SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDREN
Systemic lupus erythematosus (SLE, or lupus) is a chronic, multi-organ autoimmune condition, characterized by on-and-off inflammation triggered by antibodies against the body’s normal tissues. People of all ages may develop SLE, although it may be more severe in children in comparison to adults. The skin, kidneys, blood vessels, joints, and central nervous system are some of the commonly affected organs and tissues.
Children who are generally more susceptible to SLE have skin that is more sensitive to ultraviolet light. These skin cells can receive damage that is significant enough to lead to cell death. As damaged skin cells undergo this process, substances from inside these cells are released, including nucleic acids and proteins. Although the exact process isn’t clear yet, the body’s immune cells misread these substances (antigens) as foreign - and start to attack and multiply (by creating autoantibodies). Eventually, these antigens and autoantibodies become attached to each other to form immune complexes, which travel throughout the body via the bloodstream. Sometimes, these complexes land in certain organ systems, like the kidneys and joints, where it can cause inflammation that progresses to tissue damage.
There is no specific cause for SLE - rather, several factors play a role in determining how likely a child may develop this condition. The most significant factor is gender: nearly 90% of SLE patients are females. Experts think that hormones may be the reason behind this. Genetics is also a consideration, as most patients have close family members with the same disease or a related autoimmune condition. Environmental exposure and epigenetics are additional factors, although more research is needed to determine specific triggers. Some studies point out that high levels of stress, use of certain drugs, and exposure to smoke or viruses are examples of these external factors.
Childhood or juvenile SLE (jSLE or cSLE) is relatively uncommon. Most SLE patients are adult women. Only 20% of lupus patients were diagnosed before turning 20 years old, and there are only about 3-9 cases of SLE for every 100,000 children. In the United States, an estimated 10,000 children may have lupus. Most cases of cSLE are diagnosed between 12-14 years of age and are rarely diagnosed below 5 years old.
Children with African American, Asian and Hispanic roots are more prone to develop serious complications and may be more difficult to treat. In general, cSLE is more likely to develop liver, kidney and heart complications as compared to SLE in adults.
Signs and Symptoms
SLE is a multi-system condition; it can affect various organs and tissues in the body simultaneously, or . Signs and symptoms of SLE are not constantly present; like other immune-related diseases, there may be episodes of symptom exacerbations, as well as relatively symptom-free periods (known as periods of relapse and remission, respectively).
In childhood SLE, the most common symptoms are fever, arthralgia or arthritis, fatigue, anorexia, weight loss, changes in the skin or mucous membranes, and low blood counts. Less frequent manifestations include kidney problems, edema, nausea, vomiting, as well as lung, heart, and neuropsychiatric symptoms. Inflammation is the underlying cause of these symptoms, affecting a variety of organs and tissues.
A significant percentage of cSLE patients are female adolescents who present with fever, a malar rash, arthritis, oral ulcers, and other signs of blood vessel inflammation.
A child’s medical history and a physical examination are important in diagnosing cSLE and ruling out other conditions. The European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) have created a widely accepted list of criteria to diagnose and classify patients with SLE. This includes signs, symptoms, and laboratory tests.
Some of the criteria include fever, low white blood cell counts, low platelet counts, signs of hemolysis, joint involvement, oral ulcers, alopecia, pleural or pericardial effusion, delirium, psychosis, and seizures. For a comprehensive evaluation, some tests may be requested, such as immunologic tests (ANA and dsDNA are examples), a urinalysis, and a renal ultrasound or biopsy.
Because patients with lupus may not present with similar signs and symptoms, especially in children, there is no single criterion or test that confirms cSLE. Trained healthcare professionals assess and diagnose patients based on their medical history, physical examination, symptoms and laboratory workup.
Treatment and Secondary Prevention
Because lupus has various clinical presentations, treatment is unique for each case. Not all of the following medications are prescribed to all lupus patients. The healthcare team will decide which medications to start based on the patient’s age and medical history, affected organ systems, severity of lupus, and tolerance for medications.
Common treatment options include NSAIDs, corticosteroids, steroid-sparing immunosuppressive agents (like methotrexate and cyclophosphamide), disease-modifying antirheumatic drugs (DMARDs), monoclonal antibodies (rituximab, belimumab), anticoagulants and hydroxychloroquine. Patients are advised to avoid direct sunlight or UV exposure and to frequently use sunscreen products. Changes in lifestyle and diet must also be done. This includes decreasing stressful activities, regular hours of sleep, as well as a healthy diet with increased calcium and vitamin D.
For children, there should be additional care in managing cSLE in consideration of possible emotional and psychosocial issues arising from the diagnosis and treatment. Routine immunization schedules should be modified to exclude live vaccines, such as chickenpox and oral polio vaccines.
SLE is an evolving disease; new signs and symptoms may appear, regardless of how long it has been since the first day of diagnosis. Regular monitoring and follow-up with the healthcare team is essential to optimal management of this condition.
Lupus patients have a higher risk of developing atherosclerosis much earlier. Regular monitoring for blood pressure, body mass index, cholesterol levels should be done. Patients should also be wary of developing fever or any infection - this must be treated as early as possible since some lupus medications may decrease the body’s immune response. There is a risk of early osteoporosis; prevention includes increased vitamin D and calcium intake. Studies have shown that female SLE patients are at higher risk for myocardial infarction (MI).
There are several side effects from lupus medications. Long-term use of steroids may lead to osteoporosis, hypertension, gastrointestinal ulcers, or bone fractures. Cyclophosphamide may decrease white blood cell counts. Overall, the adjustments in quality of life due to a long-term disease such as lupus can cause frequent mood changes, emotional issues and cognitive dysfunction, especially for adolescents.
In cSLE, the survival rate for the first 5 years is at 95%, and 80-90% for the first 10 years. The most common causes of death within the first few years of lupus are complications related to:
- renal disease (glomerulonephritis)
- neuropsychiatric disease
Mothers with lupus may pass on lupus autoantibodies to their unborn children via the placenta, leading to neonatal lupus. This condition is rare, seen in only 2% of babies of mothers with SLE.
Affected newborns and infants may develop a rash on the face, scalp, or trunk within the first few months of life, especially after sunlight exposure. Low white blood cell counts, hepatitis and congenital heart block may also develop. In most cases, symptoms resolve after 3 to 4 months. Treatment involves immune globulin or corticosteroids.
Systemic lupus erythematosus is an autoimmune disease characterized by recurrent inflammatory episodes that may affect multiple organs and tissues. SLE is more likely to develop in female children with a family history of autoimmune disease. The most frequently affected areas include the skin, joints, kidneys, and the immune system itself. Treatment depends on the assessment of the healthcare team, and includes lifestyle, psychosocial and medical therapies.
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- Kliegman, R.M. et al. (2020). Chapter 183 Systemic Lupus Erythematosus. Nelson Textbook of Pediatrics 21st edition. Elsevier.
- Abraham, A.M., Matthews, S.J., Srinivasan, R., & Chaviano, K. (2021). Systemic Lupus Erythematosus in Children. American Academy of Physical Medicine & Rehabilitation. Taken from: https://now.aapmr.org/systemic-lupus-erythematosus-in-children/
- Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., Smolen, J. S., Wofsy, D., Boumpas, D. T., Kamen, D. L., Jayne, D., Cervera, R., Costedoat-Chalumeau, N., Diamond, B., Gladman, D. D., Hahn, B., Hiepe, F., Jacobsen, S., Khanna, D., Lerstrøm, K., … Johnson, S. R. (2019). 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis & rheumatology (Hoboken, N.J.), 71(9), 1400–1412. https://doi.org/10.1002/art.40930
- Justiz Vaillant AA, Goyal A, Varacallo M. Systemic Lupus Erythematosus. [Updated 2022 Mar 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK535405/
- National Health Service (2020). Lupus. NHS. Taken from: https://www.nhs.uk/conditions/lupus/
- Paz, Z. & Watcher, S. (2017). Systemic Lupus Erythematosus (Juvenile). American College of Rheumatology. Taken from: https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Systemic-Lupus-Erythematosus-Juvenile
- Mehta, J. (review)(n.d.) Pediatric Lupus. Children’s Hospital of Philadelphia. Taken from: https://www.chop.edu/conditions-diseases/pediatric-lupus
- Levy, D. (2020). Childhood-onset systemic lupus erythematosus (SLE): Clinical manifestations and diagnosis. UpToDate. Taken from: https://www.uptodate.com/contents/childhood-onset-systemic-lupus-erythematosus-sle-clinical-manifestations-and-diagnosis
- Ting, T. (review)(n.d.) Systemic Lupus Erythematosus in Children. Cincinnati Children’s Hospital Medical Center. Taken from: https://www.cincinnatichildrens.org/health/s/systemic-lupus-erythematosus
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