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

Author:

Kohno Ritsuko1,Cannom David S.23,Olshansky Brian4ORCID,Xi Shijun Cindy3,Krishnappa Darshan1ORCID,Adkisson Wayne O.1,Norby Faye L.5ORCID,Fedorowski Artur67ORCID,Benditt David G.1ORCID

Affiliation:

1. Cardiac Arrhythmia Center Cardiovascular Division University of Minnesota Minneapolis MN

2. Hospital of the Good Samaritan Los Angeles CA

3. University of Southern California Keck School of Medicine Los Angeles CA

4. Department of Medicine University of Iowa Iowa City IA

5. Smidt Heart Institute Cedars‐Sinai Heart System Los Angeles CA

6. Departments of Cardiology and Medicine (Karolinska) Karolinska University Hospital, Karoloinska Instiute, and Lund University Stockholm Sweden

7. Department of Clinical Sciences (Lund) Karolinska University Hospital, Karoloinska Instiute, and Lund University Malmo Sweden

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Cardiology and Cardiovascular Medicine

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