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Which Autoimmune Conditions Can Lead to Pulmonary Hypertension?

Posted on December 20, 2021
Medically reviewed by
Ariel D. Teitel, M.D., M.B.A.
Article written by
Joan Grossman

A growing body of research is showing important connections between some autoimmune conditions and pulmonary hypertension (PH). When your immune system attacks healthy cells, organs, and tissues in your body, you have an autoimmune condition. Autoimmune conditions are genetic disorders. Pulmonary hypertension is a chronic lung disease that is a type of high blood pressure (or hypertension).

Having both an autoimmune disease and PH is something many myPHteam members talk about. “Has anyone else who's autoimmune found out you’re a PAH (pulmonary arterial hypertension) patient? After being diagnosed with autoimmune?” one member asked.

Another member had a similar question: “Any of you have associated pulmonary arterial hypertension (APAH) — PAH secondary to scleroderma or lupus? Or some other autoimmune disorder?”

Find out how PH and autoimmune conditions are interconnected and what that could mean for you in this article.

Pulmonary Hypertension

When you have pulmonary hypertension, the right side of your heart and the arteries in your lungs are affected. Because its symptoms can resemble those of chronic obstructive pulmonary disease (COPD), getting a PH diagnosis can sometimes be difficult.

People with PH might experience symptoms such as:

  • Fatigue
  • Shortness of breath
  • Dizziness or fainting
  • Heart palpitations or pounding
  • Chest pain and pressure
  • Edema (swelling) in legs, ankles, and abdomen

Pulmonary Arterial Hypertension

The World Health Organization (WHO) sorts pulmonary hypertension into types according to the causes and characteristics of each. One such type, pulmonary arterial hypertension (PAH) is a particularly rare condition. Its exact prevalence is not known, but epidemiologists believe it occurs in approximately 2.5 to 7 cases per million people.

If you have PAH, your pulmonary arteries (blood vessels in the lungs) constrict and become stiff. This can weaken your heart muscle because your heart has to work harder to pump blood through your lungs to provide them with an adequate supply of blood. A weakened heart muscle can lead to heart failure.

The three main types of PAH are:

  • Heritable pulmonary arterial hypertension (HPAH)
  • Idiopathic pulmonary arterial hypertension (IPAH)
  • Associated pulmonary arterial hypertension (APAH)

HPAH is inherited and the cause of IPAH remains unknown. APAH happens as a result of another condition you have. (It’s “associated” with that other condition, so it’s called associated pulmonary arterial hypertension.)

Associated Pulmonary Hypertension

Associated pulmonary arterial hypertension is linked to autoimmune connective tissue diseases. Those diseases include:

  • Systemic lupus erythematosus (known as lupus)
  • Mixed connective tissue disease
  • Dermatomyositis and polymyositis
  • Primary Sjögren’s syndrome
  • Systemic sclerosis (CREST syndrome)
  • Rheumatoid arthritis
  • Thyroid autoimmune disease
  • Whipple disease
  • Antiphospholipid syndrome

Connective Tissue Diseases

When the proteins elastin and collagen in your connective tissues are attacked, you have a connective tissue disease. Connective tissues maintain the structure of your body, and include your skin, ligaments, tendons, cartilage, bones, and blood vessels.

A person who has a connective tissue disease experiences chronic inflammation. Inflammation in connective tissue can lead to symptoms — depending the condition — that can include:

  • Pain
  • Swelling
  • Stiffness
  • Fatigue
  • Shortness of breath

There is no cure for connective tissue diseases. However, your symptoms and overall disease activity may be managed with anti-inflammatory drugs. You can take these medications orally, by injection, or by infusion.

Autoantibodies and Pulmonary Hypertension

Connections between autoimmune conditions and PH have been observed for decades. More recent studies have pointed to autoimmunity existing in people with PH. There are several different causes for PH, such as heart disease and family history, but research now shows that pro-inflammatory immune cells (autoantibodies) can also be found in those diagnosed with PH. Autoantibodies — signs of immune dysfunction and autoimmunity — indicate the presence of an autoimmune condition. Even when people have idiopathic PH and no other autoimmune condition, they may have these autoantibodies.

Such findings have given researchers a new perspective on pulmonary hypertension. They hope to use these discoveries to identify new types of drug therapies that can target the overactive immune cells in people with PH — and particularly in people with PAH. Some therapies, such as endothelin antagonists, may be effective for treating both PH and certain connective tissue diseases.

Living With Comorbidities

Having a comorbidity means you have multiple diseases at the same time. Several myPHteam members have written about having the comorbidity of PH and one or more autoimmune conditions. One member said: “I have PAH, along with polymyositis, Sjögrens, hyperthyroidism, rheumatoid arthritis, and the gift of PE (pericardial effusion) in both lungs. So I have my good days and bad days.”

Another member said, “I was diagnosed with PAH and lupus, as well as scleroderma. It’s been a total change in lifestyle for me. I will always do my best to keep healthy, happy, and active.”

One myPHteam member described how their autoimmune disease affects them. “As well as having PAH as a consequence of systemic sclerosis, I also have another autoimmune disease, polymyositis. It attacks my muscles and periodically just relaxes them completely. Down I go like a puppet does when the puppeteer drops the puppet onto the stage at the end of the performance.”

Symptom Relief and Treatment

Living with PH that is associated with autoimmune disease can be challenging, but treatment strategies are evolving for these complex conditions. On myPHteam, members often share what they do to successfully manage their symptoms.

“I take Letairis for the PH, also Cartia for heart, Trelegy for lungs, and leflunomide for rheumatoid arthritis,” one member said. They went on to share that their rheumatoid arthritis (RA) caused their PH, and that their PH limits what medicines can treat their RA. That’s because, the member said, RA medicines can harm a person’s lungs. So instead of medication, that member turned to exercise for RA relief. “I have a treadmill and log as much time as possible on it,” they said.

Another member said, “I have associated pulmonary arterial hypertension, secondary to limited systemic sclerosis (scleroderma), an autoimmune disease. I take ambrisentan, Sildenafil, and furosemide for my PAH, as well as prednisone and hydroxychloroquine for my scleroderma. I also have a pericardial effusion and the prednisone and furosemide do double duty by keeping it from enlarging. I am also 100 percent pain free with this cocktail of meds.”

Said another: “I just went for my pulmonary checkup and the doc said my heart is doing very well. When I was admitted to the hospital they gave me almost no chance of making it, and wanted to put me on a transplant list. Since then, my heart is back to normal size and my blood pressure and heart rate are excellent now. I know there is no cure for the PAH, scleroderma, and lupus that I have, but I’m going to make the most of it.”

Multidisciplinary Care

If you have PH and an autoimmune disease, you have a serious medical condition that requires careful management. Each case is unique, and as such, everything from your diagnosis to your treatment and outcomes may benefit from a health care team of specialists and a multidisciplinary approach. Your team might include a cardiologist, pulmonologist, rheumatologist, and specialized nurses.

Exercise

To stay active and fit, people with PAH and autoimmune conditions can get referrals from their health care team for physical therapy or occupational therapy. Reducing your stress with mental health counseling and relaxation techniques may also help improve your quality of life and overall well-being.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension, 46,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Are you living with pulmonary hypertension and have questions about autoimmune conditions? Share your experience in the comments below, or start a conversation by posting on myPHteam.

All updates must be accompanied by text or a picture.
Ariel D. Teitel, M.D., M.B.A. is the clinical associate professor of medicine at the NYU Langone Medical Center in New York. Review provided by VeriMed Healthcare Network. Learn more about him here.
Joan Grossman is a freelance writer, filmmaker, and consultant based in Brooklyn, NY. Learn more about her here.

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