17 April 2024
Pulmonary Fibrosis and Hypertension

I know that correctly diagnosing PH or PF can sometimes take time because its symptoms, like shortness of breath, tiredness, chest pain and swelling in the legs, are similar to those of many other heart and lung conditions. I agree that it is vital to raise awareness of pulmonary hypertension to ensure that patients are able to recognise symptoms and manage their conditions. 

Patients with pulmonary fibrosis are cared for by National Health Service regional specialist Interstitial Lung Disease (ILD) services, who are commissioned by NHS England. To be referred to a specialist service patients need to be identified in primary and secondary care. Early and accurate diagnosis is a priority for NHS England, and the work to improve this area of clinical care is underway, which should have an impact on reducing delayed diagnosis of pulmonary fibrosis.

NHS England is responsible for the commissioning of services for ILD and funds the cost of anti-fibrotic treatments to treat this disease. Access to these treatments has recently been expanded to patients with non-idiopathic pulmonary fibrosis following the publication of the National Institute for Health and Care Excellence’s technology appraisal ‘Nintedanib for treating progressive fibrosing interstitial lung diseases’ in November 2021.

The NHS Long Term Plan makes a number of commitments relating to respiratory and pulmonary diseases, including increasing the number of people receiving physical health checks by 110,000 people per year, which will in turn assist with earlier diagnosis of conditions like pulmonary hypertension.

Enabling more people with heart and lung disease to complete a programme of education and exercise based rehabilitation will result in improved exercise capacity and quality of life in up to 90 per cent of patients.

The most recent audit of services across all eight specialist PH centres in England and Scotland has shown that 10 of the 15 standards were being met nationally.   However, there is always more that can be done to improve patient experience. 

Of course, raising awareness of conditions such as pulmonary hypertension is a vital part of ensuring patients receive the support they need.


Research Funding

NHS England is responsible for the commissioning of interstitial lung disease services and funds the high cost anti-fibrotic treatments which treat these diseases, including pulmonary fibrosis. Access to these treatments have recently been widened to people with non-idiopathic pulmonary fibrosis with the publication of National Institute for Health and Care Excellence Technology Appraisal 747 in November 2021.