SLE (LUPUS)

Lupus is an autoimmune disease, which means that the immune system, your body’s defence system, produces antibodies that attack the body’s own tissues, causing inflammation.

There are two main types of lupus:

• discoid lupus

• systemic lupus erythematosus (SLE)

Joint pains, skin rashes and extreme tiredness (fatigue) are the most common symptoms of lupus. Some people with lupus will only have these symptoms, though they can still have a big impact on your daily life. Fever, weight loss and swelling of the lymph glands are also quite common.

Lupus can affect many different parts of the body, and when internal organs such as the heart, lungs, brain or kidneys are involved it can be much more serious. But most people will only have one or a few of the possible symptoms, and many people will find that the symptoms come and go (see Figure 1).

Possible symptoms of lupus include:

• Skin and mouth: It’s common for a rash to develop over parts of the body that are exposed to the sun, including the face, wrists and hands. A butterfly-shaped rash over the cheeks and the bridge of the nose is especially common. Some people with lupus notice that their fingers change colour in cold weather, going first very pale, then blue and finally red. This is called Raynaud’s phenomenon and is caused by narrowing (constriction) of the blood vessels, which reduces the blood supply to the fingers or toes. You may develop groups of mouth ulcers, which may come back repeatedly.

• Hair: Some hair loss is common and can be severe in some people with lupus, but once a flare-up is brought under control the hair will usually grow back.

• Joints: Joint pain is common in lupus, especially in the small joints of the hands and feet. The pain tends to move from joint to joint and is often described as ‘flitting’. Joint pain and swelling can be the main symptoms for some people, though lupus doesn’t usually cause joints to become permanently damaged or deformed. About 1 in 20 people with lupus develop more severe joint problems. Fewer than 1 in 20 have joint hypermobility or a form of arthritis called Jaccoud’s arthropathy, which can change the shape of the joints.

• Kidneys: Around 1 in 3 people with lupus have significant inflammation of the kidneys, and kidney damage can sometimes occur. Kidney inflammation can be treated successfully in most patients if it’s identified early with regular urine, blood pressure and blood testing by your doctor. You must take any medication as prescribed by your doctor to make sure that your kidneys aren’t permanently damaged.

• Blood and blood vessel: Lupus can cause high blood pressure, particularly if the kidneys are involved. Steroid tablets, which are often used to treat lupus, can raise blood pressure particularly when used in high doses. Lupus can contribute to the development of high cholesterol, which should be checked yearly with a blood test and treated if necessary.

• Brain and nervous system: As many as 1 in 3 people with lupus may have migraines and may experience anxiety or depression. Some people have dizziness, memory loss or confusion. Rarely, lupus can cause fits (similar to epilepsy) or feelings of paranoia (similar to schizophrenia) – though these complications only affect a small number of people with lupus.

• Heart and lungs: Occasionally lupus directly affects the heart and lungs. More often, it causes inflammation in the lining tissues around the heart (pericarditis) and lungs (pleurisy), both of which cause breathlessness and sharp pains in the chest. Rarely, large amounts of fluid develop in these lining layers, causing severe breathlessness. More recently, we’ve found that lupus may also cause narrowing of the blood vessels. This can lead to an increased risk of angina, heart attacks and strokes, so close monitoring and early treatment of factors such as high cholesterol and high blood pressure are vital.

• Other organs: People with lupus can suffer swelling of the lymph glands, which may cause discomfort. Less frequently, lupus can affect the lining tissue of the gut (serositis), the gut, pancreas, liver or spleen, causing pain in the abdomen. Very rarely, lupus can affect the eyes, causing a painful red eye or changes in eyesight.

Lupus is more common in women of Chinese origin and most common in women of African or Caribbean origin. It tends to be more severe in those of Afro-Caribbean origin. Rarely, lupus can affect children, but it’s unusual before the age of five.

The immune system makes proteins called antibodies that fight infection. In lupus, the body also makes autoantibodies that attack the body’s tissues. We’re not sure why this happens, but it’s probably due to environmental, hormonal and genetic factors.

Lupus isn’t directly passed on from a parent to their children, but if you have a close relative with lupus you’re at increased risk of developing it. Similarly, if you have lupus there’s about a 1 in 100 chance of your child developing it in later life. Lupus isn’t contagious, so you can’t catch it from anyone else.

A diagnosis of lupus is made based on symptoms, a physical examination and blood tests. Tests can help to rule out other conditions.

A number of different blood tests may be used:

• Anti-nuclear antibody (ANA) test: About 95% of people with lupus are ANA positive, but the test can sometimes be positive in people who don’t have lupus, so it can’t confirm the diagnosis.

• Anti-double-stranded DNA (anti-dsDNA) antibody test: About 70% of people with lupus have these antibodies. A positive test means that lupus is highly likely as the test is hardly ever positive in people who don’t have lupus. The anti-dsDNA level usually goes up when lupus is more active so repeat tests may be helpful as a means of monitoring your condition and deciding on treatment.

• Anti-Ro antibody test: If you test positive for this autoantibody you may be more likely to get skin rashes and suffer from dry eyes or a dry mouth (Sjögren’s syndrome). This autoantibody can pass across the placenta during pregnancy. If you carry the anti-Ro autoantibody and decide to have a baby, your pregnancy will be more closely monitored.

• Antiphospholipid antibody test: A positive test for these autoantibodies may mean an increased risk of miscarriage and developing blood clots.

• Complement level test: Complement refers to a set of proteins in the blood that protect us from infections. Complement levels go down when lupus is more active.

• Erythrocyte sedimentation rate (ESR) test: This test assesses inflammation by measuring how quickly the blood cells settle at the bottom of a test tube. The ESR is often raised in lupus.

• Kidney and liver function tests: These include blood and urine tests, which are carried out regularly so any problems caused by the lupus or by drug treatment can be recognised and dealt with quickly.

• Blood cell counts: Haemoglobin, white and red blood cells and platelets are all made in the bone marrow, so blood cell counts can help to show whether the bone marrow is affected, either by the disease or the drugs.

• Other concerns: If you have symptoms such as fever, weight loss and persistent swelling of the lymph glands, your doctor may take a biopsy of lymph gland tissue to rule out cancer, which can also cause these symptoms.

A number of treatments are available that can ease pain and stiffness, but exercise and close attention to your posture are just as important to keep your spine mobile and help you to live a normal life.

The healthcare professionals in your rheumatology department can help you find treatments that are best for you. These will often include:

• drug treatments – These are given as tablets or injections to relieve pain, reduce inflammation or, if other treatments haven’t helped, to modify the condition itself.

• physiotherapy and exercise – These are very important to keep your spine strong and flexible.

Surgery is very rarely needed but may be very helpful if your hip joints are badly affected. Back surgery is even more uncommon and only used if your spine has become very bent.

A. SLE disease activity indices:

1. British Isles Lupus Assessment Group Index (BILAG)

http://www.clinexprheumatol.org/abstract.asp?a=2697

2. European Consensus Lupus Activity Measurement (ECLAM)

http://www.clinexprheumatol.org/abstract.asp?a=8761

3. Systemic Lupus Activity Measure (SLAM)

http://www.ncbi.nlm.nih.gov/pubmed/11434575 and http://www.clinexprheumatol.org/abstract.asp?a=2697

4. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI, with SELENA modification)

http://www.rheumatologie.at/pdf/sledai.pdf

https://www.thecalculator.co/health/SLE-Disease-Activity-Index-(SLEDAI)-Calculator-1119.html

5. Lupus Activity Index (LAI)

http://www.clinexprheumatol.org/abstract.asp?a=8761

B. SLE damage indices:

1. System Lupus International Collaborating Clinics/ACR Damage Index for Systemic Lupus Erythematosus (SLICC/ACEDI)

http://www.clinexprheumatol.org/abstract.asp?a=2697