Autoinflammatory diseases (AID) are caused by innate immunity dysregulation. AID pathophysiology is only partly understood, especially in the case of unclassified AID. Mast cells (MC) are innate immune cells associated with a spectrum of disease between systemic mastocytosis and mast cell activation syndrome. The implication of MC has been shown in cryopyrin associated periodic syndrome (CAPS).Our aim is to evaluate the involvement of MC in AID by assessing clinical and biological signs of MC activation and studying cutaneous and digestive biopsies.
Patients >18 years old with Auto-inflammatory diseases already followed up at the CeRéMAIA (french national reference center for autoinflamamtory diseases and AA amyloidosis) of Tenon hospital and included in the JIRcohorte
Healthy adult controls, age- and sex-matched with MAI patients, and controls with mastocytosis, an immuno-inflammatory disease.
Subject affiliated to or entitled to a social security scheme
Collection of the patient's or healthy control's non-opposition
Subjects unable to answer questions or express themselves
Subjects who do not speak French
Subject deprived of liberty or under legal protection.
Inpatients and outpatients wil be recruited in tertiary university centres of the Assitance Publique des Hopitaux de Paris. Healthy controls will be recruited from volunteer healthcare workers in Assitance Publique des Hopitaux de Paris
Cohort
Prospective
Not listed
6
Trial groupsSee each trial group below.
: presence of MCAS
presence of clinical and biological markers of MCAS
comparison of clinical symptoms of MC activation between groups
we will compare clinical symptoms of MC activation between AID patients and other groups
MC mediators associated to AID
determine which MC mediators are elevated in AID and if they correlate with inflammation
MC infiltration in biopsies from AID patients
we will study MC infiltration in digestive, renale and cutaneous biopsies from patients with AID and compare them with biopsies from the other groups from subgroups of patients who had a biopsy performed.
basophilic polynuclear activation in AID patients
we will study basophilic polynuclear activation from blood samples of AID patients
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Assistance Publique - Hôpitaux de Paris
Lead sponsor
Institut Imagine
Collaborator
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