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Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome : A Clinical Association

Kohno, Ritsuko ; Cannom, David S ; Olshansky, Brian ; Xi, Shijun Cindy ; Krishnappa, Darshan ; Adkisson, Wayne O ; Norby, Faye L ; Fedorowski, Artur LU orcid and Benditt, David G (2021) In Journal of the American Heart Association 10(17).
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... (More)

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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
mast cell, postural orthostatic tachycardia syndrome, biochemical mediators, histamine, prostaglandins
in
Journal of the American Heart Association
volume
10
issue
17
article number
e021002
publisher
Wiley-Blackwell
external identifiers
  • pmid:34398691
  • scopus:85116171390
ISSN
2047-9980
DOI
10.1161/JAHA.121.021002
language
English
LU publication?
yes
id
b63f6124-2941-466e-aeda-6af9c2168cc5
date added to LUP
2021-08-20 11:11:43
date last changed
2024-06-29 15:53:36
@article{b63f6124-2941-466e-aeda-6af9c2168cc5,
  abstract     = {{<p>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 &lt;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.</p>}},
  author       = {{Kohno, Ritsuko and Cannom, David S and Olshansky, Brian and Xi, Shijun Cindy and Krishnappa, Darshan and Adkisson, Wayne O and Norby, Faye L and Fedorowski, Artur and Benditt, David G}},
  issn         = {{2047-9980}},
  keywords     = {{mast cell; postural orthostatic tachycardia syndrome; biochemical mediators; histamine; prostaglandins}},
  language     = {{eng}},
  number       = {{17}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of the American Heart Association}},
  title        = {{Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome : A Clinical Association}},
  url          = {{http://dx.doi.org/10.1161/JAHA.121.021002}},
  doi          = {{10.1161/JAHA.121.021002}},
  volume       = {{10}},
  year         = {{2021}},
}