At least 5 million people worldwide and 1.5 million Americans have a form of lupus, according to the Lupus Foundation of America.1
What is lupus? Lupus is a chronic disease causing inflammation and pain in various parts of the body. It’s an autoimmune disease, which means the immune system — the body system that usually fights infections — attacks healthy tissue instead.2
While there’s no cure for lupus, medical interventions and lifestyle changes may help control the disease.3
What are lupus symptoms?
Symptoms of lupus usually start in early adulthood.4 4 signs of lupus may include fatigue, hair loss, pulmonary and kidney problems.5
Lupus may affect nearly all the organs in the body.6 The symptoms differ for each person with lupus. For example, 1 person with lupus may have swollen knees and fever, while another patient may be tired all the time or have kidney trouble. Someone else may suffer from rashes. Over time, new symptoms may develop, while other symptoms may happen less often.7
Lupus symptoms include, but are not limited to, this brief list:8
- Muscle and joint pain – Stiffness, swelling and pain in the muscle and joints affect most people with lupus. Common areas for muscle pain and swelling include the neck, thighs, shoulders and upper arms.
- Fever – Often caused by inflammation or infection, fevers higher than 100 degrees Fahrenheit affect people with lupus. Medicine is available to help manage and prevent fever.
- Rashes – A typical sign of lupus is a red, butterfly-shaped rash across the nose and cheeks. Rashes may occur on any part of the body exposed to the sun, including hands and arms.
- Chest pain – Lupus may cause inflammation in the lining of the lungs, which causes chest pain.
- Kidney problems – About half of people with lupus also develop kidney problems, called lupus nephritis. Symptoms include decreased kidney functions, high blood pressure, weight gain and swollen ankles.
- Mouth sores – Sores or ulcers may appear on the roof of the mouth, gums, inside the cheeks and on the lips.
- Ongoing or extreme fatigue – Fatigue may be a sign of a lupus flare-up. The patient may feel tired or exhausted despite getting enough sleep.
For people with lupus, the symptoms of the disease will fluctuate up and down. Sometimes, the disease will flare, with the symptoms worsening, making the patient feel ill. Then the disease will go into remission, when the symptoms will improve, and the patient will feel better.9
What are the types of lupus?
There are 4 types of lupus:
- Systemic lupus erythematosus (SLE), the most common form of lupus, causes tissue damage and inflammation in the affected organs. SLE can affect the joint skin, brain, lungs, kidneys and blood vessels.10
Lupus nephritis is a frequent complication in people with SLE. Lupus nephritis occurs when lupus antibodies affect structures in the kidneys that filter out waste. This causes kidney inflammation and may lead to blood in the urine, protein in the urine, high blood pressure, impaired kidney function or even kidney failure.11
- Cutaneous lupus, a form of lupus that is limited to the skin. Chronic cutaneous lupus symptoms include rashes that last a long time and may cause permanent scarring.12
The most common type of chronic cutaneous lupus is discoid lupus. Patients with discoid lupus get disk-shaped rashes that appear on the scalp, face and other body parts.13 Discoid lupus sores are usually red and scaly. The sores may cause scars, permanent hair loss or skin discoloration. Dermatologists and rheumatologists may be able to help patients with discoid lupus to prevent permanent scarring and hair loss.14
- Drug-induced lupus, a lupus-like disease caused by certain prescription drugs. The drugs that are frequently connected with drug-induced lupus are:
- Hydralazine, which is used to treat high blood pressure or hypertension.
- Procainamide, which is used to treat irregular heart rhythms.
- Isoniazid, which is used to treat tuberculosis.
Drug-induced lupus is more common in men because these drugs are prescribed more often to men. However, not everyone who takes these drugs will develop the disease.16
- Neonatal lupus, a rare condition that affects infants of women who have lupus. Neonatal lupus is not true lupus, according to the Lupus Foundation of America.17 It is a rare condition associated with anti-SSA/Ro and/or anti-SSB/La antibodies from the mother that affect the fetus. The baby may be born with a skin rash, low blood cell counts or liver problems, but after 6 months, these symptoms usually disappear with no lasting effects.18
The most serious symptom of neonatal lupus is congenital heart block, which causes a slow heartbeat. While very rare, newborns of women with lupus are at greater risk for developing this potentially life-threatening complication. Congenital heart block is usually detected when the fetus is between 18 and 24 weeks old. The condition does not disappear, and affected infants will eventually need a pacemaker.19
Advancements in testing allow physicians to identify most at-risk mothers, and the infant can be treated at or before birth. Most infants of mothers with lupus are born healthy.20
Who is at risk for developing lupus?
While anyone can develop lupus, some people are at higher risk, including:21
- Women ages 15 to 44.
- Certain racial or ethnic groups – including people who are African American, Asian American, Hispanic/Latino, Native American or Pacific Islander.
- People who have a family member with lupus or another autoimmune disease.
Healthcare professionals are not able to pinpoint why a person gets lupus because the disease likely results from a combination of genetics and environment.22 People with an inherited predisposition for lupus may develop the disease when they come in contact with something in the environment that triggers the disease. But in most cases, the cause of lupus is unknown.23
Common triggers that make lupus symptoms worsen or "flare,” making the patient feel ill, are:24
- Overworking without taking breaks.
- Exposing the body to sunlight, fluorescent or halogen light.
- Getting an infection.
- Becoming injured.
- Stopping lupus medicines.
- Using other types of medicines.
People with lupus may experience a flare, even while they are taking lupus medication. For example, lupus symptoms may flare after a patient has been out in the sun or after a long day at work, despite taking lupus medication.25
What are treatments for lupus?
Treatment of lupus depends on the patient’s symptoms. As the signs and symptoms flare and subside for the person with lupus, physicians may change medications or dosages.26
The medications most commonly prescribed to control lupus are:27
- Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs), such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be taken by the patient to treat the pain, swelling and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects of NSAIDs may include stomach bleeding, kidney problems and an increased risk of heart problems.
- Antimalarial drugs. Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), affect the immune system and may help decrease the risk of lupus flares. Side effects may include stomach upset and, very rarely, damage to the retina of the eye. Regular eye exams are recommended when taking these medications.
- Corticosteroids. Prednisone and other types of corticosteroids may minimize inflammation caused by lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious diseases that involve the kidneys and brain. Side effects include gaining weight, bruising more easily, thinning bones, increasing risk of infection, high blood pressure and diabetes. The risk of side effects increases with higher doses and long-term therapy.
- Immunosuppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep, others), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
- Biologics. Most biologic drugs are antibodies or proteins made by the immune system to attack viruses and bacteria.28 The medication belimumab (Benlysta) is administered intravenously and has been shown to reduce lupus symptoms in some patients. Side effects include nausea, diarrhea and infections. Rarely, worsening of depression may occur.
For people with lupus who haven’t experienced improvements with other medications, Rituximab (Rituxan, Truxima) may be beneficial. Side effects include allergic reaction to the intravenous infusion and infections.
In clinical trials, voclosporin has been shown to be effective in treating lupus.29
Other potential drugs are currently being studied to treat lupus, including abatacept (Orencia) and anifrolumab.30
Resources for lupus patients
For people with lupus, the disease may affect many parts of their lives for their entire lives. By taking steps to control their symptoms, prevent lupus flares and find resources to cope with the challenges of the disease, patients can live long, healthy lives.31
Tips for controlling lupus symptoms:32
- Learn to anticipate when a flare is coming.
- Schedule regular doctor visits.
- Limit time spent in sunlight or fluorescent and halogen light.
- Make time to sleep and rest.
- Create a support network of family, friends and caregivers.
While people with lupus may have limitations when the disease flares, they can stay involved with work, relationships and activities they enjoy. The Lupus Foundation of America provides a guide for coping with lupus:33
- Explain lupus to family, friends and caregivers. Start by emphasizing lupus is not contagious and not related to cancer. Describe how lupus symptoms may appear, disappear and change.
- Make adjustments as a family. Clearly communicate lupus symptoms, treatments and why the patient may not be able to participate in certain activities, including some household responsibilities. Create a manageable schedule as a family, incorporating specific time for breaks.
- Managing work with lupus. While people with lupus may need to make changes in their work environment, many are able to continue working. Depending on the patient’s symptoms and their responsibility, they may need a more flexible work schedule or work remotely. A vocational rehabilitation counselor, a job coach for people with disabilities, may help find work that is more manageable for the person with lupus.
- Find a lupus support network. From individual counselors to couples and family therapy, professionals are available to help people with lupus cope with the disease. Online support groups have been created to help patients engage with others affected by lupus to share experiences and successes.
Family and friends may ask, “What is the life expectancy of a person with lupus?” With careful follow-up and treatment, 80-90% of people with lupus can expect to live a normal life span.33 While medical science has not yet developed a cure for lupus, and some people die from the disease, for the majority of people with lupus, the disease will not be fatal.34
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