Lupus, the Heart and Blood Vessels

Lupus can significantly impact the heart and blood vessels, known as the cardiovascular system. Understanding the relationship between lupus and cardiovascular health is essential for comprehensive disease management and improving patient outcomes. Lupus, a complex autoimmune disease, is known for its diverse symptoms and manifestations. These can affect multiple organs and systems within the body, including the cardiovascular system.

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This blog post article was edited and contributed to by Donald Thomas, MD; author of “The Lupus Encyclopedia.” Parts of this blog post come from “The Lupus Encyclopedia: A Comprehensive Guide for Patients and Health Care Providers, edition 2“

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The Lupus Encyclopedia and Lupus Cardiovascular Problems

In “The Lupus Encyclopedia,” Chapters 11 and 21 delve into the intricate connections between lupus and the cardiovascular system. These chapters provide valuable insights into the various ways in which lupus can affect cardiac health and vascular function, the heart and blood vessels.

Below is an excerpt from chapter 11:

SLE can cause inflammation in the pericardium called pericarditis. Peri-­ comes from the Greek word for “around,” cardio refers to the heart, and “-­itis” means “inflammation.” Like pleuritis (chapter 10), it is one of the SLE classification criteria (chapter 1).

Up to 60% of SLE patient autopsies show evidence of pericarditis. However, only around 25% develop pericarditis symptoms, meaning that while most SLE patients get pericarditis, most of those affected do not realize it. Just as pleuritis is the most common lung prob­lem in SLE, pericarditis is the most common heart prob­lem from lupus inflammation.

The Lupus Encyclopedia (2nd ed.), p. 223

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Toni Braxton: Un-Break My Heart Singer and Lupus Heart Disease

Several well-known celebrities are affected by severe systemic lupus erythematosus. This includes Nick Cannon (lupus nephritis), Selena Gomez (required kidney transplantation for lupus nephritis), and Toni Braxton. Grammy award winner Toni Braxton received her lupus diagnosis after developing severe lupus heart involvement. Though she developed such severe SLE, she is a fighter, a true lupus warrior. Need inspiration and motivation if winning against lupus? Click on Nick Cannon’s link about and watch his story and how he overcame lupus by taking his medications but also worked on diet, exercise, etc. His lupus was severe, just jump to the 2-minute mark at the 1st episode of his lupus journey (link above). You’ll see how he had such massive edema from lupus membranous nephritis that his legs looked like elephant trunks.

Pericarditis: Inflammation Around the Heart

One of the most common cardiac manifestations of lupus is pericarditis. It is characterized by inflammation of the pericardium—the thin sac surrounding the heart. Pericarditis can cause chest pain (worse with breathing in and when lying down), shortness of breath, and other symptoms, making it an essential consideration in assessing patients with lupus. While pericarditis may occur in up to 60% of lupus patients (in autopsy studies), not all individuals will experience noticeable symptoms, highlighting the need for vigilant monitoring and early intervention. Around 25% of SLE patients develop symptoms of pericarditis.

Understanding Atherosclerosis in Lupus

Atherosclerosis, the buildup of plaque in the arteries, is another common cardiovascular complication in individuals with lupus affecting the heart and blood vessels. Chronic inflammation and immune dysregulation associated with lupus can accelerate the progression of atherosclerosis. This, in turn, increases the risk of heart attacks, strokes, and other cardiovascular events.

These cardiovascular events occur ten to twenty years sooner in SLE patients compared to people their age and sex. Complications from cardiovascular events are one of the top causes of premature death in SLE. That is why learning about these problems and how to prevent them is of utmost importance.

Managing traditional cardiovascular risk factors such as hypertension, hyperlipidemia, and smoking is crucial for reducing risk in lupus patients for the heart and blood vessels.

Know Your Numbers

Make sure to “know your numbers.” Ask your doctor your numerical goals for your blood pressure, total cholesterol, LDL cholesterol, lipoprotein a, and hemoglobin A1C. If your numbers are not at goal, ask your doctors what you can do to improve them. In addition to eating a heart-healthy diet, exercising 150 minutes a week, and not smoking, medications like statins (to lower cholesterol), blood pressure, and diabetes medicines may be needed.

However, other factors, such as blood vessel inflammation from lupus, lupus’ abnormal effects on cholesterol, and the presence of antiphospholipid antibodies, play a huge role. Controlling lupus disease activity is essential in reducing the possibility of heart attacks, strokes, and blood clots.

Taking an antimalarial drug like hydroxychloroquine (HCQ) is essential. Hydroxychloroquine reduces the risk of heart attacks, strokes, blood clots, diabetes, and antiphospholipid antibody levels in lupus patients. An Italian study (Fasano et al) showed that taking low-dose aspirin and HCQ daily greatly decreased heart attacks and strokes. Therefore, unless my patients have a contraindication to taking aspirin, I recommend that my patients take 81 mg of aspirin daily with their HCQ. Ask your doctor if it is safe for you to do so as well.

Tips in preventing heart attacks from lupus:

• Do everything to control your lupus (Follow my Lupus Secrets)
• Ask your doctor if it is safe for you to take 81 mg aspirin with your hydroxychloroquine
• Eat a healthy anti-inflammatory diet
• I like to tell my patients, “I’d love to see you become an exercise machine.” The fountain of youth is “the water fountain in the gym.” If you are unsure how to exercise safely and effectively for your health condition, ask your doctor for a physical therapy referral. They will design a personalized program for your situation.
• KNOW YOUR NUMBERS! Ask your doctor what your numerical goals are. If not PERFECT, ask what you need to do to get them perfect. This includes taking the necessary medicines. Numerical goals include:
  • LDL cholesterol
  • total cholesterol
  • lipoprotein a
  • blood pressure
  • HbA1C
  • body fat percentage on body composition testing (BMI is not accurate)

Diagnosing and Managing Cardiovascular Complications

Diagnosing and managing cardiovascular complications in lupus requires a multidisciplinary approach involving rheumatologists, cardiologists, and other healthcare professionals. Diagnostic tests such as echocardiography, electrocardiography (ECG), and cardiac MRI can help assess cardiac function and identify abnormalities. Treatment strategies may include anti-inflammatory medications, immunosuppressive therapy, and lifestyle modifications to reduce cardiovascular risk factors.

Enhancing Cardiovascular Care in Lupus

In conclusion, understanding the complex interplay between lupus and the heart and blood vessels is essential for optimizing patient care and outcomes. By recognizing the signs and symptoms of cardiovascular complications early, healthcare providers can intervene promptly and implement appropriate management strategies. Through ongoing research and collaboration, we can continue to expand our understanding of how lupus affects the heart and blood vessels. This should allow us to develop more effective treatments to improve the lives of individuals living with this chronic autoimmune condition.

Examples of Lupus Cardiovascular Problems in “The Lupus Encyclopedia”

So many problems can affect the cardiovascular system in SLE patients. Therefore, two chapters are devoted to this topic (chapters 11 and 21) in “The Lupus Encyclopedia: A Comprehensive Guide for Patients and Health Care Providers, edition 2.” Below are some excerpts:

Postural Orthostatic Tachycardia Syndrome (POTS)

Another potential cause of sinus tachycardia is when lupus affects the autonomic nervous system.

Postural orthostatic tachycardia syndrome (POTS for short) is a type of dysfunction of the autonomic nervous system, which involves nerves that we do not have conscious control over. Examples include the nerves that cause your heart to beat or cause the reflex kick of the leg when the doctor taps the knee on physical examination.

A 2015 New York study showed that POTS patients are twice as likely to have an autoimmune disease.

When we stand up, gravity usually pulls blood downward, away from the brain. To ensure that blood remains in critical organs such as the brain, the autonomic nervous system causes the arteries in the lower part of the body to squeeze when we stand. The heart does not have to work extra hard to keep blood flowing.

What happens in POTS?

In POTS, this process does not occur properly. When someone with POTS stands, the heart rate increases (tachycardia) to keep the blood flowing and blood pressure stays around normal. This can cause fatigue, weakness, dizziness, blurred vision, heart fluttering, blue fingertips (acrocyanosis), and shakiness. The gastrointestinal autonomic nerves also seem to be affected. Some patients develop bloating, diarrhea or constipation, and stomach cramps. Chronic headaches are common, and some can pass out (called syncope).

The diagnosis of POTS and related disorders can be tricky. It is best to see someone specializing in the autonomic nervous system. A tilt-table test, in which the heart rate and blood pressure are measured while a person is lying down on a table and then measured again when the table is moved upright, can be helpful. The person with POTS will have an exaggerated increase in heart rate in the upright position, while the blood pressure remains about the same or increases.

Patients with POTS should avoid dehydration, alcohol, certain medicines, and a sedentary lifestyle. Regular exercise is essential. POTS usually causes people to become less active due to fatigue, weakness, and dizziness. As a result, their muscles and cardiovascular system become less effective (called deconditioning), which, in turn, makes POTS worse, and so a vicious cycle develops. It is vital to fight it through exercise. Even though it can be hard to exercise when you feel dizzy, weak, and tired, you should force yourself. If you have trouble with upright exercise, swimming is an option. It is crucial to start off slowly. It may take several months before you see the benefits of exercise, but it can make a big difference.

What else can help?

Many people benefit from a high salt diet (greater than 8 grams a day of sodium) along with plenty of water (more than 7 cups daily). If you have high blood pressure, do not increase salt without medical advice. You should wear compression stockings or hose daily. This helps squeeze the veins in the legs, forcing fluids up to the brain and heart. Learn how to control stress (chapter 38). Medications such as fludrocortisone (Florinef), midodrine, pyridostigmine, Adderal, droxidopa, ivabradine, and beta-blockers can help.

Whether immunosuppressants helps POTS in SLE is unknown. However, studies at the Mayo Clinic, Minnesota, have shown that IVIG and Rituxan (rituximab) help POTS and other dysautonomia problems in the related autoimmune disease Sjögren’s.

Antiphospholipid (aPL) antibodies occur seven times more often in POTS than in healthy people. Antiphospholipid syndrome (APS) experts have noted improvements of POTS in some of their APS patients when treated with blood thinners such as warfarin and aspirin. Two patients with APS responded to IVIG therapy in a 2014 United Kingdom study. The use of blood thinners and IVIG in SLE patients with aPL antibodies and POTS needs more research.

KEY POINTS TO REMEMBER

1. POTS occurs when a part of the autonomic nervous system is affected, causing an exaggerated heart rate with standing. This can lead to numerous symptoms like fatigue, weakness, dizziness, blurred vision, heart fluttering, blue fingertips (acrocyanosis), shakiness, abdominal bloating, diarrhea or constipation, stomach cramps, headaches, and passing out.
2. POTS patients are more likely to have an autoimmune disease like SLE than are other people.
3. It is diagnosed by finding an exaggerated high heart rate with normal or slightly elevated blood pressure on tilt-table testing.
4. For treatment, exercise is a must. Hydration, increased salt, wearing compression stockings, and medications can help.
5. IVIG is an interesting possible treatment, but needs more study.

Immune-Related Effects of SLE

SLE and other diseases that cause systemic inflammation (such as rheumatoid arthritis, Sjögren’s, psoriatic arthritis, and gout) cause arteriosclerosis more often and at a younger age than usual.

Blood vessel inflammation increases atherosclerosis. Lupus inflammation can promote early-onset heart disease. For example, abnormal neutrophils (a type of white blood cell, chapter 3) and interferons (a type of cytokine, chapter 3) involved with lupus inflammation and disease activity promote atherosclerosis.

SLE patients with less disease activity have a lower chance for coronary artery disease than those with high disease activity. A 2010 study showed that people with SLE with active disease developed coronary artery disease four times more often than those in remission. Therefore, we know that in addition to controlling BP, cholesterol levels, weight, diabetes, and cigarette smoking, it is essential to control lupus inflammation.

People with lupus nephritis have an exceptionally high risk of accelerated arteriosclerosis. This may be due to the increased amounts of lupus-related inflammation, the HBP that occurs with lupus nephritis, and the use of high dose steroids. Kidney disease also raises homocysteine levels, which may increase atherosclerosis (discussed below).

Lupus and vasculitis

Another lupus complication associated with arteriosclerosis is vasculitis. This is not surprising because vasculitis is due to lupus directly attacking and causing inflammation and damage to blood vessels.

People with CLE (such as discoid lupus) without the systemic form are also more likely to have CV disease and die than the general population. Although we can see just a small area of inflammation affecting the skin in these individuals, there may also be enough systemic inflammation to harden the arteries.

Antiphospholipid (APL) antibodies also increase the risk of heart attacks and strokes. This is probably related to the tendency for these antibodies to cause blood clots in arteries. One type of APL (beta-2 glycoprotein I antibody, chapter 4) can bind to oxidized LDL. These combination particles can transform white blood cells into foam cells. These foam cells contribute to the plaque buildup inside arteries with atherosclerosis.

APL antibodies may play an important role, even in people who do not have SLE. A 2018 Swedish study looking at non-SLE people who had a first heart attack showed that 1 out of 10 was positive for APL antibodies (specifically anticardiolipin and beta-2 glycoprotein I antibodies). Yet, these antibodies occurred in only 1 out of 100 people who had never had a heart attack.

Doctors prescribe blood thinners such as warfarin (Coumadin) to treat heart attacks or strokes due to antiphospholipid antibody syndrome (see chapters 9, 11, and 13).

Vitamin D Deficiency

Vitamin D deficiency is more common in SLE patients and in those with more severe and active disease. SLE patients with low vitamin D are more likely to develop arteriosclerosis and CV events than SLE patients with normal levels. One small study suggested that SLE patients may end up with less arteriosclerosis by taking vitamin D.