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Pulmonary arterial hypertension (PAH)

Pulmonary arterial hypertension (PAH)

Pulmonary arterial hypertension (PAH)

What is pulmonary arterial hypertension (PAH)?


Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension (PH).1

PH is a general term used to describe high blood pressure in the lungs from any cause, whereas PAH is a serious and progressive condition characterised by increased blood pressure in the blood vessels between the heart and the lungs.2 It is a rare disease, and around 55 people per million – nearly 4,000 people – are affected in the UK.3,4 PAH can occur at any age, although on average people tend to receive a diagnosis between 30-60 years old.

Pulmonary hypertension can be split into five main groups as it can be caused by different factors:4

  • Group 1: pulmonary arterial hypertension
  • Group 2: pulmonary hypertension associated with left heart disease
  • Group 3: pulmonary hypertension associated with lung diseases and/or hypoxia
  • Group 4: pulmonary hypertension associated with chronic pulmonary artery obstruction
  • Group 5: pulmonary hypertension with unclear and/or multifactorial mechanisms

Group 1 PAH is caused when the arteries in the lungs become narrowed, thickened or stiff.1 The right side of the heart must work harder to push blood through these narrowed arteries, and this extra stress can cause the heart to become less effective at pumping blood to the lungs and body.1

There are six main types of PAH:4


  • Idiopathic PAH: Happens spontaneously, with no obvious cause.5 This is the most common type of PAH4
  • Heritable PAH: Caused by a genetic problem that runs in the family4
  • Associated PAH: Develops in association with other medical conditions, including connective tissue diseases like scleroderma and lupus, congenital heart disease, HIV and liver disease1
  • Drug- and toxin-induced PAH: Brought on by exposure to recreational drugs or other toxic substances4
  • PAH with features of venous/capillary (PVOD/PCH) involvement4
  • Persistent PH of the newborn4

What are the main symptoms of PAH?6

Shortness of breath

Finding you are out of breath going up the stairs, or walking a distance you used to be able to comfortably

Tiredness

Feeling a lack of energy disproportionate to the activity you’re doing

Feeling faint or dizzy

Light-headedness or feeling off-balance

Chest pain (angina)

Tightness, tension, or pain in the chest

A racing heartbeat (palpitations)

A higher-than-normal heartrate, due to the heart working harder

Swelling (oedema) in legs, ankles, feet or tummy (abdomen)

Swollen feet and legs, and noticing indentations from socks, or where you press on your ankles or shins


As the early symptoms of PAH are common to many other conditions, and may only happen after physical activity, it can be difficult to diagnose the disease.4,7 The onset of PAH can also be so gradual that symptoms go unnoticed for a long time, until the condition is more advanced.8 Screening people who are at risk of developing the disease can therefore lead to earlier diagnosis, support and care.4

From diagnosis to living with PAH related to systemic sclerosis - The PAH Podcast series

Janssen has worked together with patients, healthcare professionals and Scleroderma & Raynaud’s UK to produce a podcast series exploring PAH and the related condition, scleroderma.

Take a listen to the PAH podcast series below to hear from real people living and working with the disease, exploring their journeys from symptoms, to diagnosis, to living life with SSc-PAH.





Scleroderma & Raynaud's UK is the only UK health charity dedicated to improving the lives of people with Scleroderma and Raynaud’s. For more information and support around diagnosis, screening for PAH and managing the condition, visit their website.

Information about chronic thromboembolic pulmonary hypertension (CTEPH)


There are five main groups of pulmonary hypertension, as it can be caused by different factors.9 Group 4 Pulmonary hypertension can sometimes be caused by scars from previous blood clots that narrow or block the pulmonary arteries, this is called chronic thromboembolic pulmonary hypertension. A blood clot that blocks one of the blood vessels that supply your lungs is called a pulmonary embolism (PE).10,11 Janssen has developed the following materials with input from Thrombosis UK, offering further information about PE. This material is intended for use by people diagnosed with PE.

Information for patients with a confirmed diagnosis of pulmonary embolism (PE).
Recovering from a pulmonary embolism (PE): information for patients with ongoing breathlessness.
Finding your new normal: life after pulmonary embolism (PE).
  1. Pulmonary Hypertension Association. Types of PulmonaryHypertension. Available at: https://phassociation.org/patients/aboutph/types-of-ph/. Last accessed August 2024.
  2. PulmonaryHypertension Association. About Pulmonary Hypertension. Available at: https://phassociation.org/types-pulmonary-hypertension-groups/. Last accessed August 2024.
  3. Office for National Statistics. UK population. Available at: https://www.ons.gov.uk/. Last accessed August 2024.
  4. Humbert M et al, (2022), 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension; European Heart Journal; 00, 1–114; DOI: https://doi.org/10.1093/eurheartj/ehac237.
  5. European Lung Foundation. Pulmonary Arterial Hypertension. Available at: https://europeanlung.org/en/information-hub/lung-conditions/pulmonary-arterial-hypertension/. Last accessed August 2024.
  6. NHS. Pulmonary Hypertension. Available at: https://www.nhs.uk/conditions/pulmonary-hypertension/. Last accessed August 2024.
  7. ArmstrongI, et al. BMJ Open. 2012;2:e000806.
  8. Scleroderma & Raynaud’s UK. Systemic Sclerosis-Associated Pulmonary ArterialHypertension (SSc-PAH). Available at: https://www.sruk.co.uk/scleroderma/scleroderma-organ-involvement/pulmonary-arterial-hypertension-pah/. Last accessed August 2024.
  9. Asthma + Lung UK. Pulmonary hypertension causes. Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-hypertension/causes#group4. Last accessed August 2024.
  10. NHS England. Pulmonary hypertension– Causes. Available at: https://www.nhs.uk/conditions/pulmonary-hypertension/causes/. Last accessed August 2024.
  11. Asthma + Lung UK. What is a pulmonary embolism and what are the symptoms? Available at https://www.asthmaandlung.org.uk/conditions/pulmonary-embolism/what-symptoms. Last accessed August 2024.


The information on this page is not a substitute for medical advice. Please consult with a healthcare professional if you have any concerns.