Lupus

*The information on this website is not intended to diagnose, treat, cure or prevent any disease.

What is lupus?

Lupus is an autoimmune disorder with the potential to attack any part of the body. Often, this condition results in systemic inflammation and manifestations, however it may be localized to specific areas. Depending on the body systems affected, the manifestations of symptoms, or by what appears to be a triggering factor, specific subclassifications of lupus may be diagnosed, such as systemic lupus erythematosus (SLE), discoid lupus, drug-induced lupus, sub-acute cutaneous lupus, or neonatal lupus. Of these different subtypes, SLE is the most common, and ranges in severity.

In lupus, like any autoimmune disorder, the immune system inappropriately attacks normal tissues in the body. In some instances, this inappropriate immune response appears to be triggered by certain things, but often the cause of immune dysfunction is never identified. Multiple parts of the immune system appear to function abnormally in lupus. In SLE at least, there is a failure to appropriately eliminate cells which have undergone normal cell death. Instead of these cells being engulfed and removed from the body, they end up incompletely destroyed, releasing nucleic acid and proteins into circulation. These cell components are then inappropriately picked up by cells which are supposed to present foreign material to the immune system in order to begin developing an immune response. The body then begins generating antibodies against these cellular components, which then bind to cells, serving as a cue for destruction and inflammation.

Lupus affects over 1 million Americans, and about 90% of affected adults are women. Most commonly, the disease surfaces between 15-45 years of and age (mainly women of child-bearing age). Certain ethnicities are considered at higher risk, including those of African, Chinese, or Caribbean ancestry.

Due to the variability in which lupus can manifest, it can often be misdiagnosed, or a definitive diagnosis may be delayed.

Common Symptoms

There is an incredible range of symptoms and severity in lupus, and no two cases are going to be exactly the same. This contributes to the difficulty in diagnosing the disease.

Common symptoms reported include:

A butterfly-shaped rash across the nose and cheeks
Joint pain and inflammation
Fever
Raynaud’s syndrome: a condition in which the fingers and toes become white and/or blue when exposed to cold
Ulcers affecting the mouth and/or nose
Swelling of extremities or around eyes
Anemia
Hair loss
Sensitivity to sunlight
Fatigue
Chest pain
Hair loss
Kidney disease
High blood pressure
Slow blood clotting times
Seizures
Stroke
Depression
Headache

Diagnosis

Lupus can be a challenging disease to diagnose, particularly when more “classic” findings such as a facial rash are absent, or when signs are milder and easily confused with other conditions such as rheumatoid arthritis. Unfortunately, there is not a single test which can positively identify lupus, so physicians typically rely on a combination of test results, physical exam findings, and medical histories to make a diagnosis. Some laboratory tests can be very helpful in making a diagnosis of lupus. Tests which may be ordered by a physician who suspects lupus may include:

Anti-nuclear antibodies (ANAs): Anti-nuclear antibodies are common in patients suffering from lupus. They are auto-antibodies (self-directed antibodies) which target DNA. These antibodies can be detected in 97% of individuals suffering from lupus, however it is not an entirely specific test, and other conditions can also result in positive ANA results. There are many subtypes of ANAs, and evaluating specific antibody profiles can help direct a diagnosis.
Anti-phospholipid antibodies (APLs): These are antibodies directed against components of cell membranes call phospholipids. They are present in approximately 60% of patients with lupus, and can be a helpful ancillary test.
Anti-Sm Antibodies: These are a specific type of antibody directed against proteins within the cell nucleus. They are very specific for patients suffering from lupus, but not very sensitive. This means that when these antibodies are present, there is a high likelihood of patients suffering from SLE, however if they are absent, it does not exclude lupus, as most patients with SLE will not have these antibodies present. Interestingly, these antibodies may be produced in response to Epstein-Barr virus infections, which has proteins which are structurally similar to the nuclear proteins these anti-bodies are directed against.
C-Reactive Protein: This is a general marker of inflammation in the body. It may be checked in order to monitor inflammation associated with lupus, however it is elevated in many other conditions.
Biopsies: This involves surgically removing a small piece of tissue from an affected area to look for classic microscopic signs of lupus. The most common sites of biopsies are the skin and the kidneys, however biopsies can be taken from almost anywhere in the body. This can hep confirm lupus and exclude other conditions which may cause similar symptoms.
Urinalysis: Examining the urine is very important in the diagnosis and management of lupus. Kidney damage is very common in lupus patients, and monitoring kidney function may become a very important component in managing the disease.
Clotting times: The amount of time it takes for blood to clot is often increased in patients with lupus due to the destruction of platelets. This can make a person at increased risk of bleeding or suffering from a hemorrhagic stroke. Alternatively, in highly inflammatory states, bleeding times may become shortened, making it more likely a person may clot inappropriately, putting them at risk for thromboembolism or ischemic stroke.
Red Blood Cell (Erythrocyte) Sedimentation: How quickly red blood cells settle to the bottom of a test tube can be an indication of lupus. A faster rate may be more consistent with lupus, which causes the release of a compound in the blood called fibrinogen. Fibrinogen can make red blood cells stick together, making them heavier and more likely to settle to the bottom of a test tube more quickly. It is a non-specific test, however, and sedimentation rates can be increased in many other conditions.
Complete blood count: This is a measurement of your total number of red and white blood cells. Anemia is commonly associated with lupus, and a complete blood count is helpful in monitoring patients.
Echocardiogram: Occasionally lupus may affect the heart itself, resulting inflammation of the muscle (myocarditis) or pericardium (pericarditis). An echocardiogram may be helpful in assessing heart function in patients with cardiac involvement from lupus.

Treatment Options

Pharmaceuticals:

Treatment for lupus may be dictated based on the severity of clinical signs and the specific type of lupus diagnosed. Unfortunately, there is no proven cure for lupus, however management of the disease has greatly improved. Common medications which may be prescribed for lupus may include:

Corticosteroids: These are powerful anti-inflammatories and immunosuppressives which can serve to curb the overactive immune system. They work quickly, and can be important in decreasing the long-term damage to the body caused by chronic inflammation. Although effective, corticosteroids have a wide range of side effects including weight gain, mood changes, increased risk of infections, and others which makes long-term use difficult for many people
Non-steroidal Anti-inflammatories (NSAIDs): These are another class of anti-inflammatory medications. There are over-the-counter (OTC) and prescription options available. OTC options include ibuprofen (Advil) and naproxen (Aleve), while prescriptions include drugs such as meloxicam and diclofenac. They are effective for mild pain and inflammation associated with lupus.
Anti-malarial Drugs: These are drugs which are traditionally prescribed to treat malaria, but they are also effective against lupus. These medications are immune-modulatory, not immunosuppressive. They do not increase risk of infections; however, they can have other side effects. Research has shown that patients who receive anti-malarial drugs live longer than those who do not take them. They may not be effective alone for severe symptoms, and may be combined with other drugs. Examples of anti-malarial drugs include hydroxychloroquine and chloroquine. These drugs are also effective against the bacteria responsible for Lyme disease, which may support a link between the two diseases.
Antibiotics: Antibiotics may be needed, especially for those who are on immunosuppressive medications and develop infections as a consequence. However, there may be a link between Lyme disease and lupus, and antibiotic therapy could be beneficial for a subset of patients.
Immunosuppressive Drugs: There are many different mechanisms by which immunosuppressive drugs work, however they all blunt the immune response – usually by interfering with cellular replication of immune cells like B and T cells. They can take weeks to reach peak effects, and may also increase risk of infections and lifetime risk of cancer. Despite these risks, immunosuppressive medications are often a cornerstone of lupus management. Examples of immunosuppressive drugs include azathioprine, cyclosporine, and mycophenolate mofetil, leflunomide, methotrexate, and chlorambucil.
Dehydroepiandrosterone (DHEA): This is an androgenic (masculinizing) hormone that also has impacts on the immune system. It is not understood how this hormone works, as there have been no receptors identified which interact with it, however it is known to be lower in patients who are suffering from lupus. Supplementation with DHEA may be helpful in mild to moderate disease.
Monoclonal Antibodies: These are another type of drug which target the immune system. They may be used when other drugs have not effectively controlled symptoms.
Blood thinners: In patients who are found to be at increased risk of clot-formation, blood-thinning medications may be prescribed. Blood thinners include aspirin, clopidogrel (Plavix), and clotting factor Xa inhibitors such as rivaroxaban and apixaban.

Non-pharmaceutical options:

Supplements:
- Omega-3 fatty acids: Omega-3 fatty acids are anti-inflammatory in nature. Sources include fish oil and flaxseed oil. Increasing intake of omega-3’s may help reduce the symptoms of lupus.
- Curcumin/turmeric: This compound has been found to have potent anti-inflammatory properties, which may be helpful, particularly in mild to moderate cases of lupus.
- Vitamins A, E, D: These fat-soluble vitamins may be useful in improving the free-radical scavenging systems of the body, which can reduce long-term damage from inflammation. Vitamin D in particularly has been associated with many other diseases characterized by auto-immunity or immune-dysfunction.
- Evening primrose oil: This is an anti-inflammatory compound. It has been shown to be useful in rheumatoid arthritis and other inflammatory conditions which share symptoms with lupus.
- Boswellia: This is another anti-inflammatory compound which is recommended in a number of inflammatory diseases like Crohn’s, ulcerative colitis, and arthritis.
- Selenium: A powerful anti-oxidant, selenium may help decrease the severity of lupus symptoms and flare-ups. Caution should be exercised, as excessive selenium can be toxic.
- Green Tea Polyphenols: Lupus appears to have a lower incidence in countries which consume large amounts of green tea, and it has been hypothesized that a diet high in the compounds found in green tea may be protective. Increasing consumption of green tea or taking green tea extracts may be beneficial.
- Cat’s Claw (Uncaria tomentosa or Uncaria guianensis): This is a South American herb which has been used in many inflammatory conditions. It is thought to inhibit inflammatory cytokines like tumor necrosis factor (TNF-alpha). A placebo-controlled trial showed improvement in patients suffering from rheumatoid arthritis.
- Ginkgo biloba: High in anti-oxidants, ginkgo biloba may help reduce inflammation, as well as improving other measures of well-being.
Meditation: Meditation has applications for many chronic illnesses. Regular practice can help improve mental health, reduce the perception of pain, and may have physical effects as well.
Hypnosis: This is another mind-body technique which can be helpful in living with lupus. Better focus on the body and other aspects of life can greatly improve quality of life in patients with lupus.
Aromatherapy: Essential oils can greatly improve relaxation and comfort in patients with chronic diseases, include lupus. They may reduce inflammation, reduce stress, and improve circulation.
Electromagnetic Field Therapy: Research has been conducted into electromagnetic fields and their impact on fatigue in lupus. Two trials have shown improvement in fatigue in patients with moderate clinical signs.
Massage therapy: This may improve relaxation, pain, and quality of life in patients with lupus.
Yoga: This can help maintain strength and keep muscles and joints loose and active despite chronic inflammation. Yoga also intertwines closely with meditation in terms of its psychological benefits.
Chiropractic care: Spinal adjustments and managing joint and muscle pain can greatly improve some individual’s conditions with lupus.
Dietary Modifications:
- Alkaline Detox Diet: Eliminate processed foods, and inflammatory foods like fruit, gluten, legumes, and grains.
- Eliminate Nightshades: Tomatoes, potatoes, eggplants, and peppers are all members of the nightshade family and may contribute to lupus flare-ups or inflammation.
- Anti-inflammatory diet
Probiotics: Maintaining gut health is important for the body and can play an important role in regulating the immune system.

Prevention

There is no known way to prevent the development of lupus, but like other diseases, certain life-style risk factors may be able to mitigated to reduce overall risk, but many risk factors are not easily modified (such as being female, of certain ethnicities, or a history of lupus in your family). Stress, however, is a known triggering factor. Avoiding unnecessary stress, or taking healthy steps to manage stress may help reduce one’s risk of developing lupus. Meditation, exercise, and taking time for yourself away from work or stressful situations are important strategies. Sun-damage (like sun burns) are also a known triggering factor for some individuals with lupus. Wearing sunscreen is a good idea to help preventing the damage which may lead to lupus.

For those with a family history of lupus, extra care should be taken to avoid triggering factors. Make sure your physician knows that lupus runs in your family, as certain prescription drugs are associated with the development of lupus. A family history may suggest that certain individuals are more susceptible to these events.

For those who already are suffering from lupus, steps can be taken to minimize the risk of flare-ups. Eating a healthy diet which is balanced with plenty of leafy greens, omega-3 fatty acids, and avoiding the “Western” diet which is heavy in saturated fats, salt, and carbohydrates are helpful in maintaining control of lupus. Likewise, getting plenty of rest and managing stress through exercise, mindfulness practices, and avoiding sunlight. Use medications as prescribed. For many of them, prematurely stopping or tapering medications may result in a recrudescence of inflammation. Finally, infections can be major triggering events by causing immune activation. Wash your hands regularly and avoid close contact with those who are sick. Keep in mind that if you are taking immunosuppressive medications, you may be more susceptible to infections in the first place.

Causes

Usually, a definitive cause of lupus is never identified. It is also unclear why women are so much more likely to suffer from lupus compared to men. From this, many researchers believe hormones, specifically estrogen, may somehow play a role. In affected women, lupus also tends to become more severe around the times of menstruation, or during pregnancy, when estrogen levels are particularly high. However, when estrogen is supplemented (such as in birth control pills) the disease severity does not worsen, which makes the connection between estrogen and lupus less clear.

It is also clear that genetics must play a role in the development of lupus. Studies in identical twins have shown that if one twin develops lupus, there is a 1 in 4 chance the other twin will also develop lupus. A genetic link is further supported by lupus being more common in people with certain ancestries, such as African, Chinese, or Caribbean. At this point, no causal genes have been identified.

It is likely that for those individuals who are at increased risk of developing lupus, something acts as a trigger which results in the immune system attacking the body. UV damage, whether from the sun or from tanning beds, likely results in the death of many cells under the skin which may be inappropriately cleared by the body. When these cells die, the body may misinterpret the debris as foreign material, and begin producing an immune response against it. UV damage seems to be a major trigger in some people. Sun damage may not only result in the initial disease, but may also contribute to later flare-ups. Other triggers may be things such as viral infections (especially viruses like Epstein-Barr), or exposure to industrial chemicals. It has been clearly established that a number of medications have the potential to trigger lupus. These medications include some antibiotics such as penicillins, sulfa-based drugs, and tetracyclines (which also increase sensitivity to the sun as well). Finally, in the absence of other triggers, heavy stress, whether it is emotional or physical, has been associated with the development of lupus.

There is also a possible connection between lupus and Lyme disease. Both diseases can share many characteristics, such as inflamed joints and skin lesions. Lyme disease can also result in autoimmune antibodies as well. The fact that Lyme disease can be treated with anti-malarial drugs may further support Lyme disease as a possible cause of lupus, since those medications can be effective against the bacteria responsible for Lyme disease. It is reasonable to test for Lyme disease in lupus sufferers, especially because of how closely related the symptoms can be.