Synonyms
Juvenile-onset SLE; Pediatric SLE
Definition
Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder that can affect any organ of the human body. Presentation and clinical courses are highly variable which is why 11 diagnostic criteria were introduced and 4 must be fulfilled to make a diagnosis of SLE (Tsokos 2011; Hochberg 1997).
The pathophysiology of SLE is not completely understood; genetic, hormonal, and environmental factors, all contribute to the development of the disease (Hedrich et al. 2011). Women are affected more frequently with an overall female to male ratio of 10:1 (Tsokos 2011). Approximately 10–20 % of all SLE manifestations occur in the pediatric age group (before 16 years of age) and are classified as pediatric SLE (or juvenile-onset SLE) (Ardoin and Schanberg 2012; Brunner et al. 2011). Cases with a disease onset before 5 years of age, referred to as early-onset SLE, are extremely uncommon and appear to be more severe when compared to other...
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Ardoin SP, Schanberg LE. Paediatric rheumatic disease: lessons from SLE: children are not little adults. Nat Rev Rheumatol. 2012;8(8):444–5.
Aringer M, Günther C, Lee-Kirsch MA. Clin Immunol. 2012 Dec 12. doi:pii: S1521-6616(12)00291-4. 10.1016/j.clim.2012.11.012. [Epub ahead of print]
Atkinson JP, Yu CY. Systemic Lupus Erythematosus. SLE in Children. In: Robert G. Lahita, editors. 5th ed. Amsterdam: Elsevier Academic, 2011;21–45. Print.
Botto M. C1q knock-out mice for the study of complement deficiency in autoimmune disease. Exp Clin Immunogenet. 1998;15(4):231–4.
Brunner HI, Huggins J, Klein-Gitelman MS. Pediatric SLE–towards a comprehensive management plan. Nat Rev Rheumatol. 2011;7(4):225–33.
Crispín JC, Liossis SN, Kis-Toth K, Lieberman LA, Kyttaris VC, Juang YT, Tsokos GC. Pathogenesis of human systemic lupus erythematosus: recent advances. Trends Mol Med. 2010;16(2):47–57.
Flierman R, Daha MR. The clearance of apoptotic cells by complement. Immunobiology. 2007;212(4–5):363–70.
Gaubitz M, Schotte H. Early diagnosis in patients with systemic lupus erythematosus (SLE). Z Rheumatol. 2005;64(8):547–52.
Haas JP. Genetic background of juvenile idiopathic arthritis. Z Rheumatol. 2010;69(6):488–95.
Hauck F, Lee-Kirsch MA, Aust D, Roesler J, Pessler F. Complement C2 deficiency disarranging innate and adaptive humoral immune responses in a pediatric patient: treatment with rituximab. Arthritis Care Res (Hoboken). 2011;63(3):454–9.
Hedrich CM, Tsokos GC. Epigenetic mechanisms in systemic lupus erythematosus and other autoimmune diseases. Trends Mol Med. 2011;17(12):714–24.
Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, Heubner G, Lee-Kirsch MA, Gahr M. Chilblain lupus erythematosus–a review of literature. Clin Rheumatol. 2008;27(8):949–54.
Hedrich CM, Zappel H, Straub S, Laass MW, Wieczorek K, Hahn G, Heubner G, Gahr M. Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options. Clin Rheumatol. 2011;30(2):275–83.
Hersh AO, Trupin L, Yazdany J, Panopalis P, Julian L, Katz P, Criswell LA, Yelin E. Childhood-onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2010;62(8):1152–9.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725.
Lee-Kirsch MA, Chowdhury D, Harvey S, Gong M, Senenko L, Engel K, Pfeiffer C, Hollis T, Gahr M, Perrino FW, Lieberman J, Hubner N. A mutation in TREX1 that impairs susceptibility to granzyme A-mediated cell death underlies familial chilblain lupus. J Mol Med (Berl). 2007a;85(5):531–7.
Lee-Kirsch MA, Gong M, Chowdhury D, Senenko L, Engel K, Lee YA, de Silva U, Bailey SL, Witte T, Vyse TJ, Kere J, Pfeiffer C, Harvey S, Wong A, Koskenmies S, Hummel O, Rohde K, Schmidt RE, Dominiczak AF, Gahr M, Hollis T, Perrino FW, Lieberman J, Hübner N. Mutations in the gene encoding the 3′–5′ DNA exonuclease TREX1 are associated with systemic lupus erythematosus. Nat Genet. 2007b;39(9):1065–7.
Livingston B, Bonner A, Pope J. Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis. Lupus. 2011;20(13):1345–55.
Livingston B, Bonner A, Pope J. Semin Arthritis Rheum. 2012 Dec;42(3):271-80. doi: 10.1016/j.semarthrit.2012.05.001. Epub 2012 Jun 15.
Mina R, Brunner HI. Systemic Lupus Erythematosus. SLE in Children. In: Robert G. Lahita, editors. 5th ed. Amsterdam: Elsevier Academic, 2011;573–97. Print.
Namjou B, Kothari PH, Kelly JA, Glenn SB, Ojwang JO, Adler A, Alarcón-Riquelme ME, Gallant CJ, Boackle SA, Criswell LA, Kimberly RP, Brown E, Edberg J, Stevens AM, Jacob CO, Tsao BP, Gilkeson GS, Kamen DL, Merrill JT, Petri M, Goldman RR, Vila LM, Anaya JM, Niewold TB, Martin J, Pons-Estel BA, Sabio JM, Callejas JL, Vyse TJ, Bae SC, Perrino FW, Freedman BI, Scofield RH, Moser KL, Gaffney PM, James JA, Langefeld CD, Kaufman KM, Harley JB, Atkinson JP. Evaluation of the TREX1 gene in a large multi-ancestral lupus cohort. Genes Immun. 2011;12(4):270–9.
Silverman E, Eddy A. Systemic Lupus Erythematosus. Textbook of Pediatric Rheumatology. James T. Cassidy, editors. 6th ed. Philadelphia, PA: Saunders, 2011;315–43. Print.
Truedsson L, Bengtsson AA, Sturfelt G. Complement deficiencies and systemic lupus erythematosus. Autoimmunity. 2007;40(8):560–6.
Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110–21.
Zulian F, Pluchinotta F, Martini G, Da Dalt L, Zacchello G. Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus. 2008;17(9):780–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media New York
About this entry
Cite this entry
Hedrich, C.M. (2014). Juvenile Diseases: SLE in Children. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_395
Download citation
DOI: https://doi.org/10.1007/978-0-387-84828-0_395
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-0-387-84827-3
Online ISBN: 978-0-387-84828-0
eBook Packages: MedicineReference Module Medicine