Lupus is a chronic autoimmune disease that can damage any part of the body (skin, joints, and/or organs). “Chronic” means that the signs and symptoms tend to last longer than six weeks and often for many years.
In lupus, something goes wrong with the immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu).
Normally our immune systems produce proteins called “antibodies” which protect the body from these invaders.
“Autoimmunity” means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”).
As a result, it creates autoantibodies that attack and destroy healthy tissue.
These autoantibodies cause inflammation, pain, and damage in various parts of the body.
Additional facts about lupus that you should know:
- Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
- Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above. However, some treatments for lupus may include immunosuppressant drugs that are also used in chemotherapy.
- Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
- Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
- More than 16,000 new cases of lupus are reported annually across the country.
- Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
- It is believed that 5 million people throughout the world have a form of lupus.
- Lupus strikes mostly women of childbearing age. However, men, children, and teenagers develop lupus, too. Most people with lupus develop the disease between the ages of 15-44.
- Women of color are two to three times more likely to develop lupus than Caucasians.
- People of all races and ethnic groups can develop lupus.
New research indicates that belimumab, a monoclonal antibody therapy that targets a component of the immune system, provides considerable benefits to patients with systemic lupus erythematosus (SLE), a predominately female, chronic inflammatory disease that can affect virtually any organ.
The Arthritis & Rheumatology findings are encouraging because lupus is among the leading causes of death in young women.
SLE is a chronic, multisystem autoimmune disorder with a broad range of symptoms, and its development involves defective activation of B cells — the immune cells charged with producing antibodies that attack invading bacteria, viruses, and toxins.
B lymphocyte stimulator is a molecule involved in B cell differentiation, and elevated levels may contribute to the production of autoantibodies that target healthy cells and tissues.
To investigate the efficacy and safety of belimumab, which targets B lymphocyte stimulator, Andrea Doria, MD, of the University of Padova, in Italy, and her colleagues conducted a phase III, double-blind, placebo-controlled study that randomized SLE patients 2:1 to weekly subcutaneous injections of belimumab 200 mg or placebo, plus standard lupus therapy, for 52 weeks.
Belimumab is currently given by intreavenous infusion, but the subcutaneous formulation of has recently become available.
In analyses of 356 patients with certain clinical characteristics indicative of high disease activity (hypocomplementemic and anti-dsDNA-positive), more patients in the belimumab group experienced reduced lupus disease activity, as measured by what is called the SLE responder index (64.6% versus 47.2%), and there was a lower incidence of severe SLE flares (14.1% versus 31.5%).
Also, more people in the belimumab group were able to reduce their need for steroids. Side effects were similar between the groups.
“Intravenous administration of belimumab is an obstacle to treatment for many patients due to the need to go to the hospital for drug infusions.
Thus, a higher number of patients could benefit from this treatment,” said Dr. Doria.
“The self-administration of subcutaneous belimumab makes hospital access unnecessary, which leads to economic savings for patients and the community.”
An accompanying report in Arthritis & Rheumatology ranks lupus deaths among the US Centers for Disease Control and Prevention’s leading causes-of-death to determine the relative burden of SLE deaths in women.
(The agency compiles an annual leading-causes-of-death ranking based on a selected list of 113 causes, but SLE is not included on this list.)
The report notes that during 2000-2015, there were 28,411 female deaths in the United States that had SLE recorded as the underlying or contributing cause of death.
SLE ranked as the 10th leading cause of death in the 15-24-year age group, 14th in 25-34-year and 35-44-year age groups, and 15th in 10-14-year age group. Among black and Hispanic females, SLE ranked 5th in the 15-24-year, 6th in the 25-34-year, and 8th-9th in the 35-44-year age groups, after excluding three common external injury causes of death (unintentional injury, homicide, and suicide) from the analysis.
Medications used to treat lupus
A detailed guide to the types, and potential side effects of the main medications used by people with lupus.
Anti-inflammatories and over-the-counter pain relievers
Anti-inflammatory medications help to relieve many of the symptoms of lupus by reducing inflammation and pain. Anti-inflammatories are the most common drugs used to treat lupus, particularly symptoms such as fever, arthritis or pleurisy, which generally improve within several days of beginning treatment. For many people with lupus, an anti-inflammatory drug may be the only medication they need to control their lupus.
- Pain-reducer with anti-inflammatory and anticoagulant (blood-thinning) properties.
- Can control some of the symptoms of lupus.
- Can cause stomach irritation.
- Treatment for pain.
- Less stomach irritation than aspirin.
- Does not help with inflammation and cannot control lupus disease activity.
- Most people have no side effects when taking Tylenol, but in rare cases acute liver failure has occurred.
Ibuprofen (Motrin®), naproxen (Naprosyn®), indomethacin (Indocin®), nabumetone (Relafen®), and celecoxib (Celebrex®).
- Inflammation suppression.
- Especially useful for joint pain and stiffness.
- People often respond better to one particular NSAID than another so you may need to try several different products to determine the most effective one for you.
- Like aspirin, NSAIDs can cause stomach irritation.
- NSAIDs may also cause serious gastrointestinal (GI) complications, such as a bleeding ulcer. To reduce the chance of these problems, NSAIDs are usually taken with food, milk or antacids, or may be accompanied by other medications such as misoprostol (Cytotec®), omeprazole (Prilosec®), lanzoprazole (Prevacid®) and others. You may need a prescription for these medications.
- Side effects of NSAIDS, such as abnormal urine test results, occasionally may be mistaken for signs of active lupus. Recognizing this possible complication of NSAID use is important because the symptoms will go away when the drug is stopped.
- In general, you should always be cautious about taking too much of any NSAID as excessive amounts can reduce the blood flow to your kidneys and may interfere with their ability to remove waste from your body.
- NSAIDs are available over the counter and by prescription (for higher dosages).
Corticosteroids (also known as glucocorticoids, cortisone or steroids) are synthetic (man-made) prescription drugs designed to work like the body’s naturally occurring hormones produced by the adrenal glands, in particular cortisol. Hormones are the body’s chemical messengers that regulate most of the body’s functions. Cortisol helps regulate blood pressure and the immune system and it is the body’s most potent anti-inflammatory hormone. Corticosteroids prescribed for autoimmune diseases are different from the anabolic steroids that weightlifters and other athletes sometimes take to increase strength.
Steroid medications work quickly to decrease the swelling, warmth, tenderness and pain that are associated with inflammation. They do this by lessening the immune system’s response. Prednisone is the most commonly prescribed steroid for lupus. Prednisolone and methylprednisolone (Medrol®) are similar to prednisone. Some physicians prefer to prescribe these if you have liver problems.
Most people take steroids in pill form, but topical creams or gels are often used for cutaneous (skin) lupus. Steroids in liquid form are sometimes injected into muscles or directly into joints and in some cases into skin lesions. Pulse steroids are large liquid doses given intravenously (injected into a vein) over several hours; the beneficial effects can last for weeks, so pulse steroids are sometimes prescribed to control a lupus flare or for people who cannot tolerate steroids in pill form.
Your doctor will try to keep your steroid dosage at the lowest effective level. Once the symptoms of lupus have responded to treatment, the steroid dose is gradually reduced (tapered). As an alternative to tapering, or stepping down, the steroid dose, your doctor may choose to have you take steroids on an every-other day basis — one day on, one day off.
Steroids can produce a variety of side effects, including:
- A round or moon-shaped face, weight gain or hair growth
- Fluid retention and a redistribution of fat, leading to a swollen face and abdomen but thin arms and legs
- Fragile skin that bruises easily.
- Suppressed growth in children.
- Irritability, agitation, excitability, insomnia or depression.
Changes in appearance and mood are more apparent with high doses of steroids.
Long-term steroid use can produce additional side effects, including:
- Increased risk of infections poses the most danger. If you are taking steroids, you must take extra care to clean and protect any open wounds. Infections are one of the leading causes of death in people with lupus.
- Avascular necrosis of bone, which occurs most often in the hip, is the destruction of the bone itself and is quite painful. Relief from pain often requires total joint replacement.
- Osteoporosis (bones become fragile and more likely to break) leads to bone fractures, especially compression fractures of the vertebrae with severe back pain.
- Muscle weakness and cataracts also can occur.
Infections are one of the leading causes of death in people with lupus. Long-term steroid use can increase your risk of infection. If you are taking steroids, take extra care to clean and protect any open wounds.
Antimalarials are prescription drugs used in combination with steroids and other medications, in part to reduce the dose required of the other drugs. Antimalarials are most often prescribed for skin rashes, mouth ulcers and joint pain, but also can be effective in mild forms of lupus where inflammation and blood clotting are a concern.
Antimalarials improve lupus by decreasing autoantibody production, protecting against the damaging effects of ultraviolet light from the sun and other sources and improving skin lesions.
The two types of antimalarials most often prescribed today for lupus are hydroxychloroquine (Plaquenil®) and chloroquine (Aralen®). Unlike the rapid response seen with steroids, it may take months before antimalarial drugs improve your lupus symptoms.
Side effects from antimalarials are rare and usually mild; they include upset stomach and changes in skin color. Side effects usually go away after the body adjusts to the medication.
In high doses and over time, certain antimalarial drugs may damage the retina of the eye (retinal toxicity), causing vision problems. If low doses of antimalarials are used in the treatment of lupus, the risk of this complication is low. However, as a precaution, people treated with antimalarials should get a baseline eye exam before or soon after starting the drug and visit an eye doctor (ophthalmologist) annually.
Long term plaquenil users on high doses will need to monitor eye health regularly to prevent retinal toxicity from long-term use.
Pregnant women should continue to take their antimalarial medication as prescribed to avoid a lupus flare. Although this medication can cross the placenta, the possibility of eye and ear toxicity in the infant is very low. In fact, recent studies suggest that the risk of flare for the mother is greater than the risk of fetal toxicity.
Immunosuppressives (Immune Modulators)
Immunosuppressive medications are prescription drugs used to control inflammation and the overactive immune system, especially when steroids have been unable to bring lupus symptoms under control, or when a person cannot tolerate high doses of steroids. However, there can be serious side effects from these drugs.
If you are being treated with immunosuppressives, you should be carefully monitored by your physician. Immunosuppressive drugs reduce your body’s ability to fight off infections and increase the chances that you could develop viral infections such as shingles (chicken pox or herpes zoster). It is extremely important that you pay attention to even the smallest cut or wound, and let your doctor know if any sign of infection begins, such as redness, swelling, tenderness or pain. These drugs may also increase your risk for developing certain types of cancer.
Each immunosuppressive drug has unique side effects. Therefore it is important that only physicians who are experienced with the use of these medications prescribe them.
- Originally developed as a chemotherapy drug (to treat cancer) and used as an immunosuppressant (to treat lupus).
- Taken in pill form previously. Today, Cytoxan is taken through the vein (intravenously, or IV).
- Shown to improve kidney and lung disease.
- Can affect a woman’s menstrual cycle.
- Can cause bladder problems, hair loss and sterility.
- Originally developed as a chemotherapy drug (to treat cancer) and used as an immunosuppressant (to treat lupus).
- Known as the “gold standard” — the best drug — for the treatment of rheumatoid arthritis.
- Shown to be very effective in treating skin lesions, arthritis and pleuritis in people with lupus.
- Can cause sun-sensitivity, liver damage, including cirrhosis and lung infections.
- Nausea, mouth sores and headaches are the most common side effects.
- If you are taking this drug you should not drink alcohol, especially if you have a history of kidney disease. If you are taking high-dose of methotrexate, you should not use NSAIDs; caution is also advised when taking aspirin.
- Developed to prevent rejection of kidney transplants.
- Blocks inflammation pathways in lupus.
- Helps to lower steroid dosage and improve liver and kidney disease.
- Can cause pancreatitis and an allergic form of hepatitis, so liver function tests and blood counts should be done regularly.
Because blood clots can be a life-threatening symptom of lupus, these drugs thin the blood to prevent it from clotting too easily. Anticoagulant medications include low-dose aspirin and prescription heparin (Calciparine®, Liquaemin®) and warfarin (Coumadin®). In particular, if you are being treated with warfarin, you must be monitored by your doctor to be sure your blood does not become too thin. Anticoagulant therapy may be lifelong in some people with lupus. Very recent research shows that people’s genetic makeup may influence how they respond to warfarin; specifically, that people with variations in two genes may need lower warfarin doses due to differences in how the body breaks down (metabolizes) warfarin and regulates the ability of warfarin to prevent blood from clotting. Therefore the dosage and administration of warfarin must be individualized for each person
Monoclonal antibodies (mAbs)
Benlysta® (belimumab, formerly called LymphoStat-B™) was developed to disrupt activation of B lymphocytes by interfering with BLyS, a protein required for B cell activity. Benlysta is the first and only drug specifically developed for and approved to treat lupus.
Repository Corticotropin Injection (H.P. Acthar Gel)
Acthar® contains a naturally occurring, highly purified hormone called ACTH, which stands for adrenocorticotropic (a-DRE-no-cor-ti-co-TRO-pic) hormone. One way Acthar is thought to work is by helping your body produce its own natural steroid hormones, such as cortisol. These hormones may assist your immune system by helping your body defend itself against inflammation.