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Johns Hopkins Lupus Center

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    • Meet Dr. Petri
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Lupus Primer

Lupus Primer

Lupus is a disease in which the immune system begins to recognize and attack the body’s own tissues. This phenomenon is similar to “friendly fire” and causes inflammation in different organs of the body. The nature of lupus is highly individualized, and two patients may experience two sets of totally different symptoms. In the United States, lupus affects roughly 1 in 2000 people, and 9 out of 10 lupus cases occur in women. Although the disease occurs in people of all races and ethnic groups, it occurs more frequently in African Americans.

The first symptoms of lupus usually occur somewhere between the teen years and the 30s and may be mild, severe, sporadic, or continual. Common general symptoms include fatigue, fever, and hair loss. Lupus can also affect individual organs and body parts, such as the skin, kidneys, and joints.

The following pages provide introductory information on lupus for patients, loved ones, and health care providers. Think of this as “Lupus primer.”

Understanding the Signs, Symptoms & Diagnosis of Lupus

  • How is Lupus diagnosed?
  • Types of Lupus
  • Causes of Lupus
  • Signs, Symptoms, and Co-occuring conditions

How Lupus Affects the Body

  • Antiphospholipid Antibodies
  • Arthritis
  • Cardiovascular System
  • Immune System
  • Kidneys
  • Lungs
  • Nervous System
  • Skin

Lifestyle & Additional Information

  • Diet
  • Lupus and Cancer
  • Lupus and Pregnancy
  • Things to Avoid

Lupus-Specific Skin Disease and Skin Problems

Most people with lupus experience some sort of skin involvement during the course of their disease. In fact, skin conditions comprise 4 of the 11 criteria used by the American College of Rheumatology for classifying lupus. There are three major types of skin disease specific to lupus and various other non-specific skin manifestautions associated with the disease.

Lupus-Specific Skin Disease

Three forms of specific skin disease occur in people with lupus, and it is possible to have lesions of multiple types. In addition, a person can also have one of the three forms outlined below without actually having full-blown systemic lupus erythematosus (SLE), but the presence of one of these disease forms may increase a person’s risk of developing SLE later in life. Usually, a skin biopsy is used to diagnose forms of cutaneous lupus, and various medications are available for treatment, including steroid ointments, corticosteroids (e.g., prednisone), and antimalarials (e.g., Plaquenil).

Chronic Cutaneous Lupus Erythematosus (CCLE) / Discoid Lupus Erythematosus (DLE)

Chronic cutaneous (discoid) lupus erythematosus is usually diagnosed when someone exhibits signs of lupus in the skin. People with SLE can also have discoid lesions, and about 5% of all people with DLE will develop SLE later in life. A skin biopsy is used to diagnose this condition, and the lesions have a characteristic pattern known to clinicians: they are thick and scaly, plug the hair follicles, appear usually on surfaces of the skin exposed to sun (but can occur in non-exposed areas), tend to scar, and usually do not itch.

If you are diagnosed with discoid lupus, you should try to avoid sun exposure when possible and wear sunscreen with Helioplex and an SPF of 70 or higher. In addition, you doctor may prescribe medications to help prevent and curb inflammation, including steroid ointments, pills, or injections , antimalarial medications such as Plaquenil, and/or immunosuppressive medications.

Subacute Cutaneous Lupus Erythematosus (SCLE)

About 10% of lupus patients have SCLE. The lesions characteristic of this condition usually do not scar, do not appear thick and scaly, and usually do not itch. About half of all people with SCLE will also fulfill the criteria for systemic lupus. Treatment can be tricky because SCLE lesions often resist treatments with steroid creams and antimalarials. People with SCLE should be sure to put on sunscreen and protective clothing when going outdoors in order to avoid sun exposure, which may trigger the development of more lesions.

Acute Cutaneous Lupus Erythematosus (ACLE)

Most people with ACLE have active SLE with skin inflammation, and ACLE lesions are found in about half of all people with SLE at some point during the course of the disease. The lesions characteristic of ACLE usually occur in areas exposed to the sun and can be triggered by sun exposure. Therefore, it is very important that people with ACLE wear sunscreen and protective clothing when going outdoors.

Common Lupus  Skin Problems

Malar Rash

About half of all lupus patients experience a characteristic rash called the malar or “butterfly” rash that may occur spontaneously or after exposure to the sun. This rash is so-named because it resembles a butterfly, spanning the width of the face and covering both cheeks and the bridge of the nose. The malar rash appears red, elevated, and sometimes scaly and can be distinguished from other rashes because it spares the nasal folds (the spaces just under each side of your nose). The butterfly rash may appear on its own, but some people observe that the appearance of the malar rash indicates an oncoming disease flare. Whatever the case, it is important to pay attention to your body’s signals and notify your physician of anything unusual.

Photosensitivity

50% of all people with lupus experience sensitivity to sunlight and other sources of UV radiation, including artificial lighting. For many people, sun exposure causes exaggerated sunburn-like reactions and skin rashes, yet sunlight can precipitate lupus flares involving other parts of the body. For this reason, sun protection is very important for people with lupus. Since both UV-A and UV-B rays are known to cause activation of lupus, patients should wear sunscreen containing Helioplex and an SPF of 70 or higher. Sunscreen should be applied everywhere, including areas of your skin covered by clothing, since most clothing items contain an SPF of only about 5. Be sure to reapply as directed on the bottle, since sweat and prolonged exposure can cause coverage to dissipate.

Livedo reticularis

People with lupus may experience a lacy pattern under the skin called livedo reticularis. This pattern may range anywhere from a violet web just under the surface of the skin to something that looks like a reddish stain. Livedo can also be seen in babies and young women, is more prominent on the extremities, and is often accentuated by cold exposure. The presence of livedo is usually not a cause for alarm, but it can be associated with antiphospholipid antibodies.

Alopecia

About 70% of people with lupus will experience hair loss (alopecia) at some point during the course of the disease. Hair loss in lupus is usually characterized by dry, brittle hair that breaks, and hair loss is more common around the top of the forehead. Physical and mental stress can also cause hair loss, as can certain medications, including corticosteroids such as prednisone. In many cases the hair will grow back, but hair loss due to scarring from discoid skin lesions may be permanent. There is no cure-all for hair loss, but treatments such as topical steroids and Rogaine may be prescribed. Sometimes dealing with the cosmetic side effects of lupus can be difficult, but some people find using hairpieces and wigs to be an effective means of disguising hair loss.

Oral and Nasal Ulcers

About 25% of people with lupus experience lesions that affect the mouth, nose, and sometimes even the eyes. These lesions may feel like small ulcers or “canker sores.” Such sores are not dangerous but can be uncomfortable if not treated. If you experience these types of lesions, your doctor may give you special mouthwash or Kenalog in Orabase (triamcinolone dental paste) to help expedite the healing process.

Raynaud’s Phenomenon

Approximately one-third of all people with lupus experience a condition called Raynaud’s phenomenon in which the blood vessels supplying the fingers and toes constrict. The digits of people with Raynaud’s are especially susceptible to cold temperatures. Often people with the condition will experience a blanching (loss of color) in the digits, followed by blue, then red discoloration in temperatures that would only be mildly uncomfortable to other people (such as a highly air-conditioned room). It is very important that people with Raynaud’s wear gloves and socks when in air-conditioned spaces or outside in cool weather. Hand warmers used for winter sports (e.g., Hot Hands) can also be purchased and kept in your pockets to keep your hands warm. These measures are very important, since Raynaud’s phenomenon can cause ulceration and even tissue death of the fingers and toes if precautions are not taken. People have even lost the ends of their fingers and toes due to the poor circulation involved in Raynaud’s phenomenon. Cigarettes and caffeine can exacerbate the effects of Raynaud’s, so be sure to avoid these substances. If needed, your doctor may also recommend a calcium channel blocker medication such as nifedipine or amlodipine to help dilate your blood vessels.

Hives (Urticaria)

About 10% of all people with lupus will experience hives (urticaria). These lesions usually itch, and even though people often experience hives due to allergic reactions, hives lasting more than 24 hours are likely due to lupus. If you experience this condition, be sure to speak with your doctor, since s/he will want to be sure that the lesions are not caused by some other underlying condition, such as vasculitis or a reaction to medication. Your doctor will probably distinguish these lesions from those caused by vasculitis by touching them to see if they blanch (turn white).

Purpura

Approximately 15% of people with lupus will experience purpura (small red or purple discolorations caused by leaking of blood vessels just underneath the skin) during the course of the disease. Small purpura spots are called petechiae, and larger spots are called eccymoses. Purpura may indicate insufficient blood platelet levels, effects of medications, and other conditions.

Cutaneous Vasculitis

Some people with lupus may develop a condition known as cutaneous vasculitis, in which the blood vessels near the skin experience inflammation that ultimately restricts blood flow. This condition can cause hive-like lesions on the skin that may itch and do not turn white when depressed. Other skin abnormalities may also be present, including actual gangrene of the digits. If left untreated, vasculitic lesions may cause ulceration and necrosis (cell death), and dead tissue must be surgically removed. Rarely, fingers or toes with aggressive ulceration and gangrene may require amputation. Therefore, it is very important that you notify your doctor of any skin abnormalities.

Anticoagulants

Warfarin (Coumadin)
Heparin
Dalteparin (Fragmin)
Danaparoid (Orgaran)
Enoxaparin (Lovenox)
Tinzaparin (Innohep)
Fondaparinux (Arixtra)

What are anticoagulants and why are they used in lupus treatment?

Warfarin (Coumadin) and heparin are anticoagulants (“blood thinners”), medications that decrease the ability of the blood to clot. About one-third of people with lupus have antibodies to molecules in the body called phospholipids. Sometimes these antiphospholipid antibodies (called anticardiolipin, lupus anticoagulant, or anti-beta2glycoprotein I) can lead to blood clots—thromboses—such as deep venous thrombosis, stroke, or heart attack.

A blood clot by itself is called a thrombus; a blood clot that breaks off and travels elsewhere in your circulatory (blood) system is called an embolus. It is rare for a blood clot to migrate to another part of the body and block one of your blood vessels, but if it does (a condition doctors call a thromboembolism), it is always serious. Blood clots can cut off circulation to your arms, legs, lungs (pulmonary embolism), brain (stroke), and heart (heart attack).

How do anticoagulants work?

Warfarin and heparin work in slightly different ways, but both block the production of certain proteins in your liver that work together to help your blood to clot. These proteins are called “cofactors.” Vitamin K controls the creation of these cofactors in your liver, and warfarin reduces clotting in your blood by preventing vitamin K from working correctly. Heparin also works by preventing certain cofactors, namely thrombin and fibrin, from working correctly. By blocking the process early on, both warfarin and heparin ultimately help to reduce blood clots from forming in your body.

How should I take these medications?

Warfarin comes in tablet form, and heparin must be given as an injection. The amount of medication needed differs with each person and each situation, and individuals on these medications should be closely monitored by their doctors to ensure that they are being given the correct dose.

Can I take these medications while I am pregnant?

Warfarin (Coumadin) is not safe during pregnancy. It can cause birth defects and fetal bleeding. Women who take warfarin must switch to heparin or low molecular weight heparin before they become pregnant, since heparin or low molecular weight heparin (Lovenox, Fragmin) do not cross the placenta into the fetus.

What should I remember while taking anticoagulants?

Do not smoke or drink alcohol while taking anticoagulants. Smoking increases the risk of blood clots and cardiovascular disease, and cardiovascular disease is the number one cause of death in people with lupus. Alcohol can interfere with the effectiveness of anticoagulant medications, can be harmful to your liver, and can irritate your stomach (gastritis), causing bleeding.

Warfarin

Warfarin is the most widely used anticoagulant, but because everyone differs in their physiological make-up, dosage requirements differ from person to person. Blood clotting is a natural protective mechanism employed by the body to seal off damaged blood vessels; any medication that alters this natural protective mechanism must be carefully monitored. People taking warfarin must obtain a blood test every 2-4 weeks to ensure that their blood is thinning to the correct degree without bleeding complications. This test (the INR, discussed below) may be requested several times a week at the beginning of your treatment to ensure that you are started on the correct dose.

The two tests used to monitor warfarin levels in the blood are the Prothrombin Time (or, just “Pro-Time,” or “PT”) and a test called an INR (International Normalized Ratio). In actuality, Prothrombin time is the test used, and INR is simply a standardized way for medical institutions to report consistent values for Prothrombin times. The INR ratio is calculated based on comparison of blood tests against a known standard, and your physician will monitor your warfarin levels based on this INR ratio. Generally, an INR of 2.5 to 3.0 indicates that the patient is receiving the correct dose of warfarin; INR values above 5.0 can be dangerous, and those below 1.0 indicate no warfarin effect.

Heparin

Heparin works faster than warfarin, so it is usually given in situations where an immediate effect is desired. For example, this medication is often given in hospitals to prevent growth of a previously detected blood clot. This medication is also recommended for pregnant women in whom antiphospholipid antibodies have been discovered, since warfarin can be harmful to an unborn child. However, when taken for long periods of time, this medication might increase the risk of osteoporosis. Usually patients switch to warfarin when long term anticoagulant treatment is recommended.

What are the potential side effects of anticoagulants?

The two most serious side effects of anticoagulants are bleeding and gangrene (necrosis) of the skin. Bleeding can occur in any organ or tissue. Bleeding in the kidneys can cause severe back pain and blood in the urine. Bleeding in the stomach can cause weakness, fainting, black stools, or vomiting of blood. Bleeding of the brain can cause severe headache and paralysis, and bleeding of the joints can cause joint pain and swelling.

Other side effects can include rash, bloating, diarrhea, jaundice (yellowing of the eyes and skin), hair loss, itchy feet, pain in the toes, and, in the case of heparin, mild pain, redness, or warmth at the injection site. Bleeding gums, excessive bruising, nosebleeds, heavy menstrual bleeding, and prolonged bleeding from cuts may indicate an overdosing of medication. Notify your doctor if these problems occur.

Some people receiving heparin have experienced a reaction to the infusion, so contact your doctor immediately if you experience any abrupt and serious side effects, such as sudden numbness, confusion, swelling, or trouble breathing.

What medications and foods should I avoid while taking anticoagulants?

While taking anticoagulants, you should avoid supplements with vitamin K. Remember that vitamin K controls the formation of proteins in your liver involved in clotting, so taking in vitamin K can work to counteract your medication and increase your risk of blood clots. In addition, you should avoid medications that can increase your risk of bleeding. These medications include aspirin, NSAIDs, multivitamins with vitamin K, and fish oil. Any antibiotic may change the Coumadin INR test. If you must take an antibiotic, notify your Coumadin clinic so that they may schedule an additional INR check. In addition, be sure to speak with your doctor before starting any new medications. Your physician may also recommend that you carry some sort of identification to alert health professionals of your use of anticoagulants should an emergency or other situation arise.

Blood Pressure Medications (Anti-hypertensives)

25-30% of people with lupus experience hypertension (high blood pressure), which is defined as a blood pressure of greater than 140/90 mmHg. In addition, many more lupus patients have blood pressures greater than the normal 120/80 mmHg limit. 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.

High blood pressure can cause everyday headaches, but more importantly, it can lead to stroke, heart failure, and heart attack. Cardiovascular disease is the number one cause of death in people with lupus, so it is very important that your blood pressure is brought to the healthy 120/80 mmHg level and maintained. It is imperative that you take steps yourself to help achieve and maintain optimum cardiovascular health—remember, you play the most important role in your own well-being.

You can take several steps on your own to ensure that you remain as healthy as possible. Do not smoke, because smoking increases the risk of cardiovascular disease. In addition, it is important that you maintain a healthy diet and regular exercise regimen; these elements are especially significant for people taking steroid medications such as prednisone. A low-fat, low-cholesterol diet is essential for a healthy lifestyle. Focus on eating whole grains, vegetables, and lean sources of protein. Limit your sodium (i.e., salt) intake, since sodium levels are directly linked to blood pressure. In addition, try to exercise at least 30-minutes per day. This goal can be difficult for people with lupus who experience reoccurring joint and muscle pain, fatigue, and other symptoms, but engaging in low-impact daily activities such as walking, biking, yoga, Tai chi, and other forms of stretching may help to alleviate some of this pain while also helping you to maintain a healthy weight and a strong cardiovascular system. These activities also reduce the risk of osteoporosis, and people who exercise daily report that they actually feel better physically and mentally.

However, 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. Therefore, your doctor may prescribe a medication to help control your blood pressure. There are several types of medications that work to lower, control, and/or maintain blood pressure, and each works in a different way. Your doctor will work with you to evaluate and prescribe the blood pressure medication that best suits your personal condition. It is important that you remember to take your blood pressure (and other) medications as directed by your physician and notify him/her of any changes to your personal health. Take your blood pressure medications every day, including the days that you see your doctor. Do not stop taking your blood pressure medications without speaking to your doctor, since suddenly stopping your medication could put you in danger of a heart attack, stroke, or, in some cases, kidney failure.

Diuretics

Chlorthalidone (Hygroton)
Chlorothiazide (Diuril)
Hydrochlorothiazide (Esidrix, Hydrodiuril)
Indapamide (Lozol)
Metolazone (Zaroxolyn, Mykrox)
Bumetanide (Bumex)
Furosemide (Lasix)
Torsemide (Demadex)
Amiloride (Midamor)
Spironolactone (Aldactone)
Triamterene and hydrochlorothiazide (Dyazide)

Your kidneys play a very important role in helping to maintain blood pressure. A complex system of tubules in the kidney is responsible for regulating the balance of water and salts in your blood. When excess fluid and sodium build up in your kidneys, your blood pressure goes up. Diuretics (a.k.a., “water tablets,” or “water pills”) help to rid the kidneys of excess fluids and sodium through urination; in doing this, diuretics also help to reduce blood pressure. Diuretics are usually prescribed as the first line of treatment for high blood pressure but may also be given to enhance the effect of other medications.

There are actually four kinds of diuretics— thiazide diuretics, loop diuretics, potassium-sparing diuretics, and combination diuretics. Each class works on a different part of the nephron, the functional unit of the kidney that makes up its vast system of tubules. Thiazide diuretics (chlorthalidone, hydrochlorothiazide, indapamide, and metolazone) are sometimes the first drugs suggested for people with high blood pressure. These medications prevent the reabsorption of sodium and water in a specific part of the nephron called the distal convoluted tubule. In doing so, they also force more water into the urine to be removed from the body. Thiazides relax the muscles in blood vessel walls, allowing blood to flow more easily. Generally, thiazide diuretics are taken once daily. These medications can lower the amount of potassium in your body, so this factor will be monitored by your doctor.

Loop diuretics (bumetanide, furosemide, and torsemide) work on a part of the nephron called the “Loop of Henle” and prevent sodium from re-entering the blood. However, loop diuretics also interfere with the reabsorption of other salts, such as calcium, magnesium, and potassium. Proper monitoring may be necessary to prevent complications, such as low potassium. Your physican may recommend that you take a potassium supplement while taking these medications. Loop diuretics are generally less effective than thiazides for controlling blood pressure because they work for a shorter period of time and may result in a loss of blood pressure control at certain times in the day. These medications are generally taken once or twice daily.

Potassium-sparing diuretics (amiloride, spironolactone, and triamterene) are weaker than thiazides and loop diuretics but do not yield the potassium-depleting effects that loop diuretics do. These medications are sometimes used in combination with other diuretics, namely hydrochlorothazide (“HCTZ”). One example of a combination therapy is Dyazide (HCTZ/triamterene). Potassium-sparing diuretics are typically taken once daily.

Certain medications can interact with diuretics, so be sure to tell your doctor about any medications (both prescription and over-the-counter), vitamins, and supplements that you may be taking. Be especially wary of interactions with the following drugs: digitalis and digoxin (especially if your potassium level is low), certain antidepressants (especially when taking thiazide or loop-acting diuretics, other medications for high blood pressure, lithium, and cyclosporine (especially when taking a potassium-sparing diuretic).

Diuretics can cause certain side effects. Common side effects include weakness, muscle cramps, skin rash, increased sensitivity to sunlight (with thiazide diuretics), vomiting, diarrhea, cramps, lightheadedness, or joint pain. Less common side effects include impotence and irregular heartbeat. Many of these side effects can mimic lupus symptoms, so speak with your doctor if you feel that you are experiencing any new symptoms that may be caused by a new medication. However, it is important that you do not stop taking your medication unless both you and your doctor decide that this is the correct course of action. In addition to keeping your blood pressure under control, many anti-hypertensive medications also affect the function of your kidneys, so stopping your medication without appropriate approval could have serious consequences.

Angiotensin-Converting Enzyme (ACE) Inhibitors

Benazepril (Lotensin)
Captopril (Capoten)
Enalapril (Vasotec)
Fosinopril (Monopril)
Lisinopril (Prinivil, Zestril)
Moexipril (Univasc)
Perindopril (Aceon)
Quinapril (Accupril)
Ramipril (Altace)
Trandolapril (Mavik)

Angiotensin-converting enzyme (ACE) inhibitors are used to control blood pressure, treat heart failure, lower protein in the urine, and prevent kidney damage. These medications make your heart’s work much more efficient by expanding blood vessels and decreasing resistance to blood flow. ACE is a chemical in your body that converts a hormone called angiotensin-I to angiotensin-II. Angiotensin-II is involved in several actions, one of which causes the muscles surrounding the blood vessels to contract. The narrowing of your blood vessels caused by this contraction causes your blood pressure to rise. As their name suggests, ACE inhibitors work to inhibit ACE, and in doing so, they also inhibit the production of Angiotensin-II. Because of this inhibition, the blood vessels dilate (enlarge), thereby reducing blood pressure and making it easier for your heart to pump blood. ACE inhibitors have a two-fold benefit for many lupus patients, because in addition to controlling blood pressure, they also decrease protein in the urine (proteinuria), and thus help to prevent kidney scarring in lupus patients with kidney involvement. ACE inhibitors should be used carefully, however, in people with renal artery stenosis (narrowing of the artery that supplies blood to the kidney).

Most people tolerate ACE inhibitors well, but these medications do have some potential side effects. The most common side effects are a cough which may last up to a month, elevated blood potassium levels, low blood pressure, constipation, dizziness, headache, drowsiness, weakness, metallic or salty taste, and rash. Rare, but serious, side effects include kidney failure, allergic reactions, a decrease in white blood cell count, and a swelling of the tissue just below the skin (angioedema). In addition, women who are pregnant or may become pregnant should not take ACE inhibitors because they are known to cause birth defects.

Be sure to tell your doctor about any other medications, vitamins, or supplements you may be taking, since certain medications can interact with ACE inhibitors. Be especially cautious with potassium supplements, salt substitutes (which can contain potassium), and other drugs that can affect the amount of potassium in your blood, since ACE inhibitors may increase your potassium levels. ACE inhibitors may also increase the concentration of lithium (Eskalith) in the blood of people taking this medication, which can cause an increase in side effects.

Angiotensin-II Receptor Antagonists / Angiotensin Receptor Blockers (ARBs)

Candesartan (Atacand)
Eprosartan (Teveten)
Irbesartan (Avapro)
Losartan (Cozaar)
Telmisartan (Micardis)
Valsartan (Diovan)

Angiotensin-II receptor blockers (ARBs) are used to control high blood pressure, treat heart failure, lower protein in the urine, and prevent kidney failure. They are similar to ACE inhibitors, but their mechanism of action is slightly different. In your body, there are many substances that send messages to different cells and tissues to enact certain changes. However, in order for the signals to go through, there are often molecules that receive these substances to help transmit messages. Whereas ACE inhibitors block the formation of angiotensin-II, angiotensin-II receptor blockers block the molecules in your body that receive angiotensin-II so that the substance cannot transmit the signal for your blood vessels to contract. Therefore, your blood vessels remain enlarged, which keeps your blood pressure from rising and makes it easier for your heart to pump blood. Like ACE inhibitors, angiotensin receptor blockers also reduce proteinuria (excess protein in the urine) in people with lupus nephritis, which helps to keep the kidneys safe and healthy. The benefit of angiotensin-II receptor blockers is that they produce less cough as a side effect; if you experienced a cough from ACE inhibitors, you will most likely be switched to an ARB.

Like ACE inhibitors, ARBs are tolerated well by most people, but they do have some side effects. The most common side effects are elevated blood potassium levels, low blood pressure, dizziness, headache, drowsiness, weakness, metallic or salty taste, and rash. Cough can occur, but this happens less often with ARBs than with ACE inhibitors. The most serious (but rare) side effects include kidney failure, liver failure, allergic reactions, a decrease in white blood cells, and swelling of the tissue  just bellow the skin (angioedema). In addition, women who are pregnant or may become pregnant should not take ARBs because they are known to cause birth defects.

Be sure to tell your doctor about any other medications, vitamins, or supplements you may be taking, since certain medications can interact with ARBs. Be especially cautious with potassium supplements, salt subsitutes (which can contain potassium), and other drugs that can affect the amount of potassium in your blood, since ARBs may increase your potassium levels. ARBs may also increase the concentration of lithium (Eskalith) in the blood of people taking this medication, which can cause an increase in side effects.

Beta Blockers

Acebutolol (Sectral)
Atenolol (Tenormin)
Betaxolol (Kerlone)
Bisoprolol/hydrochlorothiazide (Ziac)
Bisoprolol (Zebeta)
Carteolol (Cartrol)
Carvedilol (Coreg)
Metoprolol (Lopressor, Toprol XL)
Nadolol (Corgard)
Propranolol (Inderal)
Sotalol (Betapace)
Timolol (Blocadren)
Nebivolol (Bystolic)

Beta blockers are used to lower blood pressure, treat abnormal heart rhythms and angina (chest pain and discomfort that occurs when your heart muscle does not get enough blood), and improve survival in people who have had a heart attack. Beta blockers are so-named because they block the action of adrenaline (a.k.a., epinephrine) and other substances in the body on “beta” receptors, which in turn slows the nerve impulses that travel to the heart. In doing this, beta blockers relieve stress on your heart, slow your heart beat, and lessen the force with which your heart muscle contracts. These medications also reduce the strength with which the muscles surrounding the blood vessels contract throughout the body, reducing pressure in your blood vessels and increasing blood flow. By allowing your blood to flow more easily and relieving some of the stress on your heart, beta blockers decrease heart rate, cardiac output, and ultimately blood pressure.

Beta blockers may cause certain side effects. Common side effects include drowsiness or fatigue, weakness or dizziness, and dry mouth, eyes, and skin. These medications may also cause cold hands and feet, which can be especially problematic for lupus patients experiencing Raynaud’s phenomenon. Less common side effects include wheezing or shortness of breath, slow heartbeat, trouble sleeping/vivid dreams, and swelling of the hands and feet. Rare side effects include abdominal cramps, vomiting, diarrhea, constipation, back or joint pain, skin rash, sore throat, depression, memory loss/confusion, and impotence. Tell your doctor right away if you experience any of these side effects.

While taking beta blockers, it is important that you do not drink alcohol, since alcohol can decrease the effects of these medications. In addition, avoid caffeine and over-the-counter cough and cold medicines, antihistamines, and antacids that contain aluminum. Be sure to tell your doctor about any medications, vitamins, and supplements that you may be taking, especially other blood pressure medications, anti-depressants, allergy shots, and diabetes and/or asthma medications. If you are pregnant or may become pregnant you should discuss your beta blocker medications with your doctor, since this drug should only be used during pregnancy if it is clearly needed.

Calcium Channel Blockers

Amlodipine (Norvasc, Lotrel)
Bepridil (Vascor)
Diltiazem (Cardizem, Tiazac)
Felodipine (Plendil)
Nifedipine (Adalat, Procardia)
Nimodipine (Nimotop)
Nisoldipine (Sular)
Verapamil (Calan, Isoptin, Verelan)

Calcium channel blockers, also known as “calcium antagonists,” are used to decrease blood pressure and treat angina (chest pain) and some arrhythmias (abnormal heart rhythms). They are also used to treat Raynaud’s phenomenon, a cold-induced blue/purple color change in the fingers and toes. Calcium channel blockers work by interrupting the movement of calcium into the heart and blood vessel cells, which in turn decreases the force of contraction of the myocardium (muscle of the heart) and relaxes and widens blood vessels. Many calcium channel blockers also slow down the conduction of electricity in the heart, which lowers heart rate and thus further lowers blood pressure.

Calcium channel blockers may have some side effects, the most common of which are constipation, nausea, headache, rash, edema (swelling of the legs with fluid), low blood pressure, drowsiness, and dizziness.

If you are experiencing heart failure, you should not take diltiazem (Cardizem, Tiazac) or verapamil (Calan, Isoptin, Verelan), since these drugs reduce the ability of your heart to pump blood. In addition, you should tell your doctor about any other medications, vitamins, or supplements that you may be taking, especially other heart or blood pressure medications, anti-seizure medication, and cyclosporine (Neoral, Sandimmune, Gengraf). Diltiazem and verapamil interact most with other medications because these two calcium channel blockers decrease the elimination of certain drugs from the liver, including carbamazepine (Tegretol), simvastatin (Zocor), atorvastatin (Lipitor), and lovastatin (Mevacor). Since your liver cannot eliminate these substances as efficiently, they may accumulate in your blood, an effect which can be toxic to your body.

While taking calcium channel blockers, do not eat grapefruit or drink grapefruit juice, since grapefruit may increase some of the effects of these medications. Also avoid alcohol, since it interferes with the effects of these medications and may increase their side effects. If you are pregnant, talk to your doctor about whether you should continue therapy with calcium channel blockers. No pregnancy problems or birth complications have been found to date in humans, but you should always speak with your doctor about the risks and benefits of any medications you may be taking while pregnant.

Alpha Blockers

Doxazosin mesylate (Cardura)
Prazosin hydrochloride (Minipress)
Prazosin and polythiazide (Minizide)
Terazosin hydrochloride (Hytrin)
Tamsulosin (Flomax)

Alpha blockers are sometimes used to treat high blood pressure, Raynaud’s disease, scleroderma, and other conditions. They are also helpful in men who have difficulty urinating due to benign prostatic hyperplasia (BPH). Alpha blockers are generally not the first treatment option chosen to treat high blood pressure because they have not proven to reduce long-term risk of heart attack and stroke, but they are commonly used, often in combination with other drugs, when blood pressure is difficult to control.

Like beta blockers, alpha blockers block the action of receptor molecules (called alpha receptors) from receiving certain chemical messages. Specifically, alpha blockers attach themselves to alpha receptors found on blood vessels, in the prostate, and in special blood pressure sensors called baroreceptors. In doing this, alpha blockers prevent alpha receptors from receiving signals from the hormone norepinephrine (noradrenaline) to stimulate the muscles in the walls of smaller arteries and veins and cause them to constrict. By preventing this communication, alpha blockers cause the vessels to remain open and relaxed, improving blood flow and lowering blood pressure.

Alpha blockers fall into two categories—short-acting and long-acting. Short-acting alpha blockers work quickly, but their effects do not last as long. Long-acting medications take longer to begin working, but their effects last for longer periods of time. Your doctor will advise you on the type of alpha blocker that is best for you.

When you start taking alpha blockers, you may have what is known as a “first dose” effect. This effect may cause you to experience pronounced low blood pressure and dizziness which may make you faint when rising too quickly from the sitting or lying position, a phenomenon known as orthostatic hypotension or postural hypotension. To prevent this from happening, be sure to rise slowly whenever you begin to stand up. Your doctor may try to reduce the first dose phenomenon by starting you at a low dose and gradually increasing it until you reach the proper amount. In addition, most doctors recommend that you take alpha blockers before bed, which may help to lessen this effect. Other potential side effects can include headache, pounding heartbeat, nausea, weakness, weight gain, and small increases in LDL levels (“bad cholesterol”).

If you are pregnant or may become pregnant, speak with your doctor about whether continuing treatment with alpha blockers is right for you. Also tell your doctor about any medications (both prescription and over-the-counter), vitamins, and supplements that you may be taking, especially other blood pressure medications, NSAIDs, immunosuppressives, impotence therapy agents, antibiotics, anti-fungals, HIV medications, anti-depression and anti-anxiety drugs, diabetic medications, and certain asthma medicines. Stay away from diet pills, caffeine, and over-the-counter cough, medicines, cold medicine, and anti-histamines while taking alpha blockers, since these medications may increase blood pressure. Also avoid grapefruit and grapefruit juice because grapefruit interferes with the liver’s ability to get rid of certain substances, allowing them to accumulate to toxic levels. Alcohol can have a similar effect, so people taking alpha blockers—and all people with high blood pressure—should limit alcohol use.

Central Alpha Agonists / Central-Acting Agents

Clonidine hydrochloride (Catapres)
Clonidine hydrochloride and chlorthalidone (Clorpres, Combipres)
Guanabenz acetate (Wytensin)
Guanfacine hydrochloride (Tenex)
Methyldopa (Aldomet)
Methyldopa and chlorothiazide (Aldochlor)
Methyldopa and hydrochlorothiazide (Aldoril)

Central-acting agents work to lower your heart rate and blood pressure by preventing your brain from sending signals to the nervous system to speed up heart rate and narrow blood vessels. As a result, your heart does not have to work as hard and the blood flows more easily through your blood vessels. Central-acting agents are not commonly used because they can cause strong side effects, including extreme fatigue, drowsiness/sedation, dizziness, impotence, constipation, dry mouth, headache, weight gain, impaired thinking and psychological problems like depression. Tell your doctor if experience these side effects, but do not stop taking your medication unless directed to do so by your doctor. If you are to stop taking central-acting agents, your doctor will advise you on how to gradually taper your dosage. Treatment with central-acting agents should not be stopped abruptly, since this can cause a sudden, dangerous increase in blood pressure. Also, be sure to speak to your doctor about any other medications you may be taking, since certain drugs may interfere with these medications.

Combination Therapies

Several combination therapies are available to help control, lower, and/or maintain your blood pressure. Common combination therapies are listed below. Your doctor can advise you of any special considerations regarding these medications.

General

Atenolol and chlorthalidone (Tenoretic)
Bisoprolol and hydrochlorothiazide (Ziac)
Nadolol and bendroflumethiazide (Corzide)
Propranolol and hydrochlorothiazide (Inderide)
Timolol and hydrochlorothiazide (Timolide)

ACE Inhibitor/Diuretic

Benazepril and hydrochlorothiazide (Lotensin)
Enalapril and hydrochlorothiazide (Vaseretic)
Lisinopril and hydrochlorothiazide (Prinzide, Zestoretic)
Moexipril and hydrochlorothiazide (Uniretic)
Quinapril and hydrochlorothiazide (Accuretic)

Angtiotensin II Receptor Antagonist/Diuretic

Irbesartan and hydrochlorothiazide (Avalide)
Losartan and hydrochlorothiazide (Hyzaar)
Valsartan and hydrochlorothiazide (Diovan HCT)

ACE Inhibitor/Calcium Channel Blocker

Amlodipine and benazepril (Lotrel)
Enalapril and felodipine (Lexxel)
Trandolapril and verapamil (Tarka)

Combined Alpha and Beta Blocker Action

Labetalol hydrochloride (Normodyne)

Gastrointestinal Medications

Antacids
Aluminum hydroxide, magnesium hydroxide (Mylanta, Maalox)
Calcium carbonate (Tums, Rolaids, Chooz)
Bismuth subsalicylate (Pepto-Bismol)
Sodium bicarbonate (Alka-Seltzer)

Proton Pump Inhibitors
Omeprazole (Prilosec)
Lansoprazole (Prevacid)
Rabeprazole (Aciphex)
Esomeprazole (Nexium)
Pantoprozole (Protonix)

Histamine2 Blockers
Cimetidine (Tagamet)
Ranitidine hydrochloride (Zantac)
Famotidine (Pepcid)
Nizatidine (Axid)

Promotility Agents
Metoclopramide (Reglan)

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. Occasional heartburn or acid indigestion can be treated with an over-the-counter antacid, such as Rolaids, Maalox, Mylanta, Tums, Pepto-Bismol, or Chooz. Your doctor may also include an antacid in your treatment regimen in addition to another form of GI medication. Antacids contain basic salts (ions), which interact with and neutralize the acid in your stomach on contact. Some antacids also contain simethicone, which helps relieve symptoms of gassy stomach. Antacids are effective when used to treat occasional symptoms, but you should try to avoid heartburn and acid indigestion altogether by eating smaller meals, remaining upright after eating, and cutting down on caffeine. If heartburn and acid reflux persist (e.g., for more than two weeks), you should speak with your doctor, because your heartburn symptoms could be part of a larger problem.

If you experience persistent heartburn, stomach acid, or ulcers, your doctor may prescribe a proton pump inhibitor (PPI), such as Nexium, Prevacid, Prilosec, Protonix, or Aciphex. These medications are used to treat people with heartburn, stomach or intestinal ulcers, or excess stomach acid. Proton pump inhibitors reduce acid by shutting down the tiny pumps within cells in your stomach that secrete it. Evidence also suggests that PPIs may inhibit Helicobacter pylori, a type of bacteria that can cause peptic ulcers, gastritis, and other gastrointestinal problems. Most PPIs come as over-the-counter or prescription tablets, but pantoprazole (Protonix) may also be given intravenously at the hospital for people who are admitted with a bleeding ulcer. Taking a PPI reduces the chance that an ulcer or gastrointestinal bleeding will occur again.

Your doctor may also prescribe histamine2 blockers (H2 blockers) for symptoms of GERD, esophagitis, or peptic ulcers. While both PPIs and H2 blockers suppress the production of acid in your stomach, they work in different ways and over different time periods. For example, H2 blockers work within an hour but last only about 12 hours, whereas PPIs need more time to take effect but last up to 24 hours. Also keep in mind that many PPIs and H2 blockers are available in both over-the-counter and prescription forms; while these medications vary in potency, over-the-counter forms may be more cost-effective. Talk to your doctor about these various options.

H2 blockers work to reduce the amount of acid that your stomach produces by blocking histamine2, a chemical in your body that signals the parietal cells of your stomach lining to make acid. In doing this, H2 blockers reduce the amount of acid made by your stomach. Different H2 blockers vary in potency. Over-the-counter forms are less potent, while prescription doses can be more potent.

In addition, your physician may prescribe a medication called a promotility agent if you experience GERD symptoms due to slow gastric emptying, or if H2 blockers and PPIs are not enough to control your GERD symptoms. Promotility agents help speed digestion by stimulating the movement of GI contents through your esophagus, stomach, and intestines. This helps to prevent acid from lingering in your stomach too long, thus reducing the amount of damage that acid can inflict on your GI tract and decreasing the occurrence of the acid reflux. Metoclopramide is the main promotility agent currently on the market. It works by increasing muscle contractions in the upper digestive tract, which in turn speeds the rate with which stomach contents move into the intestines.

While taking any GI medication, you should avoid drinking alcohol, since it can further upset your stomach and cause an increase in the side effects of certain medications. In addition, tell your doctor about any other medications you may be taking, since certain drugs can interact with your GI medications.

Aspirin

Why is aspirin used in lupus treatment?

Low doses of aspirin (81 milligrams, often called “baby aspirin”),* taken once a day may help to reduce the risk of blood clots and miscarriage in lupus patients who have antiphospholipid antibodies. Your doctor may recommend that you take one baby aspirin per day if you fit this category, since low doses of aspirin have been shown to lower the potential for clot-forming blood cells called platelets to stick together in narrow blood vessels. For this reason, low-dose aspirin is often grouped with the antiplatelet medications, sometimes called “platelet antagonists.” In addition, low dose aspirin may reduce the risk of heart attack and stroke.

How should I take aspirin, and what are the potential side effects?

Aspirin comes in tablets of different milligram doses. Your doctor will decide what dosage is right for you based on your symptoms of inflammation and the health of your kidneys and liver. If you take too much aspirin, you may experience an annoying ringing in your ears. In addition, if you experience stomach upset from taking aspirin or have an ulcer, you may have to stop the aspirin or take a protective medicine for your stomach such as famotidine (Pepcid).

Is there anything I should avoid while taking aspirin?

While taking aspirin, you should avoid taking ibuprofen (Advil, Motrin, Rufen), because studies have shown that this drug can counteract the benefits of aspirin therapy. If you need to take an over-the-counter medication for pain and stiffness, and your doctor has approved such a medication, you should try acetaminophen (Tylenol) or naproxen (Aleve, Naprosyn) instead.

Preparing for Surgery

Aspirin must be stopped one week before any surgery. Certain procedures, such as a colonoscopy, may also require that you stop aspirin therapy one week prior to the event. If you plan to have any surgeries or medical procedures, please discuss them with your doctor so that she/he may decide on the appropriate course of action.

∗ There is some debate as to which dose of aspirin is most effective in preventing blood clots. Usually, doctors recommend either 81 or 325 mg of aspirin daily. Go with whatever dosage your doctor recommends—both are beneficial.

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