Related Experiment Video

Updated: Jun 13, 2026

Isolation of Peritoneum-derived Mast Cells and Their Functional Characterization with Ca2+-imaging and Degranulation Assays
11:31

Isolation of Peritoneum-derived Mast Cells and Their Functional Characterization with Ca2+-imaging and Degranulation Assays

Published on: July 4, 2018

Mast cell score associates with wide-spread mast cell symptoms and comorbidities in patients with hEDS and HSD

Frances C Wilson1,2, Derek J Zangerle1, Lincoln E Rozen1

  • 1Department of Cardiovascular Medicine, Mayo Clinic, Jacksonville, Florida, USA.

Medrxiv : the Preprint Server for Health Sciences
|June 12, 2026
PubMed

Insights

A new mast cell (MC) score helps identify widespread MC symptoms in hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD). High scores correlate with more symptoms and elevated MC-related blood markers, aiding in MC activation syndrome assessment.

Area of Science:

  • Mast Cell Biology
  • Connective Tissue Disorders
  • Syndromic Conditions

Background:

  • Mast cell (MC)-associated symptoms and MC activation syndrome (MCAS) are frequently observed in patients with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD).
  • A need exists for better tools to quantify MC involvement in these patient populations.

Purpose of the Study:

  • To develop and validate a novel MC score for clinical and research use.
  • To assess MC symptoms in hEDS and HSD patients using a 11-item self-reported questionnaire.

Main Methods:

  • A cohort of 2,141 hEDS/HSD patients completed an Intake Questionnaire with 11 MC-related questions.
  • Patients were categorized into low (0-1) and high (≥5) MC score groups.
  • Symptoms, comorbidities, and blood markers were compared between high and low MC score groups.

Main Results:

  • 25% of patients had a high MC score (≥5).
  • High MC scores were associated with increased MCAS-specific symptoms (nausea, vomiting), elevated blood markers (IgE, tryptase, MBP), and higher rates of abuse/PTSD.
  • While some standard MC markers were not elevated, high MC scores impacted urinary creatinine levels.

Conclusions:

  • The novel MC score effectively identifies patients with widespread MC symptoms and higher MC activity in hEDS/HSD.
  • This tool demonstrates utility in evaluating MC activity levels for clinical and research purposes.
  • The score aids in understanding the complex interplay of MCAS in connective tissue disorders.
Abstract

Keywords:
abuseadverse childhood experienceshypermobile Ehlers-Danlos syndromehypermobility spectrum disordersmast cell activation disordersmast cell activation syndromemast cells

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