Lupus

• Skin and mouth - It is common for a rash to develop over parts of the body that are exposed to the sun: the face, wrists and hands. A butterfly-shaped rash over the cheeks and the bridge of the nose is especially common. You may develop groups of mouth ulcers, which may come back repeatedly. 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 spasm (constriction) of the blood vessels. About 1 in 8 people with lupus also experience Sjögren’s syndrome, which causes a dry mouth and eyes.
• Hair – Some hair loss is common with lupus and can be severe in some patients. 
• Joints – Joint pain is common in lupus, especially in the small joints of the hands and feet. 
  • 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.
  • Perhaps 1 in 20 people with lupus develop more severe joint problems.
  • Fewer than 1 in 20 have hypermobile and deformed joints (this is called Jaccoud’s arthropathy ).
• Kidneys – Around 1 in 3 people with lupus have significant inflammation of the kidneys. This doesn’t usually cause problems unless the disease is very severe.
• Blood pressure – Lupus can cause high blood pressure, particularly if the kidneys are involved. Steroid tablets, which are quite often used to treat lupus, can also cause an increase in blood pressure.
• Brain and nervous system
  • You may feel depressed and anxious from time to time. This may be a feature of the disease itself or a normal emotional response to having a serious illness.
  • Migraine affects as many as 1 in 3 people with lupus.
  • Lupus can sometimes cause fits (similar to epilepsy) or feelings of paranoia (similar to schizophrenia) – though this is very rare.
• Heart and lungs – Occasionally lupus affects these organs directly. 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 shortness of breath.
• Other organ involvement – Lupus can affect the bone marrow, causing anaemia and a reduction in the number of platelets (cells that help the blood to clot) and/or white blood cells. Blood-related problems like anaemia are more common in children with lupus.

People with lupus can suffer swelling of the lymph glands which may cause discomfort. Some people with lupus have a tendency to develop blood clots in veins or arteries. This is usually caused by antiphospholipid antibodies .

Some of these antibodies can also affect pregnancy, causing an increased risk of miscarriage (antiphospholipid syndrome).

Less frequently lupus can affect the gut, pancreas, liver or spleen, causing pain in the belly. Rarely, lupus can affect the eyes causing painful red eye or changes in eyesight. Fewer than 1 in 10 people with lupus develop an autoimmune disease that affects the thyroid gland, in particular the type which makes the gland underactive. Much less often people with lupus may also develop rheumatoid arthritis, or inflammation of the muscles (myositis).

We don’t know exactly why this happens, but it’s probably caused by a combination of environmental, hormonal and genetic factors.

Lupus isn’t contagious and is not directly inherited from your parents, though some of the genes inherited from parents may contribute to the development of the disease.

What are the long-term risks?

Lupus is a variable and unpredictable condition and can be life-threatening for a minority of patients whose vital organs are affected. However, with modern treatments and careful monitoring of the condition the disease can be brought under control.

Some people with lupus have an increased risk of having a heart attack or stroke. It’s important to take this into account along with any other risk factors such as smoking and raised cholesterol or blood pressure.

In young women the risk of heart attacks and stroke is usually very low, so even though the risk is increased by lupus the overall risk is still quite low.

What else should I know about lupus?

• Contraception – If you have lupus you should use contraceptive pills that contain only progesterone or low-dose oestrogen , or consider physical/barrier methods of contraception. This is because oestrogen can make the disease more likely to flare up.
• Hormone replacement therapy – If you are given hormone replacement therapy (HRT) there is a risk of the lupus flaring up. About 1 in 7 women with lupus who start HRT develop a flare of the disease.
• Keep out of the sun – Avoid sunbathing. Too much ultraviolet light can cause the skin rash, and sometimes the symptoms in the internal organs, to flare up. If you go to a hot climate, use a sun-blocking cream, factor 25 or greater. This is available on prescription for people with lupus.
• Raynaud’s phenomenon – If you experience symptoms of Raynaud’s in your hands or feet make sure you dress suitably for cold weather. Smoking is bad for the circulation and is likely to make symptoms worse whereas regular exercise will improve your circulation.
• Infections – If you have lupus, and especially if you are on immunosuppressive drugs , then you will be more prone to infection. Take sensible precautions and avoid contact with family and friends who are known to have infectious diseases like chickenpox.
• Vaccinations – We don’t recommend ‘live vaccines’ if you are on more than 20 mg prednisolone daily or immunosuppressive drugs (such as mycophenolate, azathioprine, ciclosporin, cyclophosphamide). These vaccines include yellow fever, live typhoid and live oral poliomyelitis (polio). Check with your doctor if you think you might need these or other vaccinations.
• Stress – Stress will make your illness seem worse even if it doesn’t affect the course of the disease. Learn how to manage any stress in your life. Your doctor may be able to refer you to a psychologist who can help with coping strategies.
• Fatigue – Tiredness can be a significant problem – specific causes of fatigue , such as anaemia or an underactive thyroid gland, can be identified by a blood test and treated. If no specific cause can be identified, learning to pace yourself should help.

• Anti-nuclear antibody (ANA) test – About 95% of people with lupus are ANA positive.
• Anti-DNA antibody test – 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-DNA level usually goes up when lupus is more active so repeat tests are helpful in monitoring your condition.
• Anti-Ro antibody test – If you test positive for this antibody you may be more likely to get skin rashes and to suffer from dry eyes or dry mouth (Sjögren’s syndrome).
• Antiphospholipid antibody test – A positive test for these antibodies may mean an increased risk of miscarriage and also of 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 measures how quickly the blood cells settle in a test-tube. This happens more quickly in very active lupus.
• Kidney and liver function tests – These are carried out regularly so that any problems caused by the disease itself or by the drugs being used 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 by either the disease or the drugs used to treat it.

These tests can also be helpful in monitoring the condition after diagnosis – for example, a combination of rising anti-DNA and falling complement levels (often accompanied by a high ESR test) is very helpful in predicting a flare-up of lupus.

A variety of tests are available to check the function of the heart, lungs, liver and spleen. Depending on which organs your doctor thinks may be involved, you may have x-rays, an ultrasound scan , computerized tomography (CT) scan or magnetic resonance imaging (MRI) scan.

A simple urine test can show if there is protein or blood in the urine; this can help doctors to recognize a problem in the kidneys at a very early stage. Further tests, such as kidney filtration tests, may be carried out if necessary.

If you have symptoms such as fever, weight loss and 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.

Lupus drugs

Joint pains can often be treated with non-steroidal anti-inflammatory drugs (NSAIDs) . Skin rashes can be treated with creams containing steroids and/or with hydroxychloroquine tablets. Hydroxychloroquine and similar drugs are also effective at treating tiredness caused by lupus.

If you experience Raynaud’s phenomenon drug treatments such as nifedipine or injections of prostacyclins (e.g. iloprost) are available if needed, though self-help measures are often sufficient.

Steroid injections may be tried if hair loss is a problem, though the hair often grows back by itself when the lupus is brought under control. More serious complications, such as pleurisy or pericarditis, are usually treated with steroid tablets.

These are very effective, sometimes life-saving. The dose prescribed will be kept as low as possible to minimize the risk of side-effects such as osteoporosis . Often, people with pericarditis or pleurisy will only need to take steroids for a few months.

Drugs such as bisphosphonates may be needed for people, especially older people, who would be more at risk of osteoporosis if treated with steroids.

For inflamed kidneys, severe anaemia or a very low platelet count, steroids may be required for longer and at higher doses. In these cases steroids will usually be given together with immunosuppressive drugs including azathioprine, ciclosporin, cyclophosphamide, methotrexate and mycophenolate.

These work by suppressing the immune system so that it doesn’t attack the body’s tissues so aggressively. You may need to take these drugs indefinitely, though doctors usually reduce the dose of immunosuppressive drugs as the disease becomes less active.

Immunosuppressive drugs can often control, and occasionally cure, high blood pressure caused by kidney problems. Otherwise anti-hypertensive drugs may be used to control blood pressure.

A treatment revolution

New therapeutic approaches, which target cells and molecules believed to be part of the cause of lupus, are now being used to help many patients. Although these so-called monoclonal antibody therapies have yet to be formally approved, drugs like rituximab , epratuximab and belimumab have been used with success in open studies and, in the case of the last two, large double-blind control studies (where neither the patient nor the physician knows for sure whether the patient is getting the real drug or a placebo). We now need to establish how and when these drugs can be used to the best effect.

Physical therapies

If joint pain is a particular problem, acupuncture may help. The pain relief may only last a short time to begin with but repeated treatments may bring longer-term benefit.

Other treatments

If there is a significant build-up of fluid in the lining tissues of the heart or lungs this may need to be drained using a needle and syringe.

Some people with lupus may develop kidney failure requiring dialysis or a kidney transplant. However, severe kidney damage can usually be prevented by early diagnosis and treatment of lupus.

Exercise

When the disease is active, you may not feel like doing very much and it’s important to rest when you need to. However, too much rest will cause the muscles to weaken. You need to find the right balance between rest and exercise. Walking and swimming are recommended as they can improve fitness and stamina without putting too much strain on the joints.

Diet and nutrition

There is only limited evidence available on the effect of diet in controlling lupus, though there is some evidence that a diet low in saturated fat and supplemented by fish oil (i.e. fish body oil, not fish liver oil) may be helpful.

Read more general advice on diet, nutrition and nutritional supplements.

Pregnancy

A small number of women with very severe lupus may be advised against having a baby as pregnancy can put a great strain on the heart, lungs and kidneys. If you’re thinking of having a baby, discuss your plans with your doctor or specialist nurse before conception. There is conflicting evidence about whether pregnancy is likely to cause a flare-up of lupus. If the disease is well under control at the time the baby is conceived, and you do not have kidney disease, you will probably go through pregnancy with little trouble.

If you do have kidney disease, then you may well have increased protein in your urine during the later stages of pregnancy. The doctors looking after your pregnancy check for this and consult your rheumatologist if necessary.

If you have high levels of antiphospholipid antibodies, then there is an increased risk of miscarriage. However, treatment with aspirin and/or heparin reduces this risk, and there are now many more successful pregnancies in women who have these antibodies. In pregnant women who have anti-Ro antibodies there’s a small risk (about 1 in 50) that their babies will have neonatal (newborn) lupus syndrome. This means the baby may have a rash and/or a slow heartbeat. Most babies born to mothers with anti-Ro will be fine, but it’s important to have regular scans of the baby’s heart during the pregnancy.

Doctors are naturally cautious about which drugs are used during pregnancy. It’s usually safe to use steroids, and many people have safely used hydroxychloroquine and azathioprine throughout pregnancy.

Read more general advice about pregnancy.