What Is Systemic Lupus Erythematosus?

Systemic lupus erythematosus (SLE), also known as lupus, is an autoimmune disease in which the immune system mistakenly attacks healthy tissues throughout the body. With lupus, a person’s overactive immune system causes inflammation that damages connective tissues, including cartilage and the lining of blood vessels. SLE is one of several types of lupus and the most common form. 

Here is what you need to know about SLE, including prevalence, symptoms, causes and risk factors, diagnosis, treatment, and complications.

Symptoms

The symptoms of SLE vary from person to person and change over time. The most common symptoms are:

• Severe fatigue
• Joint pain and swelling
• Headaches
• Butterfly rash on the cheeks and nose
• Hair loss
• Anemia
• Problems with blood clotting
• Raynaud’s phenomenon—a vascular disease that causes pale, blue, or red color changes to the hands and feet when exposed to cold temperatures

Some of the first symptoms of SLE are extreme fatigue, malaise (a general feeling of illness), fever, weight loss, and appetite loss. Most people with SLE will go on to develop joint pain on both sides of the body and muscle pain and weakness.

Skin problems are also common in SLE, especially a flat rash called a butterfly rash that spreads across the cheeks and on the bridge of the nose. Other skin problems include:

• Calcinosis: Calcium depositions under the skin
• Vasculitis: Damaged blood vessels
• Petechiae: Tiny red spots caused by inadequate blood clotting or capillary damage, which leads to bleeding under the skin
• Alopecia: Hair loss
• Ulcerations: Sores in the moist linings of the mouth, nose, and, rarely, genitals

Some people with SLE will develop kidney problems. In fact, up to half of the people with SLE will have renal involvement. Heart problems are also common in SLE, including inflammation of the membrane around the heart (pericarditis) and heart valve problems, which control blood flow to the heart. Heart disease is more common in people with SLE.

Inflammation from SLE can also cause damage to the nervous system and lead to peripheral neuropathy (damage to the peripheral nerves causing weakness, numbness, and pain in the hands, arms, legs, and feet), seizures, stroke, and cognitive impairment. Anxiety and depression are also common in people with SLE.

People who live with SLE have periods of the disease in which their condition worsens—called flare-ups—and other times in which the condition gets better—called remissions. SLE is a progressive condition, which means it will get worse with time and potentially cause damage to major organs of the body. While there is no cure for SLE, this condition is treatable, and medications can slow down and reduce the disease’s effects.

Causes

Much like other autoimmune diseases, an exact cause of SLE is not known. However, there are several risk factors associated with the conditions.

Genes

In most people with the disease, there isn’t a single gene mutation that causes lupus. Nevertheless, lupus and other autoimmune diseases tends to run in families.

Studies on twins, siblings, and other first-degree relatives (including parents) and second-degree relatives (including aunts, uncles, and grandparents) all indicate a strong genetic component to SLE. In studies of identical twins, if one twin has lupus, the other twin has lupus roughly half of the time.

Environment

Certain environmental triggers are believed to contribute to disease development. These include ultraviolet (UV) rays from the sun, certain medications, viruses, physical and emotional stress, and trauma.

Sex Hormones

SLE affects women far more often than men. Research indicates that sex hormones like estrogen play a part in the development of SLE, although the exact mechanisms remain unclear.

Prevalence

According to the Lupus Foundation of America, up to 1.5 million Americans and up to 5 million people worldwide have some form of lupus.

Lupus tends to affect mostly women of childbearing age, but anyone can develop lupus, even children. According to the Centers for Disease Control and Prevention (CDC), women of all ages are far more affected than men—with estimates of four to 12 women for every one man.

Lupus also affects children. Children account for 20% of all cases, according to the American College of Rheumatology.

Some ethnic groups, including African Americans, Hispanics and Latinos, Asians, and American Indians, have a higher risk in comparison to Whites.

The CDC estimates there are 16,000 new diagnoses of lupus each year.

Diagnosis

Making a diagnosis of SLE can be difficult because the signs and symptoms of the disease vary from person to person over time and overlap with other disorders. Additionally, there is no one test to confirm a diagnosis of SLE.

However, your healthcare provider can perform a physical exam, ask about medical history, including signs and symptoms, and perform laboratory tests, imaging, and/or perform a biopsy.

Physical Exam and Medical History

Your healthcare provider will look for rashes and other visible signs of SLE. Tell your healthcare provider if lupus or other autoimmune diseases run in your family.

You will be asked about symptoms of SLE you may be experiencing, such as a butterfly rash, mucous membrane ulcers, joint pain, tenderness and swelling, hair loss, and signs of lung or heart involvement, such as irregular heartbeats and heart murmurs.

Laboratory Tests

This testing includes both urine and blood tests.

A complete blood count (CBC) looks for abnormalities in white blood cell, red blood cell, and platelet counts (which are often low in lupus).

An erythrocyte sedimentation rate (ESR) blood test can indicate increased inflammatory activity in the body, but an ESR test is abnormal in a number of conditions, and not just SLE.

Other blood tests assess kidneys and look for antinuclear antibodies which, if present, may indicate that an autoimmune process is at work. Further antibody tests may be performed to look for other antibodies that are more specific to SLE.

A urinalysis looks for increased protein levels or red blood cells in urine, which are notable if SLE has affected the kidneys.

Imaging

If your healthcare provider suspects SLE is affecting your heart or lungs, imaging may reveal shadows that suggest fluid on the lungs or inflammation of the lungs.

An echocardiogram uses sound waves to produce imaging of the beating heart to check for valve problems and other heart problems.

Biopsy

If lupus affects the kidneys, your healthcare provider will want to know the type, so a sample of tissue will be taken and tested to determine the best treatment course. A skin biopsy can also help confirm a diagnosis of lupus when it affects the skin.

Treatment

There is no cure for SLE, but the condition is treatable. According the Lupus Foundation of America, goals of treatment may include:

• Controlling fatigue, joint pain, and other SLE symptoms
• Reducing inflammation
• Suppressing the effects of an overactive immune system
• Prevention and treatment of flares
• Minimizing complications, especially damage to organs

Treatment options depend on how severe a person’s symptoms are and what parts of the body SLE is affecting. Treatments may include:

• Anti-inflammatory medications, such as aspirin, Advil or Motrin (ibuprofen), Aleve (naproxen sodium and naproxen), Cambia (diclofenac), and a variety of prescription medications—to treat inflammation and joint symptoms, including pain and stiffness
• Steroid creams for rashes
• Corticosteroid therapy to reduce immune system responses
• Antimalarial drugs to address joint and skin problems
• Immunosuppressive drugs and targeted immune system biologics for severe cases of SLE
• Type 1 interferon (type I IFN) receptor antagonist Saphnelo (anifrolumab) for adult patients with moderate-to-severe SLE who are receiving standard therapy

Ask your healthcare provider about what diet and lifestyle changes you can incorporate into your treatment plan. Your healthcare provider may recommend avoiding certain foods and minimizing stress to reduce flare-ups.

Complications

SLE is known for causing damage and complications to body systems. Some of these complications are:

Kidney Problems

Kidney problems are common in people with lupus. According to the National Institute of Diabetes and Digestive and Kidney Diseases, up to half of the adults with lupus will develop a kidney condition called lupus nephritis, and up to 80% of children will develop lupus nephritis.

Heart Problems

Lupus inflammation can affect the heart in several ways. In addition, people living with lupus have increased long-term risk of developing cardiovascular disease, which is a leading cause of death.

Heart problems caused by SLE may include:

• Pericarditis: Inflammation of the sac around the heart
• Atherosclerosis: Hardening of arteries that supply blood to the heart muscle
• Endocarditis: Blood clots, or heart failure—diseases resulting from damage to the heart valve
• Myocarditis: Inflammation of the heart muscle

Lung Problems

SLE causes lung inflammation in about 50% of the people with the condition.

Lung problems associated with SLE are: 

• Pleuritis: Inflammation of the two-layered membrane surrounding the lungs.
• Acute lupus pneumonitis: A serious lung condition that causes chest pain, shortness of breath, and dry cough that may bring up blood.
• Chronic (fibrotic) lupus pneumonitis: Causing similar symptoms to acute lupus pneumonitis, this condition accumulates over time. It causes chest, lung, and breathing pain, and needs ongoing treatment so it does not get worse.
• Pulmonary hypertension: A type of high blood pressure in the main vessels between the heart and lungs. Untreated, this can lead to low oxygen levels in the body (hypoxia) and heart failure.
• Shrinking lung syndrome: A rare syndrome specific to lupus where a person feels breathlessness and experiences reduced chest expansion.

Blood-Related Problems

Blood problems are common in people with SLE, but they do not always cause obvious symptoms. Some of these problems may be severe or threatening.

These may include:

• Low numbers of circulating red blood cells (anemia), which impairs oxygen delivery to the body: Severe, life-threatening anemia can happen when lupus autoimmunity destroys red blood cells (hemolytic anemia).
• Low platelet counts (thrombocytopenia): Platelets help blood to clot. Low platelet counts increase risk of serious bleeding.
• Low white blood cell counts (leukopenia): Mild leukopenia is common in people with lupus. Severe leukopenia increases risk of infections.
• Blood clots: Lupus autoimmunity may cause dangerous blood clots in the veins (pulmonary embolism) or arteries (stroke).

Nervous System Problems

Nervous system issues related to SLE may include:

• Cognitive problems: Memory loss, concentration troubles, and errors in judgment and insight
• Tension headaches and migraines
• Seizures
• Strokes
• Other neurological problems that cause visual disturbances, dizziness, muscle problems, or painful sensations in the feet, hands, arms, or legs

Digestive Problems

Digestive system problems associated with SLE may include:

• Belly pain, often with nausea and vomiting
• Liver enlargement
• Pancreatitis: Inflammation of the pancreas
• Peritonitis: Inflammation of the sac that surrounds the intestines
• Indigestion
• Problems with swallowing
• Dry mouth

Mental Health Problems

Coping with physical and emotional effects of SLE isn’t easy. The effects can lead to difficulties in managing mental health. Mental health problems associated with SLE include:

• Anxiety, depression, or both
• Psychosis: A mental health disorder leading to delusions and/or hallucinations
• Manic behavior resulting in high levels of energy and activity, problems sleeping, and irritability, resulting from SLE treatments or high levels of inflammation. This condition can go away with treatment.   

According a 2018 report from the Korean College of Rheumatology, depression affects up to 39% of people with lupus, while mania affected around 3%. Further, anxiety disorders are common in lupus (with a prevalence of 40%) and include prominent anxiety, generalized anxiety, panic disorders, and obsessive-compulsive disorders.

Lupus psychosis is less common and most often seen in the early stages of lupus. It may be more common in people who are treating with high doses of corticosteroids.

Controlling SLE with appropriate treatment can reduce and even prevent many of these potentially life-threatening disease complications.

A Word From Verywell

Systemic lupus erythematosus severity will vary from person to person. For people who have serious flares, there is a greater chance SLE can be life-threatening. However, serious complications can be avoided by following the instructions of your treating healthcare provider, taking medications as prescribed, practicing a healthy lifestyle, and not smoking, and reaching out to your healthcare provider about serious disease flares, new symptoms, and medication side effects.

And while there is no cure for SLE the outlook for many people with the disease is good. In fact, the prognosis for lupus is better than it has been in the past and many people with SLE can expect to live a normal lifespan, unaffected by serious lupus complications.