Lupus

Understanding the type and the symptoms it causes is crucial for designing a treatment plan that will help you feel better. Depending on the type of lupus, the disease affects organs, tissues, skin and babies.

Systemic lupus erythematosus is the most common type of lupus. Systemic means the disease impacts many different parts of your body. SLE causes problems with skin, connective tissues (joints), and major organs, including the lungs, kidneys, nervous system, heart, and circulatory system. Symptoms range from mild to severe.

Drug-induced lupus erythematosus is triggered by certain prescription medications commonly used to treat heart disease, hypertension and tuberculosis. It is similar to systemic lupus except major organs are often not affected. Symptoms typically disappear within six months after stopping the medication. Drug-induced lupus is more common in men.

This form affects the skin leading to an abundance of chronic rashes and skin sores of your face, scalp or other body parts. Two types of cutaneous lupus are:

• Discoid lupus erythematosus is a chronic skin disorder characterized by round, disc-shaped rashes on the face, scalp, or other parts of the body. The rashes are often red and raised, and they may be thick and scaly. They aren’t painful or itchy, and they may last only a few days. But it’s possible for them to stick around for years and cause permanent, disfiguring scarring. Some people with discoid lupus also have systemic lupus or could develop it later.

• Subacute cutaneous lupus causes skin sores after spending time in the sun. The lesions may be ring-shaped or appear as red, scaly areas. Usually, they don’t cause itching or scarring. But they can become worse when not protected against ultraviolet (UV) light from the sun or fluorescent lighting indoors.

Lupus mostly strikes women of childbearing age. While most women with lupus who become pregnant give birth to healthy infants, some children are born with neonatal lupus. The condition can cause symptoms, such as skin rash, liver problems, or low blood cell counts. But neonatal lupus and its symptoms often go away within several months. Neonatal lupus is also linked to a rare but treatable heart condition.

Symptoms of lupus are the result of tissue inflammation throughout various parts of the body. The type and severity of symptoms vary between individuals and the type of lupus. There are times when lupus is more active (a flare-up) and periods when it is less active (in remission) when symptoms improve.

At the onset of the disease, the symptoms of lupus can be mild and vague. Note the classic butterfly-shaped rash on the face may not occur in all people with lupus.

Symptoms of lupus include:

• Abdominal pain

• Butterfly-shaped skin rash over the cheeks and nose and other skin rashes from sun exposure

• Difficulty concentrating and mild trouble with memory

• Fatigue

• Fever

• Hair loss

• Headaches

• Inflammation of the lungs and chest pain when breathing deeply

• Lesions or sores in the mouth or nose

• Muscle and joint pain and swollen joints

• Nausea and vomiting

• Photosensitivity

• Swollen lymph nodes

Complications of lupus can be serious and life threatening in some cases. Complications include damage to the brain and nervous system, digestive tract, kidneys, heart, and lungs. Seek immediate medical care (call 911) if you have any of the following symptoms:

• Bleeding while pregnant

• Blood in the urine or other bleeding symptoms

• Change in level of consciousness or alertness, such as passing out or unresponsiveness

• Change in mental status or sudden behavior change, such as confusion, delirium, lethargy, hallucinations or delusions

• Chest pain, chest tightness, chest pressure, or palpitations

• Not producing any urine

• Respiratory or breathing problems, such as shortness of breath, difficulty breathing, labored breathing, wheezing, not breathing, or choking

• Seizure

• Severe abdominal pain

What causes this autoimmune response in the body is not known, but it is thought that lupus is triggered by various environmental factors and possibly a genetic predisposition for developing an autoimmune disorder. In some cases, an individual who develops lupus has a relative with lupus or another autoimmune disease.

Past and current research highlights the combined role of genetic, hormone and environmental factors:

• Naturally occurring variations in many different genes increase the risk of developing lupus. It can run in families, but most people at risk for lupus do not have a family history of it.

• Most lupus-related genes, identified thus far, play a role in immunity, antibody function, and inflammation. When certain biologic processes are activated in people with a genetic predisposition to lupus, the body’s immune system responds resulting in inflammation.

• Scientists believe that certain hormones and environmental factors “tip the scale” and trigger the disease in people with certain gene variations.

• Factors involved in triggering lupus include the female sex hormone estrogen, viral infections, diet, stress, drug exposure, chemical exposure (such as tobacco), and sunlight.

A number of factors increase risk of developing lupus. Not all people with risk factors will develop lupus. Risk factors for lupus include:

• Family history of lupus

• Female sex between ages 15 and 44

• Genetic predisposition

• Hispanic, American Indian, Pacific Islander, or African American descent

• Use of beta blockers, which are commonly used to treated heart disease and hypertension

Conditions with symptoms and disease pathology similar to or overlapping with lupus include:

• Antiphospholipid antibody syndrome, an autoimmune disease that affects the blood

• Arthritis, joint diseases including osteoarthritis and rheumatoid arthritis

• Fibromyalgia, a painful disease involving muscles and joints

• Glomerulonephritis, a form of kidney disease that appears to be caused by problems with the immune system

• Scleroderma, an autoimmune disease affecting the skin and other connective tissues

• Sjögrens syndrome, an autoimmune disease involving the glands that make tears and saliva

Although there is no definitive lupus test, there are several clues that, when taken together, point to lupus. The most common criteria doctors use to diagnose lupus include:

• Symptoms including skin rashes (sometimes triggered by sun exposure), unexplained fatigue, mouth sores, and arthritis

• Impaired kidney function, which can show up in urine and blood tests

• Positive antinuclear antibody (ANA) test, which is not specific for lupus but can help determine if more tests are necessary

• Positive anti-dsDNA, anti-Sm, and other autoantibody tests, which are much more specific than the ANA test for lupus. Positive results can help confirm a diagnosis in patients with symptoms; however, a negative result does not exclude lupus because not all lupus patients have these antibodies.

• Abnormal blood cell counts including low red and white cells and low platelets

• Painful inflammation around the lungs or heart

• Neurologic problems including seizures or psychosis

Lupus treatment plans are multifaceted and individualized to the type and severity of lupus, and your age, medical history, and coexisting diseases and conditions. Treatment includes medications, lifestyle changes, diet, and avoiding exposure to the sun. People with lupus can be very sensitive to the sun, and sun exposure can trigger flare-ups of symptoms, including skin rashes.

Rheumatologists treat lupus patients with a wide array of medications. The goal is to relieve pain, reduce inflammation, and suppress the abnormal response of the immune system. This can relieve symptoms, minimize flare-ups, and help put the disease in remission.

Common medications include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil and Motrin), naproxen (Naprosyn and Aleve), and indomethacin (Indocin), which are very effective in treating the pain and inflammation caused by lupus. However, long-term use of NSAIDs can cause serious side effects, such as bleeding gastrointestinal ulcers and possible heart problems and cardiovascular events.

• Anticoagulants, which thin the blood and reduce the risk of developing serious blood clots

• Aspirin, which is very effective in treating the pain and inflammation caused by lupus. Aspirin also thins the blood and reduces the risk of developing serious blood clots. However, aspirin use should be closely monitored because it can cause serious side effects, such as bleeding ulcers.

• Antimalarial drugs, which can treat joint pain and inflammation as well as rashes and mouth sores

• Corticosteroids, such as prednisone, which reduce the inflammation caused by lupus. Long-term corticosteroid use has the potential for serious side effects. People taking corticosteroids should not stop taking them suddenly and should immediately report any side effects to their healthcare provider.

• Immunosuppressive medications, which suppress an overactive immune system but can increase the risk of developing opportunistic infections

• Biologics, or biologic response modifiers. Biologics are man-made drugs engineered to block or inhibit specific molecules involved in a disease.

• Disease-modifying antirheumatic drugs (DMARDs) suppress the body’s immune and inflammatory responses.

A variety of additional treatments and therapies may be recommended for lupus, including complementary therapies. These treatments, sometimes referred to as alternative therapies, are used in conjunction with traditional medical treatments. Complementary treatments are not meant to substitute for full medical care.

Other treatments and therapies for lupus may include:

• Acupuncture to help relieve joint pain

• Avoiding exposure to the sun and protecting your skin from the sun. Use sunscreen, wear a hat, and cover bare skin while in the sun. Also protect yourself from ultraviolet (UV) light from indoor fluorescent lighting.

• Meditation and biofeedback to help relieve stress and pain

Along with taking regular medical care and following your treatment plan, these lifestyle changes can help manage the disease:

• Exercise regularly including low impact options such as walking, swimming and bicycling. Exercise improves bone and heart health, reduces stress, raises energy levels, and helps people lose excess weight, which also reduces joint strain.

• Get adequate rest—at least seven hours per night—to reduce fatigue and allow your body to heal.

• Don't smoke and avoid secondhand smoke. Smoking increases the harmful effects of lupus.

• Eat a heart-healthy diet to help protect your heart from damaging inflammation

While there is no special diet for people with lupus, certain foods can help manage symptoms and may reduce your risk of complications related to lupus, including heart disease and osteoporosis. Avoiding certain foods may help reduce flares and drug side effects. Ask your healthcare provider for guidance before making significant changes to your diet.

Eat more of these in your healthy diet:

• Fish. Look for “fatty” fish, which are rich in heart-healthy omega-3 fatty acid, and eat two servings per week. Examples include salmon, albacore tuna and sardines.

• Dairy products. Low-fat milk, yogurt, cheeses and other dairy products provide calcium and vitamin D to help keep your bones strong and reduce your risk of osteoporosis.

• Leafy dark green vegetables. Spinach, broccoli and other dark green vegetables also provide calcium and vitamin D.

• Tomatoes and potatoes. Add these foods along with oranges, grapefruit, raisins and other foods high in potassium—another essential part of a heart-healthy diet to reduce your risk of coronary artery disease.

Avoid or reduce these items if you have lupus:

• Alfalfa seeds and sprouts. Compounds in alfalfa are known to trigger the immune system which can increase inflammation and cause lupus flares.

• Alcohol. Drinking alcohol may interact with your medicine making it less effective or increasing side effects. Talk with your doctor about your medications and if you should avoid drinking alcohol.

• Salt. High amounts of sodium can raise blood pressure and increase your risk of heart disease. Check labels of processed foods for sodium per serving. Most adults should aim for less than 2,300 mg of sodium per day. People age 51 or older and those who have high blood pressure, diabetes, or kidney disease should have less than 1,500 mg per day.

The chronic, progressive and unpredictable nature of lupus has a negative impact on quality of life in many areas, including physical, socioemotional, and financial well-being.

In addition to lupus symptoms, the burdens of living with lupus include:

• Higher risk of cardiovascular disease, osteoporosis, and serious infections

• High healthcare costs from disease treatment, doctor visits, and hospitalizations

• Reduced ability to work and lost productivity, particularly with highly active disease

• Greater incidence of mental health problems including mood disorders and anxiety

Lupus patients with good family and social support report less of an effect on quality of life compared to people with a poor support network. Age is also a factor; there is a correlation between older age and greater impact on quality of life.

You can best minimize life-threatening complications of lupus by following your treatment plan and seeing your healthcare provider as recommended. Complications of lupus can be serious and affect almost any organ in the body. Complications include:

• Adverse effects of treatment

• Arthritis

• Bleeding

• Blood clots

• Central nervous system damage and stroke

• Frequent infections

• High blood pressure

• Kidney damage and kidney failure

• Liver damage

• Miscarriage

• Pleurisy (inflammation of the lungs)

• Seizures

• Stroke

• Vasculitis (inflammation of the blood vessels that can lead to decreased blood flow to major organs, and such conditions as atherosclerosis and heart attack)

Most people with lupus enjoy a long life, but lupus-induced organ damage and complications can be fatal in some cases.

Here are some facts on lupus prognosis:

• Lupus is one of the leading causes of death in women ages 5 to 64.

• The 15-year survival rate for adults with lupus is 0.82, which means 82% of people who were evaluated 15 years after diagnosis were still alive. These same patients likely survived much longer, but the study did not continue past 15 years.

• Lupus-related kidney disease (lupus nephritis) reduces overall survival.

• Earlier diagnosis and advances in treatment have improved lupus survival in the last 50 years, although some fatalities are due to medication side effects.

The current number of people with lupus in the United States is not known, in part because population-based studies are lacking, the condition is still underdiagnosed, and doctors are not required to report the disease.

Older studies show a prevalence ranging from 20 to 150 lupus cases per 100,000 people. The Lupus Foundation of America reports 1.5 million Americans have some form of lupus.

In addition to lupus predominantly affecting women, more recent U.S. studies show lupus:

• Affects 5.5 per 100,000 persons (male and female) and 9.3 per 100,000 females, according to one population-based study

• Most commonly strikes women of childbearing age

• Can begin in the teen years, but it rarely affects children younger than 5. About 20% of people with lupus develop it before age 20.

• Is 3 to 4 times more common in African American than Caucasian women. It is also more common in Hispanic and Asian individuals. Black Americans with lupus have a greater risk of kidney disease than Caucasian patients.

With the profound impact of lupus on society, it is encouraging that the U.S. Department of Defense, as part of the Lupus Research Program, is funding lupus research projects identified as potentially having a high impact on understanding and treating the disease.

In addition, the Centers for Disease Control and Prevention is funding the Lupus Foundation of America and the American College of Rheumatology to heighten lupus awareness and bring greater understanding of the disease among both patients and providers.

May is Lupus Awareness Month.