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

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 patient...

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Autores principales: Kohno, Ritsuko, Cannom, David S., Olshansky, Brian, Xi, Shijun Cindy, Krishnappa, Darshan, Adkisson, Wayne O., Norby, Faye L., Fedorowski, Artur, Benditt, David G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8649306/
https://www.ncbi.nlm.nih.gov/pubmed/34398691
http://dx.doi.org/10.1161/JAHA.121.021002
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author Kohno, Ritsuko
Cannom, David S.
Olshansky, Brian
Xi, Shijun Cindy
Krishnappa, Darshan
Adkisson, Wayne O.
Norby, Faye L.
Fedorowski, Artur
Benditt, David G.
author_facet Kohno, Ritsuko
Cannom, David S.
Olshansky, Brian
Xi, Shijun Cindy
Krishnappa, Darshan
Adkisson, Wayne O.
Norby, Faye L.
Fedorowski, Artur
Benditt, David G.
author_sort Kohno, Ritsuko
collection PubMed
description 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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spelling pubmed-86493062022-01-14 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 Benditt, David G. J Am Heart Assoc Original Research 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. John Wiley and Sons Inc. 2021-08-16 /pmc/articles/PMC8649306/ /pubmed/34398691 http://dx.doi.org/10.1161/JAHA.121.021002 Text en © 2021 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Original Research
Kohno, Ritsuko
Cannom, David S.
Olshansky, Brian
Xi, Shijun Cindy
Krishnappa, Darshan
Adkisson, Wayne O.
Norby, Faye L.
Fedorowski, Artur
Benditt, David G.
Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title_full Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title_fullStr Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title_full_unstemmed Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title_short Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
title_sort mast cell activation disorder and postural orthostatic tachycardia syndrome: a clinical association
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8649306/
https://www.ncbi.nlm.nih.gov/pubmed/34398691
http://dx.doi.org/10.1161/JAHA.121.021002
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