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Mast cell activation syndrome information


Mast cell activation syndrome
SpecialtyImmunology (Allergy)

Mast cell activation syndrome (MCAS) is a term referring to one of two types of mast cell activation disorder (MCAD); the other type is idiopathic MCAD.[1] MCAS is an immunological condition in which mast cells inappropriately and excessively release chemical mediators, resulting in a range of chronic symptoms, sometimes including anaphylaxis or near-anaphylaxis attacks.[2][3][4] Primary symptoms include cardiovascular, dermatological, gastrointestinal, neurological and respiratory problems.[3][5]

MCAS is an umbrella term that describes a set of symptoms; it is not a specific diagnosis.[1][6] Multiple diagnostic schemes for MCAS have been proposed. MCAS has often been misdiagnosed, especially as medical professionals and patients don’t understand that symptoms can wax or wane, and it can be a seemingly slow buildup with symptoms occurring in multiple organ systems before anaphylaxis type episodes; most doctors are not trained to recognize its symptoms nor know much about what to do with it; after all, it was only in 2010 that diagnostic criteria was formalized. [7]

  1. ^ a b Valent P, Hartmann K, Bonadonna P, Gülen T, Brockow K, Alvarez-Twose I, Hermine O, Niedoszytko M, Carter MC, Hoermann G, Butterfield JH, Lyons JJ, Sperr WR, Greiner G, Sotlar K (2022-05-24). "Global Classification of Mast Cell Activation Disorders: An ICD-10-CM-Adjusted Proposal of the ECNM-AIM Consortium". The Journal of Allergy and Clinical Immunology. In Practice. 10 (8): 1941–1950. doi:10.1016/j.jaip.2022.05.007. hdl:10261/296655. ISSN 2213-2201. PMID 35623575. S2CID 249073293. Archived from the original on 2023-02-11. Retrieved 2023-02-10.
  2. ^ Valent P (April 2013). "Mast cell activation syndromes: definition and classification". Allergy. 68 (4): 417–24. doi:10.1111/all.12126. PMID 23409940. S2CID 43636053.
  3. ^ a b Akin C, Valent P, Metcalfe DD (December 2010). "Mast cell activation syndrome: Proposed diagnostic criteria". The Journal of Allergy and Clinical Immunology. 126 (6): 1099–104.e4. doi:10.1016/j.jaci.2010.08.035. PMC 3753019. PMID 21035176.
  4. ^ Akin C (May 2015). "Mast cell activation syndromes presenting as anaphylaxis". Immunology and Allergy Clinics of North America. 35 (2): 277–85. doi:10.1016/j.iac.2015.01.010. PMID 25841551.
  5. ^ Conway AE, Verdi M, Shaker MS, Bernstein JA, Beamish CC, Morse R, Madan J, Lee MW, Sussman G, Al-Nimr A, Hand M, Albert DA (March 2024). "Beyond Confirmed Mast Cell Activation Syndrome: Approaching Patients With Dysautonomia and Related Conditions". J Allergy Clin Immunol Pract. doi:10.1016/j.jaip.2024.03.019. PMID 38499084.
  6. ^ Gülen T, Akin C, Bonadonna P, Siebenhaar F, Broesby-Olsen S, Brockow K, Niedoszytko M, Nedoszytko B, Oude Elberink HN, Butterfield JH, Sperr WR, Alvarez-Twose I, Horny HP, Sotlar K, Schwaab J (November 2021). "Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review". The Journal of Allergy and Clinical Immunology. In Practice. 9 (11): 3918–3928. doi:10.1016/j.jaip.2021.06.011. hdl:10261/268013. ISSN 2213-2201. PMID 34166845. S2CID 235634993. Archived from the original on 2023-02-10. Retrieved 2023-02-10.
  7. ^ Vacheron N, McClinton T, Lynch-Smith DJ, Umberger R (April 8, 2020). "Mast cell activation syndrome". J Am Assoc Nurse Pract. 33 (7): 545–552. doi:10.1097/JXX.0000000000000396. PMID 32282570. Archived from the original on April 24, 2024. Retrieved April 24, 2024.

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