Lupus
Information

Lupus is a long-term ‘autoimmune’ disease, a disorder of your body’s immune system which defends against disease and infection. In autoimmune diseases, as well as protecting you, the immune system also mistakenly attacks healthy cells in your body, causing inflammation and damage.

What is Lupus?

Lupus causes inflammation that can affect one or many parts of the body: skin, joints, muscles, blood vessels, blood cells, brain and nerves, lungs, heart, kidneys, gastrointestinal tract and the linings around internal organs.

The term ‘lupus’ refers generally to systemic lupus erythematosus, or SLE. This is by far the most common form of lupus, making up around 70% of cases, and is the focus of this information. The other main forms are discoid (or cutaneous) lupus, which affects only the skin, and drug-induced lupus, which is caused by medication and usually subsides when the drug course finishes. There’s also neonatal lupus syndrome, a rare lupus-like disease which affects babies in the womb. It usually clears up by about six months after birth and isn’t linked with developing lupus in later life.

About 1 in 1000 people in the UK have lupus; the Lupus Foundation of America reckons 1.5 million have it in the US and around 5 million people have lupus worldwide. Around 9 in 10 people with lupus are women. People of black African, Caribbean and Asian origin are more likely to develop lupus than those of white European origin.

There’s no cure for lupus, but treatments can help control and ease symptoms and most people will have a near-normal lifespan. In the UK, it is rare for lupus to be fatal but those with the condition can face an increased risk of premature death, mostly due to infection or cardiovascular disease.

What are the symptoms of Lupus?

There are many different possible symptoms of lupus. You are highly unlikely to have all of them and it is rare that two people have exactly the same symptoms. Some common symptoms of lupus include:

  • extreme fatigue

  • pain in muscles and joints

  • oral or nasal ulcers

  • rashes from sunlight or UV light

  • hair loss

  • heart and lung problems

  • depression

  • headaches or migraines

  • increased risk of miscarriage

  • seizures

  • blood disorders, including anaemia

  • kidney problems

  • flu-like symptoms and night sweats

In a 2018 survey of people with lupus using the HealthUnlocked website, half of 216 respondents said muscle or joint pain was the symptom that prompted their initial GP visit and 32% said fatigue was a top reason for seeing the GP. Many cited both pain and fatigue.

Lupus symptoms aren’t always constant - they can ease off or disappear, then come back. Times when your lupus symptoms become worse are called flares or relapses. When your symptoms settle down and disease activity is low - this is often measured with autoantibody blood tests - you may be said to be in remission. Some people, however, never notice any form of remission in the illness.

Flares and remissions can happen quickly and unexpectedly. There’s often no clear cause for flares, though it is possible to identify ‘triggers’ that you know will cause problems and try to avoid them.

What causes lupus?

The causes of lupus are not fully understood, but research suggests a genetic cause and a number of possible environmental triggers. If your genes make you vulnerable to lupus, the disease could potentially be triggered by:

  • hormonal changes, such as those experienced during pregnancy, puberty or menopause

  • certain viral infections

  • exposure to UV light or sunlight

  • trauma

There are many genes involved in the development of lupus (only half of which are inherited by a child from one parent with lupus), and because environmental triggers are needed to make the disease develop, it is not common for children of someone with lupus to also have it. Only about 1 in 20 people with lupus have a close relative (mother, aunt, sister; less often father or uncle) with the condition.

Lupus diagnosis

If you suspect you have lupus you should make an appointment with your GP. If your GP feels that further investigation is needed, they will organise tests and might refer you to a specialist.

Some people with lupus may develop symptoms gradually over weeks, months or even years. This can make diagnosis difficult, especially if the GP is looking at each symptom in isolation.

“I had lupus undiagnosed since childhood,” said one mum in the 2018 HealthUnlocked lupus survey. “I’d been diagnosed as having loads of illnesses. Then I had a total collapse of everything post the birth of my second child. A load of consultants, then a talented dermatologist worked it out.”

If you think you have multiple symptoms that are common in lupus and are otherwise unexplained, it may be helpful to ask your GP for a longer appointment than usual so you have enough time to discuss them all.

Diagnostic criteria

The American College of Rheumatology established 11 ‘criteria’ which, if combined, strongly suggest that the patient has lupus. Of the 11 criteria, you need to have four or more to be classified as having lupus. These criteria were developed in 1982 and revised in 1997.

  • Malar (or Butterfly) rash

  • Rash over cheeks

  • Discoid rash

  • Red (sometimes circular) patches of skin with scaling

  • Photosensitivity

  • Skin very sensitive to sunlight

  • Oral ulcers

  • Recurrent ulcers in the mouth or nose

  • Serositis

  • Inflammation of the membranes that line the lungs or heart

  • Arthritis

  • Affecting two or more joints – causing stiffness and pain

  • Renal

  • Inflammation in the kidneys, which leads to protein leaking into your urine

  • Neurological

  • Can affect the nerves in many different ways, such as seizures or psychosis

  • Haematological

  • In the blood, low levels of haemoglobin (anaemia), white cells (which help fight infection) and/or platelets (which help in blood clotting)

  • Immunological-1

  • Presence of abnormal antibodies - anti-double-strand DNA (called anti-dsDNA), anti-Smith (referred to as anti-Sm), or antiphospholipid antibodies

  • Immunological-2

  • Presence of antinuclear antibodies (ANA) that can cause the body to attack itself - these occur in nearly all people with lupus

Tests

Various blood and urine tests are used to help diagnose lupus. Key tests include:

Antinuclear antibodies (ANA) – The ANA test is positive in around 95-98% of people with lupus. However, these antibodies are also positive in many other conditions (such as rheumatoid arthritis) and even around 10% of the healthy population. Therefore this test is not specific for SLE but a positive result can be helpful evidence towards a diagnosis.

Anti-double-stranded DNA (dsDNA) antibodies – These antibodies are very specific for lupus in that they are typically not seen in any other condition or in the healthy population. However, only about 60% of people with SLE will be positive for this test. If you test positive for these antibodies, it often means you have lupus, but if your result is negative it does not necessarily mean you don’t have lupus.

Other tests – There are also other antibodies, such as anti-Sm antibodies, which are also specific to lupus but occur only in around 10% of people with the disease. Testing for a protein called complement-3 (C3), which is not an antibody but is involved in the immune system, may also be helpful as low levels of C3 suggests lupus.

If the doctor suspects lupus is affecting your lungs or heart, you may have an X-ray or echocardiogram; if lupus affects your kidneys, you might have a biopsy, in which a sample of kidney tissue is examined.

Treatment for lupus

Lupus is treated with a range of medications. The drugs you are given will depend on your symptoms and how your lupus affects you. Most were developed to fight other conditions but have been found useful in lupus. The main medications and common side-effects you might get are explained here.

Non-steroidal anti-inflammatory drugs (NSAIDs)

NSAIDs are drugs, such as ibuprofen or naproxen, used for joint pains. There are many types and they reduce pain caused by inflammation. NSAIDs are prone to causing indigestion. They can cause peptic ulcers and bleeding from the gut - usually, another medicine is given alongside them to protect the stomach. Long-term use of NSAIDs - over many months or years - can increase the risk of heart attacks and strokes and doctors will try to give the lowest effective dose for as short a time as possible.

Antimalarials

Originally for malaria, the most commonly used drug in this group is hydroxychloroquine. It can produce a reduction in fatigue, sun-induced flares and flares of arthritis, pleurisy and fever. It is safe during pregnancy and breastfeeding. Hydroxychloroquine may, however, take 3-6 months to become fully effective and isn’t sufficient for severe lupus effects such as kidney disease and nervous system problems. In rare cases, hydroxychloroquine has been associated with eye (retinal) damage. The risk is slight, but an eye test every 12 months is recommended to identify any damage early.

Steroids

Corticosteroids such as prednisolone have been vital in the improvement of lupus care and in some cases are lifesaving. They have a profound effect on inflammation and suppress active disease. The dosage depends on the severity of your symptoms. The possible side-effects of long-term high-dose steroids include weight gain and ‘moon face’, diabetes, infections, raised cholesterol, muscle weakness and osteoporosis. The risks are greatest with higher doses for longer periods, so doctors will try to quickly reduce the dosage to a minimum or stop the steroids altogether. Injections may be an alternative to high-dose daily steroids to treat severe manifestations of lupus quickly with the least risk of side-effects.

Immunosuppressants

These drugs are used to reduce the need for steroids to control moderate and severe lupus. The most commonly used are azathioprine, methotrexate and mycophenolate mofetil. Other immunosuppressant treatments that may be used include cyclophosphamide and the biological treatments rituximab and belimumab. Except for azathioprine, these treatments are not safe during pregnancy or breastfeeding and should be stopped 3-6 months or more before trying to conceive. Immunosuppressant treatments can cause a range of side-effects including nausea, hair loss and an increased risk of infections.

Non-lupus medications

Various medications may be taken by people with lupus to help improve long-term outcomes. These include tablets to control blood pressure, anticoagulants (aspirin or warfarin) in those with an increased risk of blood clots, and anti-epileptic medication. Skin creams that may be used include corticosteroid and sun-protection creams. Patients who have had long-term steroids may be given calcium and vitamin D3 to reduce their risk of osteoporosis.

Living with lupus

“Lupus is an integral part of who I am. So it affects every bit of my life. It has meant things like no visits to hot countries with sun. It has meant things like joint replacements, cataracts, dreadful teeth, but that’s fine, it’s me. My family and friends and work colleagues have to accept that too”

HealthUnlocked lupus survey response, 2018

Lupus affects people in many different ways and can have an impact in all areas of life. But there’s plenty you can do to help manage the condition and reduce the risk of flares:

Learn about lupus

Knowing all you can about the condition will help you manage it. Information leaflets and booklets are available free to read or download from LUPUS UK. There’s also good information from the Lupus Foundation of America

Pace yourself

Fatigue is a key problem. Learning good pacing techniques can help to prevent a ‘boom and bust’ cycle with your energy levels. Learn not to push yourself too much on good days to prevent yourself from crashing.

Avoid exposure to direct sunlight and UV

Sunlight and UV can trigger lupus rashes as well as a flare of more general symptoms such as fatigue, headaches, joint pains and weakness. Use high factor sun protection (even on cloudy days), cover up, and try to stay in the shade.

Eat a healthy, balanced diet

Lupus affects people in many different ways, so there is no special diet suitable for everyone with lupus. The Mediterranean diet (plenty of fruit and vegetables, grains, nuts and seeds, two portions of fish a week and small amounts of meat and dairy produce) is probably simplest as it is is suitable for all the family. The Mediterranean diet is very similar to the UK government's healthy eating advice, which is explained simply in the Eatwell Guide.

“Eat right and exercise. It sounds like a simple solution but it really works”

HealthUnlocked lupus survey response, 2018

Take regular, gentle exercise

Just like everyone else, where possible, people with lupus need to exercise regularly. Lupus affects everybody differently and not everyone with lupus can do a wide range of exercises, but most can manage some activity - walking, swimming and cycling are all beneficial. If mobility is a problem, you can also do exercises sitting down. The amount of activity you can manage is likely to fluctuate as your lupus does. But regular physical activity can help to reduce fatigue, manage pain and reduce the risk of cardiovascular problems.

Look after your mental and emotional wellbeing

“Having a positive attitude helps to deal with the bad days”

- HealthUnlocked lupus survey response, 2018

Stress is a common trigger for lupus flares and so limiting exposure to stressful situations and learning to deal with stress are important for controlling your lupus. Because lupus can be very limiting, many people feel frustrated or angry, and low mood and depression are common. Visit the NHS Moodzone for tips on dealing with stress, anxiety and low mood.

Many people find it helps to talk to others with lupus, but talking with family, friends and colleagues is also important as it helps them to understand your condition and be better able to offer support when you need it. If you think you might be depressed, see your doctor as soon as possible.

Support groups and apps

“Join a Lupus forum online - this gives you a network of other people who live and deal with Lupus on a daily basis. I find mine to be supportive and informative”

- HealthUnlocked lupus survey response, 2018

There’s a range of online communities, including Facebook groups, and face-to-face support groups for people with lupus. Online groups let you chat with others with lupus 24/7 and they are a valuable source of information and support. Some people find they are ideal for discussing things that might seem awkward in a face-to-face group.

Local support groups generally meet once a fortnight or once a month. Some meetings are ‘coffee and chat’ sessions, others have invited speakers, and some groups organise information and awareness events.

There’s also a range of smartphone apps that you can use to help track and manage your medication, appointments and other aspects of your illness.

Online

In the UK, the LUPUS UK community at HealthUnlocked is the leading online lupus group. LUPUS UK also runs a Facebook group.

The Lupus Foundation of America runs the LupusConnect community and a Lupus Foundation Facebook group

Pain Doctor, also in the US, lists lupus Facebook groups, online communities and ways to find local support.

Local groups

Your doctor, a lupus nurse specialist, or social services may be able to suggest a group you could join. You can find local face-to-face support groups at:

  • LUPUS UK - regional group finder

  • Lupus Foundation of America - local support

Apps

There’s a wide range of smartphone apps to help you manage and learn more about your lupus. Many are free or very low cost. The apps are varied in function but typically they allow you to set reminders for taking your medicine and attending appointments. You can usually keep track of prescriptions, symptoms, diagnoses and so forth. Some let you add contact information, and many provide information about lupus. Other apps let you track UV levels, sleep patterns, pain levels, diet and so on.

You can find apps by searching for ‘lupus’ in your device’s app store - the Google Play store lists about 50 lupus-related apps.

LUPUS UK has a short article about the Lupie Diary app, and a range of apps is discussed at both the lupus website Molly’s Fund and at Lupus News Today.