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Low prevalence of idiopathic mast cell activation syndrome among 703 patients with suspected mast cell disorders.

BACKGROUND: Idiopathic mast cell activation syndrome (iMCAS) is characterised by severe, episodic systemic mast cell (MC) activation and mediator-related symptoms, event-related increase in serum tryptase levels and response to MC-targeted therapies, in the absence of underlying IgE-mediated allergy or clonal MC disorder. Studies indicating its prevalence using evidence-based diagnostic criteria are currently lacking.

OBJECTIVE: The present study aimed to assess prevalence, as well as clinical and laboratory features of patients with iMCAS.

METHODS: We conducted a retrospective evaluation of the data from 703 consecutive patients (≥18 years old) who were referred to our centre based on suspected MC disorders. Patients underwent a thorough clinical work-up including patient history, allergy tests, KIT D816V mutation analysis and/or bone marrow investigation. Disease activity was prospectively assessed during follow-up visits.

RESULTS: Thirty-one cases with confirmed iMCAS were identified. Furthermore, hereditary alpha-tryptasemia was detected in three of the cases with baseline tryptase levels >8 ng/ml. The most common clinical presentation during the MCAS episodes was mucocutaneous symptoms in patients with iMCAS, especially urticaria/angioedema. However, these symptoms were less prevalent in clonal MCAS patients (p=0.015). The duration of diagnostic delay was significantly longer in iMCAS compared to cMCAS patients (p=0.02).

CONCLUSION: Overall prevalence of iMCAS was 4.4% in the entire cohort, which indicates that iMCAS is an uncommon condition. Screening suspected patients for the three diagnostic criteria and performing a comprehensive allergy work-up including laboratory tests and ultrasensitive mutation analysis of KIT D816V followed by applying recommended diagnostic algorithms is crucial for the diagnosis of iMCAS.

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