Louis Roller takes a look at the types of lupus, its causes and treatments
Lupus is a chronic autoimmune disease that can affect many different parts of the body. There are two main types of lupus, which differ significantly in the type and severity of symptoms.
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that has the potential to affect almost any organ in the body and cause generalised and systemic symptoms. The prevalence of SLE in Australia ranges from 19 to 93 per 100 000 people, with prevalence at the higher end of the range reported in Indigenous Australians.
Females are more commonly affected by SLE than males, but peak incidence is during childbearing years (15 to 45 years of age) in women.
The clinical course of SLE is often one of remission and relapse. The prognosis of SLE depends on the nature and severity of organ involvement. Patients who present with and continue to have mild symptoms in early disease are generally unlikely to progress to more severe disease.
Patients with a younger age of onset and certain ethnic groups (eg Asians, Indigenous Australians) have an increased risk of more severe disease. Modern treatments have improved the 5-year survival of SLE to around 95%; however, the mortality rate is still higher in patients with SLE compared to the general population, with atherosclerotic cardiovascular disease the major cause of mortality in patients with SLE.
Discoid lupus (also known as chronic cutaneous lupus erythematosus) is generally milder than SLE and usually appears as a red scaly rash on sun exposed areas such as the face, scalp, arms, legs or trunk. Most people with discoid lupus have symptoms only on their skin.
However, a small number of people with discoid lupus will develop SLE.
Other milder forms of lupus include:
Subacute cutaneous lupus —the main symptoms are skin rashes, sun sensitivity and joint aches.
Drug induced lupus—this is usually a transient form that develops as a reaction to certain medications and clears up when the medications are ceased.
The cause of lupus is unknown. A combination of genetic and environmental factors contribute to the formation of the antibodies that lead to lupus. Possible triggers of disease flare ups include: hormones, certain medications and chemicals, viral and bacterial infections, exposure to UV light, dietary factors, stress and pregnancy.
Drug-induced lupus erythematosus is similar to SLE. It is an autoimmune disorder and is caused by an overreaction to a medicine.
The most common medicines known to cause drug-induced lupus erythematosus are: isoniazid, hydralazine and procainamide
Other less common drugs may also cause the condition. These may include: anti-epileptic medicines, capoten, chlorpromazine, tumor-necrosis factor (TNF) alpha inhibitors (such as etanercept, infliximab and adalimumab), methyldopa, minocycline, quinidine and sulfasalazine.
Lupus symptoms and course of the condition may be vague, variable and unpredictable and can cause many symptoms, including:
- joint pain or swelling – seen in ~50% of people with lupus
- skin rashes that get worse with sun exposure – seen in ~20% of people with lupus
- loss of appetite and weight loss
- fatigue, weakness and lethargy—these affect ~10% of people with lupus and may be severe.
Early diagnosis is important. The diagnosis of lupus is usually suspected on the basis of clinical symptoms and signs and confirmed by laboratory tests.
There are currently no cures for lupus, but there are effective medications that will bring the disease under control, and often permanently.
The aim of lupus treatments is to reduce inflammation in tissues and improve quality of life. Treatment must be individualised, taking account of the severity of the disease.
There are five main groups of drugs that are used to treat lupus:
- NSAIDs and Cox-2 inhibitors reduce inflammation and can relieve the fevers, muscle aches, and arthritis that accompany lupus.
- Anti-malarial drugs (such as hydroxychloroquine and chloroquine) are often used to reduce joint pains, skin rashes and fatigue.
- Corticosteroids (such as prednisolone) are very effective anti inflammatory medications and are the drugs of choice for treating serious complications of lupus, such as those affecting the heart, lungs and nervous system.
- Immune suppressing drugs (such as methotrexate) suppress the immune system and are generally used when serious disease is present and steroids alone are not enough to control the disease.
- Cytotoxic drugs (such as cyclophosphamide) are potent immunosuppressive agents that are used to treat serious manifestations of SLE, especially kidney inflammation.
Early detection, effective treatment and some lifestyle adjustments enable most people with lupus to feel well and live normal lives. It is only a small minority of people who find the condition substantially reduces their quality of life.
Pregnancy: Women with lupus should talk to their doctor before considering pregnancy. They should be made aware of any potential risk for themselves and the baby.
Some tips to help people with lupus to lead normal lives:
- take rests before becoming fatigued;
- commence a program of regular moderate exercise;
- avoid excessive exposure to the sun;
- avoid contact with people who have known infections;
- educate oneself about lupus;
- visit doctor regularly;
- always take medications as instructed and keep a record of medications;
- do not smoke; and
- limit alcohol intake.
Associate Professor Louis Roller, from the Faculty of Pharmacy and Pharmaceutical Sciences Monash University, was the 2014 recipient of the PSA Lifetime Achievement Award.