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Mast Cell Activation Syndrome and other Mast Cell Disorders

There are many medications that can act as mast cell activators. Below is a list of medications that either have a known or theoretical risk of activating mast cells and preferred alternatives to those medications. Note that people with Mast Cell Disorders can have variable reactions, and the medications in the "Acceptable" list may be a trigger, or one in the "Avoid" list may be fine for you. This is a general guideline. If you are taking any of the medications below, schedule an appointment or talk with your doctor on if and/or how to discontinue that medication.

 

Medication Class Avoid Acceptable
Hypnotic/sedative/Anesthetic agents Methohexital, thiopental (causes histamine release), phenobarbital dexmedetomidine, etomidate. Propofol and ketamine are likely acceptable, but cause in vitro (test tube) histamine release from mast cells.
Inhalant anesthetics Halogenated (isoflurane, sevoflurane, desflurane), nitrous oxide
Local anesthetic Amide type: Lidocaine, articaine. Ester-type: tetracaine, procaine prefer amide-type such as bupivacaine
Opioids Morphine, meperidine, codeine (all cause histamine release) fentanyl, sufentanil, alfentanil, remifentanil, buprenorphine, oxycodon, piritramid, Hydromorphone (twice the risk of fentanyl for urticaria)
Nonopioid analgesics NSAIDs (ketorolac, nefopam, aspirin, ibuprofen) cause overproduction of leukotrienes -a mast cell mediator Acetaminophen, paracetamol, metamizole
Benzodiazepines Midazolam, and diazepam are likely acceptable, but cause in vitro (test tube) histamine release from mast cells.
Muscle relaxants Nondepolarizing benzylisoquinolines (atracurium and mivacurium cause histamine release), Nondepolarizing aminosteroids (rocuronium)  Depolarizing neuromuscular blocking agents (succinylcholine), Nondepolarizing aminosteroids (pancuronium. Vecuronium is likely acceptable, but causes in vitro (test tube) histamine release from mast cells.)

Nondepolarizing Neuromuscular blocking agents (cis-atracurium)
Antibiotics Vancomycin and polymyxin B can cause histamine release, cefuroxime, Gyrase inhibitors, fluoroquinolones (contraindicated in connective tissue disorders as well) Roxithromycin, Penicillins, cephalosporins, sulfonamides, clindamycin
Selective Dopamine-and norepinephrine reuptake inhibitors Bupropion Amitriptyline, doxepine, clomipramine, maprotiline
SSRI All
Anticonvulsive agent carbamazepine, topiramate Clonazepam
Peptidergic drugs Icatibant, cetrorelix, sermorelin, octreotide, leuprolide Row 23, Cell 3
X-ray and MRI contrast medium Iodinated contrast medium, gadolinium chelate (Gd3+). Macrocyclic gadolinium chelate may be a better option (gadobutrol, gadoterate meglumine, gadoteridol. Ones that have a higher adverse incidence are linear gadolinium agents (gadopentate dimeglumine, and gadodiamide). Consider premedication if these are necessary Non-ionic contrast media: iohexol, iopamidol, iopromida, ioxilan, ioversol, idolatran, iodixanol
Plasma substitutes Hydroxyethyl starch, Gelatine Albumin solution, 0.9% NaCl solution, Ringer's solution
Cardiovascular drugs ACE inhibitors (can augment an anaphylactic reaction),  B-Adrenoceptor antagonists,  Adenosine and protamine can cause histamine release Sartans, calcium channel antagonistis, ivabradine, and much else. beta-blockers can decrease the effect of epinephrine in anaphylaxis.
Reversal of neuromuscular blockade:  Neostigmine, Sugammadex
Antiseptics Alcohol, chlorhexidine, povidone-iodine
Intravenous fluids Crystalloids, colloids, albumin, gelatin, hydroxyethyl starch
Common labor and delivery drugs Oxytocin, prostaglandins, methylergonovine, tocolytic agent (terbutaline)- Acceptable, though role of prostaglandins in causing or worsening reactions is unclear
Wound care Avoid plasters or adhesives if have significant skin reactions (such as in cutaneous mastocytosis). If they must be used, use an adhesive removal and slow removal of plaster/adhesive. Consider protecting the skin with Mepitac or Mepilex.
Misc. Alcohol containing medications and drinks atropine, glycopyrrolate, ondansetron, aprotinin (fibrin glue), blood transfusion, dyes, and latex (if no allergy)

Excipients

Excipients are inactive ingredients added to medications and are often the culprit in reactions to medications. These can be avoided by compounding the medication at a compounding pharmacy. Here is a tool that you can use to check for inactive ingredients in medications (search the specific drug name, scroll down to ingredients and appearance): https://dailymed.nlm.nih.gov/dailymed/

Other Perioperative Considerations:

  • Develop a preoperative plan with your doctor: It can be helpful to increase Mast Cell stabilizing medications prior to operations, and to take them until the day of surgery (if they need to be discontinued) and there may be premedication recommendations your doctor can recommend to discuss with the surgeon such as IV antihistamine use or steroids.
  • If you have had a reaction to a previous anesthetic, find out what it was and request a different anesthetic agent
  • Discuss preferred medications with anesthesiologist and surgeon before surgery
  • Other triggers to consider: psychological stress and anxiety, pain, environmental factors (too cold/hot of operating room, or changes in temperature), pressure (tourniquet, BP cuff), friction (tape), trauma, dry skin, histamine rich foods and odors (perfumes). It helps to minimize operative time, have optimal positioning, a quiet environment, reduce stress, control pain, maintain moderate/normal room temperate (can use heat maintenance devices, warm intravenous and irrigation fluids), moisturize skin, avoid puncturing blisters, avoid pressure and friction during procedures, request fragrance free and a single occupancy room.
  • Depending on the severity of the Mast Cell Disorder, it may be helpful to get markers before the surgery such as tryptase levels (can be a helpful reference point).

 

Resources:

  • Molderings GJ, Haenisch B, Brettner S, et al. Pharmacological treatment options for mast cell activation disease. Naunyn Schmiedebergs Arch Pharmacol. 2016;389(7):671-694. doi:10.1007/s00210-016-1247-1
  • Kumaraswami S, Farkas G. Management of a Parturient with Mast Cell Activation Syndrome: An Anesthesiologist's Experience. Case Rep Anesthesiol. 2018;2018:8920921. Published 2018 May 22. doi:10.1155/2018/8920921
  • Hepner, David. Perioperative Management of Patient with MCD. TMS training August 5, 2024
  • Pascale Dewachter, Mariana C. Castells, David L. Hepner, Claudie Mouton-Faivre; Perioperative Management of Patients with Mastocytosis. Anesthesiology 2014; 120:753–759 doi: https://doi.org/10.1097/ALN.0000000000000031

Postural Othrostatic Tachycardia Syndrome (POTS)

  • I perform an in office NASA lean test that is a screening test for POTS. If the results indicate that dysautonomia is likely, further evaluation of a tilt table and QSART testing is done.
  • Additional resources for POTS is coming soon

Hypermobility Spectrum Disorders

  • I perform diagnostic physical exams for adults and pediatrics (6 years old +) for hypermobility spectrum disorders, along with genetic testing if indicated. 
  • Additional resources are coming soon

Dietary Resources

  • Coming soon

Patient Handouts