Clinical spectrum of pediatric-onset Erdheim-Chester disease: insights from a single-center case series.

Purpose: Pediatric-onset Erdheim-Chester disease (PECD) is considerably rarer than the adult-onset Erdheim-Chester disease (AECD). This study aimed to characterize the clinical, laboratory, imaging, and outcome features of PECD and to compare similarities and differences with adult-onset cases treated at the same center. Methods: We performed a comparative analysis of PECD and AECD patients treated at the same center over the same period, examining differences in clinical presentation, laboratory and imaging findings, treatment responses, and outcomes. Results: Five pediatric and sixty-seven adult patients were included. Central nervous system and hypothalamic or pituitary involvement were observed in over 50% of children. PECD patients demonstrated distinct organ involvement patterns, including lower rates of bone involvement (60.0% vs. 97.0%; Conclusions: PECD and AECD differ in clinical manifestations and the intensity of systemic inflammation. Pediatric patients appear to have more favorable survival outcomes. These findings suggest potential age-related differences in disease presentation and inflammatory burden. Nevertheless, validation in larger multicenter cohorts is required, underscoring the necessity for large-scale, multicenter collaborative investigations to inform the development of pediatric-specific management strategies.