Lupus

Lupus... a General Overview

WHAT IS LUPUS? WHAT ARE THE TYPES OF LUPUS?

Lupus is a condition characterized by chronic inflammation of body tissues caused by your immune system. 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, for example, bacteria, and other foreign invaders. Our patients with lupus in Jacksonville, Ponte Vedra Beach,and Fleming Island with lupus produce abnormal antibodies in their blood that target tissues within their own body rather than foreign infectious agents. Because the antibodies and accompanying cells of inflammation can involve tissues anywhere in the body, lupus has the potential to affect a variety of areas of the body. 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 discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE).

Both discoid 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 age 20 to 45 years. It is more frequent in African-Americans and people of Chinese and Japanese descent.

WHAT CAUSES LUPUS? IS IT HEREDITARY?

The precise reason for the abnormal autoimmunity that causes lupus is not known. Inherited genes, viruses, ultraviolet light, and drugs may all play some role. Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as rheumatoid arthritis and thyroid disorders are more common among relatives of patients 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.

WHAT IS DRUG-INDUCED LUPUS?

Dozens of medications have been reported to trigger SLE; however, more than 90% of this "drug-induced lupus" occurs as a side effect of one of the following six drugs: hydralazine (used for high blood pressure), quinidine and procainamide (used for abnormal heart rhythm), phenytoin (used for epilepsy), isoniazid [(Nydrazid, Laniazid), used for tuberculosis], d-penicillamine (used for rheumatoid arthritis). These drugs are known to stimulate the immune system and cause SLE. Fortunately, drug-induced SLE is infrequent (accounting for less than 5% of SLE among all patients with SLE) and usually resolves when the medications are discontinued.

WHAT ARE THE SYMPTOMS AND SIGNS OF LUPUS?

In discoid lupus, only the skin is typically involved. The rash in discoid lupus often is found on the face and scalp. It usually is red and may have raised borders. Discoid lupus rashes are usually painless and do not itch but it can cause permanent hair loss. Over time, 5%-10% of patients with discoid lupus may develop SLE.

Over half of the patients with SLE develop a characteristic red, flat facial rash over the bridge of their nose. Because of its shape, it is frequently referred to as the "butterfly rash" of SLE. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by worsening of inflammation throughout the body, called a "flare" of disease.

Most patients with SLE will develop arthritis during the course of their illness. Arthritis in SLE commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, the arthritis of SLE can mimic that of rheumatoid arthritis (another autoimmune disease).

Inflammation of muscles (can cause muscle pain and weakness.

Inflammation of blood vessels, (vasculitis) that supply oxygen to tissues, can cause isolated injury to a nerve, the skin, or an internal organ. The blood vessels are composed of arteries that pass oxygen-rich blood to the tissues of the body and veins which return oxygen-depleted blood from the tissues to the lungs. Vasculitis is characterized by inflammation with damage to the walls of various blood vessels. The damage blocks the circulation of blood through the vessels and can cause injury to the tissues that the vessels supply.

Many patients with SLE experience hair loss (alopecia). Often, this occurs simultaneously with an increase in the activity of their disease.

Some patients with SLE have Raynaud's phenomenon. In these patients, the blood supply to the fingers and toes becomes interrupted upon exposure to cold, causing blanching, bluish discoloration, and pain in the exposed fingers and toes.

HOW IS LUPUS DIAGNOSED?

Since patients with SLE can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of the diagnosis of SLE, eleven criteria were established by the American Rheumatism Association. These 11 criteria are closely related to the symptoms discussed above. Some patients suspected of having SLE may never develop enough criteria for a definite diagnosis. Other patients accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested. Nevertheless, the diagnosis of SLE may be made in some settings in patients with only a few of these classical criteria. Of these patients, a number may later develop other criteria, but many never do.

The 11 criteria used for diagnosing systemic lupus erythematosus are:

1. malar (over the cheeks of the face) "butterfly" rash
2. discoid skin rash: patchy redness that can cause scarring
3. photosensitivity: skin rash in reaction to sunlight exposure
4. mucus membrane ulcers: ulcers of the lining of the mouth, nose or throat
5. arthritis: two or more swollen, tender joints of the extremities
6. pleuritis/pericarditis: inflammation of the lining tissue around the heart or lungs, usually associated with chest pain with breathing
7. kidney abnormalities: abnormal amounts of urine protein or clumps of cellular elements called casts
8. brain irritation: manifested by seizures (convulsions) and/or psychosis
9. blood count abnormalities: low counts of white or red blood cells, or platelets
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

WHAT IS THE TREATMENT FOR SYSTEMIC LUPUS?

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. Many patients with mild symptoms may need no treatment or only intermittent courses of antiinflammatory 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.

Patients with SLE need more rest during periods of active disease. Researchers have reported that poor sleep quality was a significant factor in developing fatigue in patients with SLE. During these periods, carefully prescribed exercise is still important to maintain muscle tone and range of motion in the joints.

Nonsteroidal antiinflammatory drugs (NSAIDs) are helpful in reducing inflammation and pain in muscles, joints, and other tissues. Examples of NSAIDs include aspirin, ibuprofen (Motrin), naproxen (Naprosyn), and sulindac (Clinoril). Since the individual response to NSAIDs varies among patients, it is common for a doctor to try different NSAIDs to find the most effective one with the fewest side effects. The most common side effects are stomach upset, abdominal pain, ulcers, and even ulcer bleeding. NSAIDs are usually taken with food to reduce side effects. Sometimes, medications that prevent ulcers while taking NSAIDs, such as misoprostol (Cytotec), are given simultaneously.

Corticosteroids are more potent than NSAIDs in reducing inflammation and restoring function when the disease is active. Corticosteroids are particularly helpful when internal organs are involved. Corticosteroids can be given by mouth, injected directly into the joints and other tissues, or administered intravenously. Unfortunately, corticosteroids have serious side effects when given in high doses over prolonged periods, and the doctor will try to monitor the activity of the disease in order to use the lowest doses that are safe. Side effects of corticosteroids include weight gain, thinning of the bones and skin, infection, diabetes, facial puffiness, cataracts, and death (necrosis) of large joints.

Hydroxychloroquine (Plaquenil) is an antimalarial medication found to be particularly effective for SLE patients with fatigue, skin, and joint disease. Side effects include diarrhea, upset stomach, and eye pigment changes. Eye pigment changes are rare, but require monitoring by an ophthalmologist (eye specialist) during treatment with Plaquenil. Researchers have found that Plaquenil significantly decreased the frequency of abnormal blood clots in patients with systemic SLE. Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can directly act to prevent the blood clots. This fascinating work highlights an important reason for patients and doctors to consider Plaquenil, especially for those SLE patients who are at some risk for blood clots in veins and arteries, such as those with phospholipid antibodies (cardiolipin antibodies, lupus anticoagulant, and false positive VDRL). This means not only that Plaquenil reduces the chance for reflares of SLE, but it can also be beneficial in 'thinning' the blood to prevent abnormal excessive blood clotting.

For resistant skin disease, other antimalarial drugs, such as chloroquine (Aralen) or quinacrine, are considered, and can be used in combination with hydroxychloroquine. Alternative medications for skin disease include dapsone and retinoic acid (Retin-A). Retin-A is often effective for an uncommon wart-like form of lupus skin disease. For more severe skin disease, immunosuppressive medications are considered as below.

Medications that suppress immunity (immunosuppressive medications) are also called cytotoxic drugs. Immunosuppressive medications are used for treating patients with more severe manifestations of SLE with damage to internal organ(s). Examples of immunosuppressive medications include methotrexate (Rheumatrex, Trexall), azathioprine (Imuran), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), and cyclosporine (Sandimmune). All immunosuppressive medications can seriously depress blood cell counts and increase risks of infection and bleeding. Other side effects are peculiar for each drug. For examples, Rheumatrex can cause liver toxicity, while Sandimmune can impair kidney function.

In recent years, mycophenolate mofetil (Cellcept) has been used as an effective medication for lupus, particularly when it associated with kidney disease. Cellcept has been helpful in reversing active lupus kidney disease (lupus renal disease) and in maintaining remission after it is established. It's lower side effect profile has advantage over traditional immune suppression medications.

In SLE patients with serious brain or kidney disease, plasmapheresis is sometimes used to remove antibodies and other immune substances from the blood to suppress immunity. Some SLE patients can develop seriously low platelet levels, thereby increasing the risk of excessive and spontaneous bleeding. Since the spleen is believed to be the major site of platelet destruction, surgical removal of the spleen is sometimes performed to improve platelet levels. Other treatments have included plasmapheresis and the use of male hormones. Plasmapheresis has also been used to remove proteins (cryoglobulins) that can lead to vasculitis. Endstage kidney damage from SLE requires dialysis and/or a kidney transplant.

Most recent research is indicating benefits of rituximab (Rituxan) in treating lupus. Rituximab is an intravenously infused antibody that suppresses a particular white blood cell, the B cell, by decreasing their number in the circulation. B cells have been found to play a central role in lupus activity, and when they are suppressed, the disease tends toward remission.

At the 2007 national Rheumatology meeting, there was a paper presented suggesting that low dose dietary supplementation with omega 3 fatty acids (Salvia Hispanica) could help patients with lupus by decreasing disease activity and possibly decreasing heart disease risk.

WHAT DOES THE FUTURE HOLD FOR PATIENTS WITH LUPUS?

Overall, the outlook for patients with systemic lupus is improving each decade with the development of more accurate monitoring tests and treatments. Individuals with SLE can improve their prognosis by learning about the many aspects of the illness as well as closely monitoring their own health with our ADAS providers.

WHERE CAN ONE GET MORE INFORMATION ABOUT LUPUS?

For more information about systemic lupus erythematosus, see the following sites:

The Arthritis Foundation (http://www.arthritis.org)
P.O Box 19000
Atlanta, Georgia 30326
Lupus Foundation of Minnesota (http://www.lupusmn.org)

If you would like to set up a consultation about a skin rash that you think may be lupus, click here or call 904-285-7546. Thank you and we look forward to helping you.