LIVING WITH LUPUS.....

In this day and age, the diagnosis of an illness such as lupus is not a death sentence. In fact, it is far from it. Most people with lupus have to adjust their lifestyles in some way but can expect to enjoy an almost normal life.

The key to living a good life with lupus is knowing about the condition, understanding what your limitations are and making the absolute most of what you have. So read on, learn about the condition and if there is anything you are unsure about, ask your doctor or contact your closest lupus organisation.

Links to:
- What is Lupus?
- What causes Lupus?
- Who is most often affected?
- Are all cases of Lupus the same?
- The signs and symptoms of Lupus
- How does Lupus affect the body?
- Diagnosing Lupus
- Laboratory tests
- Treating Lupus
- Lupus in different groups
- Tips for living with lupus - Help yourself to a full life
- Commonly asked questions
- Where can I go for further information and support
- The history of Lupus


What is Lupus?

Lupus is an autoimmune disease. A normal healthy immune system recognises and destroys foreign objects like bacteria and viruses. With autoimmune diseases, however, the immune system starts to attack objects that are not foreign. Hence the term "auto" (self) "immune."

With lupus, the immune system produces an excess of proteins called antibodies that attach themselves to various structures in the body. The accumulation of these antibodies in the tissues can cause inflammation, damage and pain


What causes Lupus?
Despite many years of research, the cause of lupus is still not known. Scientists believe there are several things that may trigger the formation of the antibodies, including genetic, hormonal and environmental factors. Some of the possible triggers include:

- Hormones (females between the age of 15 and 45 are most commonly affected)
- Certain medications
- Dietary factors
- Viruses and bacteria
- Stress
- Genetics
- Pregnancy
- Exposure to UV light

Who is most often affected?

Although lupus can affect anybody, 90% of lupus patients are women. Of these, 90% develop the condition during their reproductive years.

Lupus affects people the world over, although there is a higher incidence in certain regions of the world and in certain ethnic groups. For example, in the United States, African Americans, Latinos and Asians are more commonly affected than Caucasians. Lupus is more common in the Philippines and China than in Japan.



Are all cases of Lupus the same?

There are two main types of lupus:

- Systemic lupus erythematosus (SLE) – almost any organ or system of the body can be affected in this form of lupus. In most patients, only the skin and joints are affected (this is called minor organ threatening lupus). In   others, SLE also affects the kidneys, lungs, heart, blood vessels and/or brain (this is major organ-threatening lupus).

- Discoid lupus (also known as chronic cutaneous lupus erythematosus) –   usually appears as a red scaly rash on sun-exposed areas such as the face, scalp, arms, legs or trunk. Discoid lupus is generally a milder disease than SLE. It is usually diagnosed by its appearance and by skin biopsy. A small number of people with discoid lupus will develop SLE. However, most patients with discoid lupus have their condition confined to the skin only.

Other milder forms of lupus sometimes occur. In subacute cutaneous lupus, skin rashes, sun sensitivity and joint aches are the main symptoms.

Drug-induced lupus is usually a transient form that develops as a reaction to certain medications. It clears up when the medications are ceased.

 

The signs and symptoms of Lupus

Lupus is a very variable condition. While it has many characteristic symptoms, most patients will never experience all of them. Similarly, no two patients experience identical symptoms.

The severity of the disease also varies. In some patients symptoms appear suddenly and are relatively severe, while in others the disease remains at a low level for several years before diagnosis. For most patients, however, the frightening descriptions of life-threatening disease in medical textbooks never occur, and the condition remains mild and readily manageable.

Lupus runs an unpredictable course. For some people, symptoms subside after treatment of the initial acute attack. For others, periods of "remission" are punctuated by brief "flare-ups" of disease.

Initial symptoms

Several symptoms are seen in the initial stages of lupus. These include:
           - Fatigue, weakness and lethargy
           - Joint pain or swelling (experienced by about half of patients)
           - Skin rashes (around one in five patients)
           - Fever



How does Lupus affect the body?
 
Lupus can affect many parts of the body. The most common symptoms are described below. In addition, most patients feel tired or weak.

Skin Almost any type of skin rash may occur with lupus, affecting around two-thirds of patients. The characteristic lupus "butterfly" rash of the cheeks and nose is seen in about one-third of patients, while one in five patients experience mouth sores. Hives and altered skin colour (a lightening or darkening of the skin in places) can also occur. One-third of patients experience Raynaud’s phenomenon, where their fingers turn white then bluish on exposure to stress, cold or vibration.
Joints Joint pain (also known as arthralgia) and inflammation (arthritis) are common features of lupus. Nine out of 10 patients experience some form of joint pain, most commonly occurring in the hands, wrists and feet. Joint pain - which is often accompanied by stiffness and/or swelling - is most severe in the morning and eases later in the day.
Hair During active disease, significant hair loss, or alopecia, can occur. Usually the loss is only temporary and can be treated with a variety of remedies. Certain lupus medications, such as anti-malarial drugs and corticosteroids, may encourage hair growth.
Tendons When tendons are inflamed, they can tighten, causing fingers to pull into abnormal positions such as "trigger fingers". This is, however, an unusual problem.
Muscles Muscle ache, known technically as myalgia, may occur in up to two-thirds of patients. The muscles between the elbow and neck, the knee and hip are most frequently involved. Muscle inflammation or swelling is less common, but requires treatment, as unlike myalgia, it can lead to permanent muscle weakness.
Lungs A thin lining called the pleura surrounds the lungs. Inflammation of this lining is called pleuritis, a condition that affects around one-half of lupus patients at some stage during their life. This causes chest pain when deep breaths are taken.
Heart Inflammation of the sac surrounding the heart, the pericardium, is called pericarditis and affects about a quarter of those with lupus. The main symptom of pericarditis is chest pain below the breastbone that is often relieved by bending forward.
Kidney A condition called lupus nephritis sometimes develops when the delicate filtering mechanisms of the kidney, the nephrons, become inflamed.
Blood Lupus can affect the cells of the blood system and the components of the system that are involved in blood clotting. The most common complaint is anaemia, or too few red blood cells in the bloodstream. As red blood cells carry oxygen to tissues, patients with anaemia will often feel tired. Drops in the levels of white blood cells (defenders against infection) and platelets (which help the blood to clot) are also seen in some patients.
Immune system A condition known as lymphadenopathy, or swollen lymph nodes (most people know lymph nodes as "glands") can sometimes occur.
Brain and nervous system Inflammation of the blood vessels of the brain can cause a variety of symptoms, including depression, seizures and visual disturbances.

 

Diagnosing Lupus

Lupus is not an easy condition to diagnose. Symptoms are often similar to other illnesses and tend to come and go. Diagnosis is usually made on the basis of clinical symptoms and on the presence of certain antibodies in the blood. There are 11 symptoms or signs that help distinguish SLE from other conditions. Four or more of the symptoms described in the table below, not necessarily occurring at the same time, indicate SLE.

Symptoms that indicate SLE

Symptom

Description

Malar rash Rash over the cheeks
Discoid rash Scaly red or raised rash
Photosensitivity Reaction to sunlight – skin rashes that develop or become worse on exposure to sunlight
Ulcers Nose or mouth ulcers
Arthritis Inflammation of two or more joints, but the inflammation does not damage the joint
Serositis Inflammation of the lining of the heart or lungs
Renal disorder Excess protein or cells in the urine
Neurological disorder Seizures and/or abnormal behaviour when there are no other likely causes
Haematological disorder Lower numbers of red or white blood cells or blood platelets (these disorders are known as anaemia, leukopenia and thrombocytopenia, respectively)
Immunological disorder Testing positive in laboratory tests for one or more antibodies that are distinctive of lupus (eg anti-DNA antibodies, anti-phospholipid antibodies, anti-Sm)
Anti-nuclear antibody Testing positive in laboratory tests for a protein known as the "anti-nuclear antibody", a protein indicative of lupus.


Laboratory tests

A doctor will carry out a number of laboratory tests to help diagnose lupus. These include blood and urine tests (looking for urinary protein and red blood cells).

In the anti-nuclear antibody (ANA) test a sample of blood is taken and tested to see if it has anti-nuclear antibodies – antibodies that react against the nucleus of human cells.

While a positive ANA test points to lupus (virtually all people with SLE test positive), it is not diagnostic of lupus. A positive ANA test is seen in several other conditions, such as rheumatoid arthritis and some viral diseases. Sometimes even perfectly healthy people will be positive for ANA. If the ANA test is positive, the doctor will take into account whether the patient has other symptoms of lupus before diagnosing the condition. ANA-negative lupus is rare but not unheard of – patients in this situation will usually undergo more tests before their lupus is confirmed.

The anti-deoxyribonucleic acid (DNA) antibody test is similar to the ANA test. In this test, the antibodies tested for are those that react against DNA, the genetic material contained in the nucleus of human cells. It is more specific for lupus than the ANA test, as only rarely do people with other conditions test positive for anti-DNA antibodies. The drawback of this test is that the antibodies are only found in two-thirds of lupus patients.

Further blood tests may be carried out to see whether the levels of complement proteins in the blood are lowered. The presence of anti-Sm (sometimes called the extractable nuclear antigen or ENA) is also diagnostic of lupus.

 

Treating Lupus

The aim of treating lupus is to reduce inflammation in tissues and bring under control the abnormalities in the immune system that cause the inflammation. Treatment also enables the patient to live a fuller life. As no two patients are alike, treatment is tailored to the individual, taking account of the severity of the disease and the organs involved. There are four main groups of drugs used to treat lupus.

- Non-steroidal anti-inflammatory drugs

Used for pain relief and inflammatory conditions such as rheumatoid arthritis, NSAIDs are very useful for many lupus patients, Aspirin, ibuprofen and naproxin are examples of commonly used non-steroidal anti-inflammatory drugs (NSAIDs).
These drugs vary in their effectiveness a drug that one patient finds very effective may be of little or no use to another. This means several NSAIDs may have to be tried before a suitable one is found.
NSAIDs are used to relieve the fevers, headaches, muscle aches, malaise and arthritis that can accompany lupus. However, they are not disease-modifying drugs – they will not put lupus into remission or alter the underlying immune process.

The main side effect of NSAIDs is stomach irritation that can lead to ulcers. A new class of medications related to traditional NSAIDs, called COX-2 inhibitors, reduces this particular side effect.

- Anti-malarial drugs

You may well ask what malaria has to do with lupus? Nothing as it happens, except that many years ago it was fortuitously discovered that anti-malarial drugs reduced lupus symptoms in soldiers at war. Since then anti-malarial drugs have been used widely to treat lupus.
These drugs are particularly effective when the skin and joints are involved.
Two anti-malarial drugs are used in Australia to treat lupus – hydroxychloroquine and chloroquine.
Anti-malarial drugs such as hydroxychloroquine help bring lupus under control in many body systems. Skin rashes, mouth ulcers and hair loss improve in the majority of patients. Joint swelling and aching decreases.
Anti-malarial drugs have few side effects, although it may take several weeks before they start working. The most common side effect is nausea.
It is recommended that patients have their eyes examined every six months, as anti-malarial drugs, and in particular chloroquine, sometimes damage the eye.

- Corticosteroids

Corticosteroids are hormones, now produced synthetically, that have very similar effects to the steroids produced by our bodies. At their naturally low levels, they control a number of processes in our body. When used medicinally at higher doses, corticosteroids dampen inflammation and are very effective at relieving the symptoms of active lupus.

The dose of corticosteroids must be regulated to maximise benefit and minimise side effects. For patients with severe disease, they are the drugs of choice and are given at high doses. Moderate doses are taken during severe flare-ups of non-major organ-threatening disease. Patients with chronic, mild lupus receive low doses and are usually switched to another disease-modifying therapy that has fewer side effects.

At moderate to high doses, taken for weeks to months, corticosteroids can cause a variety of unwanted side effects such as heart palpitations, agitation, weight gain, changes in facial appearance, rapid heart rate and sleeplessness. Side effects from long term therapy (over a period of months to years) are more serious and include raised blood pressure, diabetes, increased risk of infection, and osteoporosis. Over time, corticosteroid doses can be reduced, but patients need to be "weaned" off the drug so that their own body can learn to produce the substance again. Prednisolone is an example of a commonly used corticosteroid.
- Immunosuppressants

As their name suggests, immunosuppressants suppress or dampen the immune system. In a disease that is caused by an over-active immune system, they are a logical and very effective choice. Commonly used immunosuppressants are cyclophosphamide and methotrexate.

Immunosuppressants can have serious side effects and for this reason they are usually reserved for severe cases of lupus. They are generally used when serious major organ-threatening disease is present and steroids alone are not enough to control the disease. They are also used when patients cannot tolerate high doses of steroids. For this reason, they are often referred to as "steroid-sparing agents", as they spare the patient the potentially toxic effects of long term steroid treatment.

Patients receiving immunosuppressants are monitored closely for such side effects as anaemia, lowered white blood cell counts and hair loss. They are also at an increased risk of infection and possibly cancer.

- Prognosis

In this day and age, with early detection and effective treatment the prognosis for lupus patients is not one of doom and gloom. The prognosis depends on a lot of things, and varies from patient to patient.
Most patients with milder forms of lupus can expect to live a normal life span. This includes subacute cutaneous lupus and SLE that is not major organ-threatening. For major organ-threatening lupus, life expectancy depends on the patient’s race, where they live (and whether they have access to good medical care), their age, their genes, and most of all how severe the disease is. Lupus is fatal in only a small percentage of cases.
- Lupus flares

Many patients experience lupus "flares" – periods where the symptoms of lupus return. Unfortunately, there is no way of knowing how long such flares will last or how severe they will be.
What triggers these flares is not entirely clear, and probably differs from person to person. Almost universally, however, emotional and lifestyle-related stresses are found to have at least some role in lupus flares. Sunlight, an infection, certain medications or even pregnancy may cause disease flares in some people. During these times it is particularly important that patients take good care of themselves.
- Lupus and pregnancy

As most women affected by lupus are of child-bearing age, it is natural that they will question whether they can become pregnant and successfully give birth to a healthy baby. The good news is that in the majority of cases, there is no reason why a woman with lupus cannot have children.
Conception

Before considering pregnancy it is important that patients talk to their doctor and are informed of any potential risks for themselves and the baby. It is preferable for lupus to be in remission and to ensure that any medication being taken will not adversely affect the pregnancy.
Lupus does not usually lower fertility, but sometimes women with lupus may have an antibody in their blood that increases the risk of miscarriage. Any woman with a history of more than two unexplained miscarriages should be tested for this antibody (the "anticardiolipin antibody"), as treatments to enable a successful pregnancy are available.


Q & A

Will lupus flare up during pregnancy or after the birth? Whether or not lupus will flare up during pregnancy cannot be predicted. However, lupus flares during pregnancy are far less likely if the illness has been quiet for the six months leading up to conception. If it does flare, it is likely to be mild and in the first trimester. After this, the foetus begins to produce hormones of its own that can help to keep the mother’s disease under control.
In the first weeks after birth new mothers may experience lupus flares but this can be controlled with corticosteroids.
Will lupus medications harm the foetus? Many medications are harmful to a developing foetus. Corticosteroids, however, are safe medications to use during pregnancy.
Will the baby be affected by lupus? A very small number of babies are born with what is known as "neonatal lupus". This is a type of lupus that is only seen when they are first born and does not persist past infancy. Whether or not a baby will develop this condition depends on whether the mother has certain antibodies in her bloodstream – if she does not, there is no risk. If she does, the risk is low but she will need to be monitored more closely during the pregnancy. In most babies neonatal lupus will clear up independently but in some it will require medical intervention.
Can babies be breast-fed? Babies can be breast-fed, although it is advisable to switch to formula three months after the birth. A breast-feeding mother cannot take most lupus medications because of the possibility they may harm the baby. The longer she breast-feeds the longer she is at risk of experiencing a disease flare.
Are my children at increased risk of lupus? While genetic factors play a role in increasing the risk of developing lupus, this illness is only "passed on" to 5-10% of successive generations. This highlights the fact that while genes are important, they do not fully explain why lupus appears in a certain individual and whether or not your children will develop lupus.
   

Lupus in different groups

Children

Although lupus is most common in young women, it is not unheard of in children. Most children are diagnosed around puberty, although there are rare cases in children younger than 5 years.
Symptoms in childhood lupus are very similar to adult lupus although they are usually more serious. For this reason, children are generally given more aggressive treatment that aims to control the disease before it involves major organs.

Men

Lupus is much more rare in men than women (occurring at a rate of approximately one man for every nine women) but does occur. Unlike women, who tend to develop the disease between the ages of 15 and 45 years, there is no distinct "risk period" where men are more likely to present with lupus. Men tend to experience slightly different symptoms that are often more severe than in women.
Older age groups

Few people develop lupus in old age and it is generally a much milder disease. Those who have had lupus for some time will usually find that their lupus calms down, with only a few symptoms such as photosensitivity and joint pain persisting.
 

Tips for living with lupus - Help yourself to a full life

Just because you have lupus does not mean you should stop living your life. Do your best to remain on top of the disease – a bit of careful planning and thought will go a long way.

Some tips for staying on top of lupus:

Tips


Make sure you get plenty of rest.
Make an effort to do some exercise this will help prevent muscle weakness and fatigue – but don’t overdo it.
Listen to your body if you feel fatigued, take a rest. This may mean taking daytime naps or restructuring your schedule.
Try to avoid becoming anxious or stressed, if you can’t avoid stressful situations, learn how to de-stress. Try yoga, meditation, walking, a relaxing bath or talk to your family, friends or support group about your anxiety.
Remember the sun can trigger flare-ups avoid excessive exposure to the sun and always cover up. Use maximum strength sunblock that protects against UVA and UVB rays
Avoid contact with people who have an infection potential sources of infection such as large crowds (buses, trains and concerts), children and babies.
Find out as much about lupus as you can this will help take away the fear of the unknown
Eat well from each food group if you are on a special diet, make sure you stick to it. Your doctor is trying to help you – so follow his or her instructions and advice.
Keep a list of your symptoms and any questions you might have for your doctor.
Always keep a record of your medications. inform your doctor regularly
Always take your medications as you have been instructed.
Visit your doctor regularly particularly if you feel as though the disease may be flaring up.
Certain things can trigger lupus see if you can work out common triggers for your symptoms. That way you can avoid them next time.
Do not smoking smoking will lower your health so try to quit
Do not over-exert yourself physically or emotionally.
You are not alone there are many other people in your situation. A support group is only a phone call away.


Commonly asked questions

Q & A

Why me? It is a good question and one that many people will ask. The short answer is – we don’t know. Scientists are working hard to find out why some people develop lupus while others do not. The answer may be the key to warding off the disease before it takes hold.
Is lupus genetic? There is good evidence that genetics play a role in deciding who will develop lupus. But just how large a role that is differs from person to person. Some people are more susceptible, genetically, to developing the disease. Whether or not they actually develop lupus may depend on their lifestyle or which viruses or medications they come into contact with.
Can other people ‘catch’ my lupus? Lupus is NOT infectious. There is no need for you to quarantine yourself.
Can I take oral contraceptives (the pill)? You will need to discuss this with your doctor, but in many women with lupus, oral contraceptives can be used without problems.
Do certain things trigger lupus flares? While there are no universally accepted triggers, you may find that sun exposure, certain foods, medications or activities affect your condition. Emotional stress is commonly implicated in lupus flares. It may be useful to keep a note of the things you did or ate prior to a lupus flare – this may help you to identify any triggers.
Am I going to have lupus for life? At present there is no "cure" for lupus, but there is effective medication that will bring the disease under control – often permanently. As you grow older, it is likely that the disease will settle down.
Is there a special diet I should follow? No, but you should eat a healthy, nutritious and well-balanced diet and try to maintain ideal body weight. If you know that a particular food triggers your lupus, it is obviously wise to avoid it.

 

Where can I go for further information and support

If you have any questions about lupus or your medication, you should ask your doctor. In addition, there are a number of lupus associations and support groups across Australia that can provide invaluable help to lupus patients.

These organisations provide support and information to patients and their families, as well as promote understanding and awareness of the condition in the community. Further research and development in lupus and its treatment is funded by the efforts of these organisations.

Contact your nearest Australian Lupus Association or support group at the address listed below:


Lupus Association of NSW Inc PO Box 89
North Ryde NSW 1670

Tel 02 9878 6055
Fax 02 9878 6049

info@lupusnsw.org.au
www.lupusnsw.org.au

Freecall (NSW Country & Interstate )
1800 802 088
Lupus Australia, Qld Inc P.O. Box 974
Kenmore QLD 4069

Tel 07 3878 9553
Fax 07 3878 9557

mail@lupus.com.au
www.lupus.com.au
Lupus Group of WA PO Box X2213
Perth WA 6847

Tel 08 9224 3144
Fax 08 9224 3144

lupus@wanet.com.au
www.lupuswa.com.au
Lupus Association of Tasmania PO Box 639
Launceston TAS 7250

Tel 03 6331 9940

lupustas@bigpond.net.au
www.users.bigpond.net.au/lupustas
Scleroderma/Lupus Support Society Inc Scleroderma/Lupus Support Society Inc.
PO Box 373
Warners Bay NSW 2282
Ph. 0249 214095
Fax 0249 855347
Janine.Furner@hnehealth.nsw.gov.au
www.haps.nsw.gov.au
   
 

The history of Lupus

- Lupus through the ages
Lupus was first described way back in the time of Hippocrates in Ancient Greece.

- The history of lupus

"Lupus" means "wolf" in Latin. There are at least two explanations why the word "lupus" was chosen to describe the disease we now know as systemic lupus erythematosus.

One explanation is that lupus was so named because the common butterfly rash seen on the cheeks and nose of many lupus patients is similar to the facial markings of a wolf. An alternative explanation relates to the early use of the word "lupus" to describe skin ulcers. In the sixteenth century, certain skin ulcers were compared to a hungry wolf eating the sufferer’s flesh.

In the 1800s doctors named another disease involving skin lesions "lupus erythematosus". These lesions were not ulcers but rather were red regions where the skin was thinner than normal.

When doctors discovered that a bacterium was responsible for many of the skin ulcers that were called lupus and that these ulcers cleared up with antibiotic treatment, they realised that lupus erythematosus was a separate condition. It was not until 1872 that doctors realised lupus erythematosus affected parts of the body other than the skin. The condition was given the name lupus erythematosus disseminatus, and the first of the symptoms were documented.

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