Systemic lupus erythematosus (SLE)

What is Systemic lupus erythematosus (SLE)?

→ Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology. 

→ SLE is occurring predominantly in young women. 

→ Common manifestations may include arthralgias (joint pain) and arthritis (joint inflammation), Raynaud syndrome (numb & cold fingers/toes), malar and other rashes, pleuritis or pericarditis (chest pains), renal (related to kidneys) or central nervous system involvement, and autoimmune cytopenias (reduced blood cell levels). 

→ Diagnosis requires clinical and serologic criteria. 

→ Treatment of severe, ongoing, active disease requires corticosteroids and immunosuppressants.

Systemic lupus erythematosus (lupus) is a chronic (long-lasting) autoimmune disease that can affect many parts of the body. Lupus occurs when the immune system, which normally helps protect the body from infection and disease, attacks its own tissues. This attack causes inflammation, and in some cases permanent tissue damage, which can be widespread – affecting the skin, joints, heart, lung, kidneys, circulating blood cells, and brain.

If you have lupus, you may experience periods of illness (flares) and periods of wellness (remission). Lupus flares can be mild to serious,and they are unpredictable. However, with treatment, many people with lupus can manage the disease.

What causes SLE?

SLE is more common in women than men by nearly 10 to 1. It may occur at any age. However, it appears most often in young women between the ages of 15 and 44. In the US, the disease is more common in African Americans, Asian Americans, African Caribbeans, and Hispanic Americans.

The cause of lupus is unknown, and researchers are still trying to learn what may trigger or lead to the disease. Doctors know that it is a complex autoimmune disease in which the body’s immune system attacks the person’s tissues and organs. Studies show that certain factors may trigger your immune system, causing the disease. These factors include:

→ Genetic

Research shows that certain genes play a role in the development of lupus. The different forms of these genes carry instructions for proteins that may affect the immune system. Researchers are studying how high levels or low levels of these proteins may be important in the development of the disease.  

→ Environmental

Exposure to certain factors in the environment – such as viral infections, sunlight, certain medications, and smoking – may trigger lupus.

→ Immune and Inflammatory Influences

Researchers think that if the body does not remove damaged or dead cells normally, this could trick the immune system into constantly fighting against itself. This process could cause an autoimmune response, which could lead to lupus. In addition, researchers are studying different cell types and how changes could lead to lupus.  

→ Hormonal

→ Certain medicines

Other risk factors:

Anyone can develop lupus, but some groups of people have a higher risk:

→ People assigned female at birth (AFAB), especially people AFAB between the ages of 15 and 44.

→ Black people.

→ Hispanic people.

→ Asian people.

→ Native Americans, Alaska Natives and First Nations people.

→ Pacific Islanders.

→ People with a biological parent who has lupus.

What are symptoms of SLE?

The symptoms of lupus vary from person to person and can range from mild to severe. You may have just a few symptoms affecting just one area of your body, or you could have many symptoms throughout your body. Symptoms may come and go, and you may develop new symptoms over time. Some symptoms happen when the disease causes inflammation in organs, such as the joints, skin, kidneys, lining of the heart and lungs, brain, and blood cells. Symptoms of lupus can include:

→ Arthritis, causing painful and swollen joints and morning stiffness.

→ Fevers.

→ Fatigue or feeling tired often.

→ A rash that appears on the face across the nose and cheeks; this is called a malar or “butterfly” rash

→ Round scaly rashes that can appear anywhere on the body.

→ Sensitivity to the sun that may cause a rash.

→ Hair loss.

→ Sores, which are usually painless, in the nose and mouth (most often on the roof of the mouth).

→ Change of color in the fingers and toes – blue-purplish, white, or red – from cold and stress (Raynaud’s phenomenon).

→ Swollen glands.

→ Swelling in the legs or around the eyes.

→ Pain when breathing deeply or lying down, from inflammation of the lining around the lungs or heart.

→ Headaches, dizziness, depression, confusion, or seizures.

→ Abdominal pain.

Lupus causes inflammation throughout the body, which can cause problems in organs, including:

→ Kidney damage that can lead to changes in kidney function, including kidney failure. This is called lupus nephritis.

→ Seizures and memory problems due to changes in the brain and central nervous system.

Heart problems:

→ Heart valve damage due to inflammation that leads to scarring.

→ Inflammation of the lining around the heart muscle, called pericarditis.

→ Inflammation of the heart muscle itself, called myocarditis

→ Inflammation of blood vessels, called vasculitis.

→ Blood clots due to high levels of certain autoantibodies referred to as antiphospholipid antibodies.

→ Low blood cell counts, including red blood cells, white blood cells, and platelets.

→ Inflammation of the tissue that surrounds the lungs, making it painful to breathe. This is called pleurisy.

Some people with lupus may be more likely to develop other conditions, such as cardiovascular disease due to inflammation of the heart and blood vessel tissues caused by lupus, which can lead to:

→ Atherosclerosis, which happens when fat and other materials attach to the blood vessel wall and form plaque. This can happen in blood vessels throughout the body.

→ Coronary artery disease, which happens when plaque builds up in the arteries that supply blood to the heart. This can interrupt blood flow when a blood clot forms or a piece of plaque breaks off, causing a heart attack.

Symptoms vary from person to person, and may come and go. Everyone with SLE has joint pain and swelling at some time. Some develop arthritis. SLE often affects the joints of the fingers, hands, wrists, and knees.

Other common symptoms include:

→ Chest pain when taking a deep breath.

→ Fatigue.

→ Fever with no other cause.

→ General discomfort, uneasiness, or ill feeling (malaise).

→ Hair loss.

→ Weight loss.

→ Mouth sores.

→ Sensitivity to sunlight.

→ Skin rash -- A "butterfly" rash develops in about half the people with SLE. The rash is mostly seen over the cheeks and bridge of the nose. It can be widespread. It gets worse in sunlight.

→ Swollen lymph nodes.

Other symptoms and signs depend on which part of the body is affected:

→ Brain and nervous system: Headaches, weakness, numbness, tingling, seizures, vision problems, memory and personality changes

→ Digestive tract: Abdominal pain, nausea, and vomiting

→ Heart: Valve problems, inflammation of heart muscle or heart lining (pericardium)

→ Lung: Buildup of fluid in the pleural space, difficulty breathing, coughing up blood

→ Skin: Sores in the mouth

→ Kidney: Swelling in the legs

→ Circulation: Clots in veins or arteries, inflammation of blood vessels, constriction of blood vessels in response to cold (Raynaud phenomenon)

→ Blood abnormalities including anemia, low white blood cell or platelet count

→ Some people have only skin symptoms. This is called discoid lupus.

What are the four different kinds of lupus?

1. Systemic lupus erythematosus (SLE): The most common form of lupus—it’s what most people mean when they refer to “lupus.”
2. Cutaneous lupus erythematosus: A form of lupus that is limited to the skin.
3. Drug-induced lupus erythematosus: A lupus-like disease caused by certain prescription drugs.
4. Neonatal lupus: A rare condition that affects infants of women who have lupus.

How is lupus diagnosed?

A healthcare provider will diagnose lupus with a physical exam and some tests. They’ll examine your symptoms and talk to you about what you’re experiencing. Tell your provider when you first noticed symptoms or changes in your body. Your provider will ask about your medical history, including conditions you may have now and how you’re treating or managing them.

Lupus can be tricky to diagnose because it can affect so many parts of your body and cause lots of different symptoms. Even small changes or issues that seem unusual for you can be a key. Don’t be afraid to tell your provider about anything you’ve felt or sensed — you know your body better than anyone.

Which tests do providers use to diagnose lupus?

There’s not one test that can confirm a lupus diagnosis. Diagnosing it is usually part of a differential diagnosis. This means your provider will probably use a few tests to determine what’s causing your symptoms before ruling out other conditions and diagnosing you with lupus.

Laboratory studies used in the diagnosis of SLE are as follows:

Blood tests to see how well your immune system is working and to check for infections or other issues like anemia or low blood cell counts. Although blood test results can help doctors diagnose lupus, they alone cannot confirm a definite diagnosis of lupus because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders. Blood tests can also indicate a low red blood cell count (anemia), a low white blood cell count, or a low platelet count. People who have anemia undergo a direct Coombs test. This test is used to detect increased amounts of certain antibodies that are attached to the surface of red blood cells and can destroy red blood cells, causing anemia.

→ CBC with differential

→ Serum creatinine

→ ESR or CRP level

Additional laboratory tests are done to detect the presence of protein or red blood cells in the urine (urinalysis) or an elevation of creatinine in the blood. These findings indicate kidney inflammation of the filtering structure in the kidneys (glomeruli), a condition referred to as glomerulonephritis. Sometimes a kidney biopsy (removal of tissue for examination and testing) is done to help the doctor plan treatment. People who have lupus should be tested frequently for kidney damage even if they have no symptoms.

→ Liver function tests

→ Creatine kinase assay

→ Spot protein/spot creatinine ratio

Other blood tests, such as measuring the level of complement proteins (proteins with various immune functions, such as killing bacteria), are also done and can help predict the activity and course of the disease in some people.

→ Complement levels

An antinuclear antibody (ANA) test looks for antibodies (protein markers that show a history of your body fighting off infections). People who have lupus usually have certain antibodies that show their immune system has been overly active. A blood test can detect antinuclear antibodies (ANA), which are present in almost all people who have lupus. However, these antibodies also occur in other diseases. Therefore, if antinuclear antibodies are detected, a test for antibodies to double-stranded DNA as well as a test for other autoimmune antibodies (autoantibodies) are done. A high level of these antibodies to DNA strongly supports the diagnosis of lupus, but not all people who have lupus have these antibodies.

→ Antinuclear antibody (ANA) test

Urinalysis to check your pee for signs of infections or other health conditions.

→ Urinalysis with microscopy

The following imaging studies may be used to evaluate patients with suspected SLE:

→ Joint radiography

→ Chest radiography and chest CT scanning

→ Echocardiography

→ Brain MRI/MRA

→ Cardiac MRI

Procedures that may be performed in patients with suspected SLE include the following:

→ Arthrocentesis (a procedure where a doctor uses a needle to take fluid out of a joint.)

→ Lumbar puncture (your healthcare provider inserts a hollow needle into the space surrounding the spinal column (subarachnoid space) in the lower back to withdraw some cerebrospinal fluid (CSF))

→ Kidney biopsy (a biopsy is taken using a very thin needle that is inserted into the kidney tissue.)

A biopsy of your skin or kidney tissue can show if your immune system has damaged them.

Women with lupus who have repeated miscarriages or have had problems with blood clots should be tested for antiphospholipid antibodies. This is an important test when planning contraceptive methods or pregnancy. This blood test, which detects antibodies to phospholipids, can also help identify people at risk of repeated blood clots. Women with positive antibodies to phospholipids should not take estrogen-containing oral contraceptives and should choose other methods of contraception.

How is lupus treated?

Your healthcare provider will suggest treatments for lupus that manage your symptoms. The goal is minimizing damage to your organs and how much lupus affects your day-to-day life. Most people with lupus need a combination of medications to help them prevent flare-ups and lessen their symptom severity during one. 

You might need:

Hydroxychloroquine: Hydroxychloroquine is a prescription antiviral medication that can relieve lupus symptoms and slow down how they progress (change or get worse).

Nonsteroidal anti-inflammatory drugs (NSAIDs): Over-the-counter (OTC) NSAIDs relieve pain and reduce inflammation. Your provider will tell you which type of NSAID will work best for you, and how often you should take it. Don’t take NSAIDs for more than 10 days in a row without talking to your provider.

Corticosteroids: Corticosteroids are prescription medications that reduce inflammation. Your provider might prescribe you pills you take by mouth or inject a corticosteroid directly into one of your joints.

Immunosuppressants: Immunosuppressants are medications that hold back your immune system and stop it from being as active. They can help prevent tissue damage and inflammation.

You might need other medications or treatments to manage specific lupus symptoms you have or other health conditions it’s causing. For example, you may need treatment for anemia, high blood pressure (hypertension) or osteoporosis if lupus causes those issues.

Can you prevent lupus?

You cannot prevent lupus because experts are not sure what causes it. Talk to a healthcare provider about your risk if one of your biological parents has lupus.

How can you prevent lupus flare-ups?

You might be able to prevent and reduce lupus flare-ups by avoiding activities that trigger your symptoms, including:

Avoiding sun exposure: Spending too much time in the sun can trigger lupus symptoms in some people. Try to avoid going outside when the sun is brightest (usually between 10 a.m. and 4 p.m.). Wear long sleeves, a hat or sun-protective clothing. Use a sunscreen that’s at least SPF 50.

Staying active: Joint pain can make it hard or painful to move. But moving and gently using your joints can be the best way to relieve symptoms like pain and stiffness. Walking, biking, swimming, yoga and tai chi are all great ways to move your body without putting too much stress on your joints. Ask your healthcare provider which types of activities are safest for you.

Getting enough sleep and protecting your mental health: Living with lupus can be frustrating. Getting the right amount of sleep (seven to nine hours for adults) and reducing your stress can help prevent flare-ups for some people. A psychologist or other mental health professional can help you develop healthy coping mechanisms.