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Lupus (Systemic Lupus Erythematosus)

Overview

Systemic lupus erythematosus (known as lupus or SLE) is an autoimmune disorder, which means that the body's immune system inappropriately attacks healthy tissue and cells, causing inflammation and tissue damage. Lupus can affect multiple bodily organs, most commonly the skin, joints, and kidneys but also the heart, lungs, blood cells, muscles, eyes, and nervous system.

Lupus is a chronic disease and requires a lifetime of management and treatment. Most people with illness can lead full and productive lives. Though it can affect women and men of all ages and ethnicities, lupus is more common in women and usually appears after puberty. Although genetics and environmental factors have been considered to play an important role in the pathogenesis of lupus, some cases can be precipitated by certain medications. Most have no known causes.

People with lupus can present a broad range of symptoms, often with individual variations. Symptoms tend to flare up periodically, alternating with periods during which symptoms become minor or go away entirely (remission).

Skin rashes, particularly in areas exposed to sunlight, are common. Severe flare-ups can cause serious injury to internal organs. Lupus may also involve the blood, resulting in anemia and a decrease in white blood cells and platelets.

Women with lupus can usually have normal pregnancies once the condition is controlled or in remission, but there is an increased risk of miscarriage and other complications. Therefore, pregnant women with lupus are considered a high-risk group.

Types

Lupus can be subdivided into several categories:

Systemic lupus erythematosus is the most typical form. It usually affects the skin and joints. When it involves the kidney and the lining around the lungs, it is called pleurisy or pleuritis. When the lining around the heart is involved, the condition is call pericarditis. The blood-producing cells that are formed in the bone marrow can be affected, causing anemia and easy bruising when the platelets are involved. Lupus of the nervous system can result in seizures, major depression, paralysis, and other serious complications.

Discoid lupus erythematosus affects only the skin, causing scaly rashes, sores, and scars, usually on the face, scalp, neck, or other areas of skin exposed to sunlight. This disorder may also cause changes in skin color and hair loss.

Drug-induced lupus erythematosus is caused by an immune reaction to certain prescription drugs, most commonly hydrazaline (used for high blood pressure), isoniazid (used for tuberculosis), doxycycline for acne, and biologic agents such as remicade (used for rheumatoid arthritis) or procainamide (used to treat heart arhythmias). Like SLE, drug-induced lupus can affect the skin, joints, and lining around the lungs and heart but rarely involves the kidney or nervous system. Symptoms of drug-induced lupus normally disappear some months after the medication is stopped.

Neonatal lupus is a very rare form that affects infants, usually born of mothers who have lupus. The most common symptom is a skin rash that goes away within a few weeks to several months. Less common symptoms may include low blood cell counts and liver problems, but these conditions also disappear entirely within several months. In rare cases, neonatal lupus may cause congenital heart block, a disorder of the heart's electrical system.

Symptoms

Because lupus may affect different parts of the body, it can cause a wide range of symptoms.

Most symptoms typically wax and wane, sometimes punctuated by severe flare-ups. Remission can occur but is not common. Common symptoms include:

• Severe fatigue.
• Fever without an infection.
• Joint pain and swelling, especially in the hands and wrists.
• Hair loss.
• Chest pain, usually worse when taking deep breaths or lying down.
• Raised, red-colored rash on the nose and cheeks (known as a malar rash).
• Mouth sores.
• Sjogren's syndrome, characterized by dry eyes or dry mouth due to inflammation of the tear and salivary glands.
• Raynaud's phenomenon, in which fingers and toes may turn blue, white, or red on cold exposure due to decreased blood flow to the extremities.
• Involvement of the nervous system, producing severe headaches, strokes, or seizures. When lupus affects the peripheral nerves in the arms and legs, numbness and weakness may result.
  

Causes and Risk Factors

Lupus is a disorder in the regulation of the body's immune system. The exact cause is unknown. There is often a genetic component to this illness. However, an environmental event or trigger may produce the symptoms and signs of lupus. In addition, certain medications can trigger lupus in predisposed individuals.

Diagnosis

Diagnosing lupus can be difficult because symptoms vary widely and could be caused by other conditions. The diagnosis is based on symptoms, physical examination, and results of specific laboratory tests. Symptoms can include:

• Raised, red-colored rash on nose and cheeks (known as a malar rash)
• Sun sensitivity (photosensitivity)
• Pain and swelling of the joints
• Chest pain due to inflammation of the lining around the heart (pericarditis) and/or lungs (pleurisy)
• Abnormal bruising of the skin due to low blood cell platelets
• Seizures
• Painful mouth sores
• Fatigue

The following tests are used to confirm a diagnosis of lupus:

• Blood tests can measure red and white blood cell and platelets.
• Urinalysis can detect abnormally high amounts of protein or red and/or white blood cells, indicating inflammation of the kidneys. Further evaluation of the kidneys may require a kidney biopsy to make a definitive diagnosis of lupus.
• Chest X-ray may reveal fluid around the lungs or heart, which indicates pericarditis or pleurisy.
• Echocardiogram can also identify fluid in the sac surrounding the heart (pericarditis).

Treatment

Treatment depends on a complete assessment of symptoms, signs, and any organ involvement by lupus. A number of different types of medication are available. Lifestyle adaptations are also extremely important. Goals of treatment include:

• Relief of pain, fatigue, and other symptoms
• Control of inflammation
• Preventing flare-ups
• Minimizing or preventing damage to internal organs

Medications

Lupus may be treated with the following types of medications:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) are the most common type of medication prescribed initially. NSAIDs reduce inflammation and pain to some extent. NSAIDs include over-the-counter products such as aspirin and ibuprofen (Advil, Motrin), as well as prescription drugs such as naproxen (Naprosyn), nabumetone (Relafen), or meloxicam (Mobic). NSAIDs carry a small risk of stomach irritation and may interact with other medications used for hypertension and heart disease.
• Corticosteroids reduce pain and inflammation by suppressing the overactive immune system in lupus. In low doses, they may be used for joint or skin symptoms, while in higher doses, corticosteroids are used for more severe life-threatening involvement of the kidney, heart, lung, brain, or blood count abnormalities. Commonly prescribed corticosteroid drugs include prednisone (Deltisone, Meticorten) and methyl-prednisolone (Medrol). With prolonged use, corticosteroid medications may cause insomnia, mood changes, weight gain, fluid retention and rounding of the face. They can cause increases in blood pressure, blood sugar, or cholesterol levels and suppress immune activity, which results in a higher risk of infection. With long-term use, corticosteroids accelerate bone loss (osteoporosis), and can cause cataracts and an uncommon condition of avascular necrosis of the large joints. Potential side effects are related to the amount and duration of the steroid dosages. Therefore, our goal is to keep you on the lowest possible dosage that will effectively treat lupus.
• Antimalarials such as hydroxychloroquine (Plaquenil) or chloroquine (Aralen) are often used to treat lupus-related skin disease such as rash and mouth sores and are beneficial for joint inflammation. Antimalarials may also help to prevent flare-ups. There is evidence that all patients should be on this class of medication. With prolonged use for over 5 years, a very rare side effect can cause deposits of this medicine in the retina of the eye, adversely affecting vision. Thus, any patient maintained on antimalarials must have periodic eye examinations after 5 years.
• Immunosuppressive medications are usually prescribed for aggressive forms of lupus and if corticosteroids have not been effective. These drugs may permit the lowering of steroid dosage and thereby minimize steroid side effects and still control lupus. They suppress the immune system and help reduce inflammation but require regular monitoring of blood counts and liver enzymes. Immunosuppressive drugs include cyclophosphamide (Cytoxan), mycophenolate mofetil (Cellcept), azathioprine (Imuran), and methotrexate (Rheumatrex). These medications carry an increased risk of infection and, in the case of cyclophosphamide, an increased risk of cancer.
• Anticoagulants such as warfarin (Coumadin) and heparin (Calciparine) prevent blood clots, a potentially serious complication of lupus in a minority of patients who have a condition called antiphospholipid syndrome. 

Drugs for seizure control, hypertension, and depression are often used as well.

It is important to keep in mind that some herbal supplements may interact with medications. Therefore, if you are considering taking herbal supplements, check with us before trying an alternative therapy. There is no evidence that these herbal supplements help lupus.

 

Lifestyle Management

If you have lupus, the following lifestyle habits and adaptations can help improve your symptoms and reduce the frequency of flare-ups:

• Avoid prolonged exposure to ultraviolet light from the sun. Wear protective clothing, including long sleeves and broad-brimmed hats. Use sunscreen that has a sun protection factor of no less than 50, and try to avoid being outside during the hours when the sun is strongest.
• Get plenty of rest. Sleep helps inflamed joints and muscles to heal. Daytime napping is restorative. Pace your activities throughout the day.
• Exercise regularly. Exercising for about 30 minutes daily supports your health, lifts your spirits, and improves muscle strength and flexibility. Avoid activity that aggravates pain and do not push yourself. Rest when you are tired. Find a form of exercise that you can enjoy.
• Eat a healthy diet. A diet that is based on fresh fruits and vegetables, whole grains, legumes, oily fish, and lean meats is usually advisable. People with lupus are at increased risk of strokes and heart attacks; therefore, your lipids, blood pressure, and blood sugar will be monitored. Moderate your alcohol consumption. 
• Do not smoke. Smoking is particularly dangerous for people with lupus because it can aggravate heart- and blood-related complications and induce skin lesion flare-ups.
• Take your medications as prescribed. Changing or skipping dosages can affect how well your medication works. If you experience side effects, tell your doctor immediately.
• Protect against infections. People with lupus are at an increased risk for infections of all kinds. Avoid close contact with people when they are sick, including family members. Wash your hands often, particularly during cold and flu season. Getting a flu vaccine and staying current with routine vaccinations is usually advisable. If you get a bacterial infection in your throat, bladder, skin, or lungs, contact us immediately.

Dealing with pain

In addition to your medications, there are techniques you can employ to help soothe pain. These include:

• Moist heat, such as a hot tub, sauna, hot shower or bath, or the application of a heated wet towel. Moist heat relaxes muscles and eases joint pain.
• Relaxation techniques, such as meditation, yoga, guided imagery, self-hypnosis, tai chi, and breathing exercises can relieve stress and lessen the experience of pain.
• Physical therapy. By helping you strengthen your muscles and increase your range of motion, physical therapy may also reduce pain and stiffness.

Confusion and depression

Memory difficulties are common lupus in lupus and other chronic illnesses. A daily task list or calendar can help you stay organized. It may also be wise to avoid multitasking, and to develop a habit of immediately writing down new information. Cognitive therapy can offer techniques to help strengthen your memory.

Many people with lupus experience depression. Psychotherapy, support groups, and antidepressant medication can help.

Coping and Support

Good communication among family members is essential. Your spouse and/or children should learn about and understand your condition and its symptoms, so that they know what to expect and can make adjustments as needed.

It can be helpful to draw up schedules of activities and chores, and to arrange home delivery of groceries and/or other supplies. The practical and emotional support of friends and neighbors can be very helpful. They may help with errands and chores and they can also provide a sympathetic and caring presence.

Traveling with lupus

Don't overdo it when you travel. Get plenty of rest, as always. Keep the pace leisurely and easy. If you will need to be sitting for a long journey, try and walk around periodically to keep your blood circulating. If you are on a plane, pump your feet back and forth for a few minutes every hour.

Keep your medication with you at all times, in original containers. If you are getting on an airplane, make sure to keep an adequate amount of your prescription drugs with you, rather than checking them with your baggage. Keep your medications in their original bottles or containers with the prescription labels. This is particularly advisable if you are traveling to a foreign country.

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If you have an emergency medical condition, call 911 or go to the nearest hospital. An emergency medical condition is any of the following: (1) a medical condition that manifests itself by acute symptoms of sufficient severity (including severe pain) such that you could reasonably expect the absence of immediate medical attention to result in serious jeopardy to your health or body functions or organs; (2) active labor when there isn't enough time for safe transfer to a Plan hospital (or designated hospital) before delivery, or if transfer poses a threat to your (or your unborn child's) health and safety, or (3) a mental disorder that manifests itself by acute symptoms of sufficient severity such that either you are an immediate danger to yourself or others, or you are not immediately able to provide for, or use, food, shelter, or clothing, due to the mental disorder.

This information is not intended to diagnose health problems or to take the place of specific medical advice or care you receive from your physician or other health care professional. If you have persistent health problems, or if you have additional questions, please consult with your doctor. If you have questions or need more information about your medication, please speak to your pharmacist. Kaiser Permanente does not endorse the medications or products mentioned. Any trade names listed are for easy identification only.