Systemic lupus erythematosus, referred to as SLE or lupus, is a chronic (long-term) disease that causes systemic inflammation which affects multiple organs.
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), heart (pericardium), and brain. Many patients experience fatigue, weight loss, and fever.
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 a remission. Yet, there is much reason for hope. Improvements in treatment have greatly improved these patients’ quality of life and increased their lifespan.
When healthy, our immune system protects the body from germs and cancers. With lupus, the immune system misfires and attacks “self”, the patient’s own tissues, in a process called autoimmunity or “loss of self-tolerance”.
In lupus as the attack goes on, all the branches of the immune system join the fight. This leads to significant and intense inflammation. The cause of lupus is unknown, as well as what drives its diverse presentation. We know that multiple factors are required, including: the “right” genetic makeup, environmental exposures and organ specific characteristics. People with lupus may also have an impaired process for clearing old and damaged cells from the body, which in turn provides continuous stimuli to the immune system and leads to abnormal immune response.
Most often, lupus starts in young females in their fertility age, although it can sometimes start during childhood (see section on Childhood-onset lupus below). The disease is more common in some ethnic groups, mainly blacks and Asians, and tends to be worse in these groups.
About 20% of people with lupus develop the disease as children or teens. When lupus starts in childhood, it is called childhood-onset SLE, or cSLE. It is rare to get lupus before age 5 years.
Lupus can be hard to detect because it is a complex disease that has many symptoms, and they can come on slowly. As experts in diagnosing and treating autoimmune diseases such as lupus, rheumatologists can best determine whether a patient has lupus and advise them about treatment options.
People with lupus often have symptoms that are not specific to lupus. These include fever, fatigue, weight loss, blood clots and hair loss. They may also have heartburn, stomach pain and poor circulation to the fingers and toes. Pregnant women can have miscarriages. Lupus can flare during pregnancy and can affect its outcome.
The American College of Rheumatology has a list of symptoms and other measures that doctors can use as a guide to decide if a patient with symptoms has lupus.
If your doctor suspects you have lupus based on your symptoms, a series of blood tests will be done in order to confirm the diagnosis. The most important blood screening test is ANA. If ANA is negative, you don’t have lupus. However, if ANA is positive, you might have lupus and will need more specific tests. These blood tests include antibodies to anti-dsDNA and anti-Sm, which are specific to the diagnosis of lupus.
The presence of antiphospholipid antibodies signals a raised risk for certain complications such as miscarriage or blood clots. 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.
There is no cure for lupus, as it is a chronic disease. The treatment goals are to suppress the overactive immune system and ultimately induce remission and prevent permanent organ damage. The types of medications required will depend on the symptoms.
Treating cSLE requires close monitoring by a rheumatology team experienced in caring for children with lupus. The team may include doctors (pediatric rheumatologists and other specialists), nurse practitioners, physician assistants, nurses, social workers, counselors, and physical and/or occupational therapists. They will partner with the family, schools and other community resources to provide a child or teen with the best care for preserving physical and psychological health.
Common treatment options include:
Lupus disease, especially when active, could lead to accelerated atherosclerosis (clogging of the arteries) which can develop in young women and could also lead to heart attacks, heart failure, and strokes. Thus, it is vital that patients with lupus, in addition to controlling their disease, exercise and lower other risk factors for heart disease, such as smoking, high blood pressure, and high cholesterol.
Renal inflammation is one of the common and most serious manifestations of lupus. It could go undetected and can lead to renal failure and dialysis. You can help prevent these serious outcomes by seeking treatment at the first signs of kidney disease. These signs include:
Most people with lupus can live normal lives. Treatment of lupus has improved, and people with the disease are living longer. Here are a couple of tips that may help you when living with lupus:
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. If you must take any of these medicines, or your disease is very active, use birth control. For more information, see Pregnancy and Rheumatic Disease.
Rheumatologists have long been concerned that the female hormone estrogen or treatment with estrogen may cause or worsen lupus, and may raise the risk of blood clots. Thus, you should avoid estrogen if your blood tests show the presence of antiphospholipid antibodies (meaning you already have a high risk of blood clots).
Children with cSLE
Hearing that your child has lupus can be frightening. However, by understanding the disease and working closely with the health care team, you can learn how best to take care of your child.
There may be times when children and teens with lupus must take time off from school to allow their bodies to heal. Otherwise, they are encouraged to live as normal a life as possible. Going to school, playing with friends, exercising, having a healthy diet and continuing family activities are all important.
Many children and teens experience several emotions related to lupus and its impact on his or her life. These can include stress, sadness, anxiety and anger. Many parents, caregivers and siblings also experience emotional challenges. It is important to recognize these feelings and provide time to talk about them. It is also important to get help when emotional challenges are causing significant distress (for example, interfering with regular activities). You can talk to members of the rheumatology team (doctor, nurse or social worker) to help identify resources for emotional health support for young patients and their families.
Updated December 2021 by the American College of Rheumatology Committee on Communications and Marketing.
This patient fact sheet is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.