What are systemic lupus erythematosus (SLE) and other types of lupus?

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Lupus refers to a range of conditions that can cause symptoms throughout the body. It is an autoimmune condition, which means it happens when the body’s immune system mistakenly attacks healthy body tissue. Symptoms can range from mild to life-threatening.

Systemic lupus erythematosus (SLE) is the most common type, and people often use the term lupus to refer to SLE. However, there are other types, depending on which part of the body lupus mainly affects.

In addition to SLE, there are cutaneous lupus (such as discoid lupus erythematosus [DLE]), drug-induced lupus (DIL), and neonatal lupus.

Systemic lupus erythematosus

SLE accounts for 70% of lupus cases. It affects multiple organs and systems throughout the body. For this reason, SLE tends to be a more severe form of lupus. The symptoms can range from mild to severe.

SLE can cause inflammation in the:

• skin
• joints
• lungs
• kidneys
• blood
• heart

This inflammation may happen in one of these areas or affect multiple areas at one time.

Discoid lupus erythematosus

In DLE, a type of cutaneous lupus, symptoms affect only the skin. DLE appears as circular lesions, typically on the scalp and face, although they can appear on other parts of the body, such as inside the ears.

The lesions tend to be red and may become thick and scaly. In some cases, the lesions lead to scarring and skin discoloration. If lesions scar on the scalp, hair may not regrow in that area.

DLE does not affect the internal organs, but around 10% of people with DLE go on to develop SLE, according to the Lupus Foundation of America. However, it is possible that these people already had SLE and their skin symptoms led to a DLE diagnosis first.

Subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body that have exposure to the sun. These lesions do not cause scarring but may cause skin discoloration.

Drug-induced lupus

Drug-induced lupus (DIL) results from the long-term use of certain medications. The symptoms tend to be similar to those in SLE but are often less severe.

Over 100 medications have been identified as potential causes of DIL, including:

• hydralazine, a hypertension medication
• procainamide, a heart arrhythmia medication
• isoniazid, an antibiotic used to treat tuberculosis
• minocycline, an antibiotic used to treat some skin conditions

DIL typically goes away within 6 months of stopping the medication.

Neonatal lupus

Neonatal lupus can affect newborns if their birth parent passes on antibodies for lupus or Sjögren’s syndrome through the placenta during pregnancy.

If the birth parent has these antibodies — whether or not they have lupus — there is a 2% an infant will develop neonatal lupus.

At birth, they may have:

• a skin rash
• liver problems
• low blood counts

The skin symptoms usually go away within a few weeks, but some infants may have a congenital heart block, in which the heart cannot regulate a normal and rhythmic pumping action. This is a more serious complication, and the infant may need a pacemaker.

The symptoms of lupus will depend to some extent on the type of lupus.

American College of Rheumatology lists 11 criteria for describing lupus. If a person meets four or more of the criteria, they will consider a diagnosis of lupus.

The 11 criteria are:

1. Malar rash: A malar rash is a butterfly-shaped rash appears across the cheeks and nose.
2. Discoid rash: Raised red patches develop on the skin.
3. Photosensitivity: A skin rash appears after exposure to sunlight.
4. Oral or nose ulcers: Ulcers appear in a person’s mouth or nose.
5. Nonerosive arthritis: This type of arthritis does not destroy the bones around the joints but does cause tenderness and swelling.
6. Pericarditis or pleuritis: Inflammation affects the lining around the heart (pericarditis) or lungs (pleuritis).
7. Kidney disorder: Tests show high levels of protein or cellular casts in the urine if a person has a kidney problem, such as lupus nephritis.
8. Neurologic disorder: A person may experience seizures, psychosis, or problems with thinking and reasoning.
9. Hematologic (blood) disorder: Blood may show a low red blood cell count (anemia), a low white blood cell count (leukopenia), or a low platelet count (thrombocytopenia).
10. Immunologic disorder: Tests show that there are anti-double-stranded DNA antibodies, anti-Smith antibodies, or antiphospholipid antibodies (APLs).
11. Positive ANA: A test detects high levels of ANA.

The symptoms of lupus occur in times of flare-ups. Between flare-ups, people usually experience times of remission, when there are few or no symptoms.

A person with lupus may also experience:

• fatigue
• muscle and joint pain
• fever
• chest pain when breathing deeply
• sensitivity to sunlight
• mouth ulcers
• memory problems
• headaches
• unusual hair loss
• pale or purple fingers or toes from cold or stress, known as Raynaud’s disease
• arthritis

Signs of lupus in females

The symptoms of lupus can vary widely, including between males and females.

Symptoms more commonly seen in females include:

• hair loss
• sensitivity to sunlight
• mouth ulcers
• arthritis
• malar rash

Signs of lupus in males

Research suggests that, while males are less likely than females to have lupus, the symptoms tend to be more severe.

Symptoms more commonly seen in males include:

• cardiovascular complications
• low blood count
• weight loss
• kidney complications
• chest pain

Effect on other body systems

Lupus can also cause the following systems, depending on which organs it affects:

• Kidneys: Swelling of the legs, feet, and face, frequent urination, and high blood pressure can result from kidney disease.
• Lungs:There is a higher risk of lung diseases, such as pleurisy and pneumonia, which can involve chest pain and breathing difficulty.
• Central nervous system: Symptoms may include brain fog, headaches, seizures, and strokes. A condition known as lupus cerebritis can cause confusion, difficulty, thinking, mood changes, seizures, lethargy, and coma.
• Blood vessels: Vasculitis (inflammation of the blood vessels) can affect circulation.
• Blood: Lupus can affect blood composition, leading to anemia, leukopenia, or thrombocytopenia.
• Heart: Inflammation can lead to myocarditis, endocarditis, or pericarditis. Symptoms include chest pain.

Other complications

Other complications that can arise from lupus include:

• a higher risk of infections, as both lupus and the treatment can weaken the immune system
• bone tissue death and bone fractures, due to the effects of lupus and medication use
• pregnancy complications, such as pregnancy loss, preterm birth, and preeclampsia

A doctor may recommend delaying pregnancy until lupus has been under control for at least 6 months.

The following images show how some lupus symptoms can appear.

Lupus is an autoimmune condition, but the exact cause is unclear.

What goes wrong?

The immune system protects the body from pathogens such as bacteria, but sometimes it mistakenly targets healthy tissue. This can cause inflammation, swelling, pain, and tissue damage.

Why does the immune system go wrong?

Researchers do not yet entirely understand what causes lupus, but they believe it is caused by multiple factors.

One possible theory relates to cell death, a natural process that occurs as the body renews its cells. Due to genetic factors, the bodies of people with lupus may not properly clear cells that have died.

The dead cells that remain may lead to the production of autoantibodies, such as antinuclear antibodies (ANAs), that go on to attack the body, causing lupus symptoms.

ANAs are commonly present in people with autoimmune conditions such as lupus. They work by targeting the nucleus of the body’s cells. The nucleus contains genetic material.

Risk factors: Hormones, genes, and environment

Lupus may develop in response to several factors. These may be:

• hormonal factors as females aged 15–44 years are nine times more likely to have lupus than males
• genetic factors, as lupus often runs in families, and scientists have identified certain genes that are common to people with lupus
• environmental factors, such as exposure to tobacco smoke or pollution or having the Epstein-Barr virus, which causes mononucleosis

Are children at risk?

According to the American College of Rheumatology, 20% of people with lupus develop the condition before the age of 20, but it rarely appears before the age of 5.

In children, lupus may have more severe symptoms. Up to 50% of children with lupus have kidney symptoms.

Treatment for lupus will depend on the type of lupus. Options include:

• protective clothing and sunscreen to shield the skin from sunlight
• medication to treat skin and joint symptoms
• immunosuppressants, such as mycophenolate or methotrexate, which dampen the action of the immune system
• biologic drugs, such as Belimumab
• a range of treatments to manage complications such as infections, seizures, skin, or kidney problems

Home remedies

Home remedies and lifestyle measures may help protect body systems and manage some symptoms.

While research is limited some evidence suggests a varied and balanced diet can help manage lupus. A doctor or dietitian can help a person with lupus make a diet plan that suits their needs.

Options may include:

• taking omega-3 fatty acids
• limiting foods that contain cholesterol and saturated fats
• reducing sodium intake
• ensuring a sufficient intake of vitamins such as vitamin D and vitamin B

Other lifestyle changes that may help include:

• avoiding or quitting smoking
• limiting alcohol intake
• doing regular, moderate exercise
• managing stress
• building or keeping up social networks to reduce the risk of social isolation

Diagnosis can be difficult because symptoms of lupus can resemble symptoms of other conditions.

A doctor will ask about symptoms, carry out a physical examination, and take a personal and family medical history. They may also request blood tests and other laboratory investigations.

Biomarkers are antibodies, proteins, genetics, and other factors that can show a doctor what is happening in the body or how the body is responding to treatment. They can help identify whether a person has a condition even when there are no symptoms.

Blood tests

Blood tests can show whether certain biomarkers are present, and biomarkers can give information about which autoimmune disease, if any, a person has.

• Antinuclear antibody: Around 95% of people with lupus will have a positive result in the ANA test, although some people test positive for ANA without having lupus.
• Antiphospholipid antibodies (APLs): These are present in around 50% of people with lupus, but they can also occur in people without lupus.
• Anti-dsDNA antibody test: Around 47% of people with lupus test positive for these antibodies, according to a study involving 1,977 people.
• Anti-Smith antibody: People with lupus may have antibodies to Sm, a type of protein.
• Anti-U1RNP antibody: Around 25–30% of people with lupus have anti-U1RNP antibodies, and fewer than 1% of people without lupus have them. However, it may be present with other autoimmune conditions.
• Anti-Ro/SSA and anti-La/SSB antibodies: These antibodies have been linked to various immune conditions, including SLE.
• Antihistone antibodies: Antibodies to histones are proteins that play a role in the structure of DNA. People with DIL or SLE may have them.
• Serum (blood) complement test: This test measures the levels of proteins that the body consumes when inflammation takes place. Low levels suggest inflammation is present, and the condition may be active.
• Nonspecific tests: Various other tests look for markers of inflammation, including C-reactive protein and erythrocyte sedimentation rate.

Further tests may include:

• urine tests, which can help identify effects on the kidneys.
• tissue biopsies, usually of the skin or kidneys, to check for damage or inflammation.
• imaging tests to reveal any organ damage

Here are some questions people often ask about lupus.

What is the main cause of lupus?

Researchers do not currently know what causes lupus. However, they believe that environmental, genetic, immunological, and endocrine factors may play a role.

What are usually the first signs of lupus?

Early signs of lupus may include:

• a malar or butterfly rash across the face
• fatigue
• joint pain
• fever
• chest pain when breathing deeply

However, the signs and symptoms of lupus vary widely and will depend on the type of lupus.

What are three triggers of lupus?

The following three factors may trigger lupus:

• stress
• exposure to toxins, such as cigarette smoke or air pollution
• having an infection, such as the Epstein-Barr Virus

What is the life expectancy of a person with lupus?

Life expectancy will depend partly on the type of lupus. Statistics suggest that 85–90% of people with Systemic lupus erythematosus (SLE) will live at least 10 years after their diagnosis, and many will live much longer.

Drug-induced lupUs (DIL) usually resolves within a few weeks after stopping the drug that caused the reaction.

Is lupus a serious disease?

Lupus is an incurable disease and symptoms can range from mild to life-threatening.

It is believed that between 10-15% of people with lupus will die prematurely due to complications of lupus. However, due to improved diagnosis and disease management, most people with the disease will go on to live a normal life span.

Lupus is an autoimmune condition that can affect a wide range of body systems. There are different types of lupus, but SLE is the most common type.

Some people may experience cycles of flare-ups and remissions, whereas others may have ongoing symptoms. The varied experiences of lupus can make it challenging for doctors to diagnose.

Once a person has a diagnosis, various treatment options can help manage symptoms, limit damage to body organs, and maximize a person’s quality of life.