Systemic Lupus Erythematosus

What is lupus?

Systemic lupus erythematosus (lupus) is a disease of the immune system. Normally, the immune system protects the body from infection.

However, in lupus, the immune system inappropriately attacks tissues in various parts of the body.

In addition to affecting the skin and joints, it can affect other organs in the body such as the kidneys, the tissue lining the lungs (pleura) and heart (pericardium), and the brain.

Most patients feel fatigued and have rashes, arthritis (painful and swollen joints) and fever.

This abnormal activity of the immune system leads to tissue damage and illness.

Who is affected by lupus?

Lupus can affect men and women of any race or age. People of African, Asian and Native American descent are more likely to develop lupus than are Caucasians.

If only women of childbearing age (14 to 45 years old) are considered, as many as 1 in 250 may develop lupus.

This suggests a possible role for female hormones influencing vulnerability to this disease.

What problems may lupus patients develop?

Many patients with active lupus feel poorly in general and complain of fever, weight loss and tiredness. Patients with lupus also develop specific problems when the immune system attacks a particular organ(s) or area(s) in the body.

The blood may also be affected during the course of lupus, resulting in low red blood cell count (anaemia, low white blood cell count and low platelet count).

Skin problems are a common feature of lupus. Some patients with lupus have a red rash over their cheeks and the bridge of their nose. Other skin problems that occur include large red, circular rashes (plaques), which may scar (called discoid lupus). Skin rashes are usually aggravated by sunlight. Hair loss and sores in the mouth or nose lasting from a few days to more than a month are also common.

Joints: Arthritis is very common in people who have lupus. There may be pain, with or without swelling. Stiffness and pain may be especially evident in the morning.

Kidney involvement in people with lupus is potentially life-threatening and may occur in up to half of the lupus patients. Kidney problems may become apparent when lupus patients feel ill with arthritis, have a rash, fever and weight loss. Less often, kidney disease may occur when there are no other symptoms of lupus. Kidney disease itself usually does not produce symptoms until it is in the advanced stages. It is important that kidney disease be diagnosed early and treated appropriately. The earliest signs of kidney disease are apparent from a urinalysis.

Blood involvement can occur with or without other symptoms. Patients may have dangerous reductions in the number of red blood cells, white blood cells or platelets (cells that help clot the blood).

Sometimes changes in blood counts may contribute to symptoms of fatigue (low red blood cell count, anaemia), serious infections (low white blood cell count), or easy bruising > (low platelet count).

However, many patients do not have symptoms that indicate blood abnormalities, so it is important for lupus patients to have periodic blood tests in order to detect any problems.

Blood clots are seen with increased frequency in lupus.

Clots often occur in the legs (a vein clot, called deep venous thrombosis deepveinthrombosisdvt, lungs (a lung clot, called pulmonary embolus pulmonary embolism, or brain.

Blood clots that develop in lupus patients may be associated with the production of antiphospholipid antibodies. These antibodies are abnormal proteins that may increase the tendency of the blood to clot.

Brain involvement is, fortunately, a rare problem in people with lupus. When present, it may cause confusion.

Heart and lung involvement is often caused by inflammation of the covering of the heart (pericardium) and lungs (pleura). When these structures become inflamed, patients may develop chest pain when breathing deeply, irregular heartbeat > and accumulation of fluid around the lungs (pleuritis or pleurisy and heart (pericarditis):

What causes lupus?

The cause of lupus is unknown.

It most likely results from a mix of inherited tendencies and things in your environment. These include viruses, sunlight and drug allergies.

People with lupus may also have an impaired process for clearing old and damaged cells from the body, which causes an abnormal immune response.

The immune system is the body’s defence system. When healthy, it protects the body by making antibodies (blood proteins) that attack foreign germs and cancers.

With lupus, the immune system misfires. Instead of producing protective antibodies, an autoimmune disease begins and makes “autoantibodies,” which attack the patient’s own tissues.

As the attack goes on, other immune cells join the fight. This leads to inflammation and abnormal blood vessels. These antibodies then end up in cells in organs, where they damage those tissues. Why this inflammatory response begins is not clear.

Female hormones are believed to play a role in the development of lupus because women are affected more commonly than men. This is especially true of women during their reproductive years, a time when hormone levels are highest.

About 10 per cent of lupus patients have a close relative with lupus.

How is lupus diagnosed?

The diagnosis of lupus is best made by an experienced clinician who fully understands the disease and other diseases with similar features that can mimic lupus.

The diagnosis is made when a patient has several features of the disease (including symptoms, findings on examination and blood test abnormalities.

The American College of Rheumatology has devised criteria to assist clinicians in making the correct diagnosis of lupus.

If your doctor suspects you have lupus from your symptoms, you will need a series of blood tests to confirm that you do have the disease.

The most important blood screening test measures ANA, but you can have ANA and not have lupus.

Therefore, if you have positive ANA, you may need more specific tests to prove the diagnosis. These blood tests include antibodies to anti-dsDNA and anti-Sm.

The presence of antiphospholipid antibodies can help doctors detect lupus.

These antibodies signal a raised risk of certain complications such as miscarriage, difficulties with memory, or blood clots that may lead to stroke or lung injury.

Doctors also may measure levels of certain complement proteins (a part of the immune system) in the blood to help detect the disease and follow its progress.

Does a positive antinuclear antibody (ANA) test mean that I have lupus?

Not necessarily. The antinuclear antibody (ANA) test > is positive in most patients with lupus, but it may also be positive in many people who do not have lupus. Therefore, a positive ANA test alone is not adequate for the diagnosis of lupus; there must be at least three additional clinical features for the diagnosis to be made.

How is lupus treated?

There is no cure for lupus, and treating lupus can be a challenge. However, treatment for lupus has improved a great deal.

The type of treatment prescribed will depend on several factors, including the person’s age, type of medications he or she is taking, overall health, medical history and location and severity of the disease.

Because lupus is a condition that can change over time and is not always predictable, a critical part of good care includes periodic visits with a knowledgeable, available physician.

Some patients with mild features of the disease do not require treatment, while patients with serious involvement (such as kidney complications) may require the use of powerful medications. Medications used to treat lupus include:

Steroids or prednisone and related derivative of cortisone. Steroid creams can be directly applied to rashes. The use of creams is usually safe and effective, especially for mild rashes.

The use of steroid creams or pills in low doses can be effective for mild or moderate features of lupus. Steroids can also be used in higher doses when internal organs are threatened. Unfortunately, high doses are also most likely to produce side effects.

Hydroxychloroquine is commonly used to help keep mild lupus-related problems, such as skin and joint disease, under control.

Cyclophosphamide is a drug that has very powerful effects on reducing the activity of the immune system. It is used to treat severe forms of lupus.

Azathioprine: It is commonly used to treat the more serious features of lupus.

Methotrexate is another chemotherapy medication used to suppress the immune system. Its use is becoming increasingly popular for skin disease, arthritis, and other nonlife.

Threatening forms of disease that have not responded to medications such as hydroxychloroquine or low doses of prednisone.

Cyclophosphamide and mycophenolate mofetil are chemotherapy drugs that have very powerful effects on reducing the activity of the immune system. They are used to treat more severe forms of lupus, especially lupus that affects the kidneys.

Belimumab is a monoclonal antibody that reduces the activity of white blood cells (lymphocytes) that make autoantibodies. Autoantibodies are important as they cause tissue damage.

Belimumab is used to treat lupus that does not involve the kidneys and has not responded to other types of treatments.

Rituximab is also a monoclonal antibody that reduces the activity of white blood cells (lymphocytes) that make autoantibodies. It is sometimes used to treat lupus that has not responded to other types of treatments.

Combination treatment: Health care providers may combine a few medications to control lupus and prevent tissue damage. Each treatment has risks and benefits. Most immune-suppressing medications, for instance, may cause major side effects. Side effects of these drugs may include a raised risk of infections as well as nausea, vomiting, hair loss, diarrhoea, high blood pressure and osteoporosis (weak bones).

Lupus flares vary from mild to serious. Most patients have times when the disease is active, followed by times when the disease is mostly quiet — referred to as remission. Yet, there is much reason for hope.

Improvements in treatment have greatly improved these patients’ quality of life and increased their lifespan.

Rheumatologists may lower the dose of a drug or stop a medicine because of side effects or when the disease goes into remission. As a result, it is important to receive careful and frequent health exams and lab tests to track your symptoms and change your treatment as needed.

Broader health impact of lupus

Even when it is not active, lupus may cause problems later. Some of these problems can be fatal. One of these problems is atherosclerosis (clogging of the arteries) that may develop in younger women or maybe more severe than usual.

This problem raises the risk of heart attacks, heart failure and strokes.

Thus, it is vital that patients with lupus lower their other risk factors for heart disease, such as smoking, high blood pressure and high cholesterol. It is also important to have as active a lifestyle as possible.

Lupus may also cause kidney disease, which can advance to kidney failure and need dialysis: High blood pressure, Swollen feet and hands, Puffiness around your eyes and Changes in urination (blood or foam in the urine, going to the bathroom more often at night, or pain or trouble urinating)

Living with lupus

Most people with lupus can live normal lives.

Treatment of lupus has improved, and people with the disease are living longer. But, it is still a chronic disease that can limit activities.

Quality of life can suffer because of symptoms like fatigue and joint pain. Furthermore, some people do not respond to some treatments.

Stay active. Exercise helps keep joints flexible and may prevent heart disease and strokes. This does not mean overdoing it. Switch off doing light to moderate exercise with times of rest.

Avoid excess sun exposure. Sunlight can cause a lupus rash to flare and may even trigger a serious flare of the disease itself. When outdoors on a sunny day, wear protective clothing (long sleeves, a big-brimmed hat) and use lots of sunscreens.

If you are a young woman with lupus and wish to have a baby, carefully plan your pregnancy. With your doctor’s guidance, time your pregnancy for when your lupus activity is low. While pregnant, avoid medications that can harm your baby. These include cyclophosphamide, cyclosporine, and mycophenolate mofetil.