What is Lupus?

There are around 50,000 people in the UK who have lupus, according to Lupus UK, which is approximately 1 in every 1,000 people. Of these, it is most often seen in women of childbearing age, with a ratio of nine women for every one man diagnosed.

Additionally, it is most often seen in people of Afro-Caribbean, Asian or Hispanic descent. It is also thought to be the most common connective tissue disease (CTD) and has different forms. Globally, lupus is more prevalent than muscular dystrophy, leukaemia and multiple sclerosis.

What is lupus?

Lupus is short for systemic lupus erythematosus (SLE) and is an autoimmune disease with no specific aetiology. What is more, there is currently no cure, so it is a life-long condition. Lupus occurs when the body’s immune system starts to attack its own organs and tissues. The inflammation this causes affects different systems in the body including the skin, joints, blood cells, kidneys, heart, lungs and brain. Because of this, it is often difficult to recognise it as the symptoms of lupus are often remarkably similar or even identical to those of other conditions or diseases.

What are the causes of lupus?

Given that lupus is an autoimmune disease, it is not contagious. However, the causes of lupus have not yet been clearly identified.

Some potential causes that have been suggested include:

• Viral infection – Having an infection has been known to bring on lupus or cause a person with lupus to relapse.
• Sunlight – Sun exposure has been known to cause skin lesions associated with lupus or induce a response internally in those susceptible to the disease.
• Certain medication – Some blood pressure medicines, antibiotics and anti-seizure medicines have been known to trigger lupus. Those with lupus brought on this way usually improve when they stop taking the medication that brought it on. Sometimes, however, the symptoms continue even if the medication has been stopped.
• Women’s health issues – puberty, childbirth and menopause – Given that lupus is much more common in women, it is not surprising that events such as puberty, menopause and childbirth could be triggering factors for the disease.
• After trauma – According to research, the risk of lupus developing is three times higher after trauma such as post-traumatic stress disorder (PTSD).

It is considered likely that lupus is not caused by one single thing but rather by a combination of factors including a person’s genetics and environment. Some suggest that those with a predisposition to lupus through their genetics may go on to develop it when triggered by something in their environment.

What are the types of lupus?

There are distinct types of lupus as well as varying degrees of severity of the condition.

Lupus.net lists four different types of lupus as well as a related condition called pre-lupus:

• Systemic lupus erythematosus (SLE).
• Cutaneous lupus erythematosus (CLE) with types and subtypes:
  – Acute cutaneous lupus erythematosus (ACLE).
  – Chronic cutaneous lupus erythematosus (CCLE).
  – Discoid lupus erythematosus (DLE).
  – Chilblain lupus erythematosus.
  – Subacute cutaneous lupus erythematosus (SCLE).
  – Lupus panniculitis (lupus profundus).
• Drug-induced lupus erythematosus.
• Neonatal lupus.
• Pre-lupus.

Systemic lupus erythematosus (SLE)

This is the most common form of lupus with around 70% of all lupus cases being of this type. ‘Systemic’ means that it can affect the whole body or any area within it. Most often, it affects the joints, kidneys and skin. For over half of people with lupus, one of the major organs is affected.

This type of lupus often brings with it some complications:

• Inflammation of the kidneys – Lupus nephritis.
• Inflammation of the brain and nervous system. This can cause memory problems, headaches, fever, seizures, strokes and confusion.
• Arteriosclerosis – Hardening of the arteries. This can cause cardiac problems including heart attacks.

Cutaneous lupus erythematosus (CLE)

Skin problems are a common symptom of SLE; however, cutaneous lupus erythematosus is a distinct form of lupus that only impacts the skin. It can occur on its own or alongside SLE. Many people believe that CLE is actually more common than SLE.

As mentioned above, CLE has a few types and subtypes. The first type, acute cutaneous lupus erythematosus (ACLE), has the characteristic butterfly (malar) rash. This can be generalised and widespread or localised and occasionally transient. It has often been diagnosed incorrectly as sunburn when it first appears.

Subacute cutaneous lupus erythematosus (SCLE) is a distinct subtype that is more common in white women of childbearing age. The lesions with SCLE are found on the upper limbs and trunk and are often triggered by exposure to sunlight. The plaques often have a central clear area and when they resolve, they do not leave scarring but do leave telangiectases (chronically dilated capillaries) and depigmentation.

Chronic cutaneous lupus erythematosus (CCLE) is another form that includes the subtypes discoid lupus erythematosus (DLE) and chilblain lupus erythematosus. Discoid lupus is the most common of these and is mostly found in people of colour. The lesions are found on the cheeks, scalps, ears and lips and heal slowly to leave depigmentation as well as scarring. Chilblain lupus erythematosus mainly involves the fingers and toes of people who smoke.

It is triggered by being exposed to a cold, moist environment. It can be associated with SLE but can also be independent of it and sporadic. The lesions are often painful and may develop ulcers and fissuring. When healed, depigmentation may also occur.

Lupus panniculitis (also known as lupus profundus) is a very rare subtype where painful nodules form under the skin in the adipose (fat) tissue. The nodules can bleed and ulcerate. Thickened plaques also appear on the body. It can happen in any part of the body and will resolve to leave skin dents due to the atrophy of the fatty tissue underneath.

Drug-induced lupus erythematosus

Around 10% of all cases of lupus are drug induced. As you would expect, high doses of some medications can cause or trigger lupus.

Some of the more common drugs that can have this effect are:

• Hydralazine and Diltiazem (drugs for high blood pressure).
• Procainamide (used to treat cardiac arrhythmia).
• Isoniazid (for malaria).
• Minocycline and sulfa drugs (antibiotics).
• TND inhibitors (anti-inflammatory drugs).

Most often, this type of lupus disappears once the drug is discontinued.

Neonatal lupus

This is a very rare form of lupus and makes up around 10% of all cases of lupus. This happens as a result of a mother’s antibodies attacking her unborn baby. The mother does not need to have lupus herself for this to occur and only around 40% of those affected had mothers who had the condition. However, despite its name, it is not considered a true form of lupus.

Pre-lupus

Many medics believe that people develop lupus from a pre-lupus condition –asymptomatic autoimmunity. This means that they might show some symptoms of lupus but not enough to get a full diagnosis. Around 20% of people who have identified pre-lupus go on to develop full-blown lupus.

Who is at risk for developing lupus?

According to the Lupus Foundation of America, four areas affect a person’s risk of developing the disease.

These are:

• Sex.
• Age.
• Race/Ethnicity.
• Family history.

As previously mentioned, women are much more likely to develop the disease, by a ratio of 9:1. Likewise, those aged between 15 and 44 are more likely to get the condition, though most are diagnosed later, with only 15% showing symptoms before adulthood.

People of colour such as those of African descent, Asians and Hispanics are more likely to develop lupus. These groups also have a larger proportion of younger people developing the disease.

Additionally, if a relative has lupus, a person’s risk is increased, and they will generally have a risk of between 5% and 13% of going on to develop the condition. However, if a child’s mother has lupus, their chances of developing it are only around 5%.

What are the signs and symptoms of lupus?

The name lupus comes from the Latin word for ‘wolf’ which was used to describe a characteristic red facial rash resembling a wolf’s bite or mask. This is often the most recognisable symptom of the disease. However, not everyone with lupus has this symptom. The different types of skin lesions are described above, as each type and subtype of lupus has distinct and characteristic patterns on the skin.

Aside from skin lesions, those with lupus often experience the following symptoms:

• Joint pain.
• Muscle aches.
• Extreme fatigue.

The less often reported symptoms include:

• Mouth ulcers.
• Headaches.
• High temperature.
• Skin sensitivity to light.
• Hair loss.
• Glandular fever.
• Claustrophobia/agoraphobia.
• Growing pains in adolescents.
• Chest pains.
• Shortness of breath.
• Toes and fingers that change to blue or white when cold or during stressful times.
• Recurrent miscarriages.
• Severe reactions to insect bites.
• Difficulty flexing finger joints.
• Migraines.
• Memory loss/confusion.
• Dry mouth/eyes.
• Menstrual cycle irregularities.
• Low lymph count.
• Raised blood ESR but normal CRP.

With such an array of symptoms, it is not surprising that cases of lupus are varied in their appearance. The symptoms can also be intermittent and can improve for a while before recurring. The severity of the symptoms can also vary.

How does lupus affect the body?

Given that lupus is an autoimmune disease, it can affect almost any system in the body.

The inflammation that lupus causes can have significant effects on the body’s internal systems:

• Kidneys – Lupus causes severe damage to the kidneys in some people. Indeed, kidney failure is one of the main causes of death for lupus patients.
• Central nervous system and brain – Symptoms such as headaches, memory loss, confusion and behaviour changes mean that the central nervous system and/or the brain are affected by lupus.
• Circulatory system and blood – Lupus can affect this body system and cause a reduction in the number of blood cells (anaemia). It can also cause increased bleeding and increased risks of blood clots. The blood vessels can also become inflamed.
• Respiratory system – The risk of inflammation of the lungs or chest cavity is increased with lupus. This means that pneumonia and lung bleeds are more likely. Breathing can become painful for some lupus sufferers.
• Heart – Lupus sometimes causes inflammation in the heart muscles, membranes and arteries. This means that those with lupus have an increased risk of heart attacks and cardiovascular diseases.
• Infections – Given that lupus is an autoimmune disease, those with the condition are more susceptible to developing conditions because the treatments for lupus can weaken the body’s immune system so that it essentially stops attacking itself.
• Skeletal issues – The bone density can reduce due to the reduced blood supply reaching the bones. This can lead to fractures and breaks.
• Cancer – Lupus slightly increases a person’s risk of developing cancer.
• Reproductive problems – Women who have lupus have an increased risk of miscarrying their babies as well as an increased risk of high blood pressure in pregnancy. This can also result in pre-term birth. Doctors often recommend delaying trying to conceive until a woman’s condition has been stable for a period of around six months or more.

How is lupus diagnosed and treated?

Given the symptoms, lupus is often difficult to diagnose. Symptoms can also vary over time, even with the same person. They are also common in other diseases and there is an overlap with other disorders. There is no single, standalone test for lupus.

Doctors usually diagnose it after a combination of blood tests, urine tests and physical examinations:

• Full blood count.
• Erythrocyte sedimentation rate test – A test that determines how quickly red blood cells sink to the bottom of a test tube. If it happens quicker than expected, this is often indicative of systemic disease, though not specifically lupus.
• Kidney and liver function tests.
• Urine tests – Urine may show increased protein or red blood cells, which often happens if lupus is affecting the kidneys.
• Antinuclear antibody tests (ANA). If someone tests positive for these antibodies, it means that the immune system is reacting. Most of those with lupus will have a positive ANA test. However, there are lots of reasons why someone may have a positive ANA test and those with lupus make up a small minority.
• Chest X-rays – These may show fluid or inflammation in the lungs.
• Echocardiogram (ECG) – This gives information on the heart rate and rhythm.
• Biopsy of skin or kidneys.

Treatment

The appropriate treatment for lupus is dependent on a person’s symptoms.

Some common medications include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen. These can be used to treat fever, pain and swelling that often occurs with lupus.
• Anti-malarial drugs such as hydroxychloroquine. These can help reduce the risk of recurrence or flare-ups.
• Corticosteroids such as prednisone. These reduce inflammation and are often used when the disease affects organs such as the brain and kidneys. There are many associated side effects with these medications, however, such as thinning bones, weight gain, diabetes and high blood pressure to name a few.
• Immunosuppressant medications such as methotrexate. These medications are heavily controlled and must be taken under frequent medical supervision. Their side effects include increased infection risk, decreased fertility, liver damage and increased cancer risk.
• Biologic medication is administered in hospital by IV. This reduces symptoms in some people who are severely affected by lupus but can also cause gastrointestinal problems and worsen depression.

What are the dos and don’ts for living with lupus?

There are a number of things that people who have lupus can do (and not do!) to help their condition:

Do:

• Use a high-factor sunscreen (SPF 50+). Those with lupus should be able to get it on prescription.
• Wear a hat and sunglasses when it is sunny.
• Learn pacing strategies to avoid fatigue.
• Stay active even when fatigued and exercise regularly.
• Learn some relaxation techniques to help manage stress and avoid flare-ups or exacerbation of symptoms.
• Eat a balanced diet and add additional calcium and vitamin D.
• Inform employers in case adjustments can be made.
• See your consultant or doctor regularly for check-ups.

Don’t

• Smoke. Stopping smoking is really important.
• Do not sunbathe or spend time in rooms with fluorescent lighting.

What support is there available for those with lupus?

Those with lupus often experience confusion about their condition as well as times of depression and anxiety. To try and cope with these feelings, they must seek support. This can be from family, friends, doctors and treating medical teams as well as by connecting to others with lupus either through support groups or online.

Here is a list of useful resources for learning more about lupus or accessing support:

• Lupus UK including Virtual Events.
• Disability Support Project – Lupus.
• North East Lupus.
• North West Lupus.
• Lupus Brighton and Hove.