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Mast Cell Activation syndrome (MCAS)

Mast cell activation syndrome (MCAS) or Mast Cell Activation Disorder (MCAD) is a condition with signs and symptoms involving the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems. It can be classified into primary, secondary, and idiopathic. 

Clinical Criteria for Diagnosis of MCAS includes episodic symptoms consistent with mast cell mediator release affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope or near syncope, tachycardia, wheezing, conjunctival injection, pruritus, and nasal stuffiness.

Other criteria include a decrease in the frequency, severity, or resolution of symptoms with anti-mediator therapy including H(1) and H(2)histamine receptor antagonists, anti-leukotrienes, or mast cell stabilizers. 

Laboratory results showing evidence of mast cell activation - Serum tryptase or urinary N-methyl histamine, or the histamine metabolites prostaglandin D2 and prostaglandin F2-alpha.

A subset of patients may have problems with their autonomic nervous system, in the form of POTS (Postural Orthostatic Tachycardia Syndrome).

Very rarely patients with EDS (Ehler-Danlos Syndrome) may have an overlap with  MCAS and POTS.  

The spectrum of Mast Cell disease is wide and includes Cutaneous Mastocytosis (Urticaria Pigmentosa) and systemic Mastocytosis.

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