Dysautonomia and joint hypermobility reflect a distinct subtype in eosinophilic esophagitis

Joint hypermobility (JH) and autonomic symptoms commonly coexist and are associated with gastrointestinal symptoms and poor quality of life. Gastric and duodenal mast cells (MCs) may be increased in these patients; however, their relevance to eosinophilic esophagitis (EoE) is unclear.

We aimed to understand the relationship between autonomic symptoms and JH in EoE with symptoms, QOL, and MCs.

Eighty EoE patients undergoing esophagogastroduodenoscopy at Lurie Children's Hospital (January 2021 to March 2024) were prospectively enrolled. Patients/caregivers completed validated surveys assessing EoE symptoms (Pediatric Eosinophilic Esophagitis Symptom Score [PEESS], QOL, and autonomic symptoms (Composite Autonomic Symptom Score 31 [COMPASS-31]). JH was determined using a published survey and Beighton score. Tryptase immunohistochemistry was quantified in gastric and duodenal biopsy samples. COMPASS-31 was compared by disease activity and hypermobility and with a published control cohort. Associations with PEESS, Pediatric Quality of Life Inventory (PedsQL), and MCs were assessed. Linear regression evaluated COMPASS-31 predictors.

COMPASS-31 was higher in EoE patients than controls (P < .001) and active EoE compared to remission (P = .02). COMPASS-31 correlated positively with PEESS scores, particularly gastroesophageal reflux, nausea/vomiting, and pain domains, and inversely with QOL. 26% of EoE patients were hypermobile and had higher COMPASS-31 (P = .003). Disease activity (P < .01) and hypermobility (P < .01) independently predicted COMPASS-31. Neither gastric or duodenal MC density was associated with COMPASS-31 or hypermobility.

In EoE patients, autonomic symptoms are increased with active disease and hypermobility, underscoring the need for clinician screening to inform management. Gastric and duodenal MC density was not associated with autonomic symptoms or hypermobility, limiting the utility of quantification.