Lecture by Dr. Anne Maitland on Mast Cell Activation Disorder in Ehlers-Danlos Syndrome

Dr. Anne Maitland’s lecture explains how mast cell activation disorder (MCAD/MCAS) commonly overlaps with hypermobile Ehlers-Danlos syndrome (hEDS) and often with POTS/dysautonomia. She reviews the wide, multi‑system symptoms caused by abnormal mast cell mediator release—flushing, hives, itching, swelling, food and drug reactions, GI upset, bladder issues, bone pain, fatigue, brain fog, anxiety, temperature intolerance, and cardiovascular symptoms. She describes how mast cells live in connective tissue and around nerves and blood vessels, so in patients with fragile connective tissue (like hEDS) their activation can worsen pain, autonomic instability, and “allergic‑type” reactivity without classic allergy test results. Maitland outlines current diagnostic challenges, emphasizing that normal skin or blood allergy tests do not rule out MCAS; instead, diagnosis relies on symptom patterns, response to mast‑cell–directed treatment, and sometimes mediator testing (e.g., serum tryptase, urinary prostaglandins and histamine metabolites), while acknowledging that available tests are insensitive and often need to be repeated around flares. She discusses management as a long‑term, stepwise process: trigger identification and avoidance (heat, friction, infections, stress, hormones, certain foods, medications, alcohol, strong smells), low‑histamine or personalized elimination diets, and careful choice of medications (second‑generation H1 antihistamines like cetirizine/loratadine, H2 blockers like famotidine, leukotriene blockers, cromolyn, sometimes low‑dose aspirin or ketotifen, and in select cases biologics like omalizumab). She stresses slow titration due to hypersensitivity, attention to excipients (dyes, fillers), and coordination between allergy/immunology, cardiology/autonomic specialists, and pain/rehab clinicians. Throughout, she encourages clinicians to take patient reports seriously even when tests are negative, and she frames MCAS in EDS as a real, physiology‑based condition that can be substantially improved with systematic trigger management, thoughtful pharmacologic therapy, and supportive measures such as hydration, compression, and graded activity for co‑existing POTS and pain.