AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management

Weiler, Catherine R.; Austen, K. Frank; Akin, Cem; Barkoff, Marla S.; Bernstein, Jonathan A.; Bonadonna, Patrizia; Butterfield, Joseph H.; Carter, Melody; Fox, Charity C.; Maitland, Anne; Pongdee, Thanai; Mustafa, S. Shahzad; Ravi, Anupama; Tobin, Mary C.; Vliagoftis, Harissios; Schwartz, Lawrence B.

doi:10.1016/j.jaci.2019.08.023

Cited by 79 publications

(117 citation statements)

References 219 publications

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“…Mast cells and their mediators can be involved in several aspects of tumor initiation and growth [21,39,[69][70][71], presumably through the production of several angiogenic and lymphangiogenic factors [19,20,75]. Systemic mastocytosis is a clonal disease associated with a somatic gain-of-function KIT mutation [56,57,123]. Mast cells, strategically located in different sections of the human heart [51,52] and atherosclerotic plaque [32,33], are involved in different phases of atherosclerosis and myocardial infarction.…”

Section: Discussionmentioning

confidence: 99%

“…Mastocytosis is a hematopoietic neoplasm characterized by abnormal expansion and focal accumulation of clonal mast cells in various organs [54][55][56]. The disease is highly heterogeneous and exhibits a complex pathology and different clinical presentations.…”

Section: Prefacementioning

confidence: 99%

“…Kovanen comprehensively reviews the complex role of mast cells throughout the progression of early to late lesions of human atherosclerosis [32]. Immunohistochemical studies in autopsied patients and studies in cell culture systems and in atherosclerotic mouse models have collectively provided evidence that mast cell mediators may promote atherogenesis at various stages of lesion development.Mastocytosis is a hematopoietic neoplasm characterized by abnormal expansion and focal accumulation of clonal mast cells in various organs [54][55][56]. The disease is highly heterogeneous and exhibits a complex pathology and different clinical presentations.…”

mentioning

confidence: 99%

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Mast Cells: Fascinating but Still Elusive after 140 Years from Their Discovery

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Section: Discussionmentioning

confidence: 99%

Section: Prefacementioning

confidence: 99%

mentioning

confidence: 99%

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Mast Cells: Fascinating but Still Elusive after 140 Years from Their Discovery

Varricchi

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“…ii) Complete resolution of symptoms or decrease in the frequency or severity of symptoms with anti-mast cell mediator therapy (antihistamines, leukotrienes modifiers and mast cell stabilizer agents). iii) Evidence of increase in a validated urinary or serum marker of MCAS (preferably with reproducible results obtained during more than one symptomatic episode) (12).…”

Section: Consensus Diagnostic Criteria For Mcasmentioning

confidence: 97%

Mast cell activation syndromes ‑ evaluation of current diagnostic criteria and laboratory tools in clinical practice (Review)

et al. 2020

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Mast cell activation syndromes (MCAS) represent a heterogeneous clinical entity caused by episodic and severe mast cell activation, including primary and secondary mast cell disorders (MCDs). The group of primary or clonal MCDs refers to systemic mastocytosis, other clonal MCAS and hereditary hypertryptasemia, while the secondary MCAS is characterized by normal mast cells (MCs) which are activated by external triggers, such as allergens or physical stimuli. Another category of MCAS is the idiopathic form, when no trigger or genetic mutation can be identified. Symptoms of mast cell activation are due to release of specific mediators and can be seen in many diseases, such as allergies, with localized or systemic clinical manifestations. Confirmation of MCAS is based on diagnostic criteria proposed by an international group of experts and the best available evidence in this field. It is generally accepted that the clinical picture of MCAS is non-specific and there are few available laboratory tools, making it difficult for clinicians to identify and confirm this entity. The diagnosis is established after exclusion of other possible clinical entities in most of the cases. Therefore, the actual diagnostic criteria of MCASs, some relevant clinical aspects and laboratory tools used in clinical practice were reviewed.

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“…Precision medicine is increasingly used as an approach to the management of allergy and anaphylaxis, thanks to progress in diagnostic tests and biomarkers now allowing thorough characterization of a patient's endotype 1 . Probability-based risk assessment and diagnostic algorithms have entered the allergists' toolbox [2][3][4] . Allergy tests must therefore offer reliable, robust, and proficient results in each patient.…”

mentioning

confidence: 99%