Lupus is a chronic condition that results from a malfunctioning immune system. It most commonly appears in women of childbearing age.
Types of lupus
The four main types of lupus are:
- systemic lupus erythematosus (SLE)
- discoid lupus erythematosus (DLE)
- drug-induced lupus
- neonatal lupus.
Systemic lupus erythematosus (SLE)
The immune system is designed to identify foreign bodies (such as bacteria and viruses) and attack them to keep us healthy. However, in the case of lupus, the immune system mistakenly attacks normal, healthy tissues (including the skin, joints, kidneys and lining of the heart and lungs), causing ongoing inflammation and pain.
SLE can range from mild to life-threatening. This very much depends on the parts of the body that are being attacked by the immune system. While pain and inflammation of the skin and joints can impact on your quality of life, the damage that SLE may cause to your major organs, such as the kidneys or nervous system, is much more serious.
The most common forms of SLE are the milder forms, and most people with lupus enjoy a full life, even though they may need to take medications.
Discoid lupus erythematosus (DLE)
Discoid lupus erythematosus (DLE) is a chronic skin condition that appears as reddened and round scaly patches that tend to develop in sun-exposed areas such as the face and hands. Sometimes, extensive lesions can develop across other areas of the body, including the neck and upper back.
It is unclear whether DLE is a separate disease or a milder version of SLE, which can affect any of the body’s organs or tissues. Around five per cent of people with DLE go on to develop SLE, and around 20 per cent of people with SLE develop DLE.
There is no cure, but the skin lesions can be managed with medication and by avoiding exposure to sunlight.
Certain medications can provoke SLE-like symptoms, which then disappear once the medications are stopped. Drugs that are known to cause lupus-like symptoms include certain hypertensive (high blood pressure) medications and drugs for heart abnormalities.
Men are more likely to experience drug-induced lupus because they take more of these particular medications than women. Some researchers suspect that genetic susceptibility may play a significant role.
This is a rare form of temporary lupus that affects a fetus or newborn baby (usually becoming obvious in the first few months of life). It occurs when the autoantibodies of a mother with SLE are passed to her child in utero. These autoantibodies can affect the skin, heart and blood of the baby.
Neonatal lupus sometimes appears as a rash (resembling DLE) developing soon after birth, and can last several months before disappearing. It is not a permanent condition, but half of all babies born with neonatal lupus may present with a heart condition.
Infants born with neonatal lupus are not at an increased risk of developing SLE later in life.
Lupus and gender
Women in their child-bearing years are the most likely to develop lupus. Nine times more women have lupus than do men.
Some researchers believe that sex hormones may play a role in the development of the disease. The principle hormones under investigation include the female hormone oestrogen, which is made by the ovaries and triggers ovulation every menstrual cycle, and the male hormones called androgens, which are responsible for masculine physical characteristics.
Research findings include that:
- Both oestrogen and androgens may be metabolised differently in people with lupus than in the general population.
- Women with lupus metabolise androgens much faster than women without lupus.
- Low levels of male hormones at pre-puberty and old age may contribute to the incidence of autoimmune diseases in males of these age groups.
Certain ethnic groups (for example, African-American and Asian people) are also more likely to develop lupus than others. However, lupus can affect men, children and older people as well.
Symptoms of lupus
A host of different symptoms can occur, but one person is unlikely to have all of them. The symptoms of lupus may include:
- skin rashes (both on the face and body)
- joint and muscle pain
- hair loss
- mouth and nose ulcers
- chest pain (as a result of inflammation of the lining of the heart or lungs)
- anaemia (a deficiency in the number or quality of red blood cells)
- poor kidney function
- seizures or visual disturbances (resulting from inflammation of the nervous system)
Symptoms of DLE
The symptoms of DLE may include:
- reddened, scaly and round lesions on the skin. Sun-exposed areas such as the face and back of the hands are the most common sites. Other areas that may be affected include the neck and upper back
- permanent bald patches (scarring alopecia), if the scalp is affected
- scars or discoloured patches left by the lesions after they heal
- blotching of the skin on the legs (reticulate telangiectasia)
- Raynaud’s phenomenon (reduced blood flow to extremities)
- joint pains
- sun sensitivity.
The cause of lupus
The cause of lupus is unknown. However it appears that genes may play a role, in conjunction with triggers such as an illness, injury or a period of stress.
Diagnosis of lupus
Lupus is a difficult condition to diagnose. There is no single medical test for lupus, and the symptoms can vary greatly from one person to another. The symptoms can often mimic those of other diseases, and it may take months or years to arrive at a diagnosis of lupus.
A diagnosis of SLE is often made through a combination of the history of your symptoms, physical examination and blood tests that focus on particular antibodies such as ANA (antinuclear antibodies test) and other blood tests such as ESR (erythrocyte sedimentation rate), c – reactive proteins and others.. As well as tests such as urine tests and tissue biopsies depending on the areas of the body affected. Test results also help rule out other conditions that may have similar symptoms.
Early diagnosis is important (as soon as possible after the appearance of symptoms), because internal organs such as the kidneys can be affected if targeted by the disease.
Usually a period of high lupus activity (a flare up) is followed by a period of remission.
Diagnosis of DLE
Diagnosis involves differentiating DLE from SLE, as the skin lesions may be the same or very similar. Tests include:
- physical examination
- medical history
- blood tests
- biopsy of a skin lesion.
Treatment of lupus
There is no way to cure or prevent lupus, but the disease and its symptoms can be well controlled with medication.
Treatment of SLE
Treatment of SLE may include:
- non-steroidal anti-inflammatory drugs (NSAIDs)
- oral cortisone
- oral hydroxychloroquine (plaquenil) – anti-malarials
- disease-modifying anti-rheumatic drugs (DMARDs)
- immunosuppressants azathioprine.
Treatment of DLE
Treatment options for DLE may include:
- topical steroid creams, applied to affected areas of skin
- plastic wrapping of the skin to increase the absorption of steroid creams
- injections of medication, in the case of exceptionally thick skin lesions that don’t respond to creams
- antimalarial drugs such as plaquenil
- other medications, such as those used for psoriasis
- oral steroids or disease-modifying anti-rheumatic drugs (DMARDs), if SLE is also present
- sun avoidance.
Staying out of the sun is perhaps the most important thing you can do to manage DLE. The ultraviolet (UV) radiation in sunlight can trigger or worsen an attack.
- Avoid exposing yourself to direct sunlight whenever possible.
- Cover as much of your skin as you can with clothes such as long-sleeved shirts, trousers, gloves, broad-brimmed hat and so on.
- Always wear sunscreen lotion on all exposed areas of skin when you go outside.
- Choose sunscreens that protect against both UVA and UVB.
- Wear sunscreen even in winter or on cloudy days – any degree of ultraviolet radiation on the skin should be avoided.
- Remember that ultraviolet radiation is not stopped by window glass and is reflected off surfaces like concrete, snow and water.
- Be aware that some fluorescent tubes emit ultraviolet radiation.
- Wind and cold temperatures may also affect some people with DLE.
- Your doctor may also check your vitamin D level to see if it is adequate.
Support for people with lupus
It is natural to feel overwhelmed by a diagnosis of lupus, as there is currently no cure and it can affect many parts of your life. You may lose your independence, self-esteem, ability to work or continue social or recreational activities. You may feel scared, frustrated, sad or angry.
It is important to acknowledge these feelings and get help if they start affecting your daily life. Your doctor, specialist or other health professional will be able to provide you with information about support that is available.
It can also be helpful to contact a Lupus Support Group and speak to other people who also have lupus. Contact Arthritis and Osteoporosis Victoria for more information.
Self-management is very important for people with lupus, including finding out as much about your condition as you can, getting enough rest, identifying triggers to reduce the incidence of flares, doing regular exercise, reducing anxiety and stress, maintaining a healthy diet and not smoking.
Where to get help
- Your doctor
- Specialist (often a dermatologist, rheumatologist, nephrologist or immunologist)
- Arthritis and Osteoporosis Victoria Tel. (03) 8531 8000 or 1800 263 265
- Musculoskeletal Help Line Tel. 1800 263 265
Things to remember
- Lupus is an autoimmune disease that can be mild or life-threatening.
- The most common forms of lupus are the milder forms, and most people with lupus enjoy a full life.
- There is no cure, but lupus and its symptoms can be controlled with medication.
- Discoid lupus erythematosus is a chronic skin condition in which reddened scaly patches develop in sun-exposed areas of the body such as the face and hands.
- Staying out of the sun is perhaps the most important thing you can do to manage DLE.
- Certain medications can provoke SLE-like symptoms (drug-induced lupus), which then disappear once the medications are stopped.
Neonatal lupus sometimes appears as a rash at birth, or developing soon after, and can last several months before disappearing.