Systemic Lupus Erythematosus

Systemic lupus erythematosus is a chronic autoimmune disease where the immune system mistakenly attacks healthy tissue throughout the body. While lupus can affect nearly every organ system, advances in treatment and care mean that most people with this condition can now live long, healthy lives with proper management.

Table of contents

• What is systemic lupus erythematosus?
• Who is at risk for developing lupus?
• Symptoms and how they vary
• Parts of the body affected by lupus
• What causes lupus?
• How is lupus diagnosed?
• Treatment approaches
• Living well with lupus
• Understanding lupus flares
• Lupus and pregnancy
• Long-term outlook

What is systemic lupus erythematosus?

Systemic lupus erythematosus, commonly called SLE or simply lupus, is an autoimmune disease, which means the body’s immune system mistakenly attacks its own healthy cells and tissues. The word “systemic” indicates that the disease can affect many different parts of the body^[1].

Lupus is a chronic condition, meaning it lasts a long time and requires ongoing management. The disease causes inflammation, which is the body’s natural response to injury or infection, but in lupus this inflammation happens when it shouldn’t. This can lead to pain, swelling, and damage in various organs including the skin, joints, kidneys, brain, heart, and lungs^[1][3].

SLE is the most common form of lupus. About 7 in 10 people with lupus have this type. When people say “lupus,” they usually mean systemic lupus erythematosus^[2].

Who is at risk for developing lupus?

Anyone can develop lupus, but certain groups of people have a higher risk. Women are much more likely to develop lupus than men, with nearly 10 women affected for every 1 man. The disease most often appears in young women between the ages of 15 and 44^[1][3].

Race and ethnicity also play a role. In the United States, lupus is more common in African Americans, Asian Americans, African Caribbeans, and Hispanic Americans than in non-Hispanic white people. Native Americans, Alaska Natives, First Nations people, and Pacific Islanders also face higher risk^[1][3].

Having a family member with lupus or other autoimmune diseases increases your risk. If you have a close family member with lupus, your risk may be slightly higher than others. Studies show that identical twins have concordance rates as high as 50%, meaning if one twin has lupus, the other has a 50% chance of developing it^[4].

Symptoms and how they vary

The symptoms of lupus vary greatly from person to person. No two cases are exactly alike, and what one person experiences may be very different from another^[1][3].

The most common symptoms include joint pain and swelling. Everyone with lupus has joint pain and swelling at some time, and some develop arthritis, which is inflammation of the joints. Lupus often affects the joints of the fingers, hands, wrists, and knees^[1].

Other frequent symptoms include:

• Extreme fatigue that doesn’t go away with rest
• Fever with no other cause
• Skin rashes, particularly a butterfly-shaped rash across the cheeks and bridge of the nose
• Sensitivity to sunlight
• Hair loss
• Mouth sores
• Chest pain when taking a deep breath
• Headaches
• Swollen glands, usually in the neck, armpits, or groin
• Weight loss
• Swelling in the arms, legs, or face

^[1][3][5]

The butterfly rash, which develops in about half of people with lupus, is one of the most distinctive signs. This rash appears over the cheeks and bridge of the nose and often gets worse with sun exposure^[1][5].

Symptoms usually develop slowly. You might notice one or two signs at first, and then more or different symptoms later on. Symptoms often come and go in episodes called flares, when they worsen for a period of time, followed by remission, when symptoms improve or disappear^[3].

Parts of the body affected by lupus

Lupus can affect nearly every organ system in the body. The specific symptoms depend on which parts are affected^[1].

When lupus affects the brain and nervous system, it can cause headaches, weakness, numbness, tingling, seizures, vision problems, and memory or personality changes. The digestive tract may be affected, leading to abdominal pain, nausea, and vomiting^[1].

Heart involvement can include valve problems, inflammation of the heart muscle, or inflammation of the sac around the heart called the pericardium. In the lungs, lupus may cause buildup of fluid in the pleural space (the area around the lungs), difficulty breathing, or coughing up blood^[1].

Kidney problems are particularly important to watch for. Lupus can cause swelling in the legs and changes in urine. This condition is called lupus nephritis, and it can affect up to 60 percent of people with lupus. For most people with lupus nephritis, symptoms develop within 5 years of when their lupus symptoms first started^[1][2].

Blood problems are also common and may include anemia (low red blood cell count), low white blood cell count, or low platelet count. Some people develop blood clots in veins or arteries, inflammation of blood vessels, or Raynaud phenomenon, where arteries constrict in response to cold or stress, causing fingers and toes to turn white or blue^[1].

What causes lupus?

The exact cause of lupus is not clearly known. Experts believe that lupus develops in response to a combination of factors both inside and outside the body^[1][2].

Genetic factors play a role. More than 100 gene locations with variations have been identified that are associated with lupus. These genes are involved in how the immune system responds to foreign substances and how it distinguishes between the body’s own tissues and outside threats. However, having these genetic variations doesn’t guarantee you will develop lupus^[4].

Hormones may influence lupus development. Reactions to certain hormones in your body, especially estrogen, may make you more likely to develop lupus. This may explain why the disease is so much more common in women^[1][3].

Environmental factors can trigger lupus in people who are genetically susceptible. These include viral infections, certain medicines, sunlight exposure, and exposure to toxins like trichloroethylene in well water or silica dust^[1][4].

Other aspects of your health history may also play a role. Smoking, stress levels, and having certain other health conditions like other autoimmune diseases might trigger lupus^[3].

How is lupus diagnosed?

Diagnosing lupus can be challenging because its symptoms often mimic those of other diseases. There is no single test that can diagnose lupus. Instead, doctors use a combination of your symptoms, physical examination findings, and laboratory test results^[1][5].

A rheumatologist, a doctor who specializes in diseases affecting the joints, muscles, and connective tissues, is usually the specialist who diagnoses lupus^[7].

The first screening test is usually the antinuclear antibody (ANA) test. Nearly all people with lupus have a positive ANA test. However, having a positive ANA alone does not mean you have lupus, as many people without the disease also test positive^[1][11].

Other blood tests may include:

• Complete blood count to check for anemia and other blood cell problems
• Antibodies to double-stranded DNA
• Complement components (C3 and C4)
• Erythrocyte sedimentation rate (ESR) to measure inflammation
• C-reactive protein (CRP) to detect inflammation
• Kidney and liver function tests
• Antiphospholipid antibodies

^[1][11]

Your doctor will also perform a complete physical exam and may order additional tests such as chest x-rays, urinalysis to check for kidney involvement, or imaging tests of affected organs^[1].

Classification criteria have been developed to help diagnose lupus. The newest criteria, proposed by the European League Against Rheumatism and the American College of Rheumatology in 2019, require a positive ANA test as an entry criterion, followed by evaluation of 22 weighted clinical features across seven domains^[6][12].

Treatment approaches

There is no cure for lupus, but treatment can control symptoms and help people live normal, healthy lives. The goal of treatment is to achieve complete remission or low disease activity, prevent flares, minimize the use of steroids when possible, and improve quality of life^[1][12].

Hydroxychloroquine, a medicine also used to treat malaria, is recommended for all patients with lupus unless there is a specific reason they cannot take it. This medication helps prevent flares, organ damage, blood clots, and increases long-term survival. It is considered the cornerstone of lupus treatment^[12][15].

For mild symptoms, treatment may include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) for joint symptoms and inflammation
• Low doses of corticosteroids, such as prednisone, for skin and arthritis symptoms
• Corticosteroid creams for skin rashes

^[1]

More severe disease involving the kidneys, heart, lungs, or nervous system often requires stronger immunosuppressive medications. These work by suppressing the overactive immune system and may include drugs like azathioprine, mycophenolate, or cyclophosphamide^[1].

Newer biological therapies have been developed that target specific parts of the immune system. Belimumab was the first drug approved specifically for lupus in over 50 years. More recently, anifrolumab and voclosporin were approved in 2021 for managing lupus^[13][12].

Treatment is individualized based on which organs are affected, how active the disease is, and how you respond to medications. Patients with active disease should be evaluated at least every one to three months^[12].

Living well with lupus

Taking care of yourself and making healthy lifestyle choices can help you manage lupus symptoms, prevent flares, and improve your quality of life^[7].

Taking your medicines exactly as prescribed is the most important step. Let your doctor know immediately if you think you are not tolerating treatments or if you are having trouble following your medication schedule^[18].

Getting plenty of rest is essential. Fatigue is one of the most common lupus symptoms. Aim for 7 to 9 hours of sleep each night and take short breaks from your activities when needed^[21][22].

Protecting your skin from the sun is critical because UV rays can trigger lupus flares. Use sunscreen with SPF 50 or higher, wear hats and protective clothing, and limit sun exposure, especially when rays are strongest. Reapply sunscreen after swimming, sweating, or toweling off^[1][22].

Managing stress through relaxation techniques like meditation, deep breathing, yoga, or engaging in activities you enjoy can help prevent flares. Stress is a known trigger for symptom worsening^[21][22].

Staying physically active is beneficial. A daily walk or swim can lower stress, improve mood, and help fight tiredness. Talk with your doctor about what types and amounts of exercise are appropriate for you^[21][22].

Eating a healthy, balanced diet that includes whole grains, fruits, vegetables, and lean proteins provides the nutrients you need. Some research suggests that foods with anti-inflammatory properties, such as fish, nuts, and leafy greens, may help reduce inflammation. Foods containing turmeric, ginger, and omega-3 fatty acids may be particularly helpful^[18][22].

Avoiding smoking is essential. Smoking can trigger flares, increase their severity, and worsen lupus symptoms overall^[21][22].

Building a support system of family, friends, healthcare providers, and others with lupus can provide emotional support and practical help. Support groups, either online or in person, can connect you with people who understand what you’re going through^[18].

Understanding lupus flares

Lupus symptoms often come and go in patterns called flares. During a flare, your symptoms get worse and you feel sick. Between flares, you may have periods of remission when you have few or no symptoms^[3][21].

Flares can happen without clear warning, but many people learn to recognize the signs that a flare is coming. You may feel more tired than usual, develop a fever, notice new rashes, or experience worsening pain^[21].

Common triggers for flares include:

• Overwork and not getting enough rest
• Stress
• Sun and UV light exposure
• Fluorescent and halogen light
• Infection
• Injury
• Stopping lupus medications
• Certain other medications

^[21][22]

Learning what triggers your flares and taking steps to avoid those triggers can help reduce how often they occur and how severe they are. Keep track of what you were doing or experiencing when flares developed to identify your personal patterns^[18].

Lupus and pregnancy

Many women with lupus give birth to healthy children, but pregnancy requires careful planning and monitoring. The key to a successful pregnancy is knowing how lupus affects your body and working closely with your healthcare team^[18].

Pregnant patients with lupus have an increased risk of spontaneous abortions, stillbirths, preeclampsia, and fetal growth restriction. Preconception counseling about these risks, planning the timing of pregnancy, and a multidisciplinary approach are essential^[12].

It’s important to see your doctor before trying to get pregnant to discuss the risks and check if your medications need to be changed. Some lupus medications are not safe during pregnancy, while others can be continued^[9].

Hydroxychloroquine should be continued during pregnancy because it helps control disease activity and may reduce pregnancy complications, including preeclampsia and cardiac problems in the baby^[12].

Long-term outlook

The outlook for people with lupus has improved dramatically over the past several decades. With early diagnosis and appropriate treatment, more than 90% of people with lupus can live normal, healthy lives. Ten-year survival rates now exceed 90%, compared to just 53% at five years in 1955^[7][20].

While lupus is a chronic disease that requires ongoing management, many people experience long periods of remission with minimal symptoms. The disease can range from mild to severe, and with proper care, even those with more serious involvement can manage their condition effectively^[1].

Regular monitoring and follow-up care are important. People with active disease should be evaluated regularly, with blood pressure checks, blood tests, urinalysis, and monitoring of kidney and liver function. This helps catch problems early and adjust treatment as needed^[12].

Certain factors can worsen lupus outcomes, including not having access to healthcare, delays in diagnosis, and not following a treatment plan. Early intervention and adherence to treatment significantly improve long-term outcomes^[7].

With ongoing research into new treatments, including advances in biological therapies and targeted medications, the future for people with lupus continues to improve. Staying informed, working closely with your healthcare team, and taking an active role in your care are the best ways to ensure the best possible outcome^[13][17].