Abstract
Purpose
Extraarticular manifestations of the joint hypermobility syndrome may include the
peripheral nervous system. The purpose of this study was to investigate autonomic
function in patients with this syndrome.
Methods
Forty-eight patients with the joint hypermobility syndrome who fulfilled the 1998
Brighton criteria and 30 healthy control subjects answered a clinical questionnaire
designed to evaluate the frequency of complaints related to the autonomic nervous
system. Next, 27 patients and 21 controls underwent autonomic evaluation: orthostatic
testing, cardiovascular vagal and sympathetic functions, catecholamine levels, and
adrenoreceptor responsiveness.
Results
Symptoms related to the autonomic nervous system, such as syncope and presyncope,
palpitations, chest discomfort, fatigue, and heat intolerance, were significantly
more common among patients. Orthostatic hypotension, postural orthostatic tachycardia
syndrome, and uncategorized orthostatic intolerance were found in 78% (21/27) of patients
compared with in 10% (2/21) of controls. Patients with the syndrome had a greater
mean (± SD) drop in systolic blood pressure during hyperventilation than did controls
(–11 ± 7 mm Hg vs. –5 ± 5 mm Hg, P = 0.02) and a greater increase in systolic blood pressure after a cold pressor test
(19 ± 10 mm Hg vs. 11 ± 13 mm Hg, P = 0.06). Patients with the syndrome also had evidence of α-adrenergic (as assessed
by administration of phenylephrine) and β-adrenergic hyperresponsiveness (as assessed
by administration of isoproterenol).
Conclusion
The autonomic nervous system–related symptoms of the patients have a pathophysiological
basis, which suggests that dysautonomia is an extraarticular manifestation in the
joint hypermobility syndrome.
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Article info
Publication history
Accepted:
March 5,
2003
Received:
July 25,
2002
Footnotes
☆Supported in part by a grant from the “Ya’el” research fund (c/o Biosence Israel, Haifa, Israel).
Identification
Copyright
© 2003 Excerpta Medica Inc. Published by Elsevier Inc. All rights reserved.
