Help and support Helpline Information and leaflets Lupus and Lupus Kidney Disease If you would like to discuss your kidney diagnosis with our trained members of staff ring the free to call number 0800 169 0936 The Helpline is open Mon-Fri 9am to 5pm Or you can E-mail us [email protected] Key points about Lupus Lupus is a condition that affects all parts of the body, and 1 in 3 people with Lupus will get some kidney disease. Kidney disease in Lupus is complicated because there are several distinct types of kidney disease that can occur. The commonest symptom of kidney disease in Lupus is water retention, causing swelling of the ankles. Kidney disease in Lupus can be treated with immunosuppressant drugs, but these may have to be taken for many years. Kidney failure caused by Lupus can be treated with dialysis or, in some cases, kidney transplantation. What is Lupus? Lupus, also called Systemic Lupus Erythematosus (or SLE), is a disease that can affect all parts of the body. The skin, joints and kidneys are commonly affected and it may develop very slowly and be present for many years. Lupus is commonest in young women, but men and older people can get it as well. It is also more common in Asian, Hispanic or Black people than in white people. There is medical treatment that may help control Lupus, but there is no cure. Why is it called Lupus? Lupus is the Latin name for wolf and was used for this condition because, before the days of drug treatments, the skin disease could eat away at the face and leave extensive damage, as if it had been attacked by a wolf. The full name is Systemic Lupus erythematosus (SLE). Systemic means it can affect all parts of the body and erythematosus means the skin can have a red colour around the rash. Drug treatments mean that the rash which gave Lupus its name is not seen nowadays, or can be controlled in its early stages. Not everyone with Lupus gets a skin rash, but the name has stuck! What causes Lupus? The cause of Lupus is not fully understood. Lupus is caused by the bodys immune (natural defence) system. The job of the immune system is to defend the body against invaders, but sometimes the immune system can go wrong and damage normal tissues in the body. A particular type of antibody, called anti-dsDNA antibody, is nearly always found in Lupus, and can be measured in the laboratory from a blood test. There is a type of Lupus where blood clots and miscarriages can occur, called confusingly by several names (anti-phospholipid syndrome, Hughes syndrome, and Lupus with anti-cardiolipin antibodies). More details about the immune system... The usual job of the immune system is to fight invaders into the body. These might be germs or bugs, or foreign objects such as splinters. The body recognises these invaders and tries to eliminate them from the body. The blood contains two main types of defence system. One is white blood cells, which stick to germs and kill them. The other type of defence is antibodies, which are smaller than the white blood cells. They work by sticking onto germs and either make them burst apart, or help the white blood cells to stick to them. The immune system is very powerful and is normally very good at recognising what is part of the body and what is not. However, sometimes it makes a mistake and attacks the body as if that part of the body was an invader. There are many different diseases caused by the immune system, and they can affect any part of the body. There are several kidney diseases caused by the immune system. Some of them affect only the kidneys, some can affect other parts of the body as well as the kidneys. It is not clear why the immune system causes diseases such as Lupus. There may be some trigger that makes the immune system go wrong, such as an infection. It is not known why Lupus occurs, except in the extremely rare cases where Lupus is a side effect of a drug. Lupus can occasionally occur in more than one family member, and is more common in people who are black, Hispanic or Asian than in Europeans. There is no scientific explanation for this at the present time. More details on the antibodies that cause Lupus... Antibodies are not all the same. Our blood contains millions of different antibodies, each one directed against only one type of chemical invader. Blood tests can measure several different types of antibody in Lupus patients which are not normally present in the body. These antibodies can attack the normal tissues of the body, instead of invaders. The most important are antibodies against double stranded DNA (called anti-dsDNA antibodies for short), and an antibody called anti-cardiolipin. Anti-ds DNA antibodies (anti-double stranded DNA). These are the most important antibodies in Lupus. DNA is the chemical inside our cells that is the code for life. Antibodies against the centre, or nucleus of the cells in our body, are very common and usually do not cause problems, but antibodies against double stranded DNA (called anti-dsDNA) do seem to be important if they are present. Nearly everyone with Lupus has anti-dsDNA antibodies, so it is an important test. Also, the amount of the antibody in the blood can be measured, and is useful in many people as a measurement of disease activity. The anti-dsDNA antibody level can therefore help to calculate the correct amount of drug treatment someone with Lupus should receive. Anti-cardiolipin antibodies (also called anti-phospholipid antibodies). These are important in Lupus because someone with this antibody is at risk of blood clots and women are at risk of miscarriage if they get pregnant. Not everyone with these antibodies will run into trouble, but if someone with Lupus wants to have a baby or has a history of blood clots, this test should be performed. If these antibodies are present, they do not usually go away with treatment, so that some people may need long term treatment to prevent blood clots. This usually means drug treatment to make the blood clot less well (anticoagulation). Warfarin tablets are most commonly used, but because warfarin can harm a baby, heparin injections are used during pregnancy. Milder cases may be able to take aspirin to reduce the chances of blood clots. Anti-nuclear antibodies. The nucleus is the central part of each cell in our body, and antibodies against the nucleus are quite common. Anti-nuclear antibodies do not mean someone has Lupus - it is necessary to go on and do other tests such as anti-dsDNA antibodies to confirm a diagnosis of Lupus. Anti-Ro and anti-La antibodies. These may also be present in Lupus. They may help confirm a diagnosis of Lupus if the anti-dsDNA antibodies are not present. Also, anti-Ro antibodies can cause problems in pregnancy, and should be tested if a woman with Lupus wants to have a baby. Crithidia antibodies. This is a very sensitive test for anti-dsDNA antibodies. It is used to confirm a diagnosis of Lupus in difficult cases, but does not have value in monitoring the effects of treatment. What are the symptoms of Lupus? Lupus has many possible symptoms; each person with Lupus is slightly different from each other one. Many of the symptoms of Lupus occur commonly in people who do not have Lupus. This is one the reasons that Lupus can be so difficult for doctors to diagnose, and why there is often a long delay between the appearance of the first symptoms and a positive diagnosis. Some people only ever have Lupus in one part of the body, in other people problems can develop slowly over years; and occasionally, a severe disease can affect all parts of the body at once. Common Lupus symptoms are tiredness and fatigue; joint pains; skin rashes. Lupus and Lupus Kidney Disease - Symptoms of Lupus The symptoms of Lupus can be:- 1) Tiredness and fatigue. Lupus patients commonly report unusual tiredness, although of course many other conditions can cause this symptom. There may be some mood changes, perhaps with excitability or depression. 2) Joints. It is common for Lupus to cause pain and swelling in joints. Any joints can be affected, but it is commonest for the hands and feet to be affected. 3) Skin. Lupus can cause several different types of skin rash. The three commonest are:- a flush across the cheeks, called a butterfly rash because it forms a shape a little like the wings of a butterfly across the cheeks (with the nose forming the body of the butterfly). patches of raised and discoloured skin, often on the face or upper body. This is called discoid Lupus, because the rash forms disc shapes on the skin small red spots on the lower legs, which are tiny little blood blisters. These form because Lupus has caused tiny blood vessels under the skin to burst. This is called a vasculitic rash, because (in medicine) blood vessels are called 'vascular', and '-itis' means inflammation 4) Kidneys. About 1 in 3 Lupus patients will get some kidney disease. 5) Brain. Lupus can cause brain disease, though fortunately serious brain disease is rare. This can be a blood clot in the brain, causing a stroke with weakness of an arm or leg. In other cases Lupus can cause tiny spots of brain damage throughout the brain, causing behaviour or mood changes. Sometimes Lupus can cause a sudden serious behaviour disturbance which requires urgent treatment. Any of these problems can cause epilepsy. This is a condition where fits occur at intervals. A fit is a collapse or loss of consciousness associated with shaking movements of the arms and legs, sometimes with incontinence of urine. 6) Eyes. Lupus can cause inflammation in the eyes, or sometimes one eye at a time. There may be pain or redness around the eye, or just a reduction in vision in the eye. Lupus disease in the eye is treatable, and it is extremely rare for blindness to occur. 7) Pregnancy. Lupus can cause miscarriage in some women while they are pregnant. In some women it causes recurrent miscarriages. Lupus can affect the baby while it is in the womb, or for a while after birth. Women who are thinking of getting pregnant should talk to their specialist about any risks to the pregnancy in their particular case, and any changes in treatment that might be necessary. 8) Blood clots. Lupus patients, especially those who have an antibody in the blood called 'anti-cardiolipin antibody', are prone to blood clots. These can cause pain or swelling in a leg. If small parts of blood clots pass up into the lungs, there can be chest pain, breathlessness and coughing up of blood. If blood clots do occur, it is necessary to take drugs to reduce the clotting of the blood (these are called anticoagulants; heparin and warfarin are most commonly used), in some cases for life. 9) Lung and heart. Fluid can collect in body cavities around the lungs or around the heart (called pleural effusion and pericardial effusion respectively). These fluid collections are often small and may not cause any problems, but sometimes need draining with a needle if they are squashing the underlying organ. Lupus can cause someone to cough up blood, and this problem must be reported to doctors immediately. The two main causes of coughing up blood are bleeding into the lungs in a Lupus crisis , and blood clots in the lungs. Lastly, Lupus can cause scarring in the lungs and breathlessness on minimal exertion. 10) Anaemia. Lupus can affect the bone marrow, with antibodies attacking blood cells. This can cause low counts of any of the three main types of cell in the blood. These three types are: the red cells which carry oxygen around (a shortage of these red cells is called anaemia); the white cells which fight infection (a shortage is called leucopaenia); the platelets which make the blood clot (a shortage is called thrombocytopaenia). All other parts of the body are occasionally affected in Lupus, though much less commonly. Why does Lupus cause kidney disease, and how many people get it? Lupus damages parts of the body because antibodies travel in the blood stream and get trapped in the tissues of the body, causing local damage (called glomerulonephritis). The kidneys have a large blood supply, and the filters of the kidneys are very delicate, so it is not surprising that the kidneys are affected in some cases. However, it is not possible to predict whether a particular individual with Lupus will get Lupus in the kidneys. To read more about glomerulonephritis Click here About 1 in 3 people with Lupus will get kidney disease at some time in their life. Sometimes kidney disease is the first sign of Lupus, sometimes kidney disease only develops after Lupus has been present for some years. What are the symptoms of Lupus kidney disease? Kidney disease in Lupus is discovered in four main ways:- Someone may feel completely well. Kidney disease is only picked up because tests are performed regularly in Lupus patients to check for kidney disease. Lupus kidney disease can cause water build up in the body, with swollen ankles, or puffy face and hands. Add nephrotic syndrome link Kidney failure can cause sickness, tiredness and itching. However, not everyone with these symptoms has kidney failure. Pain passing urine, having to go in a hurry and very frequently, may occur with urine infection. Infections may occur in people who never develop the serious forms of Lupus kidney disease, but of course infections may need urgent treatment with antibiotics. Types of Lupus kidney disease The most important kidney disease is glomerulonephritis, which is inflammation in the filters of the kidneys .There are several types of glomerulonephritis in Lupus. These have been put into 5 categories by the World Health Organisation, and a specialist can predict the long term outcome of kidney disease by knowing which type of glomerulonephritis someone with Lupus has, and how badly the kidney is affected by that type. In addition to glomerulonephritis, Lupus patients can get kidney infections and a condition associated with anti cardiolipin antibodies (also called anti-phospholipid syndrome). What treatment is needed for Lupus kidney disease? How you can help yourself?Everyone with Lupus can help themselves by keeping generally healthy and following a few simple guidelines. Can Immunosuppressant drugs be used?Because Lupus is a disease caused by overactivity of the bodys immune system (see earlier in this section for a description), reducing the activity of the immune system can reduce the activity of Lupus. Side effects of immunosuppressant drugs?All the immunosuppressant drugs used in Lupus have side effects, and people with Lupus hate taking these drugs. There are several reasons why the drugs should be used, and there are no good alternatives - before the days of good drug treatment, many people with both Lupus and kidney disease died prematurely. How long does immunosuppressant drug treatment last?People with Lupus require long term treatment. Lupus cannot be cured. So that even if all the blood tests become normal and you feel well, there is a risk of the disease coming back when treatment is stopped. However drug dosages can usually be reduced in the long term. What other drugs may be used?Many drugs from the chemist can be used in people with Lupus, to relieve simple aches and pains. People with kidney disease often require a range of drugs to reduce the blood pressure or remove the build up of water in the body. Thinning of the bones (osteoporosis) can occur with steroid therapy. The oral contraceptive and hormone replacement treatment may need to be used with caution in women who have Lupus. Can Lupus cause death or kidney failure? Lupus can be a serious disease in some people, but more often it continues for many years, causing problems without causing death. In 100 people with Lupus 70 people will not get kidney disease20 people will get troublesome but not serious kidney disease8 people will get kidney failure, some will need dialysis2 people will get severe kidney failure and a Lupus crisis (see next section) What is a Lupus Crisis? This is a term used for a severe flare of Lupus. Lupus usually has periods of time when it is not active, and then can become active, causing perhaps a skin rash and increase in protein levels in the urine. This would be called a Lupus flare. Most flares are mild, and can be treated with drugs through the outpatients clinic. Occasionally, perhaps about 1 in 100 Lupus flares, the disease is so severe it could be called a Lupus crisis. In a Lupus crisis, there is a rapid onset of severe disease in many parts of the body. There is kidney failure; disease in the lungs with breathlessness; fluid around the heart; inflammation and swelling in the brain, often causing fits. Although cases of Lupus crisis are rare, many require treatment on an intensive care unit, and about 1 in 3 people with Lupus crisis will die. Recovery from a Lupus crisis occurs in the majority of cases, but some people are left with some long term problems. Are dialysis and transplantation possible for someone with Lupus? If someone with Lupus gets kidney failure, this may be so severe that there is a life threatening build up of waste products or of water in the body. This is fatal unless artificial kidney treatment (called dialysis) is given. People with Lupus can receive dialysis in the same way as anyone else. If kidney failure becomes so severe that dialysis treatment is required, a Lupus patient may also be suitable for a kidney transplant, depending on tests for suitability. Lifestyle and pregnancy Many people with Lupus lead a normal life, however, others have to live a restricted life according to their symptoms. People with Lupus can do most of the activities they want, but sun exposure can trigger a Lupus attack in many people, so look cool in a large hat and use sunscreen. Pregnancy is often successful in women with Lupus, but there can be problems if a woman has particular types of Lupus antibodies in her blood. More information and support Other websites which can help with Lupus as it affects the body apart from the kidneys are:-www.lupus.orgwww.lupus.org.uk Download this information in PDF The National Kidney Federation cannot accept responsibility for information provided. The above is for guidance only. Patients are advised to seek further information from their own doctor.