Advertisement
Rapid Communication| Volume 224, 102637, March 2020

Prevalence of hypermobile Ehlers-Danlos syndrome in postural orthostatic tachycardia syndrome

Published:January 10, 2020DOI:https://doi.org/10.1016/j.autneu.2020.102637

      Abstract

      Despite well-established clinical associations between Hypermobile Ehlers-Danlos syndrome (hEDS) and postural orthostatic tachycardia syndrome (POTS), the precise prevalence is unknown. We therefore evaluated for hEDS in 91 POTS participants using the 2017 hEDS diagnostic checklist, which has three major criteria: 1) generalized joint hypermobility (Beighton score), 2) systemic features, family history, and 3) absence of exclusion criteria. Overall, 28 out of 91 POTS participants (31%) met clinical criteria for hEDS. An additional 24% of participants had generalized joint hypermobility without meeting hEDS criteria. Identifying the prevalence of hEDS in POTS is important for understanding possible mechanisms connecting these two syndromes.

      Abbreviations:

      EDS (Ehlers-Danlos syndromes), hEDS (hypermobile Ehlers-Danlos syndrome), OI (orthostatic intolerance), POTS (postural orthostatic tachycardia syndrome)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Autonomic Neuroscience: Basic and Clinical
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Boris J.R.
        • Bernadzikowski T.
        Demographics of a large paediatric postural orthostatic tachycardia syndrome program.
        Cardiol. Young. 2018; 28: 668-674
        • Cazzato D.
        • Castori M.
        • Lombardi R.
        • Caravello F.
        • Bella E.D.
        • Petrucci A.
        • Grammatico P.
        • Dordoni C.
        • Colombi M.
        • Lauria G.
        Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes.
        Neurology. 2016; 87: 155-159
        • Deb A.
        • Morgenshtern K.
        • Culbertson C.J.
        • Wang L.B.
        • Hohler A.D.
        A survey-based analysis of symptoms in patients with postural orthostatic tachycardia syndrome.
        Proc (Bayl Univ Med Cent). 2015; 28: 157-159
        • Juul-Kristensen B.
        • Schmedling K.
        • Rombaut L.
        • Lund H.
        • Engelbert R.H.
        Measurement properties of clinical assessment methods for classifying generalized joint hypermobility-a systematic review.
        Am J Med Genet C Semin Med Genet. 2017; 175: 116-147
        • Kavi L.N.
        • Michaela
        • Low D.A.
        • Opie M.
        • Nicholson L.M.
        • Caldow E.
        • Newton J.L.
        A profile of patients with postural tachycardia syndrome and their experience of healthcare in the UK.
        The British Journal of Cardiology. 2016; : 23
        • Malfait F.
        • Francomano C.
        • Byers P.
        • Belmont J.
        • Berglund B.
        • Black J.
        • Bloom L.
        • Bowen J.M.
        • Brady A.F.
        • Burrows N.P.
        • Castori M.
        • Cohen H.
        • Colombi M.
        • Demirdas S.
        • De Backer J.
        • De Paepe A.
        • Fournel-Gigleux S.
        • Frank M.
        • Ghali N.
        • Giunta C.
        • Grahame R.
        • Hakim A.
        • Jeunemaitre X.
        • Johnson D.
        • Juul-Kristensen B.
        • Kapferer-Seebacher I.
        • Kazkaz H.
        • Kosho T.
        • Lavallee M.E.
        • Levy H.
        • Mendoza-Londono R.
        • Pepin M.
        • Pope F.M.
        • Reinstein E.
        • Robert L.
        • Rohrbach M.
        • Sanders L.
        • Sobey G.J.
        • Van Damme T.
        • Vandersteen A.
        • van Mourik C.
        • Voermans N.
        • Wheeldon N.
        • Zschocke J.
        • Tinkle B.
        The 2017 international classification of the Ehlers-Danlos syndromes.
        Am J Med Genet C Semin Med Genet. 2017; 175: 8-26
        • Roma M.
        • Marden C.L.
        • De Wandele I.
        • Francomano C.A.
        • Rowe P.C.
        Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome.
        Auton. Neurosci. 2018; 215: 89-96
        • Rowe P.C.
        • Barron D.F.
        • Calkins H.
        • Maumenee I.H.
        • Tong P.Y.
        • Geraghty M.T.
        Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome.
        J. Pediatr. 1999; 135: 494-499
        • Shaw B.H.
        • Stiles L.E.
        • Bourne K.
        • Green E.A.
        • Shibao C.A.
        • Okamoto L.E.
        • Garland E.M.
        • Gamboa A.
        • Diedrich A.
        • Raj V.
        • Sheldon R.S.
        • Biaggioni I.
        • Robertson D.
        • Raj S.R.
        The face of postural tachycardia syndrome - insights from a large cross-sectional online community-based survey.
        J. Intern. Med. 2019; 286: 438-448
        • Sheldon R.S.
        • Grubb 2nd, B.P.
        • Olshansky B.
        • Shen W.K.
        • Calkins H.
        • Brignole M.
        • Raj S.R.
        • Krahn A.D.
        • Morillo C.A.
        • Stewart J.M.
        • Sutton R.
        • Sandroni P.
        • Friday K.J.
        • Hachul D.T.
        • Cohen M.I.
        • Lau D.H.
        • Mayuga K.A.
        • Moak J.P.
        • Sandhu R.K.
        • Kanjwal K.
        2015 heart rhythm society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope.
        Heart Rhythm. 2015; 12: e41-e63
        • Tinkle B.
        • Castori M.
        • Berglund B.
        • Cohen H.
        • Grahame R.
        • Kazkaz H.
        • Levy H.
        Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history.
        Am J Med Genet C Semin Med Genet. 2017; 175: 48-69
        • Wallman D.
        • Weinberg J.
        • Hohler A.D.
        Ehlers-Danlos syndrome and postural tachycardia syndrome: a relationship study.
        J. Neurol. Sci. 2014; 340: 99-102