Childhood-Onset Systemic Lupus Erythematosus: A Review and Update

Lupus is a chronic, autoimmune multisystem inflammatory disease that is associated with sizable morbidity and mortality. 1 Petty R.E. Lindsley C.B. Laxer R.M. Wedderburn L. Cassidy's textbook of pediatric rheumatology. 7th ed. Elsevier, Philadelphia (PA)2016: xiv Google Scholar When lupus commences in an individual less than 18 years of age, 2 Silva C.A. Avcin T. Brunner H.I. Taxonomy for systemic lupus erythematosus with onset before adulthood. Arthritis Care Res (Hoboken). 2012; 64: 1787-1793 Crossref PubMed Scopus (88) Google Scholar it is commonly referred to as childhood-onset systemic lupus erythematosus (cSLE). With a reported incidence of 0.3-0.9 per 100 000 children per year, and a prevalence of 3.3-24 per 100 000 children, 3 Kamphuis S. Silverman E.D. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol. 2010; 6: 538-546 Crossref PubMed Scopus (144) Google Scholar cSLE is rare. About 10%-20% of all patients with SLE are diagnosed during childhood. Typically, cSLE has a more severe clinical course than that seen in adults, with a higher prevalence of lupus nephritis, hematologic anomalies, photosensitivity, neuropsychiatric, and mucocutaneous involvement. 3 Kamphuis S. Silverman E.D. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol. 2010; 6: 538-546 Crossref PubMed Scopus (144) Google Scholar , 4 Malattia C. Martini A. Paediatric-onset systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2013; 27: 351-362 Abstract Full Text Full Text PDF PubMed Scopus (73) Google Scholar , 5 Tarr T. Dérfalvi B. Győri N. Szántó A. Siminszky Z. Malik A. et al. Similarities and differences between pediatric and adult patients with systemic lupus erythematosus. Lupus. 2015; 24: 796-803 Crossref PubMed Scopus (54) Google Scholar