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Lupus can affect the cardiovascular system, which includes your heart and blood vessels. In fact, cardiovascular disease, not lupus itself, is the number one cause of death in people with SLE. Therefore, it is very important that you take steps to maintain optimal cardiovascular health. Do not smoke, since smoking increases the risk of cardiovascular disease. A low-fat, low-cholesterol diet is also essential. 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. However, 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 strong cardiovascular system.

Hypertension (High Blood Pressure)

Fifty percent 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 obesity, 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 lead to kidney failure, stroke, heart failure, and heart attack. Since cardiovascular disease is the number one cause of death in people with lupus, it is very important that your blood pressure is brought to the healthy 120/80 mmHg level and kept there.

While it is important that you take steps yourself to help achieve and maintain optimum cardiovascular health, diet and exercise alone may be insufficient in controlling your blood pressure. Therefore, your doctor may prescribe a medication that works to lower, control, and/or maintain your blood pressure. Each medication works in a different way, and your doctor will work with you to evaluate and prescribe the blood pressure medication that best suits your personal condition.

Atherosclerosis

Atherosclerosis is the most common manifestation of cardiovascular disease in people with lupus. The condition is caused by the accumulation of cholesterol and other substances (known collectively as plaque) along the inner linings of arteries. Eventually, this plaque can harden and restrict the flow of blood to various parts of the body, including the heart and brain; if it ruptures, it can cause a clot to form, leading to complications like heart attack and stroke. Research has shown that being overweight and having high blood pressure are the two most important predictors of heart disease in SLE. In fact, the risk of heart attack in women with lupus aged 35-44 is 50-times greater than that of women without lupus, and for everyone with lupus the risk is increased 7 to 9-fold. Even though lupus itself contributes to the development of atherosclerosis, traditional factors, such as smoking, obesity, and high blood pressure, can exacerbate the condition and must be controlled.

Pericarditis

Pericarditis is the most common heart problem associated with active lupus and occurs in about 25% of people with SLE. The condition occurs when the pericardium—the thin membrane surrounding your heart—becomes swollen and irritated, causing it to leak fluid around the heart. People with pericarditis may experience pressure pains that are worse lying down and better sitting up. Your doctor will work with you to decide the best course of treatment for pericarditis. Often, NSAIDs or steroids are effective in reducing the inflammation associated with this condition.

Endocarditis

Libman-Sacks endocarditis occurs in about 15% of people with lupus. The condition leads to the development of growths called vegetations on the surface of heart valves. Usually this form of endocarditis is associated with antiphospholipid antibodies, which are present in about 50% of people with SLE.

Endocarditis can lead to two major complications: infection and stroke. Vegetations are prime sites for bacterial growth, and an infected valve requires surgical replacement. Visits to the dentist offer opportunities for bacteria to sneak into the blood and land on a heart valve. Therefore, people with severe heart murmurs should speak to their doctor about taking an antibiotic before receiving dental work. In addition, pieces of a vegetation can break off and travel through the blood stream, potentially blocking blood flow to the brain and causing a stroke. If you have a heart vegetation, your doctor will prescribe an anticoagulant to reduce this risk.

Myocarditis

Myocarditis, inflammation of the myocardium or heart muscle, occurs in fewer than 10% of people with lupus. People with this condition often experience a rapid heartbeat and chest pain, and x-rays may show an enlarged heart. Myocarditis may weaken your heart’s ability to pump blood to the rest of your body. Therefore, it is a serious complication that must be closely monitored and treated with high dose steroids for several weeks or months.

Sources

• “Pericardium and Pericarditis.” American Heart Association. 30 June 2009. <http://www.americanheart.org/presenter.jhtml?identifier=4683>.
• Petri, Michelle. Cardiovascular Systemic Lupus Erythematosus. In: Lahita RG, ed. Systemic Lupus Erythematosus. New York: Churchill Livingstone; 2004:905-923.
• Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford University Press, 1995. 90-98.
• “What is Atherosclerosis?” Nov. 2007. National Heart Lung and Blood Institute, Diseases and Conditions Index. 30 June 2009. <http://www.nhlbi.nih.gov/health/dci/Diseases/Atherosclerosis/Atherosclerosis_WhatIs.html>.

A blood chemistry panel is another common test used to evaluate a variety of components. Usually, it consists of about 7-25 tests. The information below is meant to provide an overview of these tests. Your doctor will counsel you regarding the results of your personal blood work and laboratory tests.

Kidney Function Tests

The creatinine blood test and blood urea nitrogen (BUN) test are used to assess kidney function in people with lupus kidney disease (nephritis).

• Creatinine: Creatinine is produced by your muscles as they breakdown creatine, a substance involved in muscle contraction. Creatinine is formed at a constant rate in the body and excreted by the kidneys, so by evaluating the amount of creatinine in your blood, your doctor can determine how efficiently your kidneys are working. Creatinine levels are measured by taking a sample of blood from your vein; then, the concentration of creatinine in your blood is compared to a standard amount for your age and sex. Increased blood creatinine levels may indicate an increase in lupus involvement of the kidney. Other conditions, such as high blood pressure or diabetes, can also cause elevated creatinine levels.
• Sometimes individuals are asked to provide a 24-hour urine sample for further assessment. The combination of blood and urine samples can be used to evaluate a creatine clearance—how effectively your kidneys filter small molecules, such as creatinine, from your blood. In addition, since creatinine is usually removed from the blood at a constant rate, blood creatinine levels can be used as a standard by which doctors can compare other urine or blood tests. Your serum (blood) creatinine level can also be combined with your age, weight, and gender to evaluate your estimated glomerular filtration rate (eGFR). Glomeruli are tiny ball-shaped structures in your kidneys that help filter blood and prevent the loss of valuable substances, such as blood cells and proteins. The eGFR is an educated estimate of the amount of blood that is filtered per minute by your glomeruli and is often used to detect kidney damage.
• Blood urea nitrogen (BUN): The BUN test measures the amount of urea nitrogen in your blood. The liver produces nitrogen in the form of ammonia (NH3) as it breaks down proteins into their constituent amino acids. From the liver, urea travels in your blood to the kidneys, which filter the urea and flush it from your body in the form of urine. To evaluate an individual’s BUN level, blood is drawn from the vein, and the concentration of urea nitrogen in the blood is evaluated and compared to a standard value for that person’s age range. Even though increased protein levels in a person’s diet can cause their blood urea nitrogen levels to increase, elevated BUN may suggest kidney involvement due to lupus or another condition such as dehydration that causes decreased blood flow to the kidneys. Low BUN levels are uncommon and are usually not as important; they can suggest certain conditions, such as malnutrition, over-hydration, or liver disease, but doctors usually use other tests to monitor these conditions.

Blood Glucose (Sugar) Test

Tests of blood glucose levels are performed to determine if an individual’s blood glucose is in normal range. This test helps to detect hyperglycemia (high blood sugar), hypoglycemia (low blood sugar), and diabetes (which can occur after long-term steroid therapy). Glucose is a simple sugar that your body gets from the food you eat. The cells of your body need glucose to obtain energy, and they cannot function without it. When we think of providing our bodies with energy, we usually think about movement and physical activity. However, glucose is also vital to the cells of your brain and central nervous system.

The amount of glucose in your blood is controlled by a feedback mechanism involving two hormones, insulin and glucagon. These hormones work to ensure that your blood contains the right amount of glucose so that your cells—including those in your brain and central nervous system—can function correctly. When your body takes in glucose after a meal, insulin is secreted by cells in your pancreas (beta cells) in order to lower your blood glucose to the appropriate level. When your blood sugar gets too low, glucagon is secreted by alpha cells of the pancreas in order to raise glucose levels. Disruptions in this feedback mechanism can be harmful to your body. In people with diabetes, the body either does not make enough insulin or does not use it properly. High or low blood sugar levels caused by diabetes or other conditions can be serious if not kept in check.
Blood glucose levels are usually evaluated when the patient is fasting, but they can also be taken at random, after a meal, or in a “challenge” test in which a person consumes a certain amount of glucose to challenge their system and track the way his/her body deals with glucose over time. Diabetics usually monitor their own blood glucose levels at home.

Fasting Lipid Profile

A lipid profile is a group of tests that includes measurements of total cholesterol, HDL-cholesterol (“good cholesterol”), LDL-cholesterol (“bad cholesterol”), and triglycerides (fats), all of which are risk factors for cardiovascular disease. It is important that your doctors perform fasting lipid profiles if your cholesterol has been elevated, because people with lupus are at an increased risk for heart disease. In fact, cardiovascular disease—not lupus itself—is the number one cause of death in people with lupus. Furthermore, medications used in lupus treatment, especially corticosteroids such as prednisone, can raise blood pressure, blood glucose, cholesterol, and triglyceride levels, exacerbating the risk factors for cardiovascular disease in people with lupus.

A fasting lipid profile is performed only when a patient is fasting (i.e., has not eaten since midnight of the previous night). Fasting ensures an accurate reading of your baseline total cholesterol, HDL, LDL, and triglyceride levels. However, please understand that it is alright to take your medications with water upon the day you are fasting—water does not affect the fasting lipid profile.

• Total Cholesterol: Cholesterol is a fatty substance made in the body and absorbed from certain foods that is essential in your body’s normal processes. It plays an important role in the membranes of your cells, is used to make hormones, and helps form the bile acids needed for your body to obtain nutrients from food. Your total cholesterol is a measurement of both types of cholesterol—LDL and HDL—and should be below 200 mg/dL. Total cholesterol levels above 240 mg/dL are considered dangerously high, especially in people with additional risk factors for cardiovascular disease, such as smoking, obesity, or family history. If your total cholesterol level is above 200 mg/dL, your doctor will most likely recommend that you follow a diet low in saturated fats and cholesterol and begin a moderate exercise regimen. If diet and exercise alone are not enough to control your cholesterol, she/he may prescribe a medication called a statin to help lower your cholesterol levels.
• Low Density Lipoproteins (LDL): Cholesterol circulates in the body in complex molecules called lipoproteins. Low density lipoproteins (LDL) are sometimes known as “bad cholesterol,” because they can deposit excess cholesterol in your arterial walls, restricting blood flow and causing a condition known as atherosclerosis. If arteries become blocked, a person can suffer heart attack, stroke, or other complications. LDL levels above 100 mg/dL are considered to be above the optimal range. If you have other risk factors for heart disease, such as a history of smoking, low HDL levels, high blood pressure, diabetes, or a personal or family history of cardiovascular disease, you should aim for lower LDL levels.
• High Density Lipoproteins (HDL): High density lipoproteins (HDL) are known as “good cholesterol” because they help to move cholesterol out of the body by carrying it to the liver where it is processed for excretion. HDL levels less than 40mg/dL are associated with an increased risk of heart disease, but a good HDL level is above 60mg/dL.

Protein

A comprehensive metabolic panel will also check the levels of certain proteins in your blood. Specifically, the test checks for albumin levels and total protein levels.

• Albumin: Albumin is a small protein made in the liver that constitutes the major protein in blood serum. Albumin performs many functions in your body, including nourishing tissues, transporting various substances through the body (hormones, vitamins, drugs, and ions), and preventing fluid from leaking out of your blood vessels. Albumin concentration will drop if a person suffers from liver damage, kidney disease, malnourishment, serious inflammation, or shock. Abumin levels allow your doctor to assess for or monitor liver or kidney disease due to lupus and other factors.
• Total Protein: In addition to albumin, your blood serum also contains a protein called globulin. In fact, globulin is actually a class of proteins that includes enzymes, antibodies, and hundreds of other proteins. A total protein test measures the combined amount of these proteins in your blood. An albumin to globulin (A/G) ratio is also computed. A person’s total protein level gives information about kidney damage, liver damage, and nutritional health. If your total protein falls outside of the normal level, your doctor will most likely order other tests to assess for liver or kidney function.

Electrolytes

Electrolytes are ions (electrically charged chemicals) in the blood and other body fluids. The concentration of electrolytes in your body depends on adequate intake of nutrients, proper absorption of nutrients by the intestines, and proper kidney and lung function. Abnormal electrolyte concentrations can indicate abnormalities in certain organs and bodily processes. For example, retention of sodium, bicarbonate, or calcium can indicate problems with kidney function. Hormones also help to control electrolyte concentrations, so abnormal electrolyte levels can also reveal certain hormone deficiencies or problems with certain hormone-regulating glands or organs. Some of the electrolytes measured in a comprehensive metabolic panel are explained below.

• Sodium (Na+): Sodium helps to regulate your body’s water balance and plays an important role in proper heart rhythm, blood pressure, blood volume, and brain and nerve function. Hypernatremia refers to having too much sodium in the blood; this can occur, for example, due to a high-salt diet. Too much sodium in your blood can cause high blood pressure, among other things. Hyponatremia refers to having too little sodium in the blood. Hyponatremia can cause confusion, restlessness, anxiety, weakness, and muscle cramps. Sodium levels in the blood are regulated by a hormone called aldosterone that is secreted by the adrenal glands. Aldosterone works to regulate sodium levels by increasing your kidneys’ reabsorption of sodium ions.
• Potassium (K+): Potassium plays a role in regulating the acid-base chemistry and water balance in your blood and body tissues. It also helps your body to synthesize proteins and make use of carbohydrates for fuel. Potassium is essential for normal muscle growth and helps sodium and calcium to maintain normal hearth rhythm and regulate the body’s water balance. Potassium also helps your muscles to contract and your nerves to send impulses. Potassium levels may be low if an individual is on a diuretic (fluid pill) such as hydrochlorothiazide (HCTC) or furosemide (Lasix). Blood potassium levels that are too high or low may lead to muscle weakness and cramping; very low levels may cause irregularities in heartbeat. Like sodium levels, potassium levels in the blood are regulated by aldosterone, which promotes potassium loss from your kidneys.
• Calcium (Ca2+): Most people recognize calcium as a part of bones and teeth, but calcium plays many other roles in the body, such as regulating heartbeat, transmitting nerve impulses, contracting muscles, and helping blood to clot properly. Blood calcium levels are regulated by parathyroid hormone, which is secreted by the parathyroid gland, and calcitonin, which is secreted by the thyroid gland. Since lupus causes an increased risk of osteoporosis and corticosteroid (e.g., prednisone) use can elevate this risk, most people with lupus should take calcium and vitamin D supplements to help maintain adequate bone density. Medications called bisphosponates may be added to help with bone integrity if osteoporosis is detected. However, it is important that you realize that a blood calcium test measures the amount of calcium in the blood, not the bones. For an adequate measurement of bone health, you will need to obtain a DEXA scan every 2 years.
• Chloride (Cl-): Chloride ions help your body in maintaining proper pH and fluid balance. It also secreted by the stomach during digestion. Excessive sweating, vomiting, or diarrhea can cause chloride levels to drop. Low chloride levels may alter the pH of your blood, cause dehydration; they may also cause you to lose potassium.
• Carbon dioxide (CO2): This test measures the amount of carbon dioxide (CO2) in the blood, which is present in the form of CO2, bicarbonate (HCO3-), and carbonic acid (H2CO3). These three forms are involved in the equilibrium that maintains the pH of your blood (7.35-7.45). Bicarbonate also works with other electrolytes to maintain a certain charge balance in your cells. The concentration of carbon dioxide in your blood is maintained by your lungs and kidneys. High or low levels of CO2 may prompt your doctor to order other tests to check your kidney and lung function, blood gases, or fluid retention.

Liver Tests

Lupus and some of the medications used to treat lupus can affect the liver. In addition, factors such as excessive alcohol intake or viral hepatitis can affect the liver in people with lupus, just as they can in the normal population. Certain tests can be performed as part of a comprehensive metabolic panel to give insight into the function of your liver. In addition, your doctor may order a test called a liver panel if she/he suspects that you have symptoms of a liver disorder. Usually these tests measure certain liver enzymes, namely alkaline phosphatase (ALP), alanine amino transferase (ALT), and aspartate amino transferase (AST). Bilirubin, a waste product of the liver that is stored in the gall bladder, is also measured. These values can be used by your doctor as a screening or monitoring tool for liver involvement. About 30-60% of lupus patients experience abnormal liver function tests; some have no symptoms of liver disorder. Generally, increased levels correlate with increased activity, but other factors can contribute to elevated levels of liver enzymes in the blood. For example, NSAIDs, acetaminophen (Tylenol), and aspirin can cause liver enzyme values to increase, especially in people with lupus. If your doctor notices abnormal liver enzyme levels, she/he may ask you to undergo additional tests for hepatitis.

The liver enzymes and substances detected in a comprehensive metabolic panel are explained in more detail below.

• Alkaline phosphatase (ALP): Alkaline phosphatase (ALP) is an enzyme—a protein that helps to bring about chemical reactions in your body—found mainly in your liver and bones. High levels of ALP in the blood may indicate bone or liver abnormalities. If high ALP values are accompanied by high values of other liver enzymes and bilirubin, then the test suggests liver involvement. Certain ratios of liver enzymes can also indicate more specific conditions. Children usually have higher ALP levels than adults because their bones are still growing.
• Alanine amino transferase (ALT): Alanine amino transferase (ALT) is another enzyme found mainly in the liver. Smaller quantities can also be found in your kidneys, heart, and muscles. Levels of this enzyme are usually assessed in conjunction with readings for other liver enzymes to determine or monitor liver involvement. Very high levels of ALT may indicate acute hepatitis.
• Aspartate amino transferase (AST): Aspartame amino transferase (AST) is an enzyme found mainly in the liver, heart, and muscles. AST is released into the blood by injured liver or muscle cells but is used primarily to detect liver damage. [Another enzyme called creatine kinase (CK or CPK) is a better indicator of heart or muscle damage.]  Levels of AST are usually viewed alongside other liver enzymes to assess for liver damage. Like ALT, very high levels of AST may suggest acute hepatitis.
• Bilirubin: Bilirubin is a yellow-brown substance formed when the liver breaks down old red blood cells. Too much bilirubin can be a sign that the liver cannot adequately remove bilirubin from the system due to blockage (e.g., gallstones, tumors), cirrhosis, or acute hepatitis. Elevated bilirubin can also indicate hemolytic anemia, a reduction in red blood cells due to abnormal breakdown of red blood cells (hemolysis). Hemolytic anemia can be inherited or acquired; about 10-15% of people with lupus develop autoimmune hemolytic anemia. Hemolytic anemia causes red blood cells to have a shortened lifespan in the blood, and since bilirubin is a product of old red blood cells, it accumulates in the body faster than it can be eliminated. [Other tests called the Coombs test, haptoglobin count, and reticulocyte count are better diagnostic tests for hemolytic anemia.] Several inherited conditions, such as Gilbert’s syndrome, can also cause a person to have too much bilirubin. These conditions may be serious or benign. Often a buildup of bilirbubin is accompanied by a yellowing of the skin called jaundice.

Thyroid Tests

The thyroid is a gland in your neck associated with your metabolism—the processes by which your body makes use of energy. Autoimmune thyroid disease can occur in people with lupus, as can other thyroid conditions. Usually, thyroid conditions cause the gland to release too much or too little hormone. Your doctor may order tests to detect the level of thyroid hormones in the blood, especially if you experience significant weight loss or gain, sweating, acute sensitivity to hot or cold, fatigue, or other symptoms. These tests can also help your doctor monitor the effectiveness of thyroid treatment. Tests for thyroid hormones are explained below in greater detail. Your doctor may request additional tests, such as tests for thyroid antibodies, to learn more about your condition.

• Thyroid stimulating hormone (TSH): Thyroid stimulating hormone (TSH) is a hormone released by the pituitary gland that signals the thyroid to release its hormones (T3 and T4) when levels in the blood get low. Together, TSH, T3, and T4 are part of a negative feedback loop that keeps levels of thyroid hormones constant in the blood. Abnormal levels of TSH in the blood can suggest a problem with the pituitary gland, such as a tumor, but this is unlikely. More often, high or low TSH levels indicate problems with the thyroid gland. The thyroid may not be responding to stimulation by TSH, or it may be releasing too much T3 and T4. Underactive thyroid (hypothyroidism) is more common in lupus, but overactive thyroid (hyperthyroidism) can also occur. Both of these conditions can be dangerous if not properly treated.
• T4 and T3: Thyroid hormone contains thyroxine (T4, 90%) and triidothyronine (T3, 10%). The primary role of these substances is to regulate your body’s metabolism. Abnormal levels of thyroid hormone can indicate hypo- or hyperthyroidism.

Sources

• “Albumin.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/albumin/test.html>.
• “BUN.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/bun/test.html>.
• “Comprehensive Metabolic Panel.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/cmp/glance.html>.
• “Creatinine.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/creatinine/test.html>.
• “Electrolytes.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009<http://labtestsonline.org/understanding/analytes/electrolytes/test.html>.
• “Glucose.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/glucose/test.html>.
• “Lipid Profile.” Lab Tests Online. 18 June 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/lipid/glance.html>.
• “Liver Panel.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/liver_panel/glance.html>.
• Parker, Janet, ed. The Encyclopedic Atlas of the Human Body: A Visual Guide to the Human Body. Chicago: Global Book, 2007.
• “Thyroid Panel.” Lab Tests Online. 8 April 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/thyroid_panel/glance-2.html>.
• “Total Protein and A/G Ratio.” Lab Tests Online. 16 May 2009. American Association for Clinical Chemistry. 12 July 2009 <http://labtestsonline.org/understanding/analytes/tp/test.html>.

The immune system is an elaborate network of cells, tissues, and organs that helps to protect the body from invaders (bacteria, viruses, fungal infections, and parasites). Usually, the immune system develops only to act upon foreign substances, and immune system cells that try to combat cells of the body are weeded out during the development process. However, in lupus and other autoimmune diseases, the immune system begins to recognize and attack “self.” In other words, the cells of the immune system begin to injure the body’s own tissues. This phenomenon is similar to “friendly fire” and can cause permanent scarring that ultimately jeopardizes the function of certain organs and systems in the body. Certain cells and processes of the immune system have been identified as playing a role in lupus.

T-cells, B-cells, and Antibodies

A group of white blood cells called lymphocytes plays a key role in the human immune response. Lymphocytes include cells called B-cells and T-cells that are responsible for flagging and fighting infections in healthy individuals.

Antigens are substances that elicit the response of T-cells and B-cells in the body. When a T-cell recognizes a specific antigen, it binds to the substance and produces chemicals called cytokines. Cytokines then cause B-cells to multiply, and some of these B cells turn into plasma cells that secrete antibodies (immunoglobulins).  [The response of B cells is referred to as the “humoral” response; T-cell activation is called the “cell-mediated” immune response.] These antibodies then circulate in the bloodstream so that when they encounter the antigen again, they bind to it, forming a complex that is then acted on by other cells of the immune system in an effort to destroy the invader. Usually, remnants of these complexes are removed from the body by a garbage disposal system that involves the spleen.

T-cells are classified as killer-T cells, helper-T cells, or suppressor T-cells. Killer-T cells have the ability to recognize and destroy infected cells in the body. Helper-T cells, however, can only identify viruses engulfed by special cells called macrophages. The macrophage presents the antigen to the helper-T cell, which responds by producing the cytokines that stimulate B cells to multiply and release antibodies.

In healthy individuals, the masses of cells that gather at an infected or injured site in the body produce factors that help fight off the infection. This process causes some inflammation and injury of healthy tissue, but usually the immune system possesses other factors that help to control this inflammatory process. In individuals with lupus, both B cells and T cells become overactive. The two main consequences of this increased activity are the production of autoantibodies (antibodies that recognize and destroy the body’s own cells) and inflammation that can lead to long-term, irreversible scarring.

The production of autoantibodies in people with lupus and other autoimmune diseases causes the immune system to target the body’s own cells for destruction. For example, about 98% of people with lupus possess antinuclear antibodies (ANA), which can attack the nucleic material of your cells. In addition, some individuals may possess anti-phospholipid antibodies, which damage proteins bound to phospholipids in the membranes of your cells. These autoantibodies are linked to pregnancy complications, stroke, heart attacks, and other blood clots.

In addition, regulatory T cells, which are supposed to control the system, are deficient in SLE.

Neutrophils

Neutrophils are the most common type of white blood cell in your body; whereas lymphocytes are involved in the ongoing immune response, neutrophils are the first line of attack against invaders. Inflammation in a healthy individual usually signals that the body’s immune system is responding appropriately to pathogens, damaged cells, irritants, or injury. However, in lupus, neutrophils cause increased inflammation due to certain interactions between an individual’s blood plasma and other immune system cells (specifically, complement, cytokines, and cell adhesion molecules). Even though increased inflammation may cause pain and discomfort, the major problem with inflammation is potential long-term irreversible scarring. It is important that you and your doctor discuss medications to curb the inflammatory processes involved in lupus in order to minimize long-term damage to important organs.

Cytokines

Cytokines are signaling molecules involved in regulation of an individual’s immune response. Some cytokines amplify the immune response, while others tone it down. Some people with lupus and similar autoimmune diseases have a greater ratio of proinflammatory to anti-inflammatory cytokines than normal individuals, which produces an unbalanced regulatory mechanism.

While an overall cause-and-effect relationship between cytokines and lupus is not yet understood, certain cytokines called interferons and interleukins are associated with the disease. In general, however, the overproduction of such molecules causes the immune system to become overactive, leading to increased inflammation and tissue injury.

Complement

Complement proteins interact in a sequential manner to clear immune complexes from your body. Deficiencies of certain complement proteins are associated with lupus. In addition, since complement proteins are consumed during inflammatory processes, low complement levels may indicate lupus activity.

Sources

• Dean, Gillian S., et al. “Cytokines and systemic lupus erythematosus.” Ann Rheum Dis 2000; 59: 243–251.
• “Systemic lupus erythematosus.” In-Depth Patient Education Reports. Ed. Harvey Simon. 21 Jan. 2008. University of Maryland Medical Center. 25 June 2009 <http://www.umm.edu/patiented/articles/what_causes_systemic_lupus_erythematosus_000063_2.htm>.
• Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 1^st ed. New York: Oxford University Press, 1995.

(Non-steroidal anti-inflammatory drugs)

• Celecoxib (Celebrex)
• Diflunisal (Dolobid)
• Etodolac (Lodine)
• Ibuprofen (Advil, Motrin, Rufen)
• Indomethacin (Indocin)
• Meloxicam (Mobic)
• Midrin
• Nabumetrone (Relafin)
• Naproxen (Naprosyn, Alleve)
• Oxaprozin (Daypro, Duraprox)
• Piroxicam (Feldene)
• Salsalate (Disalcid)
• Sulindac (Clinoril)
• Tolmetin (Tolectin)
• Trilisate
• Ketoprofen (Orudis, Oruvail)

What are NSAIDs?

NSAID stands for “non-steroidal anti-inflammatory drug.” These anti-inflammatory medications relieve some lupus symptoms by reducing the inflammation responsible for the stiffness and discomfort in your muscle, joints, and other tissues. NSAIDs are milder than many other lupus drugs and may be taken either alone to treat a mild flare or in combination with other medications. NSAIDs come in both prescription and over-the-counter forms, but you should always talk to your doctor before taking any over-the-counter medication. There are many NSAIDs currently on the market; common examples include ibuprofen (Advil, Motrin), naproxen (Naprosyn, Alleve), etodolac (Lodine), celecoxib (Celebrex), and meloxicam (Mobic). Everyone responds differently to different medications, so you and your doctor should work together to find the most effective NSAID for your lupus symptoms.

How do NSAIDs work?

NSAIDs work at a chemical level by blocking the formation of molecules in your body called prostaglandins. Prostaglandins are involved in both your body’s normal “maintenance” and your body’s inflammatory response. These prostaglandins are controlled by enzymes—proteins that help to bring about chemical changes in your body—called Cox-1 and Cox-2. Specifically, Cox-1 controls the formation of the prostaglandins involved in the normal function of many of your body’s organs. Cox-2 controls the formation of the prostaglandins involved in your body’s inflammatory response. So, by stopping your body from making prostaglandins, NSAIDs allow you to experience less swelling and less pain.

Most NSAIDs block both Cox-1 and Cox-2 enzymes. Since the Cox-1 enzyme helps some of your organ systems to function normally, you may experience some side effects when taking NSAIDs, such as upset stomach. [A full list of possible side effects can be found below.] However, some newer NSAIDs, such as celecoxib (Celebrex), block only the Cox-2 enzyme, which may help your body to avoid some of the more traditional side effects. A Cox-2 inhibitor is sometimes called a “selective” inhibitor. Your doctor will help you to decide which type of NSAID works best to treat your lupus symptoms, while minimizing the possible side effects.

What side effects can occur from taking NSAIDs?

NSAIDs can cause certain side effects. These side effects can be similar to symptoms of increased lupus, so it is important to notify your doctor if they occur. Most side effects will go away once the drug is stopped. Some side effects will only be apparent from your blood tests, so it is important to get regular complete blood counts (CBCs) every 3-4 months, including tests of your liver and kidney function.

Potential side effects of NSAIDs include:

• Upset Stomach
• Headache
• Easy bruising
• High blood pressure
• Fluid retention
• Dyspepsia (gnawing or burning pain in the pit of your stomach, sometimes with bloating)
• Gastritis (stomach inflammation) or gastric ulcers may occur, causing either hidden or apparent bleeding. This blood loss may lead to anemia.
• Increased risk of heart attack or stroke.
• Effects on blood platelets, cells that help your blood to clot normally – Most NSAIDs have some effect on blood platelets. When platelet function is reduced, it takes longer for your blood to clot. While this effect can be bad for some, it can be beneficial for others. In fact, aspirin is often prescribed to reduce the risk of blood clots in people who have antiphospholipid antibodies. However, it is important that you speak to your doctor before taking any NSAIDs if you are on low-dose aspirin therapy, since certain NSAIDs such as ibuprofen (Advil, Motrin, Rufen) can interfere with the effectiveness of this treatment. Selective inhibitors (Celebrex) do not affect platelets.
• If you already have blood pressure or kidney problems, NSAIDs may worsen kidney function. Fluid retention or further elevation in blood pressure may occur. Reduced kidney function can occur with either Cox-1 or Cox-2 inhibitors. If you have lupus nephritis, you and your doctor should closely supervise your use of NSAIDs to reduce the potential for further harm to your kidneys.
• Occasionally lupus patients may experience elevations in their liver enzyme blood tests, suggesting a mild liver inflammation (hepatitis). Usually this does not mean that the medication must be stopped, but liver tests should be performed on a regular basis.
• Worsening of symptoms in people with asthma or inflammatory bowel disease.
• Severe headache with neck stiffness may occur exclusively in people with lupus, usually only with ibuprofen.
• Skin rashes can occur, either in the form of hives or a light- or sun-sensitive rash. Sometimes these rashes can mimic a flare of your lupus, so it is important you speak with your doctor if you experience this reaction. This reaction is more common with piroxicam (Feldene)

What if NSAIDs irritate my stomach?

Always take NSAIDs with food to help protect your stomach from irritation. If you experience stomach pain or other side effects when taking NSAIDs, your doctor may prescribe medications to control these symptoms. Medications such as cimetidine (Tagamet), ranitidine (Zantac), omeprazole (Prilosec), and lansoprazole (Prevacid) help your stomach from producing acid. Misoprostol (Cytotec) may help to maintain the protective lining of your stomach wall. It is important to remember that you may experience stomach irritation from one NSAID but not another. Speak with your doctor if you are experiencing stomach pain. There could be another drug that works better for you.

What should I keep in mind if I have been taking NSAIDs for a long time?

If you have been undergoing long term NSAID therapy, you should have your blood count measured periodically (a test called a CBC) to make sure you are not experiencing any hidden gastric bleeding. This type of bleeding can cause anemia, which affects your blood’s ability to clot and can lead to easy bruising.

Limit your alcohol intake while taking NSAID medications, since alcohol can irritate the stomach. People taking NSAID medications should not smoke. In fact, individuals with lupus should not smoke at all due to their increased risk of cardiovascular disease (the number one cause of death in lupus patients).

Lastly, it is important that you talk to your doctor about any other medications—prescription and over-the-counter drugs, vitamins, and supplements—that you may be taking.

Can I take NSAIDs if I am pregnant or thinking of having a baby?

NSAIDs are forbidden during pregnancy, even while trying to conceive.

Talk to your doctor if you:

• Have decreased kidney or liver function, or an uncontrolled or undiagnosed liver problem (for example, hepatitis)
• Have ever had an ulcer, gastritis, or bleeding from the intestinal tract
• Take blood thinners (anticoagulants) like Coumadin, heparin, aspirin, or Plavix
• Take steroids such as prednisone
• Have a low platelet count
• Have Crohn’s disease or ulcerative colitis
• Have a history of stroke, heart attack, hypertension, or congestive heart failure
• Have asthma or chronic lung disease
• Are allergic to aspirin or any other NSAIDs
• Have nasal polyps
• Have ‘reflux disease,’ indigestion, or hiatal hernia
• Are pregnant, may become pregnant, or are breast feeding
• Drink more than 7 alcoholic drinks per week or more than 1 per day
• Are over 65
• Do not accept blood products due to religious or other reasons.

Get emergency help right away if you experience any of the following symptoms:

• Chest pain
• Shortness of breath
• Weakness in one part of the body
• Slurred speech.

“Arthritis” is a broad term used to describe inflammation of the joints. There are many subsets of arthritis, but the arthritis seen in lupus closely resembles rheumatoid arthritis in that it is symmetric (affects the same locations on either side of the body) and usually affects the small joints of the hands, wrists, and feet. Unlike rheumatoid arthritis, erosion of bone usually does not occur, but the hands may appear deformed if there is laxity of the tendons or ligaments. [The medical name for this condition is Jaccoud’s arthropathy. Fingers affected by this condition can be manually straightened but will curve or turn to one side if left alone.] About 95% of lupus patients will experience arthritis or arthralgias (joint pains) at some point during the course of their disease.

Polyarthralgia and polyarthritis, defined as arthralgias or arthritis affecting 5 or more joints, are the most common joint problems seen in people with SLE. In fact, over 50% of lupus patients possess this ailment upon their initial diagnosis. Morning stiffness is also common in lupus. Often people with this condition find that a warm shower in the morning helps to loosen the synovial (joint) fluid and help the body to limber up for the day.

Both large joints, such as the knees, shoulders, and elbows, and small joints, such as the toe and finger joints, can be affected by lupus arthritis. However, pain in the groin should be given special attention by your doctor [especially if you take steroid medications], since this may indicate a more serious problem known as osteonecrosis. Sometimes fluid may accumulate in a joint. In the medical realm, this buildup of fluid is called an effusion and may require draining by your doctor. However, swelling in the joints caused by lupus will improve with lupus medications.

The joint pain and swelling caused by lupus can be helped by certain medications, including NSAIDs, such as naproxen or ibuprofen. Arthritis may also be helped by daily antimalarial therapy (Plaquenil). If necessary, severe polyarthritis can be treated with certain steroids and immunosuppressive medications, such as methotrexate. Your doctor will decide which treatment plan will best alleviate your lupus arthritis.

Sources

• “Arthritis.” Medical Dictionary. 26 June 2009 <http://medical-dictionary.thefreedictionary.com/arthritis>.
• Petri, Michelle. “Lupus Information.” Nov. 2002. The Johns Hopkins Arthritis Center. 26 June 2009. <http://www.hopkins-arthritis.org/arthritis-info/lupus/>.
• Schur, Peter H. “General Symptomology.” The Clinical Management of Systemic Lupus Erythematosus. Ed. Peter H. Schur. 2nd ed. Philadelphia: Lippincott-Raven, 1996. 10.
• Ziminski, Carol M. “Musculoskeletal Manifestations.” The Clinical Management of Systemic Lupus Erythematosus. Ed. Peter H. Schur. 2nd ed. Philadelphia: Lippincott-Raven, 1996. 47-65.