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- 1. Erythematosus
- 2. Advances
- 3. Tests
- 4. Care
- 5. Medications
- 6. Psychosocial Aspects
- 7. Patient Info.
- 8. Resources
Publication Date: May 2001
Revised September 2006
Lupus: A Patient Care Guide for Nurses and Other Health Professionals
Chapter 5: Medications Used to Treat Lupus
- Patients and Providers: Working Together
- Educating Patients About Lupus Medications
- Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)
- Intravenous Immunoglobulins (IVIGs)
- Other Drugs
Medications are important for managing many systemic lupus erythematosus (SLE) patients. An array of drug therapies is now available, and this has increased the potential for effective treatment and excellent patient outcomes. Once a person has been diagnosed with lupus, the doctor will develop a treatment plan based on the person’s age, health, symptoms, and lifestyle. It should be reevaluated regularly and revised as necessary to ensure that it is as effective as possible. The goals for treating a patient with lupus include:
- reducing tissue inflammation caused by the disease
- suppressing immune system abnormalities that are responsible for tissue inflammation
- preventing flares and treating them when they do occur
- easing symptoms such as joint pain and fatigue
- minimizing complications of the disease.
People with lupus should work with their doctors to develop their medication treatment plan. Patients should thoroughly understand the reason for taking a drug, its action, dose, administration times, and common side effects. Pharmacists also can be a good resource for patients by helping them understand their medication treatment plan. If a patient experiences a problem believed to be related to a medication, the patient should notify her or his doctor immediately. It can be dangerous to suddenly stop taking some medications, and patients should not stop or change treatments without first talking to their doctor.
The array of drugs and the complexity of treatment plans can be overwhelming and confusing. Newly diagnosed patients and patients whose treatment plans have changed should be closely followed and have immediate access to a nurse or doctor if they are having problems with the prescribed medications. Most SLE patients do well on lupus medications and experience few side effects. Those who do experience negative side effects should not become discouraged, because alternative drugs are often available.
Health professionals should review drug treatment plans with the lupus patient at each office visit to determine her or his understanding of and compliance with the plan. Questions should be encouraged and additional teaching done to reinforce or provide additional information as needed. It is important to note that people with lupus often require drugs for the treatment of conditions commonly seen with the disease. Examples of these types of medications include diuretics, antihypertensives, anticonvulsants, cholesterol-lowering drugs, and antibiotics. Vaccinations are important to prevent diseases that could present a particular danger to people with lupus; however, live vaccines are not advised for people with lupus who are taking immunosuppressive drugs.
This chapter describes some of the main drugs used to treat SLE. The information presented is intended as a brief review and reference. Drug references and other medical and nursing texts provide more complete and detailed information regarding the use of each drug and the associated nursing care responsibilities.
Chapter 7 contains a Patient Information Sheet on each category of lupus medication covered here. The sheets can be used to teach people with lupus about the medications they may need to take. Each sheet contains general information about the category of drug, specific instructions on how and when to take the specific medication prescribed, information about possible side effects, and precautions.*
*Brand names included in this book are provided as examples only; their inclusion does not mean that these products are endorsed by NIH or any other Government agency. Also, if a particular brand name is not mentioned, this does not mean or imply that the product is unsatisfactory.
The NSAIDs comprise a large and chemically diverse group of drugs that possess analgesic, anti-inflammatory, and antipyretic properties. Pain and inflammation are common problems in patients with SLE, and NSAIDs are usually the drugs of choice for patients with mild SLE and little or no organ involvement. Patients with serious organ involvement may require more potent anti-inflammatory and immunosuppressive drugs.
Types of NSAIDs
There are more than two dozen different NSAIDs on the market, and many new ones are in development. Some can be purchased as over-the-counter preparations, whereas larger doses of those drugs or other preparations are available only by prescription. For example, prescriptions are required for diclofenac sodium (Voltaren®), indomethacin (Indocin®), diflunisal (Dolobid®), and nabumetone (Relafen®).
Mechanism of Action and Use
The therapeutic effects of NSAIDs stem from their ability to inhibit the release of prostaglandins and leukotrienes, which are responsible for producing inflammation and pain. NSAIDs are very useful in treating joint pain and swelling, as well as muscle pain. They may also be used to treat pleuritic chest pain. An NSAID may be the only drug needed to treat a mild flare; more active disease may require additional medications.
Although all NSAIDs appear to work in the same way, not every one has the same effect on every person. In addition, patients may do well on one NSAID for a period of time, then, for some unknown reason, derive no benefit from it. Switching the patient to a different NSAID should produce the desired effects. Patients should use only one NSAID at any given time.
Gastrointestinal (GI): dyspepsia, heartburn, epigastric distress, and nausea; less frequently, vomiting, anorexia, abdominal pain, GI bleeding, and mucosal lesions. Misoprostol (Cytotec®), a synthetic prostaglandin that inhibits gastric acid secretion, may be given to prevent GI intolerance. It prevents gastric ulcers and their associated GI bleeding in patients receiving NSAIDs. Another product, Arthrotec®, combines misoprostol with the NSAID diclofenac sodium in a single pill.
Genitourinary: fluid retention, reduction in creatinine clearance, and acute tubular necrosis with renal failure.
Hepatic: acute reversible hepatotoxicity
Cardiovascular: hypertension and moderate to severe noncardiogenic pulmonary edema. All NSAIDS now carry a warning that they may increase the risk of myocardial infarction.
Hematologic: altered hemostasis through effects on platelet function
Other: skin eruption, sensitivity reactions, tinnitus, and hearing loss
Pregnancy and Lactation
NSAIDs should be avoided after the first trimester. NSAIDs appear in breast milk and should be used cautiously by breast-feeding mothers.
This group of drugs was first developed during World War II because quinine, the standard treatment for malaria, was in short supply. Investigators discovered antimalarials could also be used to treat the joint pain that occurs with rheumatoid arthritis. Subsequent use of antimalarials showed that they are effective in controlling lupus arthritis, skin rashes, mouth ulcers, fatigue, and fever. They have also been shown to be effective in the treatment of discoid lupus erythematosus. Antimalarials are not used to manage more serious, systemic forms of SLE that affect the organs. It may be weeks or months before the patient notices that these drugs are controlling disease symptoms.
Types of Antimalarials
The drugs most often prescribed are hydroxychloroquine sulfate (Plaquenil®) and chloroquine (Aralen®).
Mechanism of Action and Use
The anti-inflammatory action of these drugs is not well understood. In some patients who take antimalarials, the total daily dose of corticosteroids can be reduced. Antimalarials also affect platelets to reduce the risk of blood clots and lower plasma lipid levels.
Central Nervous System: headache, nervousness, irritability, dizziness, muscle weakness, and tinnitus
Gastrointestinal: nausea, vomiting, diarrhea, abdominal cramps, and loss of appetite
Ophthalmologic: Visual disturbances and retinal changes are manifested by blurring of vision and difficulty in focusing. A very serious potential side effect of antimalarial drugs is damage to the retina. Because of the relatively low doses used to treat SLE, the risk of retinal damage is quite small: about 1 in 5,000. However, patients should have a thorough eye examination before starting this treatment and yearly thereafter.
Dermatologic: dryness, pruritus, alopecia, skin and mucosal pigmentation, skin eruptions, and exfoliative dermatitis
Hematologic: blood dyscrasia and hemolysis in patients with glucose 6-phosphate dehydrogenase (G6PD) deficiency
Antimalarials are usually continued during pregnancy. They do cross the placenta, but a clinical trial and case series have not found safety issues.
Corticosteroids are hormones secreted by the cortex of the adrenal gland. SLE patients with symptoms that do not improve or who are not expected to respond to NSAIDs or antimalarials may be given a corticosteroid. Although corticosteroids have potentially serious side effects, they are highly effective in reducing inflammation, relieving muscle and joint pain and fatigue, and suppressing the immune system. They are also useful in controlling major organ involvement associated with SLE. These drugs are given in much higher doses than the body produces and act as potent therapeutic agents. The decision to use corticosteroids is highly individualized and is dependent upon the patient’s condition.
Once the symptoms of lupus have responded to treatment, the dose is usually tapered until the lowest possible dose that controls disease activity is achieved. Patients must be monitored carefully during this time for flares or recurrence of joint and muscle pain, fever, and fatigue that can result when the dosage is lowered. Some patients may require corticosteroids only during active stages of the disease; those with severe disease or more serious organ involvement may need long-term treatment.
Treatment with corticosteroids must not be stopped suddenly if they have been taken for more than 4 weeks. Administration of corticosteroids causes the body’s own production of adrenal hormones to slow down or stop, and adrenal insufficiency will result if the drug is stopped suddenly. Tapering the dose allows the body’s adrenal glands to recover and resume production of the natural hormones. The longer a patient has been on corticosteroids, the more difficult it is to lower the dose or discontinue use of the drug.
Types of Corticosteroids
Prednisone (Orasone®, Meticorten®, Deltasone®, Cortan®, Sterapred®), a synthetic corticosteroid, is most often used to treat lupus. Others include hydrocortisone (Cortef®, Hydrocortone®), methylprednisolone (Medrol®), and dexamethasone (Decadron®). Corticosteroids are available as a topical cream or ointment for skin rashes, as tablets, and in injectable form for intramuscular or intravenous administration.
Mechanism of Action and Use
The frequently prescribed corticosteroids are highly effective in reducing inflammation and suppressing the immune response. These drugs may be used to control exacerbation of symptoms and are used to control severe forms of the disease. These drugs are usually administered orally. During periods of serious illness or prior to surgery, they may be administered intravenously; once the patient has been stabilized (or patient is able to have oral fluids after surgery), oral administration should be resumed.
Central Nervous System: depression, mood swings, and psychosis
Cardiovascular: congestive heart failure (CHF) and hypertension*
Endocrine: Cushing’s syndrome, menstrual irregularities, and hyperglycemia
Gastrointestinal: GI irritation, peptic ulcer, and weight gain
Dermatologic: thin skin, petechiae, ecchymoses, facial erythema, poor wound healing, hirsutism,* urticaria, and acne
Musculoskeletal: muscle weakness, loss of muscle mass, and osteoporosis*
Ophthalmologic: increased intraocular pressure, glaucoma, exophthalmos, and cataracts*
Other: immunosuppression and increased susceptibility to infection
* long-term effects
Pregnancy and Lactation
Corticosteroids cross the placenta, but can be used cautiously during pregnancy. They also appear in breast milk; patients taking large doses should not breastfeed.
Immunosuppressive agents are generally used to reduce rejection of transplanted organs. They are also used in serious, systemic cases of lupus in which major organs such as the kidneys are affected or in which there is severe muscle inflammation or intractable arthritis. Because of their steroid-sparing effect, immunosuppressives may also be used to reduce or sometimes eliminate the need for corticosteroids, thereby sparing the patient from undesirable side effects of corticosteroid therapy.
Immunosuppressives can have serious side effects. Patients need to understand, however, that side effects are dose-dependent and are generally reversible by reducing the dose or stopping the medication.
Types of Immunosuppressives
A variety of immunosuppressive drugs is available to treat lupus. Although they have different mechanisms of action, each type functions to decrease or prevent an immune response. The immunosuppressives most frequently used with SLE patients are:
- azathioprine (Imuran®). Azathioprine, one of the most widely used immunosuppressives for lupus, is an antimetabolite. Antimetabolites work by blocking metabolic steps within immune cells and then interfering with immune function. Used to control the underlying disease process, azathioprine has fewer serious side-effect risks than some other drugs used to control lupus.
- cyclophosphamide (Cytoxan®). An alkylating agent and strong immunosuppressive, cyclophosphamide is reserved for treating lupus with kidney disease or other internal organ involvement. It works by targeting and damaging autoantibody-producing cells, thereby suppressing the hyperactive immune response and reducing disease activity. It has the potential for severe side effects, including risk of serious infection.
- methotrexate (Rheumatrex®). Originally developed as a cancer treatment and later approved for rheumatoid arthritis, methotrexate, like azathioprine, is an antimetabolite. It is predominantly used for lupus arthritis. It requires monitoring of the CBC and liver function tests. To reduce toxicity, daily folic acid is prescribed.
- cyclosporine (Neoral®). Originally developed to prevent the body from rejecting transplanted organs, cyclosporine is now commonly used to treat rheumatic diseases, including lupus. Cyclosporine is an antimetabolite.
- mycophenolate mofetil (CellCept®). A strong immunosuppressive drug developed to prevent the rejection of transplanted organs, mycophenolate is sometimes used as an alternative to cyclophosphamide for lupus with kidney involvement. Mycophenolate works by keeping T and B lymphocytes from replicating.
There are many serious risks associated with the use of immunosuppressives. They include immunosuppression (resulting in increased susceptibility to infection), bone marrow suppression (resulting in decreased numbers of RBCs, WBCs, and platelets), and development of malignancies.
Dermatologic: alopecia (cyclophosphamide and methotrexate)
Gastrointestinal: nausea, vomiting, stomatitis, esophagitis, and hepatotoxicity
Hematologic: thrombocytopenia, leukopenia, pancytopenia, anemia, and myelosuppression
Respiratory: pulmonary fibrosis***
Other: increased risk of serious infections or malignancies
* temporary or reversible once drug therapy is discontinued
** recovery of function after drug is discontinued is unpredictable
*** with high doses
Pregnancy and Lactation
Use of immunosuppressives presents definite risks to the fetus. Female patients should use contraceptive measures during treatment and for 12 weeks after ending azathioprine therapy. Azathioprine may pass into breast milk, and women using this drug should consult with their doctors before breastfeeding.
IVIGs may be used to control SLE with organ involvement or vasculitis. Although the mechanism by which these products help is not well-understood, it is thought that they reduce antibody production or promote the clearance of immune complexes from the body.
Although an IVIG, like any drug, can cause potentially dangerous side effects, it doesn’t suppress the immune system the way immunosuppressives and corticosteroids do. Thus, the risk of serious infections with these drugs is less.
Dermatologic: rash, mild skin reaction at injection site
Gastrointestinal: abdominal cramps, nausea, vomiting
Musculoskeletal: chest, back or hip pain; muscle pain; joint pain
Neurologic: anxiety, chills, dizziness, fever, headache
Other: chest tightness, difficulty breathing, burning sensation in the head
In addition to these commonly used classes of medications, there are a number of other medications used, often experimentally, to treat lupus. These include thalidomide and dehydroepiandrosterone (DHEA).
- Thalidomide is a notorious sleep aid that was pulled from the market in European countries when people discovered it caused birth defects. However, it is showing effectiveness as a treatment for skin manifestations of lupus. Although its precise mechanism is not clear, thalidomide inhibits inflammatory cytokines in the skin and underlying blood vessels. Thalidomide carries the risk of many side effects, including abdominal pain, infection, chills, diarrhea, liver abnormalities, anemia, peripheral edema, hyperlipidemia, leukopenia, insomnia, sensory neuropathy, albuminuria, hematuria, ovarian failure, and thrombosis. Because of the risk of birth defects, it is strictly contraindicated in women who are pregnant or might become pregnant. Scientists are working on developing a safer version of thalidomide. A number of other drugs are used to treat specific manifestations of lupus.
- DHEA is a weak male hormone used by the body to make other hormones, including testosterone and estrogen. It appears to benefit women with lupus by helping to restore the balance of male-female hormones. The manufacturer of the DHEA product Prestara® recently completed a phase III clinical trial to determine if DHEA improves bone mineral density in women with lupus who are receiving corticosteroid therapy. There was a modest change at 1 year. An earlier study of the drug confirmed Prestara®’s ability to improve or stabilize clinical outcome and disease symptoms in women with mild-to-moderate lupus. The FDA did not approve DHEA for treating lupus. In the meantime, patients may get DHEA from a compounding pharmacist. If you have patients using DHEA, it is important to stress that they purchase a medical-grade product from a pharmacy and have regular check-ups to monitor response to the drug. DHEA is never used in men with lupus. In postmenopausal women, mammograms and PAP smears must be monitored.