If you have a connective tissue disease like scleroderma, you may be wondering how else it affects your body. You might already have swollen hands and feet, rigid joints, and gastrointestinal issues, so it may not be surprising to hear that scleroderma can also affect your lungs and cause pulmonary arterial hypertension (PAH). This condition causes high blood pressure within your lung’s arteries.

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 (RA), and discoid lupus.” (CREST syndrome is discussed in more detail below.)

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.

1. Scleroderma Is an Autoimmune Connective Tissue Disease

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 tiny organisms making you sick. Sometimes, your immune system mistakes your healthy cells as invaders and creates extra inflammation.

This is what happens in scleroderma, a rare autoimmune disease that affects your skin, muscles, joints, and internal organs. Your immune system creates specialized proteins, or antibodies, that attack your connective tissues. These tissues are primarily made of collagen, a protein that’s the basic building block for your muscles, joints, and skin. Inflammation triggers your body to make more collagen to protect itself. As a result, scleroderma causes a buildup of excess collagen or scar tissue.

Doctors and researchers aren’t quite sure what triggers scleroderma, but they believe it may be a combination of genetics, environment, and hormones. Interestingly, women are much more likely than men to develop scleroderma, according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases.

2. Scleroderma Symptoms May Be Limited or Affect Your Entire Body

There are two main types of scleroderma, localized and systemic. Localized scleroderma affects only your skin and the tissues directly beneath. Systemic scleroderma (also known as systemic sclerosis) can involve your entire body, including your lungs, heart, digestive system, and kidneys.

Systemic scleroderma is further broken down into limited cutaneous scleroderma and diffuse cutaneous scleroderma. These two forms are defined by how quickly symptoms start and which organs 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:

• Calcinosis — A buildup of calcium deposits in connective tissues
• Raynaud’s phenomenon — A condition that causes the tiny blood vessels in your hands to contract or tighten in the cold
• Esophageal dysfunction — Trouble swallowing due to muscle dysfunction
• Sclerodactyly — Skin thickening and tightening on your fingers
• Telangiectasia — Red spots on your face and hands from swollen blood vessels

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 your lungs.

3. Scleroderma Inflammation Can Lead to Pulmonary Arterial Hypertension

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 out to the rest of your body.

In systemic scleroderma, your immune system also affects the cells lining your blood vessels. The inflammation triggers them to make more collagen as well, creating scar tissue in your lungs’ arteries. The blood vessels begin to narrow and limit the amount of blood passing through. As a result, the blood pressure level in these arteries rises, leading to PAH.

The World Health Organization (WHO) classifies PAH as one of the main types of pulmonary hypertension. Idiopathic PAH — or PAH without a clear cause — accounts for around half of PAH cases, according to the WHO. The other half are associated with an underlying health condition like scleroderma. In fact, connective tissue diseases are the most common cause of associated PAH.

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:

• Fatigue
• Shortness of breath
• Chest pain
• Dizziness or fainting
• Swelling of your legs and feet from edema (fluid buildup)
• Pounding or racing heartbeat
• Hoarseness or cough

4. Some Medications Are Beneficial for Both Conditions

If you’re experiencing PAH symptoms while living with scleroderma, it’s important to talk with your doctor. They can run additional tests to make a diagnosis and get you on the right treatment plan. Examples include right heart catheterization (a procedure using 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: “My husband has systemic sclerosis, interstitial lung disease, and PAH. He takes Ofev, Opsumit, CellCept, and sildenafil, among a few other drugs.”

If you have scleroderma and PAH, your doctor may prescribe blood pressure medications for both conditions. 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 inhibitors like tadalafil (Adcirca) and sildenafil (Revatio), as well as calcium channel blockers like diltiazem (Cardizem) and nifedipine (Procardia XL, also formerly sold as Procardia).

The U.S. Food and Drug Administration (FDA) has also approved several other medications to treat systemic sclerosis-associated PAH. They include endothelin receptor antagonists (ERAs), prostanoids, and soluble guanylate cyclase (sGC) stimulators. These medications also help relax and open your pulmonary arteries to treat high blood pressure.

ERAs are pills taken by mouth once or twice daily and include bosentan (Tracleer), ambrisentan (Letairis), and macitentan (Opsumit).

Prostanoids prescribed for PAH include:

• Selexipag (Uptravi), a pill taken by mouth once daily
• Epoprostenol (Veletri, Flolan), given as an IV infusion
• Treprostinil (Tyvaso, Orenitram, or Remodulin), taken by inhalation, mouth, or infusion

The sGC stimulator riociguat (Adempas) is taken by mouth three times a day.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 51,000 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.