What is systemic lupus erythematosus?
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various part of body. It is caused by immune system that mistakenly attacks the body's own tissue, which leads to inflammation in the skin, joints, kidneys, lungs, nervous system, and other organs.
Systemic lupus erythematosus in children
Systemic lupus erythematosus typically begins to be seen in the teenage years (average age is 12). It is rarely seen in children before the age of 5. It is more common in females and in certain ethnic groups, such as African-American, Hispanic, South and Southeast Asian, and Native American.
Systemic lupus erythematosus affects children in the same ways that it affects adults except the disease can be more severe, involve more organs, and have more laboratory abnormalities.
Symptoms and Causes
What causes systemic lupus erythematosus?
The exact causes of systemic lupus erythematosus are unknown. In general, patients who have lupus are born with genetic factors that cause the immune system to become overactive. However, not all patients with a genetic predisposition develop lupus. Some environmental trigger kicks off a response in the immune system to cause lupus. These possible triggers include:
- Sun exposure
- Drug reaction
- Exposure to cigarette smoke
- Hormones during puberty
What are the signs and symptoms of systemic lupus erythematous?
Signs and symptoms include:
- Red rash on the cheeks and bridge of the nose (appears in about one-third of cases)
- Disc-shaped rash with raised patches, with possible scarring from previous outbreaks
- Skin rash due to sun exposure
- Sores in the mouth or nose
- Arthritis in two or more of the small joints
- Fluid surrounding the heart or lungs
- Kidney problems
- Seizures or psychosis
- Low red blood cell (anemia), platelet, or white blood cell counts
- Abnormal antibody testing
- High laboratory readings for anti-nuclear antibodies (ANA)
Diagnosis and Tests
How is systemic lupus erythematosus diagnosed?
Systemic lupus erythematosus is diagnosed if four or more of the above-mentioned signs and symptoms occur, either over a period of time or at the same time, with no other explanation for their appearance.
Other factors the doctor considers before making the diagnosis include:
- Family history
- Age at which the signs and symptoms occurred
- How long the signs and symptoms have been present
- Findings on physical examination
Management and Treatment
How is systemic lupus erythematosus treated?
Currently, there is no cure for systemic lupus erythematosus, but proper treatment can help greatly. The main goal of treatment is to ease symptoms and to prevent the immune system from attacking vital organs before permanent damage occurs. When treating children, this requires the attention of a team of specialists, including a pediatric rheumatologist, nurses, counselors and physical therapists who can provide necessary treatment to ensure the child's physical and psychological health.
Counseling may be necessary to help the child or teen deal with the fact that he or she has the disease, and to stay motivated to keep doctors' appointments, get regular blood tests, and take medications.
Medications will vary, depending on how severe the patient's symptoms are. Medications include:
- Corticosteroids (prednisone) to control inflammation
- Hydroxychloroquine (Plaquenil®), an anti-malaria drug used to control disease flare-ups
- Immunosuppressants such as azathioprine (Imuran®), mycophenolate mofetil (Cellcept®), methotrexate, cyclophosphamide (Cytoxan®), rituximab (Rituxan®) and Belimumab (Benlysta®)
- Pain relievers such as ibuprofen or naproxen
- Calcium and vitamin D supplements to prevent osteoporosis (a possible side effect of long-term use of corticosteroids)
What happens after treatment for systemic lupus erythematosus?
The goal of treatment is to put the disease into remission state (no active disease) in order to prevent organ damage and improve quality of the child’s life. However, the disease could flare-up, so it is important that the child closely follows the treatment plan and recommendations and sees the doctor on a regular basis.
Prednisone is one of the fastest-acting and the most effective treatments for controlling active inflammation in lupus. However, long-term use may lead to serious side effects, such as a suppressed immune system, weight gain, stretch marks, high blood pressure, osteoporosis, depression, diabetes, glaucoma, and cataracts. These conditions can have a big impact on the child's appearance, psychological well-being, and normal growth and development. Therefore, it is important for the patient to see his or her doctor on a regular basis to keep track of these side effects.
Hydroxychloroquine may, in very rare cases, cause permanent damage to the retina. All children receiving the drug should have regular checkups with an eye doctor.
Other medications, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, can decrease the number of white blood cell count or cause inflammation in liver. Blood tests for its adverse effects will be monitored regularly.
Rituximab and belimumab reduce the activity of certain immune cells, called B-cells, which are part of the body’s normal immune system to fight invading organisms. Although these drugs calm the overactive immune system that is seen in systemic lupus erythematosus, they may increase the risk of infection. Close monitoring is required.
Can systemic lupus erythematosus be prevented?
Since the causes of systemic lupus erythematosus are not fully understood, it is not known how to prevent it. Flare-ups of the condition may be reduced by following all of these recommendations:
- Avoid sun exposure.
- Wear sunscreen and protective clothing when going outdoors.
- Get enough sleep (7 to 9 hours/night).
- Take all medications as prescribed.
Outlook / Prognosis
What is the long-term outlook for children who have systemic lupus erythematosus?
Children who receive proper treatment for systemic lupus erythematosus generally have very good outcomes. Children with systemic lupus erythematosus may have a greater risk for early atherosclerosis (clogging of the arteries) and heart disease when they become young adults.
Other issues that may affect treatment and outcomes include:
- Failure to take medicines or follow the treatment plan
- Problems with the nervous system
- Other infections
- Kidney disease
- Difficulty accessing the healthcare system to receive proper care
Living with systemic lupus erythematosus
Children with systemic lupus erythematosus should live life as normal as possible. They should attend school, play sports, and participate in extra-curricular and family activities. There are no specific limitations to physical activity the children want to do (as long as it is safe). Outdoor activity could be done but your child need to be diligent with wearing sunscreen and cover skin with UV protection clothes. Exercise does not harm children with systemic lupus erythematosus, and in fact, it increases muscle strength and muscle endurances, lifts up energy and prevents the decline in the ability to do activities.
There is no special diet that has been showed to be effective for systemic lupus erythematosus nor worsen the disease. Therefore, children with systemic lupus erythematosus should eat standard well-balanced healthy diet.
Is there supporting group for systemic lupus erythematosus?
Lupus Foundation is a leading non-profit organization that is dedicated to addressing the needs of adults and children who lives with systemic lupus erythematosus in the United States. There are many local offices of Lupus Foundation throughout the US. Many local and national events are organized to educate and connect patient/family together.
More information on the Lupus Foundation can be found at https://www.lupus.org