Systemic lupus erythematosus

This is often known as ‘lupus’ for short: systemic lupus erythematosus (SLE) is classed as an autoimmune condition in which the immune system attacks healthy tissue in the body.

This mainly affects women, especially younger women.

Children can also develop lupus although this is very rare.

This section concentrates on systemic lupus erythematosus (SLE). The shorthand term ‘lupus’ is used which refers to SLE and not the other two versions of this disease. Lupus is a relatively unknown form of arthritis which attacks the tissues, organs and joints in most of the body. The joints become swollen and painful and are accompanied by a fever, mouth ulcers and a rash over the face, hands and wrists.

Lupus is discussed as follows within this section of the guide:

• Is there more than one type of lupus?
• How does lupus occur?
• Who is most likely to develop lupus?
• Causes of lupus
• Symptoms of lupus
• Diagnosis of lupus
• Tests for lupus
• Treatment for lupus
• Managing lupus
• Complications of lupus

Is there more than one type of lupus?

There are three types of lupus which are:

• Discoid lupus erythematosus
• Drug-induced lupus
• Systemic lupus erythematosus

Discoid lupus erythematosus affects the skin only, causing hard, red scaly patches of skin, hair loss and bald patches.

Drug-induced lupus is as the name says: it occurs as a side effect from many types of medication such as anti-convulsants.

Systemic lupus erythematosus is the form of lupus that is discussed in detail within this section. It occurs when the immune system turns on itself, attacking the joints of the body which causes pain and inflammation.

If the skin is attacked then a rash occurs but if the joints are targeted then the main symptoms are pain and inflammation. This is what we know as arthritis.

Basically, your immune system attacks healthy tissues and joints rather than the source of an infection.

How does lupus occur?

Systemic lupus erythematosus (SLE) occurs when something goes wrong with the immune system. But what is the immune system and how does it work?

Immune system

The immune system is your front line defence against germs, viruses and bacteria which are all causes of infections. It is a sophisticated set of biological structures such as white blood cells, organs and tissues which acts as this protection.

Their main function is to identify and destroy infectious agents which are likely to be harmful to the body.

Antibodies

When a substance or ‘antigen’ enters the body it triggers an automatic response from the immune system. The immune system views this as a threat and deals with by releasing proteins known as ‘antibodies’.

These antibodies are vital against potential disease carriers such as bacteria and viruses. But they can get it wrong, for example they attack a substance which is not harmful, e.g. pollen which then triggers an allergic reaction.

Developing an immunity

The immune system develops and strengthens from childhood through to adulthood which is why children are more prone to illnesses than adults. They have a lower level of immunity but this becomes stronger over time.

Exposure to germs and vaccinations means that your immune system has time to build a defence against these. This is known as ‘adaptive immunity’.

But in cases of lupus, the immune system releases antibodies which then attack normal healthy tissues, cells, organs and joints. It is difficult to say why this happens but something triggers this response.

Who is most likely to develop lupus?

Women are more likely to get lupus than men. Around 90% of all lupus cases are women and those of childbearing years.

There is a higher rate of lupus in women from certain ethnic backgrounds such as Asian or Chinese compared to Caucasian women.

Men can develop lupus but the majority of sufferers are women.

Lupus occurs in children although only in a few isolated cases.

(Source: NHS Choices/lupus)

This is a fairly uncommon condition but one that affects many areas of the body and with symptoms that range from minor through to life threatening.

Causes of lupus

Lupus is caused by a fault in the immune system but there are additional factors to take into account. Genetics is one possible factor as is your environment.

Find out more about these below.

Genetics

There is no ‘lupus gene’but there are several genetic mutations which increase the risk of developing this disease. Plus if one of your parents has lupus then there is strong chance you may do the same.

This is also the case with identical twins, especially if one half of these twins have lupus. If one twin does then there is a strong likelihood that the other will develop lupus.

The genetic mutations may be the reason why lupus affects more women than men. Some of these genes are found in the X chromosome: men have only the one X chromosome (along with their Y chromosome) whereas women have two X chromosomes.

So, women are twice as likely to acquire a mutated gene which along with hormonal reasons may explain why they are more prone to this form of arthritis.

Environment

There are a few environmental factors which can trigger lupus. These include fluctuations in oestrogen levels, e.g. during pregnancy which is why women are a greater risk. Other reasons include prolonged exposure to sunlight and certain medications.

But these are possible causes rather than substantive reasons so further research is needed.

Symptoms of lupus

The main symptoms of this condition are fatigue, pain in the joints and skin rashes. But these primary symptoms vary from person to person.

Some people experience only mild symptoms but others have a more serious form of this disease. It is also possible to experience what are known as ‘flare ups’in that you have a few minor symptoms which then worsen before dying down again.

These short bursts can be distressing.

Other symptoms include:

• Mouth ulcers
• Inflamed kidneys
• High blood pressure
• Swollen glands in the neck, groin and armpits
• Hair loss
• Anaemia
• Migraine
• Memory loss
• Anxiety
• Depression
• Dry eyes
• Raynaud’s phenomenon (restricted blood supply to the hands and feet).

Some people only experience the primary symptoms such as fatigue and joint pain but others suffer from many of the symptoms listed above.

Lupus can affect the internal organs of the body and if it does then it is a much more serious state of affairs. These include pleurisy and pericarditis: both of these conditions involve an inflammation of the tissues and a build up of fluid which causes pain, breathlessness and difficulty in breathing.

Lupus can affect the eyes, pancreas, liver or spleen but this is rare. A few people are unlucky enough to develop rheumatoid arthritis as well but, again, this tends to be rare.

Diagnosis of lupus

Your GP will ask you about your symptoms: what type, how often and the level of intensity (mild or severe). He/she will examine you and ask you about your current medical history.

Lupus can be inherited so he/she will ask you about your family history.

The next step is to refer you for tests to determine the progression of your condition. These tests include X-rays, MRI/CT/Ultrasound scans and blood tests.

A urine test will be performed if your GP suspects that lupus has affected your kidneys. If there is a hint of this then a kidney filtration test will be carried out to determine the extent of this damage to your kidneys.

The reason for these is that your symptoms may the sign of another similar condition. Plus it is difficult to diagnose lupus because of this similarity with other diseases.

Tests for lupus

There is a range of blood tests available which check antibody levels, blood cell counts and the amount of lupus activity in your system. These may be accompanied by tests to check kidney and liver function.

These include:

• Anti-DNA antibody test
• Anti-nuclear antibody (ANA) test
• Antiphospholipid antibody test
• Anti-Ro antibody test
• Erythrocyte sedimentation rate (ESR)
• Complement level test
• Blood cell counts
• Kidney and liver function tests

Anti-DNA antibody

The anti-DNA test is used to detect the presence of the anti-DNA antibody in your blood. If there is a high level of this antibody then it means that you may have lupus.

However, there are people with this antibody who do not have lupus. Additional tests are required to confirm this diagnosis.

If you are experiencing a ‘flare up’of your condition then your GP may expect to see an elevated levels of the anti-DNA antibody.

Anti-nuclear antibody

The anti-nuclear antibody test checks for signs of this antibody in your blood. Around 95% of all lupus cases have this antibody in their system.

Antiphospholipid antibodies

A positive test for antiphospholipid antibodies means an increased risk of blood clots, and for women, a greater chance of a miscarriage.

Anti-Ro antibodies

The presence of Anti-Ro antibodies in your blood means that you are more likely to suffer from dry eyes and mouth and skin rashes.

Erythrocyte sedimentation rate

This test monitors how quickly blood cells settle within a test tube. This happens quickly in lupus cases and flare ups.

Complement level test

The ‘complement’is the name of a chemical present in your blood as part of your immune system. The level of this chemical is checked to determine if you have lupus and if so, how active this is.

Low complement levels means a high level of lupus activity.

Blood cell counts

Your bone marrow produces platelets, red and white blood cells and haemoglobin. Haemoglobin is a protein found in red blood cells which transports oxygen around the body.

This test is carried out to see if any of these biological processes have been affected by lupus or medication prescribed to treat it.

Kidney and liver function tests

Lupus can affect internal organs of the body such as the liver and kidneys so tests are performed to see if there is any damage or side effects from medication.

Treatment for lupus

Lupus is similar to other types of arthritis in that it cannot be cured. Research is being carried out into this condition and new treatments are on the horizon but further tests are required before they are prescribed to lupus sufferers.

But there are treatments available to control the symptoms of lupus. These include medication such as non-steroidal anti-inflammatory agents (NSAID’s), hydroxychloroquine (an antimalarial drug to treat lupus related fatigue), corticosteroids and immunosuppressants.

Plus there is a new type of medication currently being trialled called ‘Rituximab’which is designed for people who have been unsuccessful with other lupus drugs. This works by destroying the cells which are responsible for producing antibodies and lupus.

But it is unlicensed at present in the UK.

Other options include acupuncture, diet and exercise and avoiding prolonged sun exposure and sunbathing. Do not use a sunbed either.

People with severe lupus can develop liver of kidney failure as a result. If this occurs then dialysis or a transplant will be needed.

But this can be prevented with early intervention of this condition.

Want to know more? Then visit our treatment for arthritis section.

Managing lupus

This condition can be controlled on a daily basis. This involves diet and exercise such as walking or swimming. Food supplements such as fish oils may help but obtain medical advice before you use this and any other type of supplement.

If you have a mild form of lupus then this can be controlled with minimal disruption to your daily life and no complications. It may mean making a few changes but it is manageable. Many people find that their symptoms improve once they reach the age of 50 or older.

Some people find it useful to talk to someone trained in this disease or with other fellow sufferers. You may find counselling or self-help groups of benefit.

If you are a woman with lupus who is thinking of starting a family then discuss this with your GP. Pregnancy may cause a flare up of lupus but the evidence to support this is debatable.

If your lupus is manageable and mild or moderate then you will probably be fine but there are risks, for example miscarriage. So it is important to talk about this with your GP before you proceed.

Find out more about these and other options in our living with arthritis section.

But severe forms of lupus can be dangerous even life threatening.

Complications of lupus

Severe cases of lupus can lead to complications which include:

• Cardiovascular disease, e.g. high blood pressure, heart disease and strokes.
• Lupus nephritis
• Autoimmune disease, e.g. Hughes Syndrome
• Rheumatoid arthritis

Cardiovascular disease is an umbrella term which encompasses heart attack, stroke, high blood pressure and atherosclerosis (hardening of the arteries).

Severe lupus can cause an inflammation of the arteries and heart which leads to some form of cardiovascular disease such as those mentioned in the paragraph above.

People with lupus are on average, seven times more likely to develop cardiovascular disease then non-lupus sufferers.

(Source: NHS Choices/lupus)

Lupus nephritis is the name given to a disease caused by an inflammation of the kidneys as a result of severe lupus. This often occurs in around half of all lupus sufferers.

Autoimmune diseases such as Hughes syndrome occur as a secondary condition to lupus. But they can also develop as a separate condition in their own right.

Hughes syndrome increases the risk of a blood clot forming in the veins (thrombosis) or arteries (heart attack).

Rheumatoid arthritis is a very common form of arthritis which causes inflammation and swelling in the joints. Find out more about this condition in our rheumatoid arthritis section.

Your GP will discuss these complications with you.