non-pharmacological breathlessness management interventions can be very helpful to reduce the impact of symptoms on quality of life and can provide an introduction to palliative care/supportive services.

What co-morbidities are commonly associated with IPF?

  • Gastro-oesophageal reflux disease;
  • Venous thromboembolism;
  • Coronary artery disease;
  • Emphysema;
  • Pulmonary hypertension;
  • Lung cancer;
  • Sleep disordered breathing;
  • Depression.

 

Further reading:

Interstitial Pulmonary Fibrosis Care Standards

Care planning in idiopathic pulmonary fibrosis

Management of idiopathic pulmonary fibrosis

British Lung Foundation (2015) Lost in the System: IPF: The Patient Experience in England.

 

Guidelines and standards

Interstitial Lung Disease Guidelines

National Institute for Health and Care Excellence (2015) Idiopathic Pulmonary Fibrosis. NICE quality standard 79.

 

Useful organisations

British Lung Foundation have useful information for patients and professionals https://www.blf.org.uk/Page/IPF

Breathing Matters: Idiopathic Pulmonary Fibrosis

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