Definition

Systemic Lupus Erythematosus (SLE), or Lupus, is a disease characterized by inflammation and damage to organs, skin, or joints. It can occur in children as well. SLE is known to have flare-up periods, during which symptoms appear, and remission periods, during which symptoms reduce or disappear.

Causes

Systemic Lupus Erythematosus (SLE) is an autoimmune disease in which the immune system attacks its own healthy cells and tissues. Many factors are known to cause SLE, usually a combination of genetic and environmental factors.

Risk Factors

Most people with lupus are young women. The age range of patients can start in late teens and extend up to 45. In children, lupus is most commonly found in those aged 15 or older. According to the Arthritis Foundation, around 25,000 children and adolescents suffer from lupus or related diseases.

Although lupus is not directly inherited from parents to children, it tends to run in families. However, this risk is relatively low, as it is rare for two siblings to suffer from lupus simultaneously.

Symptoms

Lupus symptoms are generally chronic (long-lasting), but their severity can fluctuate over certain periods in a child's life. Lupus can manifest in various ways in children, but the most common symptoms include:

• Malar rash: A butterfly-shaped rash typically appearing on the bridge of the nose and cheeks.
• Discoid rash: Raised rashes on the head, arms, chest, or back.
• Raynaud's phenomenon: A condition in which the blood vessels in the fingers and toes become stiff when exposed to certain factors such as cold, stress, or illness.

Other common symptoms that may occur in people with lupus include:

• Fever
• Joint stiffness, pain, and swelling
• Sensitivity to sunlight
• Hair loss
• Mouth sores (oral ulcers)
• Fluid around the lungs, heart, or other organs
• Weight loss
• Reduced appetite
• Low energy
• Swollen lymph nodes

In addition to the above symptoms, lupus symptoms can also resemble those of other medical conditions or disorders.

Diagnosis

SLE is challenging to diagnose because each person may experience different symptoms with varying severity. There is no single test for SLE. The diagnosis relies on the child's medical history, symptoms, physical examination, and various diagnostic and imaging tests.

Examinations may include:

• Blood tests to detect specific antibodies present in almost all people with lupus.
• Blood and urine tests to assess kidney function.
• Complement tests to measure complement levels in the blood.
• X-rays to produce images of tissues, bones, and internal organs.
• Erythrocyte sedimentation rate (ESR) to measure how quickly red blood cells settle to the bottom of a test tube.
• C-reactive protein test used to detect inflammation in the body.

Management

SLE cannot be cured, but treatments aim to manage symptoms and improve quality of life. In cases of mild severity, therapy may not be necessary aside from pain relief with NSAIDs for joint pain. Other treatment options may include:

• Hydroxychloroquine to alleviate symptoms.
• Corticosteroids to control inflammation.
• Immunosuppressive drugs to suppress the body's immune response.
• Monoclonal antibodies like belimumab and rituximab which may be used for certain patients.
• Avoiding direct sunlight exposure.
• Getting adequate rest, aiming for 8 to 10 hours of sleep per night.
• Stress reduction techniques.
• Maintaining a healthy and balanced diet.
• Prompt treatment for infections.

Children with lupus should not receive immunizations with live viruses such as chickenpox, MMR (measles, mumps, and rubella), and oral polio vaccine. It's advisable to consult a doctor regarding vaccination.

Regular monitoring by a rheumatologist is essential to ensure disease control and monitor medication side effects. Depending on the organs affected by lupus, children may also require care from nephrology, pulmonology, dermatology, neurology, or cardiology specialists.

Although lupus is not cured, symptoms can often be managed through lifestyle changes and medications. Children with lupus may benefit from consulting mental health professionals such as psychologists, psychiatrists, or competent social workers to address stress related to chronic illness.

Complications

Complications of lupus can be severe and diverse, including:

• Kidney disease (nephritis): If left untreated, nephritis can progress to the point where a child requires dialysis or even a kidney transplant, making it one of the leading causes of death from SLE in children.
• Central nervous system disorders: Brain tissue inflammation can result in mood changes, memory disturbances, seizures, and even strokes.
• Blood disorders: Anemia, a lack of red blood cells, is the most common blood-related complication. Other disorders include a decrease in white blood cells, which can lead to infections, and a lack of platelets, which can cause blood clotting disorders.
• Arthritis or joint inflammation: Long-term joint inflammation can lead to osteoporosis (bone thinning) and limited joint mobility.
• Respiratory disorders: Inflammation of the lung membrane can occur.
• Heart and blood vessel disorders: Inflammation of the heart membrane is possible.

Additionally, all children with lupus are at a heightened risk of infection due to their compromised immune system. Lupus medications suppress the body's immune response, lowering resistance to bacteria and viruses.

Prevention

There is currently no known way to prevent lupus, as no vaccines or specific diets can prevent the disease from developing. However, once the disease manifests, there are several essential strategies for managing it effectively:

• Adhering to the treatment plan prescribed by the doctor.
• Promptly informing the doctor of any new symptoms that arise.
• Taking precautions to prevent flare-ups or exacerbations of the condition.

To help manage your child's symptoms effectively, it's essential to ensure they maintain a healthy diet, get an adequate amount of sleep, and engage in regular exercise. Additionally, it's important to avoid potential triggers for flare-ups, particularly excessive exposure to sunlight.

When to see a doctor?

If your child displays symptoms such as fever, fatigue, joint stiffness, and skin rash, particularly a malar rash, it could indicate lupus. In such cases, you must consult your child's doctor promptly.

For children already diagnosed with lupus, it's important to contact the doctor if there are sudden changes in symptoms or the appearance of new ones. Additionally, infections can pose a greater risk to children with lupus. If your child experiences fever or worsening malaise, it's essential to inform the doctor immediately.

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