Table 5 Treatment options for mast cell activation disease.

Basic therapy (continuous oral combination therapy to reduce mast cell activity)

• H₁-histamine receptor antagonist (to block activating H₁-histamine receptors on mast cells; to antagonize H₁-histamine receptor-mediated symptoms)

• H₂- histamine receptor antagonist (to block activating H₂-histamine receptors on mast cells; to antagonize H₂-histamine receptor-mediated symptoms)

• Cromolyn sodium (stabilising mast cells)

• Slow-release Vitamin C (increased degradation of histamine; inhibition of mast cell degranulation; not more than 750 mg/day)

• If necessary, ketotifen to stabilise mast cells and to block activating H₁-histamine receptors on mast cells

Symptomatic treatment options (orally as needed)

• Headache⇒ paracetamol; metamizole; flupirtine

• Diarrhea⇒ colestyramine; nystatin; montelukast; 5-HT₃ receptor inhibitors (eg. ondansetron); incremental doses (50-350 mg/day; extreme caution because of the possibility to induce mast cell degranulation) of acetylsalicylic acid; (in steps test each drug for 5 days until improvement of diarrhea)

• Colicky abdominal pain due to distinct meteorism ⇒ metamizole; butylscopolamine

• Nausea⇒ metoclopramide; dimenhydrinate; 5-HT₃ receptor inhibitors; icatibant

• Respiratory symptoms(mainly increased production of viscous mucus and obstruction with compulsive throat clearing) ⇒ montelukast; urgent: short-acting ß-sympathomimetic

• Gastric complaints⇒ proton pump inhibitors (de-escalating dose finding)

• Osteoporosis, osteolysis, bone pain⇒ biphosphonates ([51]; vitamin D plus calcium application is second-line treatment in MCAD patients because of limited reported success and an increased risk for developing kidney and ureter stones; [52])

• Non-cardiac chest pain⇒ when needed, additional dose of a H₂-histamine receptor antagonist; also, proton pump inhibitors for proven gastroesophageal reflux

• Tachycardia⇒ verapamil; AT1-receptor antagonists; ivabradin

• Neuropathic pain and paresthesia⇒ α-lipoic acid

• Interstitial cystitis⇒ pentosan, amphetamines

• Sleep-onset insomnia/sleep-maintenance insomnia⇒ triazolam/oxazepam

• Conjunctivitis⇒ exclusion of a secondary disease; otherwise preservative-free eye drops with glucocorticoids for brief courses

• Hypercholesterolemia⇒ (does not depend on the composition of the diet) therapeutic trial with HMG-CoA reductase inhibitors (frequently ineffective)

• Elevated prostaglandin levels, persistant flushing⇒ incremental doses of acetylsalicylic acid (50-350 mg/day; extreme caution because of the possibility to induce mast cell degranulation)