Cardiovascular risk is high in rheumatic diseases, and it has been shown that Sjögren’s disease (Sjögren’s) severity is associated with increased cardiovascular risk.1,2 In one study, the prevalence of cardiovascular involvement in patients with Sjögren’s was 61.9% compared to 29.7% in controls without autoimmune disease,2 which aligns with the 53% of patients who reported having cardiovascular involvement in the Living with Sjögren’s patient survey.3
This article provides an overview of heart health in people with Sjögren’s disease, including common heart attack and stroke symptoms which can mimic certain symptoms of Sjögren’s.
Common Types of Heart Disease
Heart disease is a term for the conditions that affect the heart’s structure and function. The most common types of heart disease are:
- Coronary artery disease: occurs when plaque builds up in the arteries
- Congenital heart disease: conditions that are present at birth, like fetal heart block
- Arrhythmias: irregular heartbeats caused by structural or electrical abnormalities
- Heart valve disease: disease of the heart valves
Heart disease is the leading cause of death in the United States and globally. It can ultimately lead to heart failure, which is a condition where the heart can no longer function normally and pump enough blood to the rest of the body.
Common Causes of Heart Disease
- Hypertension: a condition where the blood pressure in your arteries is too high
- Arrhythmias: irregular heart beats
- Atherosclerosis: buildup of plaque (cholesterol, blood cells, and other substances) in the arteries
- Pericarditis: inflammation of the protective sac around the heart, which can cause fluid buildup called pericardial effusion
- Myocarditis: inflammation of the heart muscle
- Myocardial fibrosis: the heart muscle develops excess scar tissue, making it stiff and less able to pump blood
- Myocardial ischemia: the heart muscle doesn't receive enough blood flow or oxygen, usually caused by atherosclerosis of the coronary artery
- Damage to heart valves
- Diabetes
- Genetic abnormalities
Sjögren’s Disease and the Heart
Although not usually thought of as a clinical manifestation of Sjögren’s, heart disease and other cardiovascular (associated with the heart and/or blood vessels) events can be major complications of autoimmune disease-related mechanisms.
Cardiovascular manifestations in Sjögren’s are believed to be due to the presence of pro-inflammatory cytokines (small signaling proteins that can cause inflammation, i.e. TNFα, IL-1β, and IL-6), mediation of autoantibodies (antibodies that attack the body’s tissues and organs, i.e. anti-Ro/SSA), and chronic inflammation. These inflammatory mechanisms can lead to atherosclerosis and other causes of cardiovascular disease, such as conduction disturbances which occur when the heart’s electrical system is not working properly (also known as heart block).4
As a result of inflammation, endothelial dysfunction plays a key role in atherosclerosis and is considered an early marker for cardiovascular disease. Endothelial dysfunction is a condition where the inner lining of the blood vessels cannot function properly and leads to narrowing of the blood vessels. It can also lead to arrhythmia due to lack of blood flow and oxygen to regions of the heart responsible for electrical signaling pathways.5
Beyond endothelial dysfunction, arterial stiffness also contributes to the development of atherosclerosis andincreases cardiovascular risk. Arterial stiffness can reduce the ability of the arteries to contract and expand. Consequently, arterial stiffness increases the risk of heart disease by increasing blood pressure and impairing coronary (blood vessels supplying blood and oxygen to the heart) blood flow. This can lead to the development of heart failure with preserved ejection fraction (HFpEF), which is where the heart muscle pumps normally (preserved ejection fraction) but cannot fill with blood properly because it is too stiff.5,6,7 HFpEF can cause fluid to back up in the lungs, vessels, and tissues of the body, which can result in shortness of breath and swelling in areas of the body including the legs.
While the inflammatory burden on people with Sjögren’s may not be as severe as other autoimmune diseases, accumulating evidence suggests that even low-grade inflammation is associated with increased risk of cardiovascular involvement.4
Common Cardiovascular Conditions in Sjögren’s
In Sjögren’s, cardiovascular involvement can manifest in many ways. Some common manifestations:
- Hypertension
- Pericardial effusion/pericarditis
- Myocardial ischemia
- Heart block
- Valvular disease
- Myocarditis
- Myocardial fibrosis
- Vasculitis
- Abnormalities in the annular apparatus- muscles that anchor the heart valves
- Valvular regurgitation
- Valvular thickening including the mitral, tricuspid, and aortic valves
- HFpEF
Incidence of Common Cardiovascular Conditions in Sjögren’s
Chronic inflammation is thought to be a cause for several cardiovascular manifestations in Sjögren’s including hypertension, arrhythmias, atherosclerosis, and pericarditis.7,8,9
Untreated hypertension increases the risk of heart attacks and strokes, which can weaken the heart muscle and contribute to heart disease. In the Living with Sjögren’s patient survey, 36% of patients reported being diagnosed with hypertension3 and other studies have shown hypertension to be prevalent in 28-50% of the Sjögren’s patient population.7
Arrhythmias can be caused by dysautonomia (abnormal response of the autonomic nervous system) or conduction disturbances. Common arrhythmias include bradycardia (slow heartbeats), atrial fibrillation (upper chambers of the heart beat irregularly), and tachycardia (rapid heartbeats). Tachycardia was reported by 16% of patients.3 Tachycardia can prevent the heart from filling with blood in between heartbeats, attribute to high blood pressure, and, ultimately, lead to heart disease. However, it may also be caused by other underlying heart conditions.
A common overlapping condition with Sjögren’s called postural orthostatic tachycardia syndrome (POTS) is associated with arrhythmia caused by dysautonomia. POTS is characterized by an excessive increase in heart rate upon standing up from a sitting or lying position. Currently, there is no direct evidence that POTS causes heart disease or heart failure.10
Atherosclerosis is a known complication for rheumatic diseases, where plaque can cause blockages in arteries and changes in blood pressure. In a small study of 37 patients with Sjögren’s, 50% of patients had atherosclerosis which was associated with circulating anti-SSA antibodies and leukopenia (lower than normal white blood cells).6
Severe complications like acute pericarditis and myocarditis, which can affect the heart’s ability to pump blood, are rare and only 1% of patients with Sjögren’s reported having pericarditis in the Living with Sjögren’s patient survey.3,7
Chronic cardiovascular conditions, such as the ones mentioned above, caused by inflammatory responses and subsequent damage to the heart can lead to heart attacks, stroke, and heart failure.
While these cardiovascular complications are serious, many of them can be managed with a healthy diet and drug treatments specific to each condition, like blood pressure medication, anti-arrhythmic medications, and others. If you suspect cardiovascular involvement, be sure to discuss these immediately with your physician.
Signs and Symptoms of Heart Attack and Stroke
Since the symptoms of Sjögren’s are complex and often mimic symptoms of other conditions, it is important to be aware of the sudden appearance of any of the following symptoms of heart attack and stroke. Some heart attacks can be intense and immediate, while others can slowly start with mild pain or discomfort. It is also important to note that heart attack and stroke can present differently in men and women.
Heart Attack Symptoms
A heart attack, also known as a myocardial infarction, occurs when blood flow is reduced or stopped to the heart. The lack of oxygen can cause heart cells to die, weaken the heart, and prevent normal function.
Symptoms11:
- Chest discomfort: discomfort feeling like pressure, fullness, or pain in the center of the chest that can last for more than a few minutes, which can be intermittent and come back
- Discomfort in the arm, back, neck, jaw, or stomach: can be pain, numbness, and/or pressure
- Shortness of breath: can occur with or without chest discomfort
- Lightheadedness
- Cold sweat
- Nausea
- Rapid or irregular heartbeat
- Fatigue
Differences between men and women
Women often have a challenging time recognizing that they are having a heart attack as their symptoms may be more subtle. Men often have chest pain as their first indicator of a heart attack, while women may experience anxiety, upset stomach, pain in the shoulder, back, or arm, and unusual fatigue before other more common signs of a heart attack.11
Since many symptoms of Sjögren’s disease overlap with those of a heart attack, it can be challenging for patients to recognize a true cardiac event. If you have chest pain that lasts more than 15 minutes and isn't relieved by rest, you should call 911.
Stroke Symptoms
A stroke, also known as a cerebrovascular event, occurs when oxygen is reduced or stops flowing to the brain. Without oxygen to the brain, brain cells can die causing brain damage, disability, or death. During a stroke, a condition known as stroke-heart syndrome can also occur where not only is the brain damaged, but this damage can lead to disruption in the neural pathways that regulate the heart and cause the heart to function abnormally. The subsequent damage to the heart can lead to arrhythmias, heart failure, and sudden cardiac death.12
Symptoms12:
- Dizziness
- Numbness
- Weakness on one side of the body
- Difficulty speaking, writing, or understanding language
- Drooping on one side of the face
- Arm weakness or drift when raising both arms
- Blurred vision
- Severe headache
The American Stroke Association recommends F.A.S.T. as an acronym to recognize a stroke and act accordingly.13
F.A.S.T.:
- Face drooping: one side of the face starts to droop or become numb; try to smile and if you cannot then proceed with calling 911
- Arm weakness: one arm can be weak or numb; if you raise your arms and one arm drifts downward, this could be a sign of a stroke
- Speech difficulty: unable to speak, slurred speech
- Time to call 911: if any of these symptoms are present, even if they go away, call 911 and get to a hospital immediately
Cardiovascular involvement, as with many other conditions, is greatly underdiagnosed in women and in Sjögren’s. For a more in depth look at Heart Disease and Sjögren’s, you can read more in our article here.
References:
- Garcia A, Trevisani V, Dardin L, et al. AB0547 Cardiovascular Risk in Patients with Sjogren's Syndrome. Annals of Rheumatic Diseases. 2015;74:1082-1083.
- Santos CS, Salgueiro RR, Morales CM, Castro CÁ, Álvarez ED. Risk factors for cardiovascular disease in primary Sjögren's syndrome (pSS): a 20-year follow-up study. Clin Rheumatol. 2023;42(11):3021-3031. doi:10.1007/s10067-023-06686-6
- Living with Sjögren’s: Summary of Patient Survey. The Sjögren’s Foundation; 2022. www.sjogrens.org
- Sun G, Fosbøl EL, Yafasova A, et al. Long-term risk of heart failure and other adverse cardiovascular outcomes in primary Sjögren's syndrome. J Intern Med. 2023;293(4):457-469. doi:10.1111/joim.13595
- Casian M, Jurcut C, Dima A, Mihai A, Stanciu S, Jurcut R. Cardiovascular Disease in Primary Sjögren's Syndrome: Raising Clinicians' Awareness. Front Immunol. 2022;13:865373. Published 2022 Jun 9. doi:10.3389/fimmu.2022.865373
- Vaudo G, Bocci EB, Shoenfeld Y, et al. Precocious intima-media thickening in patients with primary Sjögren's syndrome. Arthritis Rheum. 2005;52(12):3890-3897. doi:10.1002/art.21475
- Bartoloni E, Baldini C, Schillaci G, Quartuccio L, Priori R, Carubbi F, et al. Cardiovascular Disease Risk Burden in Primary Sjögren’s Syndrome: Results of a Population-Based Multicentre Cohort Study. J Intern Med. 2015;278(2):185–92. doi: 10.1111/joim.12346
- Juarez M, Toms TE, De Pablo P, Mitchell S, Bowman S, Nightingale P, et al. Cardiovascular Risk Factors in Women With Primary Sjögren’s Syndrome: United Kingdom Primary Sjögren’s Syndrome Registry Results. Arthritis Care Res. (Hoboken) 2014;66:757–64. doi: 10.1002/acr.22227
- Augusto KL, Bonfa E, Rodrigues Pereira RM, Bueno C, Piren Leon E, Santos Trindade Viana V, et al. Metabolic Syndrome in Sjögren’s Syndrome Patients: A Relevant Concern for Clinical Monitoring. Clin Rheumatol. 2016;35:639–47. doi: 10.1007/s10067-015-3072-1
Cardiovascular risk is high in rheumatic diseases, and it has been shown that Sjögren’s disease (Sjögren’s) severity is associated with increased cardiovascular risk.1,2 In one study, the prevalence of cardiovascular involvement in patients with Sjögren’s was 61.9% compared to 29.7% in controls without autoimmune disease,2 which aligns with the 53% of patients who reported having cardiovascular involvement in the Living with Sjögren’s patient survey.3
This article provides an overview of heart health in people with Sjögren’s disease, including common heart attack and stroke symptoms which can mimic certain symptoms of Sjögren’s.
Common Types of Heart Disease
Heart disease is a term for the conditions that affect the heart’s structure and function. The most common types of heart disease are:
Heart disease is the leading cause of death in the United States and globally. It can ultimately lead to heart failure, which is a condition where the heart can no longer function normally and pump enough blood to the rest of the body.
Common Causes of Heart Disease
Sjögren’s Disease and the Heart
Although not usually thought of as a clinical manifestation of Sjögren’s, heart disease and other cardiovascular (associated with the heart and/or blood vessels) events can be major complications of autoimmune disease-related mechanisms.
Cardiovascular manifestations in Sjögren’s are believed to be due to the presence of pro-inflammatory cytokines (small signaling proteins that can cause inflammation, i.e. TNFα, IL-1β, and IL-6), mediation of autoantibodies (antibodies that attack the body’s tissues and organs, i.e. anti-Ro/SSA), and chronic inflammation. These inflammatory mechanisms can lead to atherosclerosis and other causes of cardiovascular disease, such as conduction disturbances which occur when the heart’s electrical system is not working properly (also known as heart block).4
As a result of inflammation, endothelial dysfunction plays a key role in atherosclerosis and is considered an early marker for cardiovascular disease. Endothelial dysfunction is a condition where the inner lining of the blood vessels cannot function properly and leads to narrowing of the blood vessels. It can also lead to arrhythmia due to lack of blood flow and oxygen to regions of the heart responsible for electrical signaling pathways.5
Beyond endothelial dysfunction, arterial stiffness also contributes to the development of atherosclerosis andincreases cardiovascular risk. Arterial stiffness can reduce the ability of the arteries to contract and expand. Consequently, arterial stiffness increases the risk of heart disease by increasing blood pressure and impairing coronary (blood vessels supplying blood and oxygen to the heart) blood flow. This can lead to the development of heart failure with preserved ejection fraction (HFpEF), which is where the heart muscle pumps normally (preserved ejection fraction) but cannot fill with blood properly because it is too stiff.5,6,7 HFpEF can cause fluid to back up in the lungs, vessels, and tissues of the body, which can result in shortness of breath and swelling in areas of the body including the legs.
While the inflammatory burden on people with Sjögren’s may not be as severe as other autoimmune diseases, accumulating evidence suggests that even low-grade inflammation is associated with increased risk of cardiovascular involvement.4
Common Cardiovascular Conditions in Sjögren’s
In Sjögren’s, cardiovascular involvement can manifest in many ways. Some common manifestations:
Incidence of Common Cardiovascular Conditions in Sjögren’s
Chronic inflammation is thought to be a cause for several cardiovascular manifestations in Sjögren’s including hypertension, arrhythmias, atherosclerosis, and pericarditis.7,8,9
Untreated hypertension increases the risk of heart attacks and strokes, which can weaken the heart muscle and contribute to heart disease. In the Living with Sjögren’s patient survey, 36% of patients reported being diagnosed with hypertension3 and other studies have shown hypertension to be prevalent in 28-50% of the Sjögren’s patient population.7
Arrhythmias can be caused by dysautonomia (abnormal response of the autonomic nervous system) or conduction disturbances. Common arrhythmias include bradycardia (slow heartbeats), atrial fibrillation (upper chambers of the heart beat irregularly), and tachycardia (rapid heartbeats). Tachycardia was reported by 16% of patients.3 Tachycardia can prevent the heart from filling with blood in between heartbeats, attribute to high blood pressure, and, ultimately, lead to heart disease. However, it may also be caused by other underlying heart conditions.
A common overlapping condition with Sjögren’s called postural orthostatic tachycardia syndrome (POTS) is associated with arrhythmia caused by dysautonomia. POTS is characterized by an excessive increase in heart rate upon standing up from a sitting or lying position. Currently, there is no direct evidence that POTS causes heart disease or heart failure.10
Atherosclerosis is a known complication for rheumatic diseases, where plaque can cause blockages in arteries and changes in blood pressure. In a small study of 37 patients with Sjögren’s, 50% of patients had atherosclerosis which was associated with circulating anti-SSA antibodies and leukopenia (lower than normal white blood cells).6
Severe complications like acute pericarditis and myocarditis, which can affect the heart’s ability to pump blood, are rare and only 1% of patients with Sjögren’s reported having pericarditis in the Living with Sjögren’s patient survey.3,7
Chronic cardiovascular conditions, such as the ones mentioned above, caused by inflammatory responses and subsequent damage to the heart can lead to heart attacks, stroke, and heart failure.
While these cardiovascular complications are serious, many of them can be managed with a healthy diet and drug treatments specific to each condition, like blood pressure medication, anti-arrhythmic medications, and others. If you suspect cardiovascular involvement, be sure to discuss these immediately with your physician.
Signs and Symptoms of Heart Attack and Stroke
Since the symptoms of Sjögren’s are complex and often mimic symptoms of other conditions, it is important to be aware of the sudden appearance of any of the following symptoms of heart attack and stroke. Some heart attacks can be intense and immediate, while others can slowly start with mild pain or discomfort. It is also important to note that heart attack and stroke can present differently in men and women.
Heart Attack Symptoms
A heart attack, also known as a myocardial infarction, occurs when blood flow is reduced or stopped to the heart. The lack of oxygen can cause heart cells to die, weaken the heart, and prevent normal function.
Symptoms11:
Differences between men and women
Women often have a challenging time recognizing that they are having a heart attack as their symptoms may be more subtle. Men often have chest pain as their first indicator of a heart attack, while women may experience anxiety, upset stomach, pain in the shoulder, back, or arm, and unusual fatigue before other more common signs of a heart attack.11
Since many symptoms of Sjögren’s disease overlap with those of a heart attack, it can be challenging for patients to recognize a true cardiac event. If you have chest pain that lasts more than 15 minutes and isn't relieved by rest, you should call 911.
Stroke Symptoms
A stroke, also known as a cerebrovascular event, occurs when oxygen is reduced or stops flowing to the brain. Without oxygen to the brain, brain cells can die causing brain damage, disability, or death. During a stroke, a condition known as stroke-heart syndrome can also occur where not only is the brain damaged, but this damage can lead to disruption in the neural pathways that regulate the heart and cause the heart to function abnormally. The subsequent damage to the heart can lead to arrhythmias, heart failure, and sudden cardiac death.12
Symptoms12:
The American Stroke Association recommends F.A.S.T. as an acronym to recognize a stroke and act accordingly.13
F.A.S.T.:
Cardiovascular involvement, as with many other conditions, is greatly underdiagnosed in women and in Sjögren’s. For a more in depth look at Heart Disease and Sjögren’s, you can read more in our article here.
Glossary
References: