What are the symptoms and treatment options for systemic lupus erythematosus (SLE) in pediatric patients?

Symptoms of Systemic Lupus Erythematosus in Children

Pediatric SLE presents with more severe and active disease at onset compared to adults, with renal involvement, hematologic abnormalities, and cutaneous manifestations being the most common initial features. 1

Common Presenting Symptoms

Musculoskeletal and Constitutional Features

• Musculoskeletal symptoms occur in 74% of children at presentation, including arthritis and joint pain 2
• Constitutional symptoms affect 67% of children, presenting as fever, fatigue, and weight loss 2, 3
• Polyarthritis is present in approximately 68% of pediatric cases 4

Cutaneous Manifestations

• Skin involvement occurs in 72% of children at diagnosis 2
• Butterfly (malar) rash is significantly more common in children (61%) compared to adults (35.5%) 4
• Photosensitivity affects 20% of pediatric patients 4
• Mucosal ulceration occurs in 11.4% of children 4

Renal Involvement

• Lupus nephritis is present in 28-43% of children at presentation, representing a significantly higher rate than in adults 2, 4
• Children have 1.5-2.4 times increased risk of renal involvement compared to adults 1
• Proteinuria or cellular casts are found in 44% at initial presentation 2
• Renal involvement increases with age at disease onset, being more common in postpubescent children 5

Neuropsychiatric Manifestations

• Neurologic symptoms occur in 28% of children at presentation 2
• Approximately 25% of children with SLE develop neuropsychiatric manifestations, which are a major cause of morbidity and mortality 6
• Unusual presentations include quadriplegia, chorea, and severe headaches 2
• Seizures and cognitive dysfunction are among the more common neuropsychiatric features 1

Hematologic Abnormalities

• Hematologic disorders affect 57% of pediatric patients, significantly higher than the 36.4% in adults 4
• Anemia is present in 72% at diagnosis 2
• Lymphopenia occurs in 59% of children 2
• Leukopenia affects 31% at presentation 2

Other Manifestations

• Lymphadenopathy is present in 15% of children 2
• Raynaud's phenomenon occurs in 10% 2
• Splenomegaly is more common in prepubescent children 5

Laboratory Findings at Presentation

Serologic Markers

• Antinuclear antibodies (ANA) are positive in 97% of children 2
• Anti-double-stranded DNA antibodies are present in 95% 2
• Low complement levels (C3 or C4) occur in 77% of cases 2
• Elevated erythrocyte sedimentation rate is found in 87% 2

Critical Diagnostic Consideration

• 85% of patients with atypical presentations have abnormal complete blood count or urinalysis results at presentation 2
• One-third (33%) of children present with features not initially suggestive of SLE, requiring high clinical suspicion 2

Age-Related Differences in Presentation

Postpubescent children (13-18 years) show significantly more renal involvement and lymphopenia with lower complement levels compared to prepubescent children 5

• Prepubescent children more commonly present with splenomegaly 5
• Disease activity (SLEDAI-2K score) correlates positively with age at disease onset 5
• Female predominance exists across all age groups (2.5-7.0:1 ratio) 5

Comparison with Adult-Onset Disease

Children with SLE tend to have more active disease, receive more intensive immunosuppressive treatment, and accrue more damage over time, often related to glucocorticoid toxicity 1

Key differences include:

• Higher frequency of lupus nephritis in children (43% vs 26.4% in adults) 4
• More common butterfly rash (61% vs 35.5%) 4
• Greater prevalence of hematologic disorders (57% vs 36.4%) 4
• Less frequent neurological symptoms in children (6% vs 30% in adults) 4
• Lower rates of polyarthritis in children (68% vs 86%) 4

Clinical Pitfalls to Avoid

SLE should be considered in the differential diagnosis of any multisystem disease, particularly in non-Caucasian ethnic groups 3

• Do not dismiss unusual presentations such as parotitis, persistent cough, severe abdominal pain, or dizziness as these can be initial SLE manifestations 2
• Always obtain complete blood count and urinalysis in suspected cases, as these are abnormal in the vast majority of atypical presentations 2
• Maintain high clinical suspicion when fever, fatigue, and multisystem involvement occur together 3