Pediatric Systemic Lupus Erythematosus, Dermatomyositis, Scleroderma, and Vasculitis

Pediatric SLE (pSLE) is a chronic multisystem autoimmune disease with remitting, relapsing course and onset of symptoms before age 18 years, accounting for approximately 20% of all SLE. This clinically heterogeneous disease is characterized by a distinct spectrum of autoantibodies including antinuclear antibody (ANA), double-stranded deoxyribonucleic acid (dsDNA), and antibodies against extractable nuclear antigens (ENAs). In genetically susceptible hosts B cell–mediated autoimmune processes lead to a variable combination and severity of clinical symptoms including antibody-mediated vasculitis, direct antibody binding to target cells, and thrombotic organ dysfunction. The American College of Rheumatology (ACR) classification criteria for adults with SLE are commonly applied to children with pSLE. The classification of neuropsychiatric SLE (NPSLE) in children and adolescents remains a challenge. The 1990 ACR NPSLE nomenclature and case definitions appear to have limited applicability for children in certain domains.