Systemic lupus erythematosus (SLE) is a chronic (long-term) autoimmune disease where the body’s immune system attacks healthy tissues in many parts of the body including the joints, skin, and other organs. SLE is also referred to as “lupus” but it is important to understand that there are other types of “lupus”.
SLE causes a wide range of symptoms that can be very different from person to person. It is called the disease of 1000 faces as it can happen 1000 different ways in 1000 different people. No two people with SLE are the same! Symptoms can come and go and can range in severity from mild to severe. It is a tricky disease to diagnose because it can mimic the symptoms of other illnesses, and because of how symptoms can unpredictably change.
Among all of the possible symptoms, the most common features of SLE are feeling tired and pain. These can occur when the body’s immune system is “activated” and is busy attacking the body’s tissues. Another common symptom is a type of rash on the face called a malar rash or butterfly rash, which will occur in about half of people with the disease.
The reason that the immune system attacks the body’s tissues in SLE is not well understood. Experts believe that a wide number of factors including the environment and genetic factors may involved.
SLE mostly affects women of childbearing age. Women get it about nine times more often than men. It is estimated that about 1 in 1,000 people have SLE.
SLE causes a wide range of symptoms because of how the body’s immune system is attacking healthy tissues in many parts of the body including the joints, skin, and other organs. The specific symptoms, their severity, what parts of the body get attacked, and how symptoms come and go can vary greatly between two different people with SLE.
Even though are a huge spectrum of symptoms in lupus, it’s very common for patients to experience a relatively consistent personal pattern in terms of which symptoms might appear during a flare, and what things might trigger their flare.
The first thing people with lupus usually notice is fatigue or extreme tiredness. Another very common symptom is pain. This can either affect the joints (with or without swelling), or it can affect the whole body.
People with Lupus might feel some (but rarely all) of the following symptoms:
- Fatigue (tiredness)
- Different types of skin rashes that may be triggered by sunlight
- A particular type of red skin rash that appears on the face called a malar rash or butterfly rash may occur in about half of people who get lupus
- Joint aches – also known as arthralgia
- Joint swelling – also known as arthritis
- Swelling in the legs or eyes
- Hair loss
- Raynaud’s phenomenon – fingers and toes that turn white or blue in the cold
- Mouth ulcers or canker sores
- Swollen lymph nodes (glands)
- Pain in the chest when taking a deep breath in (this is called pleurisy)
- Muscle pain
Symptoms can sometimes be very serious when the immune system severely attacks vital parts of the body. This can include organs such as the kidneys, lungs, heart, or brain; or important cells like blood cells.
Lupus tends to be the worst in the first few years (2-5 years). It’s important for patients to minimize damage to their body as much as possible during this period.
Systemic Lupus Erythematosus (SLE) can be properly diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease, or another specialist who knows a lot about lupus. Lupus can be tricky to diagnose and having someone experienced is very useful.
To diagnose SLE, physicians will take a careful and thorough history. They will ask tons of questions tons of questions because lupus can have so many symptoms. Next, they will perform a complete physical examination including the head and neck, lungs, heart, abdomen, joints, and skin.
Finally, special blood tests are ordered to see the patient’s body is making antibodies to itself.
Looking for antibodies that attack the body: the anti-nuclear antibody (ANA) test is the most important blood test to do first if lupus is suspected. This test helps determine if the body is making too many antibodies to itself (a small amount is normal) and if they are attacking unusual places. If this test comes back negative in a patient, then it’s reasonable to conclude that they don’t have lupus. If it comes back positive, then more tests should be run to confirm lupus because a positive ANA test is possible both in healthy people and in people with other diseases.
Looking for antibodies that attack DNA: the Anti-Double Stranded DNA (anti-dsDNA) test measures antibodies that attack DNA, a special protein that is the “building block of life” and contains genetic code (genes). This is a very specific test for lupus and if it is very positive there is a good chance the diagnosis is lupus..
Looking for antibodies that attack certain proteins: The Extractable Nuclear Antigen (ENA) Panel is a test that measures antibodies to 6 or 7 other proteins in the body. The antibodies in this panel may include: anti-Ro (also called anti-SSA), anti-La (also called anti-SSB), anti-Sm, anti-RNP, anti-Jo-1, anti-Scl70, and anti-centromere. Things get a bit more tricky here and it’s best for patients to discuss this test and its results with a rheumatologist.
Looking for inflammation: Sjoogren’s Syndrome causes inflammation in the body so tests for inflammation are expected to have abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).
Looking at kidney function: Creatinine is a common blood test that looks at how well the kidneys are working. Abnormally high levels of creatinine can indicate a problem and can mean that the kidneys are involved.
Looking for kidney involvement: urinalysis testing to detect protein or blood in the urine can give a sign that the kidneys might be involved.
Looking for lung involvement: Chest x-ray and/or CT scans can help confirm if the lungs are involved.
Looking for nerve involvement: EMG or Nerve Conduction Studies can be performed to look for nerve involvement.
Lupus is an autoimmune disease that occurs when the body’s immune system is “turned on” and begins to attack itself. When the immune system is turned on it makes you feel tired and achy. The best way to think of it is like you have the flu 24 hours a day 7 days a week. Except with lupus, you’re not fighting the flu.
Lupus causes the immune system to attack its own tissues. This attack produces inflammation. The word inflammation comes from the Latin word inflammare which means to light on fire. The tissues in the body are essentially “on fire”. In people with lupus, inflammation is extremely variable and can occur in almost any tissue of the body. The “fire” doesn’t have to be that hot – it can be a smouldering fire that slowly causes damage.
There are many medications that can be used to treat lupus. The choice of medications will depend on a person’s specific symptoms.
The goals of treatment are:
- Prevent flares
- Treat flares when they occur
- Reduce organ and tissue damage, and other problems.
One of the best things someone with lupus can do to help with treatment is to learn their lupus. Even though the disease can present a huge spectrum of symptoms, it usually shows a consistent pattern in a given person, and flares tend to follow predictable patterns. Once someone with lupus gets an understanding of their disease, it tends to keep doing the same thing.
Our advice to patients is: your lupus is your lupus. Learn what your lupus looks like and what triggers your flares.
Lupus tends to be the worst in the first few years (2-5 years). It’s important to get through this time with as little damage to the body as possible.
Treating the Symptoms of Lupus
Fatigue is one of the most common symptoms in people with lupus. It’s also one of the most difficult symptoms to treat. It can go a long way for someone with lupus to learn how to balance the demands in their life with their need to rest. Medications such as prednisone can be helpful but can also have side effects, especially in high doses or when it’s used for a long time.
Treating Swollen and Inflamed Joints
Swollen and inflamed joints may be treated with the same types of medications that are used to treat rheumatoid arthritis. These include Disease-Modifying Anti-Rheumatic Drugs (DMARDs) like methotrexate or Plaquenil (hydroxychloroquine). For joints that ache but are not swollen, analgesics and/or Plaquenil (hydroxychloroquine) might help.
Treating Chronic Pain
Chronic pain can be treated with a variety of medications – if one doesn’t work for you, try another.
Treating Skin Rash
Skin rash can be treated with topical medications applied directly to the skin. Other times, medications taken by mouth such as Plaquenil (hydroxychloroquine), Aralen (chloroquine), Imuran (azathioprine), and Cellcept (mycophenolate) can be useful. Prednisone can also be helpful to get skin rashes under control.
Treating Hair Loss
Hair loss is difficult to treat. The best solution is to keep lupus under control to prevent flares in the first place. Some medicines can also cause hair loss.
Treating Mouth Ulcers
Like skin rash, mouth ulcers are treated by directly applying a medication to the sore. Sometimes prednisone can help.
Treating Immune System Attacks Against the Organs
When lupus affects the organs, treatment with DMARDs or biologics and high doses of prednisone may be needed to quickly get the inflammation under control.
Medications Used to Treat Lupus
Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)
NSAIDs help the pain and swelling of the joints caused by lupus. There are about 20 different anti-inflammatory medications available, so if one doesn’t work for a person, their doctor can give them another one to try.
Prednisone is a very effective medication that can help control inflammation, and helps control many of the symptoms of lupus including fatigue, arthritis, skin rash, and mouth sores. High doses are typically used when any organs are involved.
In the short-term, prednisone works very well to rapidly control symptoms. When used for long periods of time, prednisone can have side effects. People with lupus should to discuss the risks and benefits of using prednisone with their rheumatologist.
Some people with lupus also benefit from cortisone injections directly into their affected joints. This treatment option should be discussed with a rheumatologist.
Analgesics (Pain Relievers)
Analgesic medications only control pain. They do nothing to control lupus or to prevent damage to tissues such as the joints. Analgesics can range from simple things like acetaminophen (paracetamol, Tylenol) to more potent narcotics like morphine.
Disease Modifying Anti-Rheumatic Drugs (DMARDs)
DMARDs are medications that control symptoms and prevent long-term damage. Examples include methotrexate, Plaquenil (hydroxychloroquine), Aralen (chloroquine), Arava (leflunomide), Imuran (azathioprine), Cellcept (mycophenolate mofetil) or Myfortic (mycophenolic acid), and Cytoxan (cyclophosphamide).
DMARDs have been around for a long time and can work very well to control symptoms such as inflamed joints. They may be used in combination with prednisone when organs are involved.
In some people, DMARDs such as Plaquenil (hydroxychloroquine), Aralen (chloroquine), or Imuran (azathioprine) can be used to treat joint pain, skin rashes, and fatigue. Methotrexate and Arava (leflunomide) can be used to treat arthritis.
When lupus is severe, powerful medications like Cytoxan (cyclophosphamide) can be very effective in controlling the disease.
Most DMARDs take some time before they begin to work. Some people might not feel any effect when they first start taking them. It’s important for people taking DMARDs to be patient and keep taking the medication as prescribed, to help get the inflammation of lupus under control.
Biologics are a newer class of medications that are often very effective, and can make a big difference for people living with lupus. Like DMARDs, biologics control symptoms and prevent long-term joint damage.
The types of biologics used to treat lupus target a type of white blood cell that plays a role in the body’s immune system called the B-cell. Two biologics are used to treat lupus: Rituxan (rituximab) and Benlysta (belimumab). If one doesn’t work for a person, their rheumatologist may suggest trying the other.
Biologics are often given in combination with DMARDs or prednisone, because combination therapy has been shown to work better than treatment with either medication alone. Biologics can also help reduce the dose of prednisone that is used over the long-term.
Chronic Pain Medications
Several different types of medication are available for the relief of chronic pain. Low doses of Elavil (amitriptyline), Cymbalta (duloxetine), Lyrica (pregabalin), gabapentin, and cyclobenzaprine are a few examples. With so many chronic pain medications to choose from, if one doesn’t work for someone with lupus, their rheumatologist can recommend another one to try.
A drug called intravenous immunoglobulin (IVIG) is sometimes used to treat lupus. It doesn’t work in everyone, but when it does work in a given person, it can work extremely well.
Living with SLE
The pain and stiffness caused by SLE can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of SLE on their work and daily routine.
We recommend adjusting features of the workplace to help make working with SLE easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have SLE while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with SLE can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with SLE.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for SLE, and they can interact with many medications.
People with SLE that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with SLE. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like SLE already increases this risk without a big extra boost from smoking.
Travel is still possible when you have SLE.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though SLE does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with SLE can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Systemic Lupus Erythematosus (SLE) has no effect on fertility. The chances of getting pregnant for people with SLE are the same as with people that don’t have it. SLE also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with SLE and pregnancy are:
Patients with SLE might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with SLE who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause SLE to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with SLE. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of SLE or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help SLE in any significant way.
People with SLE should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with SLE to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Systemic Lupus Erythematosus is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.