Clinical Approach to MCAS Diagnosis and Cutaneous Mastocytosis
Table 4. Pharmacotherapy for mast-cell derived symptoms
[2, 3, 10, 11, 13]
Organ | Symptoms | Mediators | Treatment/Management |
Pulmonary Upper airway
Lower airway | Sneezing Rhinorrhea Nasal congestion Conjunctivitis Stridor Dyspnea Chest tightness Wheezing Hypoxia | Histamine PDG2 PAF Cysteinyl LTs | -H1 anti-histamines (oral, nasal) -Corticosteroids (nasal, inhaled) -Inhaled epinephrine -Anti-leukotrienes -Omalizumab |
Mucocutaneous | Angioedema Urticaria Flushing Pruritis | Histamine PDG2 PAF Cysteinyl LTs | -H1 and H2 anti histamines -Ketotifen -Rupatadine -Aspirin, NSAIDs -Omalizumab |
Gastrointestinal (GI) | Nausea Emesis Diarrhea Abdominal cramping | Histamine PAF Cysteinyl LTs | -H2 anti-histamines -Cromolyn (oral) -Corticosteroids |
Cardiovascular (CV) | Dizziness Lightheadedness (Pre)syncopeHypotension | Histamine PGD2 PAFCysteinyl LTs | -If emergency, see treatment below-H1 and H2 anti histamines -Anti-leukotrienes -Omalizumab -Venom immunotherapy, if applicable |
Anaphylaxis (≥ 2 organ systems, hypotension following exposure to known allergen [32]) | See above – also, reports of uterine contractions | Histamine PGD2 PAF Cysteinyl LTs | Dependent upon symptoms: -Epinephrine (IM, gtt) -IV fluids -Oxygen -Albuterol, ipratropium, racemic epinephrine -Position (supine with legs elevated, L decubitus if pregnant – caution with resuming upright position [empty vena cava]) [33] -Glucagon (if on beta blocker and non responsive to epinephrine, IVF; caution re: airway as this can cause emesis) -May consider beta-2 agonists to treat uterine contractions, NSAIDs for prevention of future uterine contractions (as long as no concern NSAID-related symptoms worsening) [34] |