Sjogren's patients have greater incidence of cardiovascular diseases (CVDs), which are a major factor in early mortality (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. The article, Heart Disease and Sjogren's by Dr. Nancy Carteron can be printed and shared with clinicians. For tips on how to choose and share Sjogren's information with your clinicians, see the Handouts For Clinicians Page.
CVD comorbidities in Sjogren’s include
CVDs are increased in most rheumatic and autoimmune diseases (220). This is thought to be caused by systemic inflammation. 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) below. Explanation: 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 to rheumatoid arthritis, and ankylosing spondylitis, but lower than systemic lupus erythematosus (SLE) and systemic sclerosis. CVD risk increases with the number of autoimmune disorders. About 50% of people with Sjogren's will have two or more autoimmune/autoinflammatory diseases.
Sjogren’s is a systemic inflammatory disease. This is true even when C-reactive protein (CRP) is not elevated. CRP is usually normal in Sjogren’s (42), even in patients with severe systemic involvement. If the CRP is elevated, this is usually not related to Sjogren’s disease activity, but rather, from non-Sjogren’s causes of inflammation (217, 221). Researchers have also discovered other inflammatory markers in Sjogren's patients (104, 217), although testing in the normal clinic setting does not typically include those markers. Sjogren's and high CRP are both associated with increased CVD risk.
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 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) is less prevalent in Sjogren’s than it is in SLE but can occur. APS increases the risk of strokes, DVT, PE, and miscarriages. Note that 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.
The following CVDs may be direct manifestations of Sjogren’s (218)
This topic has been poorly studied in Sjogren’s. There may be subclinical changes that are subtle/ not detected such as pericardial effusions.
myocarditis
pericarditis, /pericardial effusion
valvular abnormalities
cardiac arrhythmias including prolonged QT interval.
heart failure
pulmonary hypertension/ pulmonary arterial hypertension (Ph and PAH impact both heart and lungs) (218)
Congenital heart block occurs in about 2% of babies born to SSA positive mothers (75, 218).
Sjogren’s manifestations that impact the heart and blood vessels but are not usually categorized as CVDs
Raynaud’s impacts 15-30 % with Sjogren’s.
Vasculitis of the skin and organs is a serious systemic feature, impacting about 10% (3, 12).
Autonomic disorders impacting the cardiovascular system such as POTS and OH are prevalent neurological manifestations of Sjogren’s.
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
IMPORTANTLY, HCQ appears to lower the risk of cardiovascular disease. by lowering inflammation, cholesterol, blood sugar, and the tendency to form blood clots.
Women of reproductive age Discuss pregnancy planning and/or prevention, especially if you are SSA positive or have been diagnosed with APS.
Importantly, 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 w/SARS-Cov2 infection (Covid-19 is the illness caused by infection), even in mild cases. Vaccines lower hospitalizations and death but are insufficient to prevent infection and long-term sequelae. The public health messaging around prevention often does not reflect the current science. Covid-19 is airborne, spread mostly through aerosols that linger in unventilated indoor air for hours. 6-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.
Primary care providers (PCPs) often take the lead on monitoring and managing CVD risk.
Be sure to let your PCP know that Sjogren’s disease is an independent risk factor for CVD. Many are unaware that Sjogren's is a systemic inflammatory disease. You might want to print out an Open Access article such as 217 or 218 to share with them. See the Clinician Handouts page for tips and strategies for sharing information.
What about hydroxychloroquine (HCQ) and the heart?
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.
Need help with terminology? Check out the Glossary, always a click away at the bottom of every Sjogren's Advocate page.
~ Sarah Schafer, MD and Sjogren's patient