- severe fatigue.
- joint pain.
- joint swelling.
- headaches.
- a rash on the cheeks and nose, which is called a “butterfly rash”
- hair loss.
- anemia.
- blood-clotting problems.
What Is Lupus?
Lupus is an autoimmune disease whereby a misdirected immune system leads to inflammation and injury to one’s own body tissues.
Signs of Lupus
Lupus can involve many different parts of the body, including:
- Skin
- Joints
- Heart
- Lungs
- Kidneys
- Brain
There is no specific cure for lupus, but treatments are effective at minimizing lupus damage and improving function. Approximately 80% of lupus patients are women.
Lupus Symptoms: Joint Pain
Though the first signs of the lupus disease can be rash, there are often pains in the muscles and joints. Both sides of the body tend to be affected. Hands, wrists, knees, and feet are commonly affected. The joints can become swollen, warm, and have limited range of motion.
Lupus Symptoms: Butterfly Rash
A classic lupus rash involves the cheeks and over the bridge of the nose. This is referred to as a “butterfly-shaped” lupus rash. It is also common for the skin to be very sensitive to burning and irritation after sun exposure. This is referred to as photosensitivity.
Lupus Symptoms: Nail Changes
Lupus rash can cause a ruddy discoloration of the backs of the hands and fingers. There can also be poor circulation to the nail beds that leads to irregularities of the fingernails. Inflammation at the nail bed can cause swelling and puffiness.
What are risk factors and causes of systemic lupus erythematosus? Is lupus contagious? 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 caused by an infectious microorganism and is not contagious from one person to another.
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 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. 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.
People with SLE need more rest during periods of active disease. Researchers have reported that poor sleepquality was a significant factor in developing fatigue in people with SLE. These reports emphasize the importance for people and physicians to address sleep quality and the effect of underlying depression, lack of exercise, and self-care coping strategies on overall health. During these periods, carefully prescribed exercise is still important to maintain muscle tone and range of motion in the joints.
To protect from sun sensitivity, sunscreens, sun avoidance, and sun protection clothing are used. Certain types of lupus rash can respond to topical cortisone medications.
Nonsteroidal anti-inflammatory 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, 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 affected. 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 the tissues in large joints.
Hydroxychloroquine (Plaquenil) is an antimalarial medication found to be particularly effective for SLE people with fatigue, skin involvement, and joint disease. Consistently taking Plaquenil can prevent flare-ups of lupus. Side effects are uncommon but 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 people with systemic lupus. Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can directly act to prevent the blood clots. This fascinating study highlights an important reason for people and doctors to consider Plaquenil for long-term use, especially for those SLE people 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 venereal disease research laboratory test). This means not only that Plaquenil reduces the chance for re-flares of SLE, but it can also be beneficial in thinning the blood to prevent abnormal excessive blood clotting. Plaquenil is commonly used in combination with other treatments for lupus.
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 described below.
Medications that suppress immunity (immunosuppressive medications) are also called cytotoxic drugs. They are sometimes referred to as chemotherapy because they are also used to treat cancer, generally in much higher doses than those used to treat lupus. Immunosuppressive medications are used for treating people with more severe manifestations of SLE, such as 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. Immunosuppressive medications may not be taken during pregnancy or conception because of risk to the fetus. Other side effects are specific for each drug. For examples, methotrexate can cause liver toxicity, while cyclosporine can impair kidney function.
In recent years, mycophenolate mofetil (CellCept) has been used as an effective medication for lupus, particularly when it is 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. Its lower side-effect profile has advantage over traditional immune-suppression medications.
In SLE patients with serious brain (lupus cerebritis) or kidney disease (lupus nephritis), plasmapheresis is sometimes used to remove antibodies and other immune substances from the blood to suppress immunity. Plasmapheresis is a process of removing blood and passing the blood through a filtering machine, then returning the blood to the body with its antibodies removed. Rarely, people with SLE 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 certain harmful proteins (cryoglobulins) that can lead to vasculitis. End-stage kidney damage from SLE requires dialysis and/or a kidney transplant.