Managing Sjögren's Disease in Pediatric Patients.

Abstract

Over the past decade, therapeutic development in adult Sjögren's disease (SjD) has accelerated substantially. Multiple targeted agents directed at B-cell survival, co-stimulation, autoantibody recycling, and immune regulation are advancing through late-phase clinical development. As of August 2026, there are no US Food and Drug Administration-approved disease-modifying or immunomodulatory therapies specifically indicated to treat the underlying autoimmunity of SjD. US Food and Drug Administration approvals in adults are limited to symptomatic treatment of sicca (dryness) symptoms, particularly xerostomia (dry mouth). Treatment of childhood SjD remains off-label and is supported by a limited evidence base consisting largely of case reports, case series, registry abstracts, and clinician survey data. B-cell-targeted medicines and conventional disease-modifying anti-rheumatic drugs have been used off-label in SjD, including childhood SjD. In this review, we compare the clinical phenotype of childhood SjD with adult SjD, summarize the therapies currently used in children and the evidence supporting them, and propose a framework for prioritizing adult SjD therapeutics for pediatric development. The inflammatory phenotype of childhood SjD provides a strong biologic rationale for the study of selected targeted therapies, while underscoring the need for staged pediatric development, phenotype-enriched trials, and outcome measures suited to glandular and systemic disease in children. The highest-priority candidates for pediatric study are discussed based on available adult data and current approval in children for other pediatric diseases.