Journal of the American Academy of Dermatology · 2026
Progression of skin-limited pediatric-onset discoid lupus erythematosus to diagnosis of systemic lupus erythematosus: Results of a multicenter, retrospective cohort study.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Arkin Lisa M, Buhr Kevin A, Ardoin Stacy P, Faith Esteban Fernandez, Lee Lara Wine, von Scheven Emily, Brandling-Bennett J Heather, Castelo-Soccio Leslie, Chiu Yvonne E, Co Dominic O, Diaz Lucia Z, Garcia-Romero Maria T, Hunt Reagan D, Oza Vikash S, Schoch Jennifer J, Paller Amy S, Vleugels Ruth Ann, Chong Benjamin F, Werth Victoria P, Ardalan Kaveh, CARRA-PeDRA Lupus Dermatology Work Group
Аннотация
BACKGROUND: Pediatric skin-limited discoid lupus erythematosus (DLE-only) is rare, with limited data on risk factors for progression to systemic lupus erythematosus (SLE).
OBJECTIVE: To assess incidence, risk factors, and phenotype of pediatric DLE-only progression to SLE.
METHODS: In this 17-site retrospective cohort of pediatric DLE, the primary outcome was time to SLE diagnosis (American College of Rheumatology classification criterion ≥4). Kaplan-Meier estimates for 1-, 2-, and 5-year progression to SLE were generated. Cox proportional hazards modeling identified baseline predictors of progression to SLE.
RESULTS: The 1-year progression rate from DLE-only to SLE was 14.4% (95% CI, 9.6-18.9). Progression to SLE was most strongly associated with baseline antinuclear antibody positivity (hazard ratio, 3.71) and older age (hazard ratio, 1.11/y). Antiphospholipid antibodies and cytopenias also predicted progression to SLE in multivariable analysis. The SLE phenotype was relatively mild, with most patients meeting mucocutaneous and laboratory criteria (22/236, 9%) and a few patients in whom other end-organ diseases developed (7/236, 3%).
LIMITATIONS: Retrospective design, missing data.
CONCLUSION: Antinuclear antibody-positive patients with DLE-only warrant close monitoring for progression to SLE, especially within the first year. Severe end-organ disease in patients with DLE who progress to SLE is uncommon. Future studies should test whether early recognition and intervention in DLE-only slows progression to SLE.
OBJECTIVE: To assess incidence, risk factors, and phenotype of pediatric DLE-only progression to SLE.
METHODS: In this 17-site retrospective cohort of pediatric DLE, the primary outcome was time to SLE diagnosis (American College of Rheumatology classification criterion ≥4). Kaplan-Meier estimates for 1-, 2-, and 5-year progression to SLE were generated. Cox proportional hazards modeling identified baseline predictors of progression to SLE.
RESULTS: The 1-year progression rate from DLE-only to SLE was 14.4% (95% CI, 9.6-18.9). Progression to SLE was most strongly associated with baseline antinuclear antibody positivity (hazard ratio, 3.71) and older age (hazard ratio, 1.11/y). Antiphospholipid antibodies and cytopenias also predicted progression to SLE in multivariable analysis. The SLE phenotype was relatively mild, with most patients meeting mucocutaneous and laboratory criteria (22/236, 9%) and a few patients in whom other end-organ diseases developed (7/236, 3%).
LIMITATIONS: Retrospective design, missing data.
CONCLUSION: Antinuclear antibody-positive patients with DLE-only warrant close monitoring for progression to SLE, especially within the first year. Severe end-organ disease in patients with DLE who progress to SLE is uncommon. Future studies should test whether early recognition and intervention in DLE-only slows progression to SLE.