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

Study Overview
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Brief Summary
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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.

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

Conditions
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Ehlers Danlos Hypermobile Syndrome

Intervention / Treatment
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* Other: Determine the presence of mast cell activation syndrome


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 14 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Other: Determine the presence of mast cell activation syndrome|Mast cell activation syndrome will be confirmed if the following three conditions are met (according to Valent et al, 2012) : At least two clinical signs compatible with MAS before the first consultation (V1) Improvement of symptoms on 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 consistent with MAS before the first consultation (V1) Improvement of symptoms with antihistamines within 2 months of initiation of treatment No significant increase in tryptase during crisis In all other cases, mast cell activation syndrome is unlikely.|

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Genetic disease, Collagen abnormality, Nausea, Heartburn, Ehlers Danlos hypermobile syndrome, Mast cell activation syndrome (MCAS), Allergology