Abstract
Mast cell activation syndrome (MCAS) is defined by international authorities as an illness with severe episodic symptoms due to mast cell activation affecting at least two body systems concurrently with laboratory evidence of mast cell mediator release and a response to anti-mast cell mediator treatments. It is one example of a mast cell activation disorder. There is currently no high quality evidence that MCAS is associated with dysautonomias, hypermobility or both although symptoms may overlap and some patients with postural orthostatic tachycardia syndrome (PoTS) and hypermobility Ehlers Danlos syndrome (hEDS) benefit symptomatically with anti-mast cell mediator medication, including antihistamines. A trial of treatment can therefore be considered in the appropriate clinical context.
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References
Holgate ST, Church MK, Broide DH, Martinez FD, editors. Allergy, 4th edn. Elsevier Saunders, Edinburgh; 2012.
Rich RR, Fleischer TA, Shearer WT, Schroeder HW, Frew AJ, Weyland CM, editors. Clinical Immunology: Principles and Practice, 5th edn. Elsevier; 2019.
Simons FER, Ebisawa M, Sanchez-Borges M, Thong BY, Worm M, et al. 2015 update of the evidence base: World allergy organization anaphylaxis guidelines. World Allergy Organization Journal. 2015;8:32.
Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010;126(6):1099–104.
Akin C. Mast cell activation syndromes. J Allergy Clin Immunol. 2017;140(2):349–55.
Valent P. Mast cell activation syndromes: definition and classification. Allergy. 2013;68(4):417–24.
Afrin LB. Mast cell activation disease and the modern epidemic of chronic inflammatory disease. Transl Res. 2016;174:33–59.
Valent P, Akin C, Bonadonna P, Hartmann K, Brockow K, Niedoszytko M, Nedoszytko B, Siebenhaar F, Sperr WR, Oude Elberink JNG, Butterfield JH, Alvarez-Twose I, Sotlar K, Reiter A, Kluin-Nelemans HC, Hermine O, Gotlib J, Broesby-Olsen S, Orfao A, Horny HP, Triggiani M, Arock M, Schwartz LB, Metcalfe DD. Proposed diagnostic algorithm for patients with suspected mast cell activation syndrome. J Allergy Clin Immunol Pract. 2019;7(4):1125–33.
Weiler CR, Austen KF, Akin C, Barkoff MS, Bernstein JA, Bonadonna P, Butterfield JH, Carter M, Fox CC, Maitland A, Pongdee T, Mustafa SS, Ravi A, Tobin MC, Vliagoftis H, Schwartz LB. AAAAI mast cell disorders committee work group report: mast cell activation syndrome (MCAS) diagnosis and management. J Allergy Clin Immunol. 2019;144(4):883–96.
Horny HP, Akin C, Arber DA Peterson LC, Tefferi A, Metcalfe DD, Bennett JM, Bain BJ, Escribano L, Valent P. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL, et al. editors. World Health Organization (WHO) classification of tumours. Pathology & Genetics. Tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press; 2016.
Lyons JJ, Yu X, Hughes JD, Le QT, Jamil A, Bai Y, Ho N, Zhao M, Liu Y, O’Connell MP, Trivedi NN, Nelson C, DiMaggio T, Jones N, Matthews H, Lewis KL, Oler AJ, Carlson RJ, Arkwright PD, Hong C, Agama S, Wilson TM, Tucker S, Zhang Y, McElwee JJ, Pao M, Glover SC, Rothenberg ME, Hohman RJ, Stone KD, Caughey GH, Heller T, Metcalfe DD, Biesecker LG, Schwartz LB, Milner JD. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nat Genet. 2016;48(12):1564–9.
Valent P, Bonadonna P, Hartmann K, Broesby-Olsen S, Brockow K, Butterfield JH, Triggiani M, Lyons JJ, Oude Elberink JNG, Arock M, Metcalfe DD, Akin C. Why the 20% + 2 Tryptase formula is a diagnostic gold standard for severe systemic mast cell activation and mast cell activation syndrome. Int Arch Allergy Immunol. 2019;180(1):44–51.
Weiler CR. Mast cell activation syndrome: Tools for diagnosis and differential diagnosis. J Allergy Clin Immunol Pract. 2020;8(2):498–506.
Ravi A, Butterfield J, Weiler CR. Mast cell activation syndrome: improved identification by combined determinations of serum tryptase and 24-hours urine 11 & #x03B2;-PGF2α. J Allergy Clin Immunol Pract. 2014;2:775–8.
Kohn A, Chang C. The relationship between hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS). Clin Rev Allergy Immunol. 2020;58(3):273–297.
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Grattan, C.E.H. (2021). Mast Cell Activation Syndrome (MCAS). In: Gall, N., Kavi, L., Lobo, M.D. (eds) Postural Tachycardia Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-54165-1_21
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DOI: https://doi.org/10.1007/978-3-030-54165-1_21
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