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Systemic steroids for lupus


If you feel overwhelmed or frustrated with some of the outward effects of your medications, your doctor can help you to come up with some strategies to minimize side-effects. However, it is important to realize that you play the most important role in helping yourself to stay as healthy as possible. There are many things you can do on a daily basis to help minimize the side effects of both steroid medications and your lupus symptoms.

A healthy diet is important for everyone, but it is especially important for people with lupus and those taking steroid medications. While taking steroids, your cholesterol, triglyceride, and blood sugar levels may increase. For these reasons, it is absolutely essential that you not increase your calorie intake and follow a low sodium, low-fat, and low-carbohydrate diet.

You do not need to cut out all of the foods you love, but concentrate on eating whole grain breads and cereals and lean sources of protein such as chicken and fish. If you need something to accompany your vegetables, try lighter dips like hummus. It is also important that you minimize alcohol intake when taking steroid medications, since steroids may already irritate your stomach.

In fact, it is best not to drink alcohol at all, because combining alcohol with certain lupus medications can be very harmful to your liver. Steroids may deplete certain vitamins in your body, such as vitamins C, D, and potassium. Your doctor may recommend for you to take supplemental vitamins or increase your intake of certain foods in order to make up for these deficiencies. Usually it is beneficial to take a multivitamin every day, but speak with your doctor to see which one is right for you, since some vitamins can adversely affect certain conditions.

For example, people with antiphospholipid antibodies, especially those taking anticoagulants such as warfarin Coumadin , should avoid vitamin K because it can increase the risk of blood clots. Steroids can also contribute to a thinning of the bones known as osteoporosis, which may put you at an increased risk for bone fractures.

Your doctor may prescribe a drug for osteoporosis or advise you to take a calcium or hormone supplement. Bisphosphonates such as Actonel, Fosamax, and Boniva are commonly prescribed, as are parathyroid hormone Forteo and other medications. To help keep your bones as strong as possible, try to increase your intake of calcium and vitamin D. Calcium helps to keep bones strong and vitamin D helps your body make use of calcium. Foods high in calcium include milk and milk products, tofu, cheese, broccoli, chard, all greens, okra, kale, spinach, sourkraut, cabbage, soy beans, rutabaga, salmon, and dry beans.

In addition to increasing your risk of osteoporosis, steroid medications can weaken your muscles. Staying as active as possible will help you to maintain strong muscles and bones. Weight-bearing activities such as walking, dancing, and running will help your muscles stay strong and healthy.

Many people report that these activities make them feel better mentally as well. However, you should never put yourself through more than reasonable discomfort when exercising. People with lupus should never smoke due to their increased risk of cardiovascular disease. Steroid medications increase this risk by upping blood pressure, triglycerides, and cholesterol. Smoking, steroids, and lupus make a very bad combination. Steroid medications can also increase the risk of infection; this risk increases if you are also taking immunosuppressive drugs.

For this reason, it is important that you try to avoid colds and other infections. Washing your hands regularly is perhaps the best way to keep germs at bay. More serious infections can lead to serious—even fatal—illness. The infections that most worry doctors are kidney infection, a type of skin infection called cellulitis, urinary tract infections, and pneumonia.

It is important to be on the lookout for any changes in your health, because people taking steroids may not run a fever even though they are very ill. If these infections go untreated, they could enter the bloodstream and pose an even bigger threat, so it is important that you notify your doctor at the first signs of an infection or illness. In addition, live virus vaccines, such as FluMist, the small pox vaccine, and the shingles vaccine Zostavax should be avoided because they may cause disease in individuals taking steroid medications.

Finally, since medications can increase your risk of cataracts and aggravate glaucoma, try to get an eye exam twice a year. Notify your doctor of any major changes in your vision. You should not stop taking steroids abruptly if you have been taking them for more than 4 weeks. Once your body has adjusted to taking steroids, your adrenal glands may shrink and produce less natural cortisone.

Therefore, it is important to slowly reduce the dosage of steroids to allow the adrenal glands to gradually regain their ability to produce cortisone on their own. Steroids are often given in high doses, which may increase the risk of side effects. However, as their name suggests, immunosuppressive work to suppress the immune system, so when taking these drugs, it is important to watch out for infection and notify your doctor at any sign of illness.

If you do acquire an infection, you may be prescribed an antibiotic or other medication, but be sure to stay away from Bactrim, since this medication can cause flares in some people with lupus. Because of the risk of osteoporosis, your doctor may also prescribe a bisphosphonate such as Actonel, Fosamax, or Boniva. Your doctor may also prescribe a diuretic to deal with bloating, fluid retention, and hypertension high blood pressure.

In addition, since cortisone can cause elevated cholesterol, your doctor may prescribe statins such as Lipitor, Crestor, Vytorin, or Caduet. These medications work to lower cholesterol. 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. It is also a major cause of morbidity. Studies conducted worldwide have suggested that somewhere between 6 and 10 percent of patients with systemic lupus erythematosus have clinically recognized premature atherosclerosis.

The pathogenesis of premature atherosclerosis is almost certainly multifactorial and includes direct effects of the disease and side effects of treatment. Longitudinal regression analyses 14 have shown that increasing the dosage of prednisone increases serum cholesterol, weight and blood pressure. Patients with systemic lupus erythematosus have higher levels of homocysteine, a known risk factor for atherosclerosis.

Corticosteroid treatment increases the levels of cardiovascular risk factors, including weight, blood pressure, cholesterol and homocysteine levels. Antiphospholipid antibody syndrome is one of the most common causes of acquired hypercoagulability in the general population and is much more common in patients with systemic lupus erythematosus Table 8. About one half of patients with systemic lupus erythematosus make antiphospholipid antibodies, including anticardiolipin antibody and lupus anticoagulant.

These antibodies often fluctuate over time, as does disease activity in general. Patients who have antiphospholipid antibodies have an increased risk of antiphospholipid antibody syndrome, a hypercoagulable state that can present with venous thrombosis, arterial thrombosis, recurrent pregnancy loss or thrombocytopenia. The two clinically important antiphospholipid antibodies are lupus anticoagulant and anticardiolipin antibody. Presentations of antiphospholipid antibody syndrome include thrombosis, recurrent or late pregnancy loss, and thrombocytopenia.

Long-term management of patients who have had a thrombotic event resulting from antiphospholipid antibody syndrome includes high-intensity warfarin Coumadin therapy INR of 3 to 4. The diagnosis of antiphospholipid antibody syndrome requires one of the four clinical presentations mentioned previously and the presence of either lupus anticoagulant or moderate- or high-titer anticardiolipin antibody.

In patients with systemic lupus erythematosus, the presence of lupus anticoagulant appears to be more specific for the syndrome than the presence of anticardiolipin antibodies, 16 but high-titer anticardiolipin antibody of the IgG or IgM class is also a risk factor. Patients with systemic lupus erythematosus who have already had a manifestation of antiphospholipid antibody syndrome require treatment.

Patients who have had venous or arterial thrombosis appear to benefit from maintenance therapy with high-intensity International Normalized Ratio of 3 to 4 warfarin Coumadin. Anemia in patients with systemic lupus erythematosus is most often associated with chronic disease or is related to iron deficiency.

Classic autoimmune hemolytic anemia can present acutely and severely or as a chronic condition. Severe hemolytic anemia is treated initially with intravenous methylprednisolone, 1, mg per day for three days. Leukopenia, which frequently occurs in patients with systemic lupus erythematosus, is usually not severe i.

Severe, life-threatening thrombocytopenia is treated with high-dose intravenous methylprednisolone but may also require intravenous immunoglobulin therapy. The long-term management of patients with severe thrombocytopenia may also include danazol Danocrine , vincristine Oncovin , immunosuppressive drugs and, in rare instances, splenectomy.

Twenty years ago, women with systemic lupus erythematosus were often told not to become pregnant. Today, most women with lupus erythematosus can have a successful pregnancy, although the potential for maternal and fetal complications does exist Table 9. Patients who require warfarin or cyclophosphamide therapy should not become pregnant because of the teratogenic potential of these drugs. Some patients with lupus erythematosus who have the anti-Rh o and anti-La antibodies do have a higher risk of congenital heart block in the fetus.

Patients with antiphospholipid antibody syndrome have an increased risk of pregnancy loss and may require heparin and low-dose aspirin therapy to have a successful pregnancy. Patients with active lupus erythematosus and renal disease, or those who require higher dosages of prednisone greater than 20 mg per day have an increased risk of preterm birth. Early losses are usually due to active lupus erythematosus or unknown factors. Second- or third-trimester losses are usually due to antiphospholipid antibody syndrome.

Mother usually has both anti-Rh o and anti-La antibodies. Most babies survive, but some have important morbidity. Monitoring the next pregnancy with serial four-chamber fetal echocardiograms may allow early detection of fetal heart block. Risk factors include active lupus erythematosus, maintenance therapy using prednisone dosages of more than 20 mg daily, renal disease and hypertension.

Increased risk of premature rupture of the membranes. Pre-eclampsia may be difficult to differentiate from renal flare caused by systemic lupus erythematosus. Already a member or subscriber? Log in. Michelle Petri, M. Petri received a medical degree from Harvard Medical School, Boston. After training in internal medicine at Massachusetts General Hospital, Boston, she completed fellowships in rheumatology, and allergy and immunology at the University of California, San Francisco, School of Medicine.

Address correspondence to Michelle Petri, M. Monument St. Reprints are not available from the author. Scopelitis E.

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