What is systemic Lupus Erythematosus
(SLE) or Lupus?
Systemic lupus erythematosus, also known as SLE, or simply lupus, is a disease that is characterized by periodic episodes of inflammation of and damage to the connective tissues and organs, including the heart, lungs, blood vessels, brain, kidneys, and skin. The kidneys, heart, lungs, and brain are the organs most affected. Lupus affects each individual differently and the effects of the illness range from mild to severe. Lupus potentially can be fatal.
Lupus is more common in young women (late teens to 30s). This may be due to the fact that estrogen (a female hormone) seems to be associated with SLE in some cases. Men can also be affected and have severe disease. Lupus affects more African Americans, Asian Americans, Latinos, and Native Americans than Caucasian Americans. Lupus in children occurs most often at the age of ten and older; lupus is rare in children younger than 5 years of age.
The disease is known to have periods of flare-ups and periods of remission (partial or complete lack of symptoms). Children with lupus frequently have kidney involvement. The severity of the kidney involvement can alter the survival rate of patients with lupus. In some cases, kidney damage is so severe it leads to kidney failure.
What causes lupus?
Lupus is an autoimmune disorder, which means the body's immune system attacks its own healthy cells and tissues.
Lupus is considered to be a multifactorial condition. Multifactorial means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Multifactorial traits do occur in families because they are partly caused by genes. Females are affected with lupus three to 10 times more often than males.
What are the symptoms of lupus and how is it diagnosed?
Lupus symptoms are usually chronic and recurring. The following are the most common symptoms of lupus, however, each child may experience symptoms differently. Symptoms may include:
malar rash - a rash shaped like a butterfly that is usually found over the bridge of the nose and on the cheeks.
discoid rash - a raised rash found on the head, arms, chest, or back.
inflammation of the joints
fluid around the lungs, heart, or other organs
anemia, low white blood cell, or low platelet count
Raynaud's phenomenon - a condition in which the blood vessels of the fingers and toes go into spasm and cause them to change color to purple or white when triggered by factors such as cold, stress, or illness.
nerve or brain dysfunction
Symptoms of lupus may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.
Lupus is difficult to diagnose because the symptoms may be vague. There is no single test that can diagnose lupus. To fullfil criteria for a diagnosis of lupus, a child must have a specific combination of certain symptoms, physical exam findings, and laboratory tests. Some of these tests include: a CBC (complete blood count), blood chemistries (including liver and kidney function), inflammatory blood tests (ESR or erythrocyte sedementation rate and CRP or c-reactive protein), urine analysis (to check for blood or protein in the urine), complement levels (blood proteins that help to destroy foreign substances and are often low in active lupus). Specific tests for lupus include autoantibodies such as ANA (antinuclear antibody test), double-stranded DNA, SSA/SSB, RNP, sm, and SCL-70. Further tests including x-rays, ultrasound of the heart, lung function tests, skin biopsy or kidney biopsy may be needed depending on the presentation of the disease.
How is lupus treated?
There is no cure for lupus. The goal of treatment in lupus is to control inflammation to prevent long-term organ damage and scarring. Pediatric rheumatologists use a team approach to caring for patients with lupus. This involves both active participation on the part of the child and the parents in the decision-making process of treatment. The medications used in treatment will vary depending on the amount and severity of organ involvement.
Treatment of lupus begins with an understanding of the disease and the different options available for children. Counseling is an important part of each hospital or clinic visit. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occupational therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). All patients with lupus should avoid sun exposure during the peak sunlight hours of the day (from 10 am to 3 pm) and wear sunscreen with SPF rating of at least 30. If the kidneys are affected by lupus, pediatric nephrologists (kidney doctors) will often be involved as well.
There are many different medications used in treating lupus. A pediatric rheumatologist will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating lupus include nonsteroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen), anti-malarial medicines (such as hydroxychlorquine or plaquenil), blood pressure medications (such as ACE inhibitors like lisinopril), corticosteroids (such as prednisone), and immunosuppressive agents (such as cyclophosphamide or cytoxan, and mycophenolate mofetil or cellcept). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.