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Table 5 Treatment options for mast cell activation disease.

From: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

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)
  1. All drugs should be tested for tolerance in a low single dose before therapeutic use, if their tolerance in the patient is not known from an earlier application.