Mast cell activation disorder and postural orthostatic tachycardia syndrome: A clinical association

Ritsuko Kohno, David S. Cannom, Brian Olshansky, Shijun Cindy Xi, Darshan Krishnappa, Wayne O. Adkisson, Faye L. Norby, Artur Fedorowski, David G. Benditt

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

BACKGROUND: Recently there has been increased interest in a possible association between mast cell activation (MCA) disorder and postural orthostatic tachycardia syndrome (POTS). This study examined the frequency with which symptoms and laboratory findings suggesting MCA disorder occurred in patients diagnosed with POTS. METHODS AND RESULTS: Data were obtained from patients in whom symptoms and orthostatic testing were consistent with a POTS diagnosis. Individuals with <4 months symptom duration, evident ongoing inflammatory disease, suspected volume depletion, or declined consent were excluded. All patients had typical POTS symptoms; some, however, had additional nonorthostatic complaints not usually associated with POTS. The latter patients underwent additional testing for known MCA biochemical mediators including prostaglandins, histamine, methylhistamine, and plasma tryptase. The study comprised 69 patients who met POTS diagnostic criteria. In 44 patients (44/69, 64%) additional nonorthostatic symptoms included migraine, allergic complaints, skin rash, or gastrointestinal symptoms. Of these 44 patients, 29 (66%) exhibited at least 1 laboratory abnormality suggesting MCA disorder, and 11/29 patients had 2 or more such abnormalities. Elevated prostaglandins (n=16) or plasma histamine markers (n=23) were the most frequent findings. Thus, 42% (29/69) of patients initially diagnosed with POTS exhibited both additional symptoms and at least 1 elevated biochemical marker suggesting MCA disorder. CONCLUSIONS: Laboratory findings suggesting MCA disorder were relatively common in patients diagnosed with POTS and who present with additional nonorthostatic gastrointestinal, cutaneous, and allergic symptoms. While solitary abnormal laboratory findings are not definitive, they favor MCA disorder being considered in such cases.

Original languageEnglish (US)
Article numbere021002
JournalJournal of the American Heart Association
Volume10
Issue number17
DOIs
StatePublished - Sep 7 2021

Bibliographical note

Funding Information:
Dr. Benditt was supported in part by a grant from the Dr. Family in support of Heart-Brain research.

Publisher Copyright:
© 2021 The Authors.

Keywords

  • Biochemical mediators
  • Histamine
  • Mast cell
  • Postural orthostatic tachycardia syndrome
  • Prostaglandins

PubMed: MeSH publication types

  • Journal Article
  • Research Support, Non-U.S. Gov't

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