Table 2 Proposed criteria for mast cell activation syndrome (all three criteria must be present) (Akin, 2017)
Mast cell activation disease (MCAD), includes individual mast cell activation disorders and mast cell activation syndrome (MCAS) | |
|---|---|
Individual mast cell activation disorders | Skin: urticaria, angioedema, flushing |
Gastrointestinal: nausea, vomiting, diarrhea: abdominal cramping/pain, gastroesophageal reflux disease | |
Cardiovascular: hypotensive syncope or near syncope, tachycardia | |
Neuropsychiatric: brain fog, anxiety, depression, paresthesia, lightheadedness | |
Respiratory: wheezing | |
Naso-ocular: conjunctival injection, pruritus, nasal congestion, posterior rhinorrhea | |
Genitourinary: pain with urination, urinary frequency; females—pain with intimate relations; heavy, painful menses | |
Musculoskeletal: bone pain, muscle pain, degenerative disc disease, osteopenia/osteoporosis | |
MCAS diagnosis: three criteria | (1) In addition to two or more of the above-mentioned MCA disorders |
(2) An elevated biomarker for mast cell activation syndrome, which can include semi tryptase or urinary mediators, such as prostaglandin and histamine metabolites (prostaglandin 1)2, 11 Beta Prostaglandin F2 Alpha, or methylhistamine | |
(3) A decrease in the frequency, severity, or resolution of symptoms with histamine receptor antagonists or other mast cell-targeted medications, such as ketotifen, omalizumab, cromones, or tricyclic agents | |