Prevalence of Mast Cell Activation Syndrome in Patients With EDS With Digestive Disorders (SAMED)

Prevalence of Mast Cell Activation Syndrome in Patients With Ehlers Danlos Hypermobile Syndrome With Digestive Disorders

The aim of the study is to confirm the association between hypermobile Ehlers Danlos syndrome (hEDS) and mast cell activation syndrome (MCAS) in patients with digestive disorders managed in allergology departments.

Study Overview

• Status: Recruiting
• Conditions: Ehlers Danlos Hypermobile Syndrome
• Intervention / Treatment: Other: Determine the presence of mast cell activation syndrome

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

• Study Contact: Name: Marie DE SOLERE; Phone Number: +33320225269; Email: drci@ghicl.net
• Study Contact Backup: Name: William's VAN DEN BERGHE, CRA; Phone Number: +33320225731; Email: VanDenBerghe.Williams@ghicl.net

Study Locations

• France
  • Nord
    • Lille, Nord, France, 59020
      • Recruiting
      • Saint-Vincent-de-Paul Hospital
      • Contact: Juliette CARON, Md; Phone Number: 07 70 18 45 64; Email: caron.juliette@ghicl.net

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Subjects with EDSh with digestive disorders

Description

Inclusion Criteria:

• with hypermobile Ehlers Danlos syndrome
• with digestive disorders
• 14 years of age or older (minimum age for the MOS-SF 36 questionnaire)
• able to answer the questionnaires
• whose participation in a day hospital at Saint Vincent de Paul dedicated to the assessment of "functional digestive pain" is planned
• who do not object to the use of their health data for research purposes

Additional criteria for minors :

- No objection to the use of health data for research purposes by parents/guardians

Exclusion Criteria:

• under guardianship or curatorship

• having taken a long-term high-dose antihistamine treatment (H1 or H2) during the last two months, according to the following thresholds :

  • Desloratadine (>5 mg/jour)
  • Bilastine (>20 mg/jour)
  • Cetirizine (>20 mg/jour)
  • Ebastine (>10 mg/jour)
  • Fexofenadine (>150 mg/jour)
  • Levocetirizine (>5 mg/jour)
  • Loratadine (>10 mg/jour)
  • Exocetiridine (5 mg/jour)
  • Mizolastine (>10 mg/jour)
  • Rupatadine (>10 mg/jour)
  • Polaramine (>10 mg/jour)
  • Oxomemazine (>2 mg/jour)
  • Hydroxyzine (>25 mg/jour)
  • Doxylamine (>15 mg/jour)
  • Cimetidine (>200 mg/jour)
• Patients deprived of liberty, pregnant or nursing women

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Prevalence of mast cell activation syndrome in patients with hypermobile Ehlers Danlos syndrome with digestive disorders	The presence of mast cell activation syndrome will be described in three modalities : confirmed, possible, and unlikely. Mast cell activation syndrome will be confirmed if the following three conditions are met : at least two clinical signs compatible with mast cell activation syndrome (MCAS) before the first consultation (V1); improvement of symptoms with antihistamines within 2 months of initiation of treatment; significant increase in tryptase during crisis Mast cell activation syndrome will be possible if: at least two clinical signs compatible with MCAS before the first consultation (V1); improvement of symptoms under antihistamine at 2 months of the introduction of the treatment; no significant increase in tryptase during crisis In all other cases, mast cell activation syndrome is unlikely.	2 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Frequency of the symptoms after treatment with anti-histamines	Symptoms are those established by Valent et al, 2012. For each clinical sign the patient will rate the frequency of the symptom: more than once a day, once a day, 2 to 6 times a week, once or less a week, never, over the two months prior to visit 1 (V1), and between V1 and the 2-month follow-up visit .	2 months
Medical Outcome Study Short-Form 36 (MOS SF-36 ) survey after antihistamine treatment	MOS SF-36 consists of eight scaled scores, which are the weighted sums of the questions in their section. Each scale is directly transformed into a 0-100 scale on the assumption that each question carries equal weight. The lower the score the more disability	2 months
Frequency of consumption of analgesics	The frequency of consumption of different analgesics will be studied over the two months preceding V1 and between V1 and V2.	2 months
Frequency of intolerance according to particular food consumption	For a given food, at V1 and V2, intolerance will be qualified by the presence of digestive disorders following the consumption of the food among : cow's milk, goat's milk, sheep's milk, fresh cheeses, cow's milk cheese or yogurt, goat's milk cheese or yogurt, sheep's milk cheese or yogurt, wheat/gluten, dry sausage, ham, turkey, chicken, pork, beef, raw egg, cooked egg, smoked fish, canned fish fresh fish, shellfish, mollusks, tomatoes, potatoes, spinach, peas, cabbage, lentils, beans, lemons, oranges, strawberries, raspberries, pineapple, mango, apple, pear, nectarine, peach, banana, kiwi, nuts, peanuts, spices, beers, wines, spirits, other.	2 months

Collaborators and Investigators

• Sponsor: Lille Catholic University
• Investigators: Principal Investigator: Juliette CARON, Md, Lille Catholic University

Study record dates

Study Major Dates

• Study Start (Actual): September 28, 2022
• Primary Completion (Anticipated): January 1, 2025
• Study Completion (Anticipated): March 1, 2026

Study Registration Dates

• First Submitted: May 16, 2022
• First Submitted That Met QC Criteria: May 16, 2022
• First Posted (Actual): May 19, 2022

Study Record Updates

• Last Update Posted (Estimate): January 11, 2023
• Last Update Submitted That Met QC Criteria: January 10, 2023
• Last Verified: January 1, 2023

More Information

Terms related to this study

• Keywords: Nausea; Heartburn; Genetic disease; Collagen abnormality; Ehlers Danlos hypermobile syndrome; Mast cell activation syndrome (MCAS); Allergology
• Additional Relevant MeSH Terms: Pathologic Processes; Skin Diseases; Immune System Diseases; Neoplasms, Connective and Soft Tissue; Neoplasms by Histologic Type; Neoplasms; Disease; Hypersensitivity; Neoplasms, Connective Tissue; Immune Complex Diseases; Syndrome; Digestive System Diseases; Mastocytosis
• Other Study ID Numbers: RC-P00115

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No