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If you have a connective tissue disease like scleroderma, you may be wondering how it affects your body. You might have symptoms like swollen hands and feet, rigid joints, and digestive issues, so it may not be surprising to hear that scleroderma can also affect your lungs and cause a condition called pulmonary arterial hypertension (PAH). PAH, one form of pulmonary hypertension (PH), occurs when your lungs’ arteries narrow, causing high blood pressure in your lungs.
Several myPHteam members have reached out to others, asking about their experiences living with scleroderma and PAH. “Does anyone have an autoimmune issue causing PH?” asked one member.
Another replied, “My PAH is related to my connective tissue diseases. I have CREST syndrome (a form of scleroderma), rheumatoid arthritis, and discoid lupus.”
In this article, we’ll cover what scleroderma is and how it’s related to PAH. We’ll also go over why you’re more likely to develop PAH if you’re living with scleroderma and how your doctors treat these conditions. A pulmonologist (lung specialist) and rheumatologist (joint and muscle specialist) can help.
Your body’s immune system works hard to protect you from invading bacteria and viruses. When you have an infection, it kicks into overdrive and creates inflammation to kill off the organisms making you sick. However, this process can sometimes go wrong. This happens when your immune system mistakenly targets your own healthy cells, resulting in autoimmune disease.
This is what happens with scleroderma, a rare autoimmune disease that can affect your skin, joints, muscles, and internal organs. Doctors and researchers aren’t quite sure what triggers scleroderma, but they believe it may be a combination of genetics, environment, and hormones.
However, health experts know that scleroderma arises when the immune system is stimulated. It causes an overproduction of collagen — a protein found in your skin, bones, and muscles that provides structure and support to your tissues — and leads to symptoms like skin and joint pain. Women are more likely than men to develop scleroderma, according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases.
There are two main types of scleroderma:
Localized scleroderma affects only the skin and the tissues directly beneath it. Types include morphea, which causes oval or round patches of hard, discolored skin, and linear scleroderma, where the skin thickens in a band or line, often on the arms, legs, or forehead.
Systemic scleroderma (also known as systemic sclerosis) can involve the entire body, including the lungs, heart, digestive system, and kidneys.
Systemic scleroderma has two forms — limited cutaneous scleroderma and diffuse cutaneous scleroderma. They are defined by which areas of the skin they affect.
Limited cutaneous scleroderma tends to develop slowly and is generally milder than the diffuse form. The limited form affects the skin covering your face, hands, fingers, lower arms, and lower legs. This condition is also referred to by the acronym CREST, which stands for:
Diffuse cutaneous scleroderma symptoms, on the other hand, come on suddenly. You’ll likely first notice thickened skin covering your fingers and toes, then spreading to your arms and legs. This type of scleroderma is associated with more internal organ damage, including the lungs.
Your lung arteries and blood vessels are responsible for carrying oxygen-poor blood away from your heart to your lungs. There, the blood is replenished with oxygen and sent back to your heart to be pumped to the rest of your body.
In systemic scleroderma, changes in the cells that line your blood vessels cause them to narrow and become inflamed. This leads to increased blood pressure in these arteries, a condition known as PAH.
PAH is one of the main types of pulmonary hypertension. According to the World Health Organization (WHO), idiopathic PAH — or PAH without a clear cause — accounts for around half of PAH cases. The other half are caused by underlying health conditions like scleroderma. Connective tissue diseases are the most common cause of associated PAH, or PAH caused by other health conditions.
Studies estimate that between 7 percent and 19 percent of people with systemic scleroderma have PAH. If you’re living with scleroderma, it’s important to be aware of common PAH symptoms. In the beginning, most cases are too mild to cause noticeable signs. As your disease progresses, you may begin to experience symptoms like:
If you experience PAH symptoms while living with scleroderma, it’s important to talk with a healthcare provider. They can perform an exam and run additional tests to make a diagnosis. PAH diagnosis tests include a right heart catheterization (a procedure that uses a small, thin tube to examine your heart) and an echocardiogram to measure the pressure in your pulmonary arteries.
Fortunately, many scleroderma treatments are also effective for managing PAH. Your doctor, pulmonologist, and rheumatologist can work together to create the best treatment plan for you.
One myPHteam member asked others, “Does anybody else have PAH related to scleroderma? And if so, what medications do you take to treat both diseases?”
Another shared part of their spouse’s medication list, stating, “My husband has systemic sclerosis, interstitial lung disease, and PAH.”
If you have scleroderma and PAH, your doctor may prescribe medications that lower pulmonary blood pressure. These drugs can help open your blood vessels to relieve high blood pressure in your lungs’ arteries and treat Raynaud’s symptoms. Examples include phosphodiesterase 5 (PDE5) inhibitors and calcium channel blockers.
The U.S. Food and Drug Administration (FDA) has also approved several other types of medications to treat systemic sclerosis-associated PAH. They include endothelin receptor antagonists (ERAs), prostacyclin therapies, activin signaling inhibitors, and soluble guanylate cyclase (sGC) stimulators. These medications help relax and open your pulmonary arteries to treat high blood pressure.
Other medications used to treat scleroderma — including immunosuppressive drugs — do not directly lower pulmonary blood pressure. Instead, they help control inflammation and fibrosis in the skin, joints, and lungs. In some people with scleroderma-related lung disease, this may help slow the progression of pulmonary hypertension.
If you’re living with scleroderma, it’s important to be aware of how the condition can affect your lungs and overall health. PAH may not cause obvious symptoms at first, but catching it early can make a big difference.
If you notice new or worsening signs like shortness of breath, fatigue, or swelling in your legs, talk to your doctor. Working with a care team that includes both a rheumatologist and a pulmonologist can help you find the right treatment plan to manage both conditions and improve your quality of life.
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, over 57,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.
Are you living with scleroderma and pulmonary arterial hypertension? What symptoms do you experience? What treatments have helped you manage these conditions? Post your suggestions in the comments below, or start a conversation on your Activities page.
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I Have PAH And Sjogen Syndrome. The Doctor Tells Me My Next Step Is Ramogalin (could Be Spelled Wrong) With A Constant Pump. Scares Me.
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My scleroderma has manifested around my eye. This is causing a vision problem. Is there a way to lessen the skin thickness?
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