Systemic lupus erythematosus (SLE)

by | Dermatology

Systemic lupus erythematosus, (also known as lupus or SLE) is a type of autoimmune disease, which means that the body’s immune system mistakenly attacks its own healthy tissues and organs. Lupus is a chronic (long-term) condition that can affect many different parts of the body, including the joints, skin, heart, lungs and brain, causing pain and inflammation in the affected areas.

SLE is one form of lupus. Another type of lupus just affects the skin, and is known as discoid lupus. When people talk about ‘lupus’ they are usually referring to SLE.

Lupus symptoms

The symptoms of lupus can vary widely. They may come and go or stay constant; they can be mild, moderate or severe; they may start suddenly or develop slowly over time.

Most people with lupus have times when the symptoms get worse (known as a flare or flare-up) and times when the symptoms are very mild or disappear (known as remission).

The signs and symptoms of lupus will also vary depending on what parts of the body are affected by the disease. People with lupus don’t usually experience all the symptoms – so different people with lupus will have different sets of symptoms.

The most common signs and symptoms include:

  • Fatigue;
  • Fever;
  • Joint pain, stiffness and swelling;
  • ‘Butterfly rash’ – a skin rash on the face that is shaped like a butterfly and covers the cheeks and nose (also known as malar rash);
  • Skin lesions or a skin rash that appears with sun exposure (also known as photosensitivity);
  • Poor circulation in the fingers and toes (also known as Raynaud’s phenomenon): causes the fingers and toes to turn white or blue when exposed to cold or during times of stress;
  • Shortness of breath;
  • Chest pain, especially with deep breathing;
  • Nose, mouth or throat sores;
  • Dry eyes;
  • Headaches, confusion and memory loss;
  • Weight loss;
  • Bald patches or hair loss.

Causes of SLE

The exact cause of lupus is unknown. It most likely occurs due to a combination of factors both inside and outside the body. For example, many experts think that some people have a higher risk of developing SLE because of their genes (a genetic predisposition). Something in the environment, like an illness or infection, may then trigger the disease to develop in these people. Other triggers may include exposure to sunlight, smoking and hormonal changes that occur during puberty. The use of certain medicines can sometimes cause side effects that are similar to SLE.

Does lupus always go into remission?

SLE is unpredictable and affects each person differently. Some people have long-lasting symptoms, while others experience many flares and periods of remission throughout their lives. Some people have long periods of remission with symptoms every few years, while others have frequent flares of symptoms and shorter periods of remission. With treatment, most people with lupus can lead a full and active life.

Who gets SLE?

SLE is an uncommon condition, affecting around 20-40 people out of every 100,000. Around 90 per cent of people with SLE are women. SLE can affect people of any age, but it is usually diagnosed in people aged between 15 and 40 years.

Complications and effects of lupus

People with SLE are more likely to develop other problems, including:

  • Kidney problems: SLE can cause long-term inflammation in the kidneys and a serious kidney disease called lupus nephritis.
  • Infection: People with SLE have a weakened immune system and are therefore more likely to develop infections, including respiratory infections, yeast infections, urinary tract infections, herpes and shingles.
  • Cardiovascular disease: People with SLE are more likely to develop problems in their heart and arteries (known as cardiovascular disease or CVD).
  • Pregnancy complications. SLE does not usually affect a woman’s ability to get pregnant, but it can increase the risk of problems such as miscarriage, pre-eclampsia (a condition that causes high blood pressure during pregnancy), and premature birth.

Tests and diagnosis

Lupus can be difficult to diagnose because the symptoms may be similar to other more common medical conditions. Symptoms also vary from person to person and change over time, so this can make a diagnosis challenging.

To make a diagnosis of SLE, your doctor will check whether you have several key symptoms, combined with high levels of certain antibodies found in a blood test.

In someone suspected of having lupus, the following blood tests can help make the diagnosis:

  • ANA antibodies. The presence of antinuclear antibodies (ANA). Antibodies are small proteins produced by the immune system when it detects harmful substances. Most people with SLE have the antinuclear antibody in their blood. However, it is possible to have ANA without SLE, so it is not used to diagnose the condition on its own.
  • Anti-DNA antibodies. The presence of anti-DNA antibodies in their blood. Many people with SLE also have this antibody in their blood.
  • Complement. The level of a chemical known as complement. This is part of the immune system, and can show how active the SLE is.
  • ESR. Erythrocyte sedimentation rate (ESR) is not a diagnostic test specifically for lupus, but it can show if inflammation is present in the body.

Treatment

Currently there is no cure for SLE, but the symptoms can usually be controlled with treatment. This involves the use of medicines and lifestyle changes.

The type of treatment will depend on how bad the symptoms are, whether you are having a flare, and what parts of your body are affected.

Medicines for lupus

The most common medicines for systemic lupus erythematosus include:

  • Non-steroidal anti-inflammatory drugs (NSAIDs): Used to help with joint pain and swelling. Examples include ibuprofen, naproxen and diclofenac. They should not be used in people with kidney problems.
  • Hydroxychloroquine (brand name Plaquenil): Used to treat skin problems, tiredness and joint pain that isn’t relieved with NSAIDs. People using this medicine usually have their vision checked before starting and then regularly each year.
  • Steroid tablets (e.g. prednisone): Used to reduce inflammation and swelling in people with severe symptoms. Steroids may cause side-effects if taken for long periods, so the lowest possible dose is recommended.
  • Immunosuppressant medicines: Used to reduce immune system activity. Examples include azathioprine, cyclosporin, cyclophosphamide (e.g. brand names Cycloblastin, Endoxan), methotrexate.
  • Belimumab (brand name Benlysta): A monoclonal antibody used to reduce the activity of the immune system in people with severe SLE. This medicine is given as an infusion into a vein by a doctor or nurse.

Lifestyle factors: how to reduce symptoms of lupus

If you have lupus, several self-help measures may help to reduce symptoms and flares:

  • Avoiding the sun: Exposure to sunlight can aggravate symptoms of SLE, so it is important to avoid the sun whenever possible. People with SLE should use a sunscreen with an SPF of 50+ and wear long-sleeved clothing and a wide-brimmed hat on sunny days.
  • Avoiding infections: People with SLE are more prone to infection, especially if they use steroids or medicines that reduce immune system activity. It is therefore important to try to avoid contact with people who have infections.
  • Having regular check-ups: People with SLE should see their doctor regularly, not just when symptoms get worse. The doctor can make sure the most appropriate treatment is being used.
  • Getting enough rest: Fatigue is a common symptom of SLE, so it is important to get plenty of rest and not overdo things.
  • Not smoking: Smoking can worsen the effects of SLE on the heart and blood vessels.

Lupus specialists

Lupus is a complex condition and is treated by a medical specialist called a rheumatologist, sometimes in conjunction with other specialists, and a person’s general practitioner.

Support groups for lupus

There are a number of support groups for people with lupus. Lupus support groups offer a range of services which may include telephone counselling, meetings, help with information and regular newsletters. Talking to other people with lupus may help with feelings of isolation and may provide you with emotional support.

Getting pregnant with SLE

A woman with SLE may have a higher risk of complications during pregnancy, so it is important to plan and talk to a doctor in advance if possible. The risk of complications is higher if a woman becomes pregnant when symptoms are severe, so doctors will usually recommend waiting until the symptoms are in remission.