WIP 153 - Drug-Specific Presentations and Outcomes of Drug Reaction with Eosinophilia and Systemic Symptoms (DReSS) in Children: A Scoping Review

Poster Number: WIP 153
Publication Number: WIP 153.2644

Background: Effective treatment of Drug Reaction with Eosinophilia and Systemic Symptoms (DReSS) is especially challenging in children due to a low incidence rate, heterogeneous clinical presentation, and incomplete understanding of the pathophysiology. While several classes of drugs are commonly associated with pediatric DReSS, there is a lack of data on drug-specific differences on the clinical presentation and disease course.

Objective: To identify drug-specific aspects of the clinical presentation and outcome of pediatric DReSS.

Design/Methods: A scoping review of pediatric DReSS was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In total, 491 patients aged 0-18 years with a single implicated drug were included for analysis. Associations between the most common medications (e.g., carbamazepine [CBZ], lamotrigine [LMT], phenobarbital [PB], phenytoin [PT], sulfamethoxazole [SMX], dapsone [DAP]) and clinical variables, including demographics, clinical presentation, organ involvement, mortality, and long-term sequelae, were investigated. Data from the 236 individuals analyzed thus far shows that DReSS manifests differently depending on the culprit drug. Compared to the other medications, PB was associated with shorter latency (median = 14 days [IQR = 11-21] vs 25 days [15-30]), higher rates of mucosal involvement (50% vs 31%), and flaring during treatment tapering (19% vs 9%). In contrast, DAP was linked with longer hospitalization (median = 18 days [IQR = 12-39] vs 11 days [7.5-17.5]), high proportions with anemia (73% vs 25%), and a high fatality rate (13% vs 2%). SMX was associated with the highest rates of auto-immune sequelae (autoimmune thyroid disease, alopecia areata, and vitiligo; 29% vs 4%). This study highlights that key differences exist between the manifestations and outcomes of pediatric DReSS depending on the causative drug. This data could inform targeted assessment and monitoring of pediatric DReSS, and hopefully lead to improved clinical care and management for children and their families.