Maryann Lee, MDUPG Rheumatology Staten Island, NY
Systemic lupus erythematosus is a chronic inflammatory diseaseof unknown cause that can affect any organ in the bodyincluding the skin, joints, kidneys, lungs, nervous system, andthe heart.
Lupus affects more women than men usually in their 20s and30s during their child bearing years.It is characterized by periods of remissions and chronic or acuterelapses or “flares”Common symptoms are fatigue, fever, weight loss, joint andmuscle pain, sores in the mouth or nose, rashes the mostcommon of which is the “butterfly” or malar rash after sunexposure, and Raynauds or microvasospasm of the small bloodvessels especialy in the hands or feet.Lupus can affect any organ especially the kidneys and heart soyour Rheumatologist is always on the lookout for any signs ofproblems in these organ systems.
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/
Something derails the normal message and the body attacks itself in lupus andmakes antibodies that reacts with itself leading to the symptoms/disease oflupus. These antibodies seem to only be markers of lupus and an immunesystem problem . These antibodies have not been found to cause lupus on theirown and so the cause of lupus is still unknown.
These antibodies can then travel throughout thebody and trigger other chemical signals whichleads to inflammation in different parts of thebody and can cause damage in these differentorgans including the heart in various ways
Valvular diseaseThe heart has 4 valves that control the flow of blood circulating throughyour bodyThese valves can be injured by bacterial infection or by immune complexesof 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 ultimatelydamage the valvesOften this is asymptomatic to the patient but over time valves can becomestiff or floppy and not work as effectively leading to heart failure
These damaged valves are also more prone to bacterial infectionor sometimes fragments of deposits on the valves caused by thelupus inflammatory process can break off and cause embolileading to strokesPatients with antiphospholipid antibodies are also at a higherrisk for endocarditis perhaps from increased deposition ofantibody complexes
Antibiotic prophylaxis for lupus patients with valvular lesionsis not an absolute recommendation by the AHA but is suggestedespecially when the patient is undergoing procedures that maycause transient bacteremia which include:Invasive dental procedures/surgeriesSurgery of the respiratory tract such as tonsillectomy orbronchoscopyInvasive surgery/procedure of the GI tract
Steroids and other immunosuppressive therapy does not seemeffective for these valve deposits.Anticoagulation may be helpful for Antiphospholipid antibodyassociated valve deposits.Patients with cardiac symptoms suggestive of valve diseaseshould undergo echocardiogram testing however screening ofasymptomatic patients is not recommended
It is usually asymptomatic and found incidentally but is the most frequentcause of symptomatic cardiac diseasePericarditis refers to inflammation of the lining around the heart .Symptoms include positional substernal chest pain with abnormal heartsounds on exam.Pericardial effusion will occur at some point at least half of lupus patientsPericarditis/pericardial effusion usually occurs when lupus is active
Usually it is a benign condition and is treated withNSAIDS/anti-inflammatories and sometimes steroids withsuccessIf fluid has built up significantly around the heart surgicaldrainage would be requiredMyocarditis occurs when the heart muscle is inflamed and is amuch more serious condition but fortunately occurs rarely butcan be treated with immunosupressant medications and wouldneed be treated aggressively to prevent irreversible heartdamage
Conduction defects where the electrical system of the heart isdisrupted sometimes occurs as a result of myocarditis episode.Usually it is a result of inflammation and fibrosis of theconduction system of the heart and can lead to arrythmias suchas heart block.Most often it is a first degree heart block and often short termand reverses with time. Higher degree heart block are notusually seen in adult lupus patients.
Neonatal lupus is observed in 1-2 % of babies born to mothers withautoimmune disease. There seems to be an association with Ro and Laantibodies carried by the mother being transferred the the fetusThere is an increased incidence up to 17% in mothers with Ro and Laantibodies who already had a previous child with heart block. Still this isa minority of births.Symptoms include a facial/body rash that can occur up to 4 months oldCardiac manifestations such has heart block usually occurs in utero or inthe neonatal period. It is less common after birth ( a Toronto based studyreported an incidence after birth of only 5%) This heart block isirreversible and life threatening and requires aggressive fetal monitoringduring pregnancy.
Patients with SLE have been found to have an increased risk ofaccelerated atherosclerosis leading to coronary heart diseaseand heart attackSeveral studies have given reports of up to double the risk ofheart disease in young women with SLE in their 30s and 40s asopposed to other women at that age without SLE
With improvements in treatments of other life-threateningorgan disease caused by lupus such as kidney failure coronaryartery disease is emerging as the leading cause of morbidity andpremature death in patients with SLEReasons for this are likely multifactorial:Traditional risk factors such as diabetes, highcholesterol, hypertension, family history, obesity, sedentarylifestyle, cigarette smoking are still important modifiable riskfactorsOther factors special to lupus include steroid use and lupusitself are factors as well
We know now that atherosclerosis itself is an inflammatoryprocess with activation of the immue system and inflammationdriven processes that lead to the plaques that causes blockagesin the arteries of the heart Image from medscape.org
We also know that Lupus specific factors likely play a role here.Certain antibodies associated with lupus stimulates the productionand storage of cholesterolLupus also affects your blood vessels and how they respond to stressand 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
Numerous studies have shown an increased prevalence in Lupuspatients of certain risk factors for heart disease includingHypertensionDiabetesPremature menopauseSedentary lifestyleAt risk body habitusMetabolic syndrome (central obesity, high cholesterol, diabetes)
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 SLEActive SLE which indicates an active inflammatory state mayalso in itself be a risk factor for accelerated atherosclerosis andheart disease
Cholesterol screeningStress test and echocardiogramsCT of the heart looking for calcificationCarotid artery ultrasoundCardiac mri
Check your cholesterol levels regularlyCurrent recommendations suggest treating SLE patients as having acardiac equivalent which means treating to goals of a LDL cholesterolless than 100 with cholesterol lowering drugsCertain trials have shown statins which are effective cholesterollowering drugs also have an anti-inflammatory effectPlaquenil which is a useful medication in the treatment of severalmanifestations of lupus has also been shown to have lipid loweringeffects – C. Tang, T. Gofrey, et. al, Int Med Journal, July 2012; 968-978.
Diabetes – control glucose with medications and diet and lifestyle changesHypertension control with medications, diet and lifestyle changesIF possible reduce steroid dose as much as possibleStop smokingWeight control and reducing obesityHealthy well balanced diet – low fat, high fiber diet incorporating fresh fruitsand vegetables and lean proteins is the way to go. there is no proven “anti-inflammatory diet”Exercise regularly – improves blood pressure, lowers cholesterol, improvesfatigue, aids weight loss