The relationship between mast cell activation syndrome, postural tachycardia syndrome, and Ehlers-Danlos syndrome

Allergy Asthma Proc. 2021 May 1;42(3):243-246. doi: 10.2500/aap.2021.42.210022.

Abstract

Background: Postural tachycardia syndrome (POTS), hypermobile Ehlers-Danlos syndrome (EDS), and mast cell activation syndrome (MCAS) can occur in the same patient. In this study, we investigated the relationship among these three syndromes. Objective: To establish the relationship of MCAS in patients diagnosed with POTS and hypermobile EDS as well as characterize the demographics of the patients affected by these syndromes. Methods: A total of 195 medical records of patients by using a diagnostic codes data base search for disorders of autonomic dysfunction were identified. The demographics of the patients and diagnoses of POTS, EDS, or MCAS were recorded. Confidence intervals of the proportion of patients MCAS within a population of patients with POTS and EDS were compared with the proportion of patients with MCAS and without POTS and EDS. Odds ratios were also calculated within these groups. Results: The percentage of MCAS within the group of POTS and EDS was 31% in comparison with 2% within the non-POTS and EDS group. The 95% confidence interval calculated for the MCAS in the POTS and EDS group did not overlap with 2%, which showed a statistically significant result. The odds ratio between the two groups was found to be 32.46. Conclusion: There was a marked percentage of MCAS among the patients with diagnoses of POTS and EDS.

MeSH terms

  • Ehlers-Danlos Syndrome* / complications
  • Ehlers-Danlos Syndrome* / diagnosis
  • Ehlers-Danlos Syndrome* / epidemiology
  • Humans
  • Mast Cell Activation Syndrome* / complications
  • Mast Cells
  • Odds Ratio
  • Postural Orthostatic Tachycardia Syndrome* / diagnosis
  • Postural Orthostatic Tachycardia Syndrome* / epidemiology

Supplementary concepts

  • Ehlers-Danlos syndrome type 3