Comorbidities in Ehlers-Danlos syndromes and hypermobile spectrum disorders.

INTRODUCTION

hypermobile spectrum disorders (HSD) and Ehlers-Danlos syndromes (EDS) are connective tissue disorders often associated with systemic manifestations such as mast cell activation syndrome (MCAS), postural orthostatic tachycardia syndrome (POTS), irritable bowel syndrome (IBS), and autoimmune conditions, including spondyloarthritis (SpA). The overlap of these conditions complicates diagnosis and treatment. This study investigates the prevalence of MCAS and other comorbidities, as well as patterns of medication use, in individuals with HSD/EDS.

MATERIAL AND

METHODS

this cross-sectional study included 37 participants diagnosed with HSD or EDS based on the 2017 Diagnostic Consensus Criteria. Participants were divided into two groups: 23 with SpA + EDS and 14 with EDS-only. Demographic variables, comorbidities, and medication use (biologics, disease-modifying antirheumatic drugs [DMARDs], and nonsteroidal anti-inflammatory drugs [NSAIDs]) were analyzed using comparative statistical methods.

RESULTS

MCAS was significantly less prevalent in SpA + EDS participants (13%) compared to the EDS-only group (85.7%, p < 0.0001). POTS (60.9% vs 78.6%) and IBS (60.9% vs 85.7%) occurred at similar frequencies in both groups. The use of immunomodulators was higher in SpA + EDS (73.9%) than EDS-only (42.8%, p = 0.003). Biologic use was more common in SpA + EDS (34.8% vs 7.1%, p = 0.050), whereas NSAID use was higher in EDS-only participants (47.4% vs 30.4%, p > 0.05).

CONCLUSIONS

the lower MCAS prevalence in SpA + EDS may reflect symptom overlap or suppression due to immunomodulatory treatments. Differences in medication use highlight variations in diagnostic and therapeutic strategies. Comprehensive evaluations are essential to ensure accurate diagnoses and optimal treatment approaches.