Pulmonary arterial hypertension (PAH)
PAH is a potential complication of scleroderma, that can affect your lungs.1 Here you can find out what it is and what to be aware of.
2 min read
PAH happens because fibrosis (scarring) narrows the small blood vessels in the lungs. This causes the blood pressure to rise in the pulmonary artery (the big blood vessel that carries blood to the lungs), as it is trying to force blood through narrower blood vessels.1,2
The result of PAH is that less blood gets to the lungs, so less oxygen can get from them into the bloodstream. The heart tries to fix the problem by making its right side, which pumps blood to the lungs, work much harder.1,2
Symptoms of PAH may include:2
- Getting out of breath doing simple tasks like climbing stairs.
- Feeling very tired when going about your normal day.
- Feeling dizzy.
- Chest pain.
- Swollen legs.
- Bluish colour to your lips and skin (cyanosis).
- Racing pulse or heart palpitations.
Your doctor will be monitoring you regularly to check on the health of your heart and lungs. If you do develop PAH, your doctor will help you manage it and recommend a treatment that suits you. Some treatments for the condition are already available.3
Contact your doctor
Feeling short of breath doesn't mean you have PAH, but you must tell a doctor.4
The facts:
people with severe scleroderma might develop serious lung symptoms that affect how they go about their life.3
of people who will be affected by lung problems develop symptoms within 3 years of having scleroderma.3,5-7
of people with scleroderma are affected by PAH.8
You may also like to read:
Lung problems
Monitoring scleroderma
Treatment
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University of Michigan. Scleroderma Program. Pulmonary Hypertension in Scleroderma. Available: https://www.uofmhealth.org/conditions-treatments/rheumatology/pulmonary-hypertension-scleroderma [Accessed December 2021].
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Mayo Clinic. Pulmonary hypertension symptoms and causes. Available: http://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697 [Accessed December 2021].
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McNearney TA, Reveille JD, Fischbach M, et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum. 2007; 57:318–326.
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Scleroderma of the lungs. Royal Brompton & Harefield NHS Foundation Trust. Available: https://www.rbht.nhs.uk/our-services/scleroderma [Accessed December 2021].
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Jaeger V, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One 2016;11(10):e0163894.
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Steen V, et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994;66(6):1625–35.
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Nihtyanova S, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheum 2014;66(6):1625–35
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Denton and Hachulla. Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening. Eur Respir Rev. 2011 Dec;20(122):270-6.
"Scleroderma and pulmonary involvement show me how good it is to be alive, how good it is to wake up in the morning and breathe. We do this every day and we don't value it, and I value it a lot more now."
– Rute