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The following medications are often used by doctors to treat other conditions that commonly occur in people with lupus. Although these drugs do not specifically address the underlying cause of lupus, they are used to treat other conditions that may be compounded or indirectly caused by lupus. Since lupus affects different people in different ways, treatment courses are highly individualized. Please remember to take your medications exactly as directed by your physician and notify him/her of any concerns upon your next visit. Never take any medications until they are approved by your doctor – in other words, do not self-medicate!

• Aspirin Low doses of aspirin are often recommended for lupus patients who have antiphospholipid antibodies and may reduce the risk of heart attack and stroke.
• Antidepressents Anti-depressant medications are used to treat depression and anxiety, present in almost half of all people who have lupus. It is important that you speak with your doctor if you feel you are experiencing clinical depression, because many people who are physically ill respond well to anti-depressant medications. In addition, your doctor may treat your depression in different ways depending on the cause.
• Antiplatelet Medications (Platelet Antagonists) Some lupus patients are at an increased risk for blood clots due to the prevalence of a condition known as antiphospholipid antibody syndrome (APS). Platelet antagonists help prevent these clots and in doing so, also help to prevent heart attack, stroke, and other complications.
• Osteoporosis Medications (Bisphosphonates) Bisphosponates are medications used to treat and prevent osteoporosis. People with lupus are at an increased risk for this condition due to the inflammation they experience with the disease. Certain medications taken by lupus patients also increase the risk of osteoporosis, especially corticosteroids such as prednisone.
• Blood Pressure Medications (Anti-hypertensives) 25-30% of people with lupus experience hypertension (high blood pressure). The most common causes of high blood pressure in people with lupus are kidney disease and long-term steroid use. Other medications, such as cyclosporine (Neoral, Sandimmune, Gengraf) can also cause elevations in blood pressure. It is important to remember that while diet and exercise are extremely important for optimal cardiovascular health, these elements alone may be insufficient in controlling your blood pressure; in this case, your doctor will prescribe a medication.
• Anticoagulants Anticoagulants (“blood thinners”) are medications that decrease the ability of the blood to clot and are used in lupus patients with antiphospholipid antibodies to reduce the risk of deep venous thrombosis (DVT), stroke, and heart attack.
• Gastrointestinal Medications Many people with lupus suffer from gastrointestinal problems, especially heartburn caused by gastroesophageal reflux disease (GERD). Peptic ulcers can also occur, often due to certain medications used in lupus treatment, including NSAIDs and steroids. Certain medications may be prescribed or recommended by your doctor to control these conditions.
• Cholesterol Medications (Statins) Statins are medications that lower the level of cholesterol in your blood by reducing the production of cholesterol in the liver. People with high levels of cholesterol in their blood face an increased risk of cardiovascular disease, which can lead to chest pain, heart attack, stroke, and peripheral vascular disease. Studies have shown that people with lupus are more likely to have clogged arteries that can lead to heart attack and stroke at a younger age. This increased risk is caused by elevated cholesterol levels, high blood pressure, diabetes, and inflammation, conditions that occur often in people with lupus. Certain medications, such as corticosteroids (e.g., prednisone) can provoke or compound these symptoms. For this reason, the cholesterol-lowering properties of statins are commonly called upon for lupus patients.
• Thyroid Medications Autoimmune thyroid disease is common in lupus. It is believed that about 6% of people with lupus have hypothyroidism (underactive thyroid) and about 2% have hyperthyroidism (overactive thyroid). A thyroid gland that is functioning improperly can affect the function of organs such as the brain, heart, kidneys, liver, and skin. Hypothyroidism can cause weight gain, fatigue, depression, moodiness, and dry hair and skin. Hyperthyroidism can cause weight loss, heart palpitations, tremors, heat intolerance, and eventually lead to osteoporosis. Treatment for both underactive and overactive thyroid involves getting your body’s metabolism back to normal.
• Fibromyalgia Medications Fibromyalgia is a chronic disorder characterized by widespread pain and tenderness, general fatigue, and non-restful sleep. Many people with lupus have fibromyalgia; in fact, much of the pain that people with lupus feel is due to this condition. Three medications are used to reduce some of the physical and emotional symptoms of fibromyalgia.
• Restasis (Dry Eye Medication) Restasis is an immunosuppressive medication used to treat eye symptoms related to Sjogren’s syndrome, a chronic autoimmune disorder in which the glands that produce tears and saliva do not function correctly.

About 50% of people with SLE will experience lung involvement during the course of their disease. Five main lung problems occur in lupus: pleuritis, acute lupus pneumonitis, chronic (fibrotic) lupus pneumonitis, pulmonary hypertension, and “shrinking lung” syndrome.

Pleuritis (Pleurisy)

Pleuritis, also known as pleurisy, is characterized by inflammation of the pleura, the two-layered membrane surrounding the lungs. If fluid forms and escapes the membrane, the condition is referred to as pleural effusion. Since inflammation of the pleura puts pressure on the lungs, people with pleuritis often experience pain when taking deep breaths. A pleural effusion is detectable on an x-ray, and pleurisy or a pleural effusion can be treated with NSAIDs or steroid medications. Antimalarials like Plaquenil may also be effective in preventing or fending off the condition.

Acute Lupus Pneumonitis

Acute lupus pneumonitis is a serious condition that affects anywhere from 1-10% of lupus patients. The condition is characterized by chest pain, shortness of breath, and a dry cough that may bring up blood. Acute lupus pneumonitis requires immediate treatment with high dose steroids and possibly immunosuppressive medications. Yet, even with successful treatment, some people develop lung scarring. Thus, it is very important that you speak with your physician if you experience any unfamiliar lung pains or symptoms.

Chronic (Fibrotic) Lupus Pneumonitis

As the name suggests, chronic lupus pneumonitis is the creeping cousin of acute lupus pneumonitis. Whereas the acute form comes on quickly, chronic lupus pneumonitis accumulates subtly over years. Some people with chronic lupus pneumonitis may have had acute lupus pneumonitis as some point during their lives, while others have not. Often the disease takes so long to develop that people do not realize or complain of the initial symptoms. While current inflammation can be treated with steroid medications, any scarring that occurs over time is permanent. Thus, it is very important that you remember to tell your doctor about any chest, lung, or breathing pain during regular visits. If caught early, chronic lupus pneumonitis can be treated.

Pulmonary Hypertension

In order for the organs in your body to work, they must receive oxygen. The right side of your heart is responsible for pumping blood from your veins back to your lungs to receive oxygen; the oxygenated blood then returns to the left side of the heart, where it is pumped to the rest of the body for use. Pulmonary hypertension is a unique form of hypertension (high blood pressure) in which the blood vessel that carries blood from the heart to the lungs thickens, reducing the amount of blood that can move through it in a given amount of time. In order to counter this resistance, your heart must pump harder and faster. When the heart fails to keep up, the rest of the body suffers by receiving less and less oxygen (a condition called hypoxia).

Pulmonary hypertension occurs in about 10% of people with lupus. On rare occasions pulmonary hypertension responds to corticosteroids and immunosuppressive treatment. However, if it is not reversible (meaning, if it is not due to active lupus), then your doctor can discuss with you one of the FDA-approved medications for pulmonary hypertension.

“Shrinking Lung” Syndrome

“Shrinking lung” syndrome is another rare lupus complication. People with the condition feel a sense of breathlessness and reduced chest expansion. Some people with shrinking lung syndrome improve with corticosteroid or immunosuppressive therapy.

Sources

• D’Cruz, D, Khamashta, MA, Hughes, G. Pulmonary Manifestations of Systemic Lupus Erythematosus. In: Wallace, DJ, Hahn, BH, eds. Dubois’ Lupus Erythematosus. 7^th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2007:678-699.
• Medsger, Thomas A. “Pulmonary Manifestations.” The Clinical Management of Systemic Lupus Erythematosus. Ed. Peter H. Schur. 2nd ed. Philadelphia: Lippincott-Raven, 1996. 87-94.
• “Pulmonary hypertension.” American Heart Association. 1 July 2009. <http://www.americanheart.org/presenter.jhtml?identifier=11076>.
• Wallace, Daniel J. “Pants and Pulses: The Lungs and Heart.” The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford University Press, 1995. 81-98.

• Prednisone
• Prednisolone
• Hydrocortisone
• Methylprednisolone (Medrol)
• Dexamethasone (Decadron)
• Triamcinolone IM
• IV methylprednisolone (Solu-Medrol)
• Topical Steroids

What are steroids, and why are they used to treat lupus?

Steroids are a group of chemicals that make up a large portion of the hormones in your body. One of these steroids, cortisone, is a close relative of cortisol, which the adrenal glands in your body make as a natural anti-inflammatory hormone. Synthetic cortisone medications are some of the most effective treatments for reducing the swelling, warmth, pain, and tenderness associated with the inflammation of lupus. Cortisone usually works quickly to relieve these symptoms. However, cortisone can also cause many unwelcome side effects, so it is usually prescribed only when other medications—specifically NSAIDs and anti-malarials—are not sufficient enough to control lupus.

The word “steroid” often sounds frightening because of the media attention given to the anabolic steroids that some athletes use to put on muscle. However, it is important to remember that steroids make up a large group of molecules with different functions, and the steroids given to treat lupus—specifically, corticosteroids—are different than those you may hear about on the news.

How do corticosteroids work to reduce inflammation in the body?

Inflammation is the body’s natural response to events such as injury, infection, and the presence of foreign substances—things your body doesn’t recognize as a part of itself. Sometimes, however, as with lupus, your body’s immune system does not function properly, and the inflammatory response works to damage your own tissues, causing stiffness, swelling, warmth, pain, and tenderness in different parts of the body. Corticosteroids help to slow and stop the processes in your body that make the molecules involved in your inflammatory response. These steroids also reduce the activity of your immune system by affecting the function of cells in your blood called white blood cells. In reducing inflammation and immune response, corticosteroids help to prevent damage to the tissues in your body.

What steroid medications are commonly prescribed for lupus?

Prednisone is the steroid most commonly prescribed for lupus. It is usually given as tablets that come in 1, 5, 10, or 20 milligram (mg) doses. Pills may be taken as often as 4 times a day or as infrequently as once every other day. Usually, a low dose of prednisone is about 7.5 mg per day or less, a medium dose is between 7.5 and 30 mg per day, and a dose of more than 30 mg qualifies as a high dose. Your doctor may also prescribe a similar drug called prednisolone, especially if you have had any liver problems. Prednisolone and prednisone are very similar. In fact, the liver must convert prednisone to prednisolone before the body can use it.

Sometimes lupus flares can be treated with an intra-muscular (IM) injection of a drug called Triamcinolone. These injections are usually given at your doctor’s office, and they often reduce flares without some of the side-effects that would accompany an increase in the dosage of an oral steroid like prednisone. Usually, the only noticeable side effect of these injections is a dimple or loss of pigmentation at the injection sight.

Steroids can also be given intravenously (IV) in the form of methylprednisolone (Solu-Medrol), and your doctor may prescribe higher doses of methylprednisolone (1000 mg) given over 3-5-day period. These treatments are often referred to as “pulse steroids.” Other forms of steroid medications commonly given for lupus are hydrocortisone, methylprednisolone (Medrol) dose packs, and dexamethasone (Decadron) tablets. These medications vary in potency. For example, hydrocortisone is weaker than prednisone, methylprednisolone is stronger, and dexamethasone is very potent. Ointments containing corticosteroids are also commonly prescribed for lupus rashes.

What are the side effects of steroid medications?

Steroid medications can have serious long-term side effects, and the risk of these side effects increases with higher doses and longer term therapy. For this reason, steroid medications are usually prescribed only after other less potent drugs have proven insufficient in controlling your lupus. Your doctor will work with you to determine the lowest dose of steroids necessary to control your lupus symptoms and will prescribe steroids for the shortest possible amount of time. Steroids are sometimes combined with other drugs to help reduce some of these side effects.

Possible side effects of taking these steroid medications are:

• Changes in appearance
  • Acne
  • Development of round/moon-shaped face (sometimes called “Cushing’s syndrome” after the physician who first described it)
  • Weight gain due to increased appetite
  • Redistribution of fat, leading to swollen face and abdomen, but thin arms and legs
  • Increased skin fragility, leading to easy bruising
  • Hair growth on the face
• Psychological problems
  • Irritability
  • Agitation, psychosis
  • Euphoria/depression (mood swings)
  • Insomnia
• Increased susceptibility to infections
• Stomach irritation, peptic ulceration
• Irregular menses (periods)
• Potassium deficiency
• Aggravation of the following preexisting conditions:
  • Diabetes
  • Glaucoma
  • High blood pressure
• Increase in:
  • Cholesterol
  • Triglycerides
• May suppress growth in children
• Long term side effects:
  • Avascular necrosis of bone (death of bone tissue due to lack of blood supply):
    • Usually associated with high doses of prednisone taken over long periods of time.
    • Produces pain, including night pain. Pain relief usually requires either a core bone biopsy or total surgical joint replacement.
    • Occurs most often in hip, but can also affect shoulders, knees, and other joints.
  • Osteoporosis
    • Thinning of the bones.
    • Can lead to bone fractures, especially compression fractures of vertebrae with severe back pain.
  • Cataracts
  • Glaucoma
  • Muscle weakness
  • Premature atherosclerosis – narrowing of the blood vessels by cholesterol (fat) deposits.
  • Pregnancy complications –Doses of 20mg or more have shown to increase pregnancy and birth complications, such as preeclampsia.

What can I do to stay as healthy as possible while taking my steroid medications?

While taking steroid medications such as prednisone, it may seem that your body’s reactions to the things you do and the food you eat are out of your hands. 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.

Diet

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.* When you need a snack, look to vegetables—they are low in sugar and calories and provide the perfect food for “grazing.” Try to eat them without Ranch dressing or vegetable dip, because these items carry lots of fat and calories. 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.

Osteoporosis

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.

Staying Active

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. In fact, there are actually chemicals in your brain triggered by significant exercise (usually about 30 minutes per day) that help you to attain a “natural high.” Your doctor can help you to assess your personal condition and decide on an exercise routine that is best for you. However, you should never put yourself through more than reasonable discomfort when exercising.

Smoking

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.

Infection

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.

Eye Exams

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.

Do not abruptly stop taking steroids

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.

Are there other drugs that I might take while taking steroids?

Steroids are often given in high doses, which may increase the risk of side effects. Medications called “immunosuppressive” drugs are sometimes prescribed in addition to steroids to help spare some of these undesirable 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. She/he may also recommend taking calcium or vitamin D supplements to reduce bone thinning. 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.

∗ The omega 3 fatty acids in fish and fish oil also have anti-inflammatory properties, which may help to reduce some of the discomfort in your joints and muscles.

Where are the kidneys, and what do they do?

The kidneys are two bean-shaped organs that rest under your abdominal cavity, towards your lower back. Most people think of the kidneys as organs responsible for filtering blood and producing urine. However, the kidneys play other vital roles in the body, including the regulation of blood volume, blood pH, and blood pressure. The functional unit of the kidney is called the nephron. Each nephron functions like a mini-kidney to remove waste and unneeded substances from your body, while also ensuring that you retain vital nutrients and water.

How does lupus affect the kidneys?

About one half of people with lupus experience kidney involvement, and the kidney has become the most extensively studied organ affected by lupus. The part of the kidney most frequently troubled by lupus is a part of the nephron called the glomerulus, a tuft of capillaries that functions to filter substances from of the blood. For this reason, the type of kidney inflammation most commonly experienced in lupus is called glomerulonephritis. Fortunately, the prognosis for patients experiencing kidney lupus is improving due to the growing number of medications that physicians have in their arsenal.

What are the signs of kidney lupus? How is it diagnosed and classified?

Swelling of the ankles and abdomen may be a sign of kidney disease, but the problem with kidney lupus is that often people are not aware that it is occurring until they visit the doctor for lab tests and blood work. Testing for the disease includes urinalysis, which your doctor will check for items such as red blood cells, red blood cell casts, and protein that has leaked from your kidneys into your urine. In addition, she/he will most likely ask you to perform a 24-hour urine test and determine the efficiency with which your kidneys are filtering blood by measuring your creatinine clearance. Lastly, your doctor may also recommend you to a kidney doctor (called a nephrologist) to perform a biopsy to determine the class and degree of your kidney involvement. There are 6 levels of kidney lupus (Normal glomeruli, Mesangial disease, Focal proliferative glomerulonephritis, Diffuse proliferative glomerulonephritis, Membranous nephropathy, and Sclerosing nephropathy) and the class in which your disease falls will help your doctor to design a specific course of treatment.

What is the treatment course for lupus nephritis?

The treatment of lupus nephritis is highly individualized, but several drugs provide an arsenal from which your physician may choose. Corticosteroids are often used to reduce the inflammation involved in lupus nephritis, especially milder forms. Oral prednisone can be used, as can higher doses of methylprednisolone given in “pulses.” Another class of medications called immunosuppressives can also be used. These medications help to reduce your immune system’s attack on your kidneys by suppressing certain cells involved in this response. However, both corticosteroids and immunosuppressives make you more susceptible to infection, so it is important to speak to your doctor if you feel that you are developing one.

How is kidney lupus monitored?

As you return to your doctor’s office, she/he will most likely keep close watch on your kidneys to ensure that your kidney involvement is not worsening. There is no all-encompassing test for kidney function; instead, your doctor will use a number of tests to monitor your blood and urine. By obtaining a blood urea nitrogen (BUN) levels and blood creatinine levels, she/he can monitor how well your kidneys are filtering blood. In addition, you blood pressure will be monitored, since high blood pressure is an indication of kidney degeneration. High levels of protein in the urine (proteinuria) can indicate increased kidney involvement, since protein usually does not cross from your blood vessels into your kidney tubules and thus should not appear in your urine. In addition, tests of blood complement and anti-DNA antibodies can suggest inflammation that may be affecting the kidneys.

An important note about medications and blood pressure

Due to the silent nature of kidney lupus, it is very important that you take your medications daily as prescribed by your doctor. Even if you do not feel sick, inflammation in your kidneys and other organs can lead to permanent, irreversible scarring. Your medications can combat this inflammation right now, but permanent scarring will never go away. If kidney disease is allowed to progress without treatment, your kidneys will fail, and you will need to go to dialysis three times per week to have your blood filtered. The only way to get off dialysis is to have a kidney transplant, and organ transplant opportunities are not easy to come by. This information is not meant to frighten you, but merely to ensure that you understand how important it is to take your medications!

In addition, since the kidneys are tied intimately to the regulation of your blood pressure, it is imperative that you maintain a healthy blood pressure of 120/80 mmHg. If your doctor prescribes a blood pressure medication to keep your kidney lupus in check, be sure to take it exactly as prescribed. Limit your intake of salt to less than 3 grams per day, and try to eat a healthy, well-balanced diet. The prognosis for people with lupus nephritis does not have to be grim; it is important to remember that you play the most important role in keeping your body as healthy as possible!

Sources

• Balow, JE, Boumpas, DT, Austin, HA. Renal Disease. In: Schur, PH, ed. The Clinical Management of Systemic Lupus Erythematosus. 2nd ed. Philadelphia: Lippincott – Raven; 1996:109-126.
• Dooley, MA. Clinical and Laboratory Features of Lupus Nephritis. In: Wallace, DJ, Hahn, BH, eds. Dubois’ Lupus Erythematosus. 7^th ed. Philadelphia: Lippincott Williams & Wilkins; 2007:1112-1130.
• Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford University Press, 1995. 139-145.

Because lupus can produce a variety of symptoms in different individuals, it may take some time for a physician to actually make the diagnosis. Often a doctor will say that lupus might be present, but that the current symptoms are insufficient to signify a firm diagnosis. In this event, s/he will likely monitor the patient’s symptoms, signs, and lab tests closely over time and have him/her return for regular visits.

No single finding qualifies an individual as having SLE. Instead, the American College of Rheumatology (ACR) has devised certain classification criteria, and four or more of these criteria must be present for a classification of lupus. [The term “classification” is not synonymous with “diagnosis.” “Classification” means that reasonable certainty exists for the diagnosis of lupus for research purposes.] Although, these criteria are currently being updated, they are believed to be about 90% effective. The ACR criteria include malar rash; discoid rash; photosensitivity (development of a rash after sun exposure); oral or nasal ulcers; arthritis of multiple joints; serositis: (inflammation of the lining around the lungs or heart); kidney disease indicated by protein or casts in the urine; neurological disorders such as seizures and psychosis; and blood disorders such as hemolytic anemia, leukopenia, and lymphopenia. Other signs that are common but not included in the classification criteria are hair loss or breaking, especially around the forehead, and Raynaud’s Phenomenon, a two- or three-color change of the fingertips upon cold exposure.

Although no one symptom qualifies someone as having lupus, certain clinical techniques can be used to narrow down the diagnosis. For example, a test for antinuclear antibodies (ANAs) in the blood is probably the first tool a physician will use. A positive ANA test does not necessarily mean that someone has lupus; in fact, one out of five normal women has a positive ANA. However, a negative ANA test greatly reduces the suspicion.

Disease indices

Other sets of criteria, known as disease activity indices, exist for the monitoring of lupus. These forms allow a physician examining a patient to check for the improvement or worsening of the disease. These forms include the BILAG (British Isles Lupus Assessment Group Index), SLEDAI (Systemic Lupus Erythematosus Disease Activity Index), SLAM (Systemic Lupus Activity Measure), ECLAM (European Consensus Lupus Activity Measurement), and the Lupus Activity Index (LAI). Sometimes these indices will show no signs of lupus, even when the patient feels badly. This is because some of the problems that occur in lupus, such as chronic fatigue and pain, are not tracked by the indices. Instead, these symptoms represent a co-occuring problem called fibromyalgia.

Other similar conditions

Since other diseases and conditions appear similar to lupus, adherence to classification can greatly contribute to an accurate diagnosis. However, the absence of four of these criteria does not necessarily exclude the possibility of lupus. When a physician makes the diagnosis of SLE, s/he must exclude the possibility of conditions with comparable symptoms, including rheumatoid arthritis, systemic sclerosis (scleroderma), vasculitis, dermatomyositis and arthritis caused by a drug or virus.

Sources

• Salmon, Jane E., and Robert P. Kimberly. “Systemic Lupus Erythematosus.” Hospital for Special Surgery Manual of Rheumatology and Outpatient Orthopedic Disorders: Diagnosis and Therapy. 5th ed. Philadelphia: Lippincott Williams & Wilkins, 2006. 221-38.
• Schur, Peter H. “General Symptomology.” The Clinical Management of Systemic Lupus Erythematosus. Ed. Peter H. Schur. 2nd ed. Philadelphia: Lippincott-Raven, 1996. 9-16.
• Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 3^rd ed. New York: Oxford University Press, 2005. 259.