Up to 3 in every 20 people with a connective tissue disease (CTD) will go on to develop pulmonary arterial hypertension (PAH). This page has been designed to provide further information and useful resources to help you learn more about CTD-PAH.
On this page you will find information on:
There are different sub-types of PAH. This page aims to provide helpful and reliable information to support you in understanding these, by explaining the conditions, their signs and symptoms, as well as their connection to other conditions.
While there is ongoing research into the cause of PAH, we do know there are four main sub-types.
Associated PAH is when the disease occurs because of the impact of living with other conditions. PAH can be associated with a variety of other conditions including; diseases that affect the tissues holding the body together (also known as connective tissue disease), birth defects that affect the heart (also known as congenital heart disease), HIV and liver disease.
Connective tissue disease (CTD)- PAH
Portopulmonary hypertension (PoPH)
If a person is at risk of developing a certain condition, a doctor may carry out tests at regular appointments, even if you are not showing any obvious symptoms, to check the likelihood of the disease developing. Screening is the term used to describe these tests.
Early diagnosis of PAH can be challenging, particularly because the main symptoms, such as breathlessness, dizziness, and tiredness can be confused with more common conditions like asthma and chronic obstructive pulmonary disease (COPD). In some cases, it can take a while to be diagnosed, and because PAH can get worse over time, the symptoms at diagnosis may be more severe and harder to manage with treatment.
Earlier screening can help improve the journey to treatment by reducing the time to a PAH diagnosis and therefore providing the opportunity for the disease to be better managed or for its progression to be slowed.
Learning the signs and symptoms of PAH is important so you know when to speak to your doctor.
For more information about how PAH is diagnosed, click here.
Collagen is a protein found in the tendons, ligaments, skin, eyes, bone, and blood vessels.
Elastin is a stretchy protein that resembles a rubber band and is the major component of ligaments and skin.
There are more than 200 different types of CTD, although those most commonly associated with PAH include:
Localised, affecting just the skin.
Systemic sclerosis (SSc) where blood vessels and internal organs can also be affected. If the heart and lungs are affected by SSc, it can put you at increased risk of developing PAH. You can find out more about SSc and PAH below:
The symptoms of scleroderma can usually be controlled by a range of different treatments, however, while treatments exist, there is currently no cure.
Also known as lupus, is a disease that can cause inflammation of the connective tissue in every organ of the body, from the brain, skin, blood, to the lungs. While numbers vary, it is estimated that about one in 12 people with SLE may develop PAH, and it is nine times more common in women than in men.
If you have been diagnosed with SLE or MCTD, make sure you speak with your doctor if you have any concerns about PAH, or are showing symptoms as highlighted above. They will be able to support you in providing guidance and carry out the appropriate tests. If you have SSc, you can find further information on SSc-PAH below.