DESCRIPTION

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by production of autoantibodies to various components of the cell nucleus and tissues, resulting in a variety of clinical manifestations including organ injury/dysfunction.

• Chronic cutaneous lupus (CLE) and most commonly discoid lupus are skin diseases that can occur in isolation or as part of SLE. All patients with discoid lupus should be evaluated for SLE.
• Drug-induced lupus erythematosus (DILE) is an autoimmune phenomenon where a certain drug exposure leads to the development of systemic lupus-like symptoms. The mainstay of treatment is recognition and discontinuation of the offending drug.
• Neonatal lupus erythematosus (NLE) is an acquired condition in newborns due to maternal autoantibodies (usually Sjögren syndrome A [SSA] or Sjögren syndrome B [SSB] antibodies) that cross the placenta (see section on NLE for further description).

EPIDEMIOLOGY

• Age: 20% of SLE begins in childhood, but it is very rare in those who are <5 years old; If lupus is diagnosed <5 years of age, genetic testing for monogenic causes of lupus should be pursued.
• Female-to-male ratio: between 3 to 5:1 (prepubertal) and 9 to 10:1 (postpubertal)
• The incidence and prevalence of SLE are higher in Afro-Caribbean, Hispanic, Asian, and Native American populations.
• Peak incidence: between ages 15 and 40 years
• Incidence in children is from 10 to 20 cases per 100,000 children per year
• Prevalence: 5,000 to 10,000 children in the United States

ETIOLOGY

Lupus is an autoimmune disease, with multiple genetic, environmental, and hormonal factors that contribute to pathogenesis.

RISK FACTORS