Health Talk: Systemic Lupus Erythematosus (SLE or Lupus)

Systemic Lupus Erythematosus Facts: 

• Systemic lupus erythematosus (SLE) is an autoimmune disease.
• SLE is characterized by the production of unusual antibodies in the blood.
• SLE is eight times more common in women than men.
• The cause(s) of SLE is (are) unknown, however, heredity, viruses, ultraviolet light, and drugs all may play some role.
• Up to 10% of people with lupus isolated to the skin will develop the systemic form of lupus (SLE).
• Eleven criteria help doctors to diagnose SLE.
• Treatment of SLE is directed toward decreasing inflammation and/or the level of autoimmune activity.
• People with SLE can prevent "flares" of disease by avoiding sun exposure and not abruptly discontinuing medications and monitoring their condition with their doctor.

What is systemic lupus erythematosus?

Lupus is an autoimmune disease characterized by acute and chronic inflammation of various tissues of the body. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. The immune system is a complex system within the body that is designed to fight infectious agents, such as bacteria and other foreign microbes. One of the ways that the immune system fights infections is by producing antibodies that bind to the microbes. People with lupus produce abnormal antibodies in their blood that target tissues within their own body rather than foreign infectious agents. These antibodies are referred to as autoantibodies.

Because the antibodies and accompanying cells of inflammation can affect tissues anywhere in the body, lupus has the potential to affect a variety of areas. Sometimes lupus can cause disease of the skin, heart, lungs, kidneys, joints, and/or nervous system. When only the skin is involved, the condition is called lupus dermatitis or cutaneous lupus erythematosus. A form of lupus dermatitis that can be isolated to the skin, without internal disease, is called discoid lupus. When internal organs are involved, the condition is referred to as systemic lupus erythematosus (SLE).

Both discoid lupus and systemic lupus are more common in women than men (about eight times more common). The disease can affect all ages but most commonly begins from 20-45 years of age. 

What causes systemic lupus erythematosus ?

The precise reason for the abnormal autoimmunity that causes lupus is not known. Inherited genes, viruses, ultraviolet light, and certain medications may all play some role.

Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis, and autoimmune thyroid disorders are more common among relatives of people with lupus than the general population. Some scientists believe that the immune system in lupus is more easily stimulated by external factors like viruses or ultraviolet light. Sometimes, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure.

It also is known that some women with SLE can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of SLE, suggests that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.

What are lupus symptoms and signs?

People with SLE can develop different combinations of symptoms and organ involvement. Common complaints and symptoms include:

• Fatigue
• Low-grade fever
• Loss of appetite
• Muscle aches
• Hair loss (alopecia)
• Arthritis
• Ulcers of the mouth and nose
• Facial rash ("butterfly rash")
• Unusual sensitivity to sunlight (photosensitivity)
• Inflammation of the lining that surrounds the lungs (pleuritis) and the heart (pericarditis),
• Poor circulation to the fingers and toes with cold exposure (Raynaud's phenomenon).

Complications of organ involvement can lead to further symptoms that depend on the organ affected and severity of the disease.

The following are 11 criteria used for diagnosing systemic lupus erythematosus:

1. Malar (over the cheeks of the face) "butterfly" rash
2. Discoid skin rash (patchy redness with hyperpigmentation and hypopigmentation that can cause scarring)
3. Photosensitivity (skin rash in reaction to sunlight [ultraviolet light] exposure)
4. Mucous membrane ulcers (spontaneous sores or ulcers of the lining of the mouth, nose, or throat)
5. Arthritis (two or more swollen, tender joints of the extremities)
6. Pleuritis or pericarditis (inflammation of the lining tissue around the heart or lungs, usually associated with chest pain upon breathing or changes of body position)
7. Kidney abnormalities (abnormal amounts of urine protein or clumps of cellular elements called casts detectable with a urinalysis) Note: Ultimately, in patients with kidney disease from systemic lupus erythematosus (lupus nephritis), a kidney biopsy may be necessary to both define the cause of the kidney disease as being lupus-related as well as to determine the stage of the kidney disease in order to optimally guide treatments. Kidney biopsies are often performed by fine needle aspiration of the kidney under radiology guidance, but in certain circumstances, a kidney biopsy can be done during an open abdominal operation.
8. Brain irritation (manifested by seizures [convulsions] and/or psychosis, referred to as "lupus cerebritis")
9. Blood-count abnormalities: low white blood count (WBC) or red blood count (RBC), or platelet count on routine complete blood count testing)
10. Immunologic disorder (abnormal immune tests include anti-DNA or anti-Sm [Smith] antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test)
11. Antinuclear antibody (positive ANA antibody testing [antinuclear antibodies in the blood])

What is the treatment for systemic lupus erythematosus?

There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation and/or the level of autoimmune activity in the body. The precise treatment is decided on an individual basis. Many people with mild symptoms may need no treatment or only intermittent courses of anti-inflammatory medications. Those with more serious illness involving damage to internal organ(s) may require high doses of corticosteroids in combination with other medications that suppress the body's immune system.

The goal of treatment for mild lupus is to prevent symptom flares-when fatigue, joint pain, and rash get worse. Maintain a schedule of regular checkups with your doctor, instead of waiting until your disease flares. When flares do occur, the goal is to treat them rapidly to limit any damage to body organs.

Treatment for mild lupus includes:

• Avoiding the sun. If you must be in the sun, cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering both UVA and UVB rays) with a high sun protection factor (such as SPF 50) to protect your skin.
• Applying corticosteroid cream for rashes.
• Taking acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) and getting plenty of rest for mild joint or muscle pain and fever.
• Taking antimalarial medicines, especially for skin rashes, but also for fatigue and joint and muscle pain.
• Taking low-dose corticosteroids if NSAIDs aren't effective in controlling your symptoms.

For more severe cases of lupus, treatment may include:

• Higher-dose corticosteroids, either in pills or by injection.
• Medicines that suppress the immune system (immunosuppressants).
• People with lupus have a greater risk of heart attack and stroke than people without lupus. To help prevent heart attack and stroke, your doctor will help you manage risk factors such as high blood pressure and high cholesterol.

Medicine Choices

If you have mild disease or symptoms that affect your quality of life but don't have organ-threatening problems, your doctor may prescribe:

1. Acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs), sometimes in combination with antimalarial drugs.
2. Antimalarial drugs such as hydroxychloroquine (Plaquenil).
3. Low-dose corticosteroids and/or topical corticosteroid creams or ointments.

If you have more severe disease, your doctor may prescribe:

1. Corticosteroids, such as prednisone.
2. Immunosuppressive medicines, such as azathioprine, belimumab, cyclophosphamide, mycophenolate mofetil, or methotrexate.

HOME CARE

Good self-care is essential to managing lupus (systemic lupus erythematosus, or SLE). Learn to recognize your body's warning signs of a flare. Warning signs may include increased fatigue, joint pain, rash, or fever. When you notice any of these signs, take steps to control your symptoms.

Stress may trigger lupus symptoms. Keep your stress level as low as you can.

• Keep your daily schedule as simple as possible.
• Keep your list of obligations to others to a bare minimum.
• Delegate to others.
• Exercise regularly. A daily walk, for example, can reduce stress, clear your head, improve your mood, and help fight fatigue.
• Use relaxation techniques such as meditation, yoga, and guided imagery to calm your body and mind.

Fatigue is common in people with lupus. To fight fatigue:

• Get plenty of rest. Some people with lupus need up to 12 hours of sleep every night.
• Pace yourself. Limit tiring activities.
• Ask others for help. Don't try to do everything yourself.
• Take short breaks from your usual daily activities. Consider cutting down on work hours or getting help with parenting responsibilities, at least during periods when lupus symptoms are severe.
• Exercise regularly. Physical activity boosts energy and helps you stay in good condition. Walking and swimming are good forms of exercise for people with lupus.
• If you suspect that depression is contributing to your fatigue, get prompt treatment from your doctor, a mental health professional, or both.

Take care of your skin. 

• Ask your doctor about the use of corticosteroid creams to relieve skin symptoms that are particularly troublesome. If you are bothered by the way a lupus rash looks on your face or if you have scars from lupus, you can try makeup, such as Covermark, to cover the rash or scars.
• Ultraviolet (UVA and UVB) light triggers disease flares in most people with lupus. Exposure to ultraviolet light, as from sunlight, can trigger or start skin rash, joint pain, or fatigue, or it can make these symptoms worse. To minimize your exposure to ultraviolet light:
•  Avoid the sun. If you must be in the sun, cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering both UVA and UVB rays) with a high sun protection factor (50 SPF or higher) to protect your skin. Reapply sunscreen after swimming, sweating, or toweling off. Experiment with sunscreens. Some may irritate your skin or wash off too easily.
• Avoid going out when the sun's rays are the strongest. In most areas, this is between the hours of 10 a.m. and 4 p.m., especially during the summer.

Good general care is essential. A healthy lifestyle not only improves your quality of life but may also reduce your chances of having more frequent and severe flares. Taking good general care of yourself also helps decrease the risk of heart attack and stroke. Good care includes:6

• Getting vaccinations to help protect you from illnesses such as pneumonia and the flu.
• Treating high blood pressure and high cholesterol.
• Taking medicine to help prevent osteoporosis caused by corticosteroids.
• Preventing plaque in the arteries (atherosclerosis) that can be made worse by corticosteroids.
• Protecting yourself against infections you can get more easily due to decreased immune system function.

Sources: webmd.com and medicinenet.com