Sjogren's and Heart Health
- Sarah Schafer, MD
- Oct 11, 2022
- 4 min read
Updated: Mar 20
If you have Sjogren's, you have a higher risk of heart and blood vessel problems, which doctors call cardiovascular disease (CVD) or heart disease. These problems are a big reason why people with Sjogren's might die earlier than expected (153, 201, 218). Although the heart and blood vessels may be directly affected by Sjogren's disease, most CVDs in Sjogren's patients are comorbidities. The good news is that much can be done to lower CVD risk.
CVD comorbidities in Sjogren’s include:
The following CVDs may be direct manifestations of Sjogren’s:
myocarditis
pericarditis /pericardial effusion
valvular abnormalities
cardiac arrhythmias, including a prolonged QT interval
heart failure
pulmonary hypertension (PH) / pulmonary arterial hypertension (PAH) (both impact the heart and lungs) (218)
Congenital heart block occurs in about 2% of babies born to SSA positive mothers (75, 218).
These Sjogren’s manifestations impact the heart and blood vessels but are not usually categorized as CVDs:
Raynaud’s, which impacts 15-30% of people with Sjogren’s,
vasculitis of the skin and organs, a serious systemic feature that impacts about 10% of Sjogren's patients (3, 12), and
POTS and OH, autonomic disorders that impact the cardiovascular system (prevalent neurological manifestations of Sjogren’s).
NOTE: Comorbidities are not the same as manifestations. See Manifestations vs. Comorbidities to learn the difference between the two.
Need help with terminology? Check out the GLOSSARY, always a click away, at the bottom of every Sjogren's Advocate page.
CVDs occur at higher rates in people with rheumatic and autoimmune diseases (220).
CVDs are thought to be caused by systemic inflammation. Sjogren's is a systemic inflammatory disease, even if C-reactive protein (CRP) levels are normal. People with Sjogren’s are at increased risk of CVD even if they do not have other risk factors. Having more than one autoimmune rheumatic disease increases risk, as illustrated by Cannon et. al, (220) in Table 1, below.
Table 1: The dotted vertical line is the control group. The red bars to the right of that line show increased risk of CVD. Sjogren's carries a similar increased risk of CVD to rheumatoid arthritis and ankylosing spondylitis, but a lower risk than systemic lupus erythematosus (SLE) and systemic sclerosis. CVD risk increases with the number of autoimmune disorders one has. About 50% of people with Sjogren's will have two or more autoimmune/autoinflammatory diseases.
Sjogren’s features associated with special CVD risks.
SSA and/or SSB positive individuals appear to have a higher risk of developing CVD (221). However, Sjogren’s patients who do not have these autoantibodies should also be considered at increased risk of CVD and monitored accordingly.
Raynaud’s phenomenon, seen in 20-30% of Sjogren’s patients, is associated with an increased risk of developing pulmonary arterial hypertension (218).
Antiphospholipid syndrome (APS) increases the risk of strokes, DVT, PE, and miscarriages. APS is is less prevalent in Sjogren’s than it is in SLE but can occur. Having anti-phospholipid antibodies (aPL) does not mean you have APS. Experts disagree about whether all Sjogren’s patients should be routinely screened for aPL.
Talk with your rheumatologist or primary care clinician about monitoring and management of CVD risk factors.
Modifiable CVD risk factors include:
high blood pressure,
high cholesterol,
high triglycerides,
diabetes / prediabetes,
obesity,
sedentary lifestyle,
obstructive sleep apnea (↑ risk in Sjogren’s) (222),
medications such as corticosteroids, certain hormones, cancer treatments (discuss with your clinician),
smoking,
alcohol,
some over the counter medications and recreational drugs
pregnancy in SSA-positive patients (congenital heart block risk)
SARS Cov-2 (Covid 19) infection (see the explanation at the very end of this post).
What about hydroxychloroquine (HCQ) and the heart?
Hydroxychloroquine (HCQ) appears to lower the risk of cardiovascular disease by lowering inflammation, cholesterol, blood sugar, and the tendency to form blood clots. One study has shown a decreased risk of atherosclerosis-related heart events (e.g., heart attacks) in Sjogren’s patients (219) who take HCQ. This has been well established in SLE. There are few studies of HCQ in Sjogren’s; more research is needed. HCQ may be associated with adverse events such as cardiomyopathy and arrhythmias from QT prolongation (218), although this risk is not elevated in new users of HCQ (223). Patients with symptoms such as palpitations or syncope should be evaluated by a cardiologist. Sjogren's patients with known heart conditions should discuss the risks and benefits of HCQ use with their rheumatologist and may benefit from consultation with a cardiologist. See this blog post to learn more about benefits and risks of HCQ in Sjogren’s.
Primary Care Providers (PCPs) often take the lead on monitoring and managing CVD risk.
But many PCPs are unaware that Sjogren's is a systemic, inflammatory disease and don't know to look for CVD in Sjogren's patients. Be sure to let your PCP know that Sjogren’s disease is an independent risk factor for CVD.
Self-Advocacy Resources
Print and share "Heart Health and Sjogren's Disease" by the Sjogren's Foundation.
Print and share "Heart Disease and Sjogren's" by Dr. Nancy Carteron.
Print and share an Open Access article such as 217 or 218. For tips on how to choose and share Sjogren's information with your clinicians, see HANDOUTS FOR CLINICIANS.
See CLINICIAN HANDOUTS for even more tips and strategies for sharing information with clinicians.
Why you should avoid infection or reinfection with SARS Cov-2 (Covid-19).
Cardiovascular diseases including blood clots, strokes, and heart attacks increase 2-3-fold the year following infection with SARS-Cov2 infection (Covid-19 is the illness caused by infection), even in mild cases. There are many long term risks of infection beyond the impact on the heart and blood vessels. For a quick summary, see Your Vascular System and Covid.
Covid-19 is airborne, spread mostly through aerosols that linger for hours in unventilated indoor air.
Six-foot distancing and using hand sanitizers do little to stop the spread. This 1-minute YouTube video from the John Snow Project shows how the virus is spread and how to protect yourself and others. The public health messaging around prevention often does not reflect the current science.
Vaccines lower hospitalizations and death but are insufficient to prevent infection and long-term sequelae.
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