Current Treatment Options in Allergy

, Volume 3, Issue 4, pp 384–400

Mast Cell Activation Syndromes

Urticaria and Atopic Dermatitis (M Ferrer-Puga, Section Editor)

DOI: 10.1007/s40521-016-0100-6

Cite this article as:
Bonamichi-Santos, R. & Castells, M. Curr Treat Options Allergy (2016) 3: 384. doi:10.1007/s40521-016-0100-6
Part of the following topical collections:
  1. Topical Collection on Urticaria and Atopic Dermatitis

Opinion statement

Mast cell activation syndromes(MCAS) are disorders associated with mast cell activation (MCA), and include Primary MCAS, Secondary MCAS and Idiopathic MCAS. MCAS are characterized by clinical symptoms of MCA in cutaneous, gastrointestinal, respiratory, cardiovascular, musculoskeletal and neurological organs. Mast cell (MC) mediators such as tryptase in serum, and/or histamine or prostaglandin urinary metabolites are typically elevated at base line or transiently during episodes of MCA, and there is a total or partial response to mast cell mediators controller medications. In primary MCAS an activating point mutation at codon 816 of KIT on MC is present in most cases of Systemic Mastocytosis (SM) and Monoclonal Mast Cell Activation Syndrome (MMCAS) and is absent in secondary and Idiopathic MCAS. MCAS might be the underlying cause of unexplained symptoms when several organ systems are involved, such as the gastrointestinal tract and the skin. It is especially important to be able to recognize the constellation of clinical features because response to anti-MC mediator medications is often excellent. This update on mast cell disorders (MCD) provides an insight into the classification, clinical presentations, diagnosis, treatment and management. We describe associated conditions, such as Hymenoptera Reactions, Familial Tryptasemia, Postural Orthostatic Tachycardia Syndrome and Ehlers-Danlos Syndrome.

Keywords

Mast cellMast cell activationTryptaseHistamineAllergyAnaphylaxisAntihistamines

Abbreviations

CM

Cutaneous mastocytosis

EDS

Ehlers-Danlos syndrome

HV

Hymenoptera venom

IMCAS

Idiopathic mast cell activation syndrome

MC

Mast cell

MCA

Mast cell activation

MCAS

Mast cell activation syndromes

MCT

Mast Cell T

MCTc

Mast cell TC

MMAS

Monoclonal mast cell activation syndrome

POTS

Postural tachycardia syndrome

SM

Systemic mastocytosis

VIT

Venom immunotherapy

Copyright information

© Springer International Publishing AG 2016

Authors and Affiliations

  1. 1.Division of Rheumatology, Immunology and Allergy; Department of MedicineBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Clinical Immunology and Allergy DivisionUniversity of São PauloSão PauloBrazil