About systemic lupus erythematosus
What is systemic lupus erythematosus?
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, not abruptly discontinuing medications, and monitoring their condition with their doctor.
What is systemic lupus erythematosus? What are the types of lupus?
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 by rash, 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. Statistics demonstrate that lupus is somewhat more frequent in African Americans and people of Chinese and Japanese descent.
What causes systemic lupus erythematosus? What are risk factors for lupus? Is lupus hereditary?
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. Lupus is not contagious.
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. Moreover, it is possible to have more than one autoimmune disease in the same individual. Therefore, "overlap" syndromes of lupus and rheumatoid arthritis, or lupus and scleroderma, etc., can occur.
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.
Research has demonstrated evidence that a key enzyme's failure to dispose of dying cells may contribute the development of SLE. The enzyme, DNase1, normally eliminates what is called "garbage DNA" and other cellular debris by chopping them into tiny fragments for easier disposal. Researchers turned off the DNase1 gene in mice. The mice appeared healthy at birth, but after six to eight months, the majority of mice without DNase1 showed signs of SLE. Thus, a genetic mutation in a gene that could disrupt the body's cellular waste disposal may be involved in the initiation of SLE.
What is drug-induced lupus?
Dozens of medications have been reported to trigger SLE. However, more than 90% of cases of "drug-induced lupus" occurs as a side effect of one of the following six drugs: hydralazine (Apresoline) is used for high blood pressure; quinidine (Quinidine Gluconate, Quinidine Sulfate) and procainamide (Pronestyl; Procan-SR; Procanbid) are used for abnormal heart rhythms; phenytoin (Dilantin) is used for epilepsy; isoniazid (Nydrazid, Laniazid) is used for tuberculosis; and d-penicillamine (used for rheumatoid arthritis). These drugs are known to sometimes stimulate the immune system and cause SLE. Fortunately, drug-induced SLE is infrequent (accounting for less than 5% of all people with SLE), and it usually resolves when the medications are discontinued.
What are the symptoms for systemic lupus erythematosus?
A typical sign of lupus is a red, butterfly-shaped rash over your cheeks and nose, often following exposure to sunlight.
No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus have mild disease characterized by episodes — called flares — when signs and symptoms get worse for a while, then improve or even disappear completely for a time.
The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. The most common signs and symptoms include:
- Joint pain, stiffness and swelling
- Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body
- Skin Lesions that appear or worsen with sun exposure
- Fingers and toes that turn white or blue when exposed to cold or during stressful periods
- Shortness of breath
- Chest pain
- Dry eyes
- Headaches, Confusion and memory loss
What are the causes for systemic lupus erythematosus?
As an autoimmune disease, lupus occurs when your immune system attacks healthy tissue in your body. It's likely that lupus results from a combination of your genetics and your environment.
It appears that people with an inherited predisposition for lupus may develop the disease when they come into contact with something in the environment that can trigger lupus. The cause of lupus in most cases, however, is unknown. Some potential triggers include:
- Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people.
- Infections. Having an infection can initiate lupus or cause a relapse in some people.
- Medications. Lupus can be triggered by certain types of blood pressure medications, anti-seizure medications and antibiotics. People who have drug-induced lupus usually get better when they stop taking the medication. Rarely, symptoms may persist even after the drug is stopped.
What are the treatments for systemic lupus erythematosus?
Treatment for lupus depends on your signs and symptoms. Determining whether you should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor.
As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages. The medications most commonly used to control lupus include:
- Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter , such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be used to treat pain, swelling and fever associated with lupus. Stronger are available by prescription. Side effects of may include stomach bleeding, kidney problems and an increased risk of heart problems.
- Antimalarial drugs. Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), affect the immune system and can help decrease the risk of lupus flares. Side effects can include stomach upset and, very rarely, damage to the retina of the eye. Regular eye exams are recommended when taking these medications.
- Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones, high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.
- Immunosuppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep, others), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
Biologics. A different type of medication, belimumab (Benlysta) administered intravenously, also reduces lupus symptoms in some people. Side effects include nausea, diarrhea and infections. Rarely, worsening of depression can occur.
Rituximab (Rituxan, Truxima) may be beneficial for some people in whom other medications haven't helped. Side effects include allergic reaction to the intravenous infusion and infections.
In clinical trials, voclosporin has been shown to be effective in treating lupus.
Other potential drugs to treat lupus are currently being studied, including abatacept (Orencia), anifrolumab and others.
What are the risk factors for systemic lupus erythematosus?
Factors that may increase your risk of lupus include:
- Your sex. Lupus is more common in women.
- Age. Although lupus affects people of all ages, it's most often diagnosed between the ages of 15 and 45.
- Race. Lupus is more common in African Americans, Hispanics and Asian Americans.
Is there a cure/medications for systemic lupus erythematosus?
SLE affects people differently. Treatments are most effective when you start them soon after symptoms develop and when your doctor tailors them to you. It’s important that you make an appointment with your doctor if you develop any symptoms that concern you.
Living with a chronic condition can be difficult. Talk to your doctor about support groups in your area. Working with a trained counselor or support group can help you reduce stress, maintain positive mental health, and manage your illness.