Systemic Lupus Erythematosus (SLE) MedicationA more recent article on systemic lupus erythematosus is available. Steroid sparing drugs for lupus survival rates have improved, over one half of patients with systemic lupus erythematosus have permanent drugz in one or more organ systems. Arthritis and cutaneous manifestations are most common, but renal, hematologic and neurologic manifestations contribute largely to morbidity and mortality. Treatment approaches emphasize dugs a combination of drugs to minimize chronic anavar (oxandrolone) comprar to corticosteroids. Systemic lupus erythematosus has fascinated physicians for almost a century and remains the prototypic autoimmune disease.
Medications | The Lupus Alliance of LIQ has been helping those with Lupus since
A more recent article on systemic lupus erythematosus is available. Although survival rates have improved, over one half of patients with systemic lupus erythematosus have permanent damage in one or more organ systems.
Arthritis and cutaneous manifestations are most common, but renal, hematologic and neurologic manifestations contribute largely to morbidity and mortality. Treatment approaches emphasize using a combination of drugs to minimize chronic exposure to corticosteroids. Systemic lupus erythematosus has fascinated physicians for almost a century and remains the prototypic autoimmune disease.
Although it is estimated to affect one out of every 1, white persons and one out of every black women from 18 to 65 years of age, 1 systemic lupus erythematosus is certainly not the most common example of autoimmunity encountered by physicians. Positive antinuclear antibodies are extremely common in the general population, occurring in as many as 10 to 20 percent of young women.
Because systemic lupus erythematosus is a chronic disease, patients require extensive health education in terms of their responsibility in managing their condition. This requires compliance with office visits and medications, and lifestyle modifications to reduce or prevent associated problems such as hyperlipidemia, obesity and hypertension. An ongoing partnership between the primary care physician and the rheumatologist is essential in the long-term management of patients with systemic lupus erythematosus.
The cause of systemic lupus erythematosus remains elusive. Predisposing factors include genetic factors certain types of human leukocyte antigens and null complement alleles , environmental factors including sun exposure, some drugs such as sulfa antibiotics, and hormonal factors. Systemic lupus erythematosus is more common in blacks than in whites and is obviously more common in women than in men ratio: The pathophysiology of systemic lupus erythematosus is not completely understood.
The production of abnormal antibodies by B cells remains the hallmark sign of lupus erythematosus. Some of the autoantibodies, such as anti—double-stranded DNA and anti-Smith, are very specific for systemic lupus erythematosus. Whether the B cells themselves are intrinsically abnormal is a subject of current research. One of the underlying defects in systemic lupus erythematosus may center on apoptosis, or programmed cell death. In patients with systemic lupus erythematosus, cellular antigens exposed during apoptosis incite an immune response.
The diagnosis of systemic lupus erythematosus requires a thorough history, a physical examination and laboratory tests, including a complete blood cell count, chemistry panel and urinalysis.
Serologic tests such as antinuclear antibodies, anti-Rh o , anti-La, anti-RNP, anti-Sm, anti-dsDNA and antiphospholipid antibodies are helpful to confirm the diagnosis. The American College of Rheumatology ACR has developed criteria to classify patients with a diagnosis of systemic lupus erythematosus for research studies Table 1. Four of the 11 criteria must be met.
Before a patient can be classified with systemic lupus erythematosus, at least four of the following 11 disorders must be present:. The revised criteria for classification of systemic lupus erythematosus. Arthritis Rheum ; Systemic lupus erythematosus is a disease that continues to evolve over time.
Thus, a patient who presents with skin and joint disease remains at risk for renal disease even after having lupus erythematosus for decades. Continued monitoring, even when the disease appears to be clinically inactive, is essential. It is very important that a partnership be established between the primary care physician, the rheumatologist and any other physicians caring for the patient.
This partnership has been summarized in the guidelines from the ACR. The major challenge for physicians managing patients with lupus erythematosus is to treat the active phase without allowing the treatment itself to cause long-term damage.
This intent has led to a major change in treatment approach, with the goal of limiting corticosteroid exposure, if possible. As a result, physicians are now less reluctant to turn to immunosuppressive drugs such azathioprine Imuran or cyclophosphamide Cytoxan. Treatment for active systemic lupus erythematosus differs, depending on the organ systems involved and disease severity. Current treatment often includes a combination of drugs. At some point, over 90 percent of patients with systemic lupus erythematosus have polyarthralgias or polyarthritis because of the disease.
Nonsteroidal anti-inflammatory drugs NSAIDs remain the mainstay of treatment in these patients, especially those who have mild polyarthralgias or polyarthritis Table 2. In addition, NSAIDs may adversely affect renal function, a special concern because 50 percent of patients with systemic lupus erythematosus develop associated nephritis.
Hydroxychloroquine Plaquenil is the antimalarial drug used most often in the United States; ophthalmologic monitoring is recommended every six to 12 months. Methotrexate Rheumatrex or azathioprine Imuran can be used as steroid-sparing drugs; methotrexate cannot be used during pregnancy. Antimalarial agents, especially hydroxychloroquine Plaquenil , are frequently used in the treatment of polyarthritis.
Hydroxychloroquine is very safe; only 1 percent of patients using it develop retinopathy. Retinopathy as a result of hydroxychloroquine use is usually reversible when the drug is discontinued. In spite of NSAID and antimalarial therapy, some patients require corticosteroids to control severe polyarthritis. An extremely severe flare-up of polyarthritis can be treated with intravenous methylprednisolone sodium succinate A-Methapred, Solu-Medrol , 1, mg administered over 90 minutes, given daily for three days.
This therapy will usually abruptly stop the flare, allowing the patient to stay on a low-maintenance dosage of prednisone. If the maintenance dosage of prednisone is greater than 10 mg per day, additional steroid-sparing drugs can be added to the regimen. Low dosages of methotrexate Rheumatrex , such as 7. It is now standard practice to use folic acid to counter some of the minor side effects of methotrexate.
Additional musculoskeletal complications of systemic lupus erythematosus include osteonecrosis and osteoporosis Table 3. Osteonecrosis, also called avascular necrosis of bone, occurred in 14 percent of patients in one study. Occurs in 14 percent of patients with systemic lupus erythematosus. Most commonly affects the hip joints.
Early detection requires MRI. Core decompression of bone is an effective treatment in early stages of the disease. Occurs in 64 percent of patients with systemic lupus erythematosus. Osteoporosis of the lumbar spine is associated with the highest dosage of prednisone and the cumulative effects of prednisone. Calcium, vitamin D, calcitonin and bisphosphonates are effective treatments even in premenopausal women with osteoporosis. The major risk factors for osteonecrosis include a prednisone dosage greater than 20 mg per day for one month or longer, and the presence of Raynaud's disease or vasculitis.
The early detection of avascular necrosis of bone usually requires an MRI scan of the hip. The same study reported that only 35 percent of premenopausal women with lupus erythematosus had normal bone mineral density. Guidelines from the ACR have suggested the use of estrogen supplementation in premenopausal women with corticosteroid-induced osteoporosis, but this therapy has not yet been shown to be safe in patients with systemic lupus erythematosus.
Over 90 percent of patients with systemic lupus erythematosus eventually have a cutaneous manifestation of the disease, including malar rash, discoid lupus erythematosus, alopecia or aphthous stomatitis. The usual therapy for cutaneous lupus erythematosus is strict use of sun block, judicious use of topical steroids although fluorinated topical steroids should not be used on the face and antimalarial therapy Table 4.
Some patients with very severe cases of discoid lupus erythematosus may not respond adequately to the usual dosage of hydroxychloroquine, which is mg per day for a normal-sized adult. Quinacrine, in a dosage of mg per day, can be added without increasing the risk of retinopathy, or the patient can be switched to chloroquine HCl Aralen , in a dosage of mg per day. Use of combination antimalarial therapy hydroxychloroquine [Plaquenil] and quinacrine or chloroquine Aralen , which has more risk of retinopathy, is sometimes necessary.
Use of corticosteroids may be necessary as part of initial therapy for severe discoid lupus or for lupus vasculitis; intradermal corticosteroids are helpful for individual discoid lesions, especially in the scalp. Methotrexate Rheumatrex or azathioprine Imuran is used as steroid-sparing drug. One of the most effective drugs for treatment of discoid lupus, but teratogenicity and neuropathy will limit its acceptance and use.
Because chloroquine therapy carries an increased risk of retinopathy compared with hydroxychloroquine therapy, patients taking it should undergo ophthalmologic monitoring every three months. In patients who cannot tolerate antimalarials, dapsone or retinoids are additional therapeutic choices. It is important that glucosephosphate dehydrogenase status be checked in black patients before they begin dapsone therapy.
Retinoids should not be used in patients who may become pregnant. Patients with very severe cutaneous lupus erythematosus, including lupus vasculitis, may require high dosages of corticosteroids. If the maintenance dosage of corticosteroids is greater than 10 mg per day, the addition of steroid-sparing agents, such as methotrexate or azathioprine, should be considered.
One of the most effective drugs in the treatment of cutaneous lupus erythematosus, including discoid lupus erythematosus, is thalidomide Synovir ; however, because of its teratogenic effects and the increased risk of peripheral neuropathy in patients taking it, this agent will probably never have widespread use.
One of the major worries for physicians and patients with systemic lupus erythematosus is lupus nephritis Table 5. Overall, 50 percent of patients with systemic lupus erythematosus have some manifestation of lupus nephritis; this figure reached 75 percent in black patients who were followed prospectively by the author. Occurs in approximately 50 percent of patients with systemic lupus erythematosus. Renal biopsy can be helpful in identifying the activity of lupus nephritis and the degree of chronicity scarring.
Cyclophosphamide Cytoxan is more effective than corticosteroids alone for the treatment of severe forms of lupus nephritis diffuse proliferative glomerulonephritis. Not all lupus nephritis is severe. Patients with milder forms, including mesangial glomerulonephritis and focal proliferative glomerulonephritis, may respond to corticosteroid therapy alone or with steroid-sparing drugs such as azathioprine. However, the patient with rapidly progressive lupus nephritis, with diffuse proliferative glomerulonephritis on biopsy, or severe proteinuria and active urine sediment, will initially need high-dose corticosteroid therapy and should be a candidate for cyclophosphamide therapy.
Clinical trials at the National Institutes of Health have been instructive in the development of protocols for the administration of cyclophosphamide with minimal long-term toxicity.
Patients receiving this therapy may later be at risk for the development of malignancies, but the risk of leukemia and lymphoma appears to be very small. Premature ovarian failure is a significant complication and occurs in up to 60 percent of women over 30 years of age. The central nervous system manifestations of systemic lupus erythematosus can present in many forms and are often difficult to diagnose Table 6.
No gold standard diagnostic test currently exists. Characteristic abnormalities are frequently found on brain MRI, lumbar puncture and electroencephalogram. Psychosis and seizures are usually easy to diagnose and respond well to antipsychotics or anticonvulsants, as well as to corticosteroid treatment for systemic lupus erythematosus. However, many patients with systemic lupus erythematosus present with cognitive function difficulties, making it a challenge for the physician to differentiate between what is related to active lupus erythematosus, what is related to corticosteroid treatment, and what may be related to depression or chronic fatigue syndrome.
Fibromyalgia, a musculoskeletal syndrome characterized by generalized pain, fatigue and a variety of associated symptoms, is found in as many as 30 percent of patients with systemic lupus erythematosus and is frequently associated with chronic fatigue. Patients with central nervous system manifestations of lupus erythematosus who present with status epilepticus, organic brain syndrome or coma can be treated with intravenous methylprednisolone pulse therapy.
However, it is usually necessary to rule out other conditions that may mimic central nervous system manifestations of systemic lupus erythematosus, including infection and toxic metabolic states.
One of the major complications of systemic lupus erythematosus is premature or accelerated atherosclerosis Table 7. This complication is one of the causes of later mortality, in the perimenopausal and early postmenopausal years.