This document discusses systemic lupus erythematosus (SLE) and its effects on the heart. SLE is a chronic inflammatory disease that can affect multiple organs, including the heart. It can cause valvular heart disease, pericarditis, myocarditis, and conduction defects. SLE is also associated with an increased risk of accelerated atherosclerosis and coronary heart disease. Reasons for this include traditional risk factors, steroid use, inflammation from SLE, and certain autoantibodies that affect blood vessels and cholesterol levels. Screening and monitoring of cardiac involvement is important for lupus patients.

1. Maryann Lee, MD
UPG Rheumatology
Staten Island, NY

2. Systemic lupus erythematosus is a chronic inflammatory disease
of unknown cause that can affect any organ in the body
including the skin, joints, kidneys, lungs, nervous system, and
the heart.

3. Lupus affects more women than men usually in their 20s and
30s during their child bearing years.
It is characterized by periods of remissions and chronic or acute
relapses or “flares”
Common symptoms are fatigue, fever, weight loss, joint and
muscle pain, sores in the mouth or nose, rashes the most
common of which is the “butterfly” or malar rash after sun
exposure, and Raynauds or microvasospasm of the small blood
vessels especialy in the hands or feet.
Lupus can affect any organ especially the kidneys and heart so
your Rheumatologist is always on the lookout for any signs of
problems in these organ systems.

4. Lupus can be like the game of telephone.
The initial order from
your body’s immune
system may be protective
but somewhere down the
road the order gets
changed and things take
a wrong turn.
http://www.flickr.com/photos/fuffer2005/3006563299/lightbox/

5. Something derails the normal message and the body attacks itself in lupus and
makes antibodies that reacts with itself leading to the symptoms/disease of
lupus. These antibodies seem to only be markers of lupus and an immune
system problem . These antibodies have not been found to cause lupus on their
own and so the cause of lupus is still unknown.

6. These antibodies can then travel throughout the
body and trigger other chemical signals which
leads to inflammation in different parts of the
body and can cause damage in these different
organs including the heart in various ways

7. Valvular disease
The heart has 4 valves that control the flow of blood circulating through
your body
These valves can be injured by bacterial infection or by immune complexes
of antibody/antigen reactions that occur during lupus activity.
More inflammation is then triggered and in the process of healing
, fibrosis, scarring and calcification can occur, which can ultimately
damage the valves
Often this is asymptomatic to the patient but over time valves can become
stiff or floppy and not work as effectively leading to heart failure

8. These damaged valves are also more prone to bacterial infection
or sometimes fragments of deposits on the valves caused by the
lupus inflammatory process can break off and cause emboli
leading to strokes
Patients with antiphospholipid antibodies are also at a higher
risk for endocarditis perhaps from increased deposition of
antibody complexes

9. Antibiotic prophylaxis for lupus patients with valvular lesions
is not an absolute recommendation by the AHA but is suggested
especially when the patient is undergoing procedures that may
cause transient bacteremia which include:
Invasive dental procedures/surgeries
Surgery of the respiratory tract such as tonsillectomy or
bronchoscopy
Invasive surgery/procedure of the GI tract

10. Steroids and other immunosuppressive therapy does not seem
effective for these valve deposits.
Anticoagulation may be helpful for Antiphospholipid antibody
associated valve deposits.
Patients with cardiac symptoms suggestive of valve disease
should undergo echocardiogram testing however screening of
asymptomatic patients is not recommended

11. It is usually asymptomatic and found incidentally but is the most frequent
cause of symptomatic cardiac disease
Pericarditis refers to inflammation of the lining around the heart .
Symptoms include positional substernal chest pain with abnormal heart
sounds on exam.
Pericardial effusion will occur at some point at least half of lupus patients
Pericarditis/pericardial effusion usually occurs when lupus is active

12. Usually it is a benign condition and is treated with
NSAIDS/anti-inflammatories and sometimes steroids with
success
If fluid has built up significantly around the heart surgical
drainage would be required
Myocarditis occurs when the heart muscle is inflamed and is a
much more serious condition but fortunately occurs rarely but
can be treated with immunosupressant medications and would
need be treated aggressively to prevent irreversible heart
damage

13. Conduction defects where the electrical system of the heart is
disrupted sometimes occurs as a result of myocarditis episode.
Usually it is a result of inflammation and fibrosis of the
conduction system of the heart and can lead to arrythmias such
as heart block.
Most often it is a first degree heart block and often short term
and reverses with time. Higher degree heart block are not
usually seen in adult lupus patients.

14. Neonatal lupus is observed in 1-2 % of babies born to mothers with
autoimmune disease. There seems to be an association with Ro and La
antibodies carried by the mother being transferred the the fetus
There is an increased incidence up to 17% in mothers with Ro and La
antibodies who already had a previous child with heart block. Still this is
a minority of births.
Symptoms include a facial/body rash that can occur up to 4 months old
Cardiac manifestations such has heart block usually occurs in utero or in
the neonatal period. It is less common after birth ( a Toronto based study
reported an incidence after birth of only 5%) This heart block is
irreversible and life threatening and requires aggressive fetal monitoring
during pregnancy.

15. Patients with SLE have been found to have an increased risk of
accelerated atherosclerosis leading to coronary heart disease
and heart attack
Several studies have given reports of up to double the risk of
heart disease in young women with SLE in their 30s and 40s as
opposed to other women at that age without SLE

16. With improvements in treatments of other life-threatening
organ disease caused by lupus such as kidney failure coronary
artery disease is emerging as the leading cause of morbidity and
premature death in patients with SLE
Reasons for this are likely multifactorial:
Traditional risk factors such as diabetes, high
cholesterol, hypertension, family history, obesity, sedentary
lifestyle, cigarette smoking are still important modifiable risk
factors
Other factors special to lupus include steroid use and lupus
itself are factors as well

17. We know now that atherosclerosis itself is an inflammatory
process with activation of the immue system and inflammation
driven processes that lead to the plaques that causes blockages
in the arteries of the heart
Image from medscape.org

18. We also know that Lupus specific factors likely play a role here.
Certain antibodies associated with lupus stimulates the production
and storage of cholesterol
Lupus also affects your blood vessels and how they respond to stress
and injury and affect their inherent repair mechanisms
SLE-Related Risk Factors
Standard Risk Factors
Systemic inflammation
Age
Autoantibodies to endothelium,
Hypertension
HDL, phospholipids
Hyperlipidemia
Circulating immune complexes
Hyperglycemia
Activated complement products
Smoking
Nephritis
Genetic factors
Dyslipidemia
Image from
Hahn, B. n engl j
med
349;25, december
Macrophage Monocyte
18, 2003
Calcium
Foam cell
Endothelium
Oxidized
LDL
HDL
Smooth-muscle cells

19. Numerous studies have shown an increased prevalence in Lupus
patients of certain risk factors for heart disease including
Hypertension
Diabetes
Premature menopause
Sedentary lifestyle
At risk body habitus
Metabolic syndrome (central obesity, high cholesterol, diabetes)

20. Steroid use- can cause or worsen traditional risk factors like
high cholesterol, diabetes, and obesity
- dose and duration of steroid use may be an important
factor with higher dose and longer use may also be a
maker of more active SLE
Active SLE which indicates an active inflammatory state may
also in itself be a risk factor for accelerated atherosclerosis and
heart disease

21. Cholesterol screening
Stress test and echocardiograms
CT of the heart looking for calcification
Carotid artery ultrasound
Cardiac mri

22. Check your cholesterol levels regularly
Current recommendations suggest treating SLE patients as having a
cardiac equivalent which means treating to goals of a LDL cholesterol
less than 100 with cholesterol lowering drugs
Certain trials have shown statins which are effective cholesterol
lowering drugs also have an anti-inflammatory effect
Plaquenil which is a useful medication in the treatment of several
manifestations of lupus has also been shown to have lipid lowering
effects – C. Tang, T. Gofrey, et. al, Int Med Journal, July 2012; 968-978.

23. Diabetes – control glucose with medications and diet and lifestyle changes
Hypertension control with medications, diet and lifestyle changes
IF possible reduce steroid dose as much as possible
Stop smoking
Weight control and reducing obesity
Healthy well balanced diet – low fat, high fiber diet incorporating fresh fruits
and vegetables and lean proteins is the way to go. there is no proven “anti-
inflammatory diet”
Exercise regularly – improves blood pressure, lowers cholesterol, improves
fatigue, aids weight loss