Medications and Systemic Lupus Erythematosus

Medications play an important role in the care of most people with systemic lupus erythematosus (SLE). This pamphlet discusses the principal drugs used in the primary management of lupus (Table 1). The choice of drugs is highly individualized and typically changes often during the course of the disease. Factors that are considered in treatment decisions include the type and severity of lupus symptoms, the person's response to treatment, and risks of drug side effects.
In addtion, it is important to note that people with lupus often require other drugs for the treatment of conditions commonly seen with the disease. Examples of these types of medications include: diuretics for fluid retention, anti-hypertensive drugs for increased blood pressure, anti-convulsants for seizure disorders, antibiotics for infections, and drugs for osteoporosis.
Anti-Inflammatory Drugs
Anti-inflammatory drugs relieve the symptoms of lupus by reducing the inflammation responsible for the pain and discomfort. By far, anti-imflammatory medications are the most commonly used drugs for lupus treatment, particularly for symptoms such as fever, arthritis, or pleurisy. Improvement in symptoms is generally noted within several days of beginning treatment. In the majority of people with lupus, anti-inflammatory drugs are the only medication that is ever required to control their lupus.
Anti-inflammatory drugs fall into two categories: non-steroidal anti-inflammatory drugs (NSAIDs) or corticosteroids (Table 1). The NSAIDs include both salicylates (aspirin) and related drugs that may be purchased over the counter (Advil, Nuprin, Aleve) or that require a physician's prescription. NSAIDs are especially useful for musculoskeletal symtpoms (arthritis, arthralgia, joint stiffness or pain), fever and chest pain from mild peurisy (inflammation of the lining of the lung) or pericarditis (inflammation of the sac around the heart). For reasons that are not known, people often respond better to one non-steroidal drug than another. Thus, it may be necessary to treat a person with several different drugs to determine the most effective one.
The most common side effect of non-steroidal medications is irritation of the stomach or bowel which causes abdominal pain. Infrequently, this may lead to serious complications such as an ulcer with bleeding. To reduce the chance of these problems, non-steroidal drugs are usually taken with meals or given along with other medications that protect the stomach and bowel.
Occasionally, side effects form non-steroidal drugs may be mistaken for signs of active lupus. For instance, non-steroidal drugs may cause fever, skin rashes, abnormal urine studies, or severe headache that might mimic findings seen in lupus. Recognition of these side effects is important since the symptoms are promptly reversed simply by stopping the drugs.

Anti-Malarial Drugs
Drugs used for the treatment of malaria are widley used in the management of lupus sypmtoms. Anti-malarials are particularly effective in the treatment of lupus arthritis, skin, rashes, and mouth ulcers. Other possible benefits of anti-malarials include reducing the risk of blood clots and lowering cholestrol levels. The drug hydroxychloroquine (Plaquenil) is the most commonly used of the anti-malarial agents. Anti-malarials are considered to have a snall risk of causing birth defects and are generally not recommended for women who want to become pregnant.
The side effects of low-dose anti-malarial therapy include gastric symptoms (stomach pain or dyspepsia), rashes or darkening of the skin, and muscle weakness. Shortly after starting treatment, there may be a temporary mild blurring of vision. This goes away entirely on its own.
In high doses (such as those used in the treatment of malaria), certain anti-malarial drugs may damage the retina of the eye, causing vision problems. With the low doses of anti-malarials used in the treatment of lupus, the risk of this complication is extremely low. However, as a precaution, people treated with anti-malarials generally have a thorough eye examination before the drug is started and then every 6-12 months during therapy. This is so that any sign of damage to the retina can be detected early and, if neededn the drug can be stopped.
Corticosteroids (Cortisone)
Corticosteroids are naturally occuring hormones with very potent anti-inflammatory properties; in high doses they also suppress immune functions. They may be used as creams or ointments for lupus skin rashes or given as pills or injections. Most signs of lupus respond rapidly to corticosteroids treatment. At times, corticosteroids may actually be life saving.
The decision to begin corticosteroids, and the details as to what type and dose of corticosteroid to use, are highly individualized. For example, a person may have symptoms such as fever, arthritis, or pleurisy that have not responded to non-steroidal drugs. He/she could then be treated with low doses of an oral corticosteroid, such as prednisone or methylprednisolone (prednisolone). Individuals with more severe or serious lupus symptoms, such as kidney disease, seizures, anemia, or low platelets (thrombocytopenia) may require high doses of corticosteroids. In most instances, the corticosteroid is given as a single dose in the morning.
Other ways of giving corticosteroids include doses given several times each day, doses only on alternate days, or high doses intravenously (bolus therapy). Once the symptoms of lupus have responded to treatment, the dose of corticosteroids is gradually reduced (tapered). Meanwhile the individual is carefully watched for evidence of relapse of the disease. The longer a person has been on corticosteroids, the more difficult it becomes to lower the dose. It is very important that corticosteroids are taken exactly as prescribed. Treatment should never be stopped abruptly without consulting with a physician.
There are many complications of corticosteroid treatment. the risks of these complications are increased when high doses of corticosteroids are required, or when corticosteroids are used for an extended peroid. Corticosteroids may produce changes in physical appearance such as weight gain, puffy cheeks, thinning of the skin and hair, and easy bruising. Stomach discomfort such as dypepsia or heartburn are common. These may be minimized by giving the drug with meals or along with medications that prevent stomach damage. Marked changes in mood while taking corticosteroids include both depression and mood swings. Corticosteroids may cause diabetes, and may increases the risk of infections, muscle weakness, or cataracts. Corticosteroids may have an effect on the bones, producing damage of the hips, knees, or other joints (ostenonecrosis). Corticosteroids may also produce osteoporosis (thinning of bone) when given over long periods. In most people, calcium or other medications to prevent osteoporosis are given along with the corticosteroids.
Cytotoxic Drugs
Cytotoxic, or immunosuppressive, drugs are used to suppress the immune sustem in people with lupus. The most commonly used drugs of this type are azathioprine (Imuran), cyclophosphamide (Cytoxan), methotrexate (Rheumatrex), and cyclosporine (Sandimmune, Neoral). These drugs are genreally reserved for people with more serious manifestations of lupus-lupus nephritis or neurologic disease-in whom treatment with corticosteroids has failed.
It is very important that cytotoxic drugs only be given by physicians who are experienced with the use of these medications. The possible toxicites of cytotoxic drugs are considerable and individuals treated with these drugs must be very carefully monitored. The drugs have a major effect on cells produced by the bone marrow, including white blood cells, red blood cells, and platelets. Thus, people treated with cytotoxic drugs must have regular complete blood counts (CBCs) to make certain that levels of these cells do not become too low. In addition, cytotoxic drugs reduce a person's ability to fight off infections. Those receiving cytotoxic drugs are more likely to contract viral infections such as shingles (herpes zoster), and other more serious infections may also develop.
There are distinct toxicites that are unique to each cytotoxic drug. Cyclophosphamide, for instance, amy cause hair loss, bladder complications, and sterility. Azathioprine may cause an allergic-type of hepatitis and pancreatits. Methotrexate may cause liver damage, including cirrhosis, as well as a serious lung toxicity. Cyclosporine commonly produces hypertension and may lead to kidney damage. All cytotoxic drugs are thought to increase a person's risk for developing cancer.
Investigational Drugs
A number of investigational (research) drugs for lupus are currently being studied in clinical trials. Promising new treatments include hormone modifications, more selective immunosuppressive drugs, and biologic agents.
Studies have suggested that using the hormone dehydroepiandrosterone (DHEA) and blocking secretion of the hormone prolactin with bromocriptine, amy improve lupus symptoms and reduce the need for corticosteroids. Preliminary studies indicate that newer, more potent immuonsuppressive drugs may be effective in people with lupus nephritis. Drugs such as mycophenolate mofetil (CellCept) and 2-chlorodeoxyadenosine how less risk of serious side effects compared to more conventional cytotoxic drugs. Finally, researchers are using biologic agents to selectively block the immune system from forming autoantibodies that develop in lupus. This represents a new and exciting approach for both treating and preventing the disese.
There is a need for people with lupus to know about the drugs used in the treatment of their disease. This pamphlet briefly reviews the major drugs currently being used. If you have any questions about the information presented here, you should check with your physician, your pharmacist, or call the Lupus Foundation of America.