Anifrolumab for Adolescent Discoid Lupus Erythematosus

This case series describes the outcomes among adolescent patients with systemic lupus erythematosus and refractory discoid lupus erythematosus treated with anifrolumab.


Introduction
Anifrolumab, a human monoclonal antibody targeting type I interferon receptor, was approved in 2021 for adults with systemic lupus erythematosus (SLE) and has since emerged as an efficacious agent for refractory discoid lupus erythematosus (DLE). 1 Rapid clinical improvement has been attributed to anifrolumab's selective antagonism of type I interferon signaling, a known factor associated with DLE disease activity. 2However, its utility for adolescents with SLE and recalcitrant DLE remains unknown.

Methods
This multicenter retrospective case series used medical records of adolescent patients treated with anifrolumab at Boston Children's Hospital, Hassenfeld Children's Hospital, and University of Wisconsin Hospital.The study was deemed exempt by each participating site's institutional review board, and consent was waived as data were deidentified.Inclusion criteria were adolescent patients with SLE and recalcitrant DLE seen between August 1, 2022, and June 30, 2023, who received 1 or more dose of anifrolumab.Electronic health records were reviewed for demographics, clinical features, treatment data, and adverse events.This study followed the reporting guideline for case series.Analyses were performed using R, version 4.2.2 (R Project for Statistical Computing).P values were calculated using the Mann-Whitney test, and 2-sided P <.05 was considered significant.

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Discussion
DLE is characterized by irreversible scarring and disfigurement in cosmetically sensitive areas.Rapid and effective treatment is critical to minimize long-term cosmetic sequelae that may affect selfesteem and psychosocial functioning, particularly among adolescent patients. 3ifrolumab recently emerged as a promising therapeutic option for adult patients with refractory DLE, as demonstrated in several case series and a retrospective cohort study from our group. 1,2We report first outcomes data of adolescent patients with SLE and refractory DLE treated with anifrolumab.Not only did we observe significant improvement in skin disease with just 1 dose of anifrolumab (including mean 18-point reduction in CLASI-A score, when a reduction by 3-4 points is considered clinically meaningful), but clinical improvement occurred within a matter of weeks.
Infusions were well tolerated.We did observe 1 case of recurrent HSV-1 reactivation, which is consistent with increased risk for viral infections reported in original phase 3 trials among adult patients with SLE. 4 Additionally, while all patients demonstrated improvement in overall SLE disease activity, 3 patients experienced SLE manifestations (pericarditis, worsening proteinuria, or progressive organic brain syndrome) requiring additional therapy (Table ).All 3 patients had a history of severe SLE not in remission at time of anifrolumab initiation, and 2 patients reported nonadherence to adjunctive mycophenolate mofetil prior to worsening of kidney disease and neurologic involvement, respectively.
Our findings suggest that anifrolumab is associated with rapid and sustained improvement of recalcitrant DLE among adolescent patients with SLE.Although this study is limited by its small sample size and retrospective nature, it is the first series, to our knowledge, to describe skin disease response to anifrolumab in this population.

Figure . F
Figure.Clinical Improvement of Recalcitrant Discoid Lupus Erythematosus in Adolescent Patients With Systemic Lupus Erythematosus Treated With Anifrolumab Black or African American, and White patients between 14 and 20 years of age.Race and ethnicity were classified by the parent or by self-reporting in the electronic medical record.
a This cohort included Asian, b Prior therapies were considered a failure if undertaken for 12 weeks without adequate disease control or not tolerated by the patient for any reason.