Children can develop lupus, medically termed Childhood-Onset Systemic Lupus Erythematosus (cSLE). Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease where the immune system mistakenly produces autoantibodies that attack the body’s healthy tissues and organs. This leads to widespread inflammation affecting nearly any part of the body, including the skin, joints, kidneys, and brain. Although more commonly diagnosed in adults, roughly 15% to 20% of all SLE cases begin before age 18. Onset typically occurs during adolescence, with the median age of diagnosis often falling between 11 and 12 years.
Distinguishing Features of Childhood Lupus
Childhood-Onset Systemic Lupus Erythematosus is generally a more aggressive form of the disease compared to adult-onset lupus. This increased severity means children often experience a more rapid and intense progression of symptoms. Children with lupus are more likely to have significant involvement of major internal organs early in the disease course.
Between 50% and 75% of children develop lupus nephritis (inflammation of the kidneys), often within the first two years of diagnosis. This rate is substantially higher than seen in adults, making kidney disease a prominent concern. Central nervous system involvement, manifesting as seizures, severe headaches, or mood disorders, is also reported more frequently in the pediatric population.
The female-to-male sex ratio is lower in childhood than in adult lupus cases. While adult SLE overwhelmingly affects women, the ratio in cSLE is closer to 4.5:1, and it is nearly equal in children younger than five years old. Due to the disease’s severity, children with cSLE often receive more aggressive therapeutic regimens, including higher doses of corticosteroids and immunosuppressive drugs, to control inflammation and prevent irreversible organ damage.
Recognizing Signs and Symptoms in Children
The signs of lupus in children can be broad and nonspecific, making early diagnosis challenging. One common constitutional complaint is persistent, overwhelming fatigue that does not improve with rest, often accompanied by unexplained fever and weight loss. These general symptoms, along with decreased appetite, are frequently the first indications of systemic inflammation.
Musculoskeletal symptoms are highly prevalent, with a majority of children experiencing joint pain (arthralgia) or actual joint swelling (arthritis). This joint involvement is often symmetrical, affecting the same joints on both sides of the body. The pain can sometimes shift from one joint to another. Unlike some other forms of childhood arthritis, the joint damage from lupus is typically non-deforming.
Dermatological manifestations are a hallmark of the disease, most notably the malar rash, commonly referred to as the “butterfly rash.” This distinct red rash spreads across the bridge of the nose and cheeks, often sparing the folds next to the nose and mouth. Many children with cSLE also experience photosensitivity, where their skin reacts severely to sunlight, which can trigger or worsen a rash.
Other observable symptoms include patchy hair loss (alopecia) due to scalp inflammation. Painless ulcers inside the mouth or nose are also common and can appear repeatedly. Swelling, particularly around the eyes and in the legs, may be noticed in cases of severe disease activity, serving as an early sign of kidney involvement.
Diagnosis and Management Approaches
Diagnosing Childhood-Onset Systemic Lupus Erythematosus requires clinical observation combined with specific laboratory tests, as no single test confirms the condition. Diagnosis often begins with blood work to look for autoantibodies, particularly the antinuclear antibody (ANA), which is nearly always positive in lupus patients. Further testing looks for highly specific autoantibodies, such as anti-double-stranded DNA (anti-dsDNA) and anti-Smith (anti-Sm) antibodies, which strongly support a lupus diagnosis.
Inflammation markers, like the erythrocyte sedimentation rate (ESR), are typically elevated. Complement proteins (C3 and C4) are often found at low levels during active disease flares. A complete blood count checks for cytopenias, which are low counts of blood cells, such as hemolytic anemia, leukopenia, or thrombocytopenia.
Since kidney involvement is common, urine tests are essential to check for protein and blood in the urine. A kidney biopsy is often performed to determine the exact type and severity of lupus nephritis.
Management of cSLE focuses on controlling disease activity, reducing inflammation, and preventing cumulative organ damage while minimizing medication side effects. The individualized treatment plan frequently involves a multidisciplinary team, including a pediatric rheumatologist, nephrologist, and other specialists.
Antimalarial drugs, such as hydroxychloroquine, are a standard part of therapy recommended for almost all children with lupus to help moderate the immune system. Glucocorticoids (steroids) are used to rapidly suppress inflammation during flares, though long-term use is limited due to potential side effects like growth delay and osteoporosis.
For more severe organ involvement, especially lupus nephritis, stronger immunosuppressive medications like mycophenolate mofetil or cyclophosphamide are used. Newer biologic agents, which target specific immune pathways, are also increasingly used to reduce the reliance on steroids.