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Review
. 2023 Mar 1;35(2):68-81.
doi: 10.1097/BOR.0000000000000913. Epub 2022 Oct 25.

Current views on lupus in children

Eve M D Smith  1   2 , Hanna Lythgoe  3 , Christian M Hedrich  1   2
Affiliations
Review

Current views on lupus in children

Eve M D Smith et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: This manuscript provides an update on clinical and pathophysiological features of juvenile-onset systemic lupus erythematosis (jSLE), challenges applying adult-derived classification criteria, and recent advances in treatment and care.

Recent findings: Significant scientific advances have improved the understanding of genetic factors (both genetic causes and risk alleles) and associated phenotypic features. Panels of urine/blood biomarker candidates aid in diagnosing jSLE, monitoring disease activity and predicting treatment response. Available classification criteria have been extensively assessed, with differences in clinical and immunological phenotypes of patients across age groups and ethnicities affecting their performance in jSLE. Therapeutic options remain limited and are based on protocols for adult-onset SLE patients. International efforts to inform development of a treat-to-target (T2T) approach for jSLE have yielded cohort-level evidence that target attainment reduces the risk of severe flare and new damage, and treatment compliance.

Summary: Recent studies have significantly improved our understanding of jSLE pathogenesis, highlighting important differences between jSLE and adult SLE, and providing the basis of biomarker development and target-directed individualized treatment and care. Future work focused on development of a T2T approach in jSLE is eagerly awaited.

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References

    1. Tsokos GC. Systemic lupus erythematosus. New Engl J Med 2011; 365:2110–2121.
    1. Hedrich CM, Smith EMD, Beresford MW. Juvenile-onset systemic lupus erythematosus (jSLE) - pathophysiological concepts and treatment options. Best Pract Res Clin Rheumatol 2017; 31:488–504.
    1. Massias JS, Smith EM, Al-Abadi E, et al. Clinical and laboratory phenotypes in juvenile-onset systemic lupus erythematosus across ethnicities in the UK. Lupus 2021; 30:597–607.
    1. Massias JS, Smith EMD, Al-Abadi E, et al. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups. Lupus 2020; 29:474–481.
    1. Smith EMD, Lythgoe H, Midgley A, et al. Juvenile-onset systemic lupus erythematosus: update on clinical presentation, pathophysiology and treatment options. Clin Immunol 2019; 209:108274.

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