Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome

Brent P. Goodman, Amy Crepeau, Priya S. Dhawan, Julie A. Khoury, Lucinda A. Harris

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Objective: To describe the spectrum of autonomic dysfunction in a uniformly evaluated cohort of patients with Sjögren syndrome. Methods: A series of 13 patients underwent a comprehensive evaluation for suspected autonomic impairment, including a neurological examination, autonomic testing, and laboratory studies. A diagnosis of Sjögren syndrome was established as the cause of autonomic dysfunction in all. Clinical features, findings on autonomic testing, and laboratory results are described. Results: All patients in this series reported postural lightheadedness and syncope or near-syncope. Autonomic testing confirmed the presence of orthostatic hypotension on tilt-table testing in 5 patients and an excessive postural tachycardia and/or hypertensive response in 8 patients. Only 2 of the patients with orthostatic hypotension had a significant sensory neuropathy. Symptoms suggestive of gastrointestinal and genitourinary impairment were seen in nearly all patients, with abnormal motility testing (most frequently esophageal dysmotility) in 5 of 6 patients who underwent formal testing. Patients in this series treated with immune-modulating therapy experienced significant improvement. Conclusions: A diagnosis of Sjögren syndrome should be aggressively pursued in patients with signs and symptoms suggestive of autonomic nervous system impairment. Although the spectrum of adrenergic failure is variable, ranging from orthostatic hypotension to an excessive postural tachycardia, most patients do have symptoms of more generalized autonomic failure. Patients who were treated with immune-modulating therapy did improve.

Original languageEnglish (US)
Pages (from-to)127-130
Number of pages4
JournalNeurologist
Volume22
Issue number4
DOIs
StatePublished - Jan 1 2017

Fingerprint

Autonomic Nervous System
Orthostatic Hypotension
Syncope
Tachycardia
Esophageal Motility Disorders
Neurologic Examination
Dizziness
Adrenergic Agents
Signs and Symptoms

Keywords

  • autonomic nervous system
  • autonomic neuropathy
  • orthostatic hypotension
  • postural orthostatic tachycardia syndrome
  • Sjögren syndrome

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Goodman, B. P., Crepeau, A., Dhawan, P. S., Khoury, J. A., & Harris, L. A. (2017). Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome. Neurologist, 22(4), 127-130. https://doi.org/10.1097/NRL.0000000000000134

Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome. / Goodman, Brent P.; Crepeau, Amy; Dhawan, Priya S.; Khoury, Julie A.; Harris, Lucinda A.

In: Neurologist, Vol. 22, No. 4, 01.01.2017, p. 127-130.

Research output: Contribution to journalArticle

Goodman, BP, Crepeau, A, Dhawan, PS, Khoury, JA & Harris, LA 2017, 'Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome', Neurologist, vol. 22, no. 4, pp. 127-130. https://doi.org/10.1097/NRL.0000000000000134
Goodman BP, Crepeau A, Dhawan PS, Khoury JA, Harris LA. Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome. Neurologist. 2017 Jan 1;22(4):127-130. https://doi.org/10.1097/NRL.0000000000000134
Goodman, Brent P. ; Crepeau, Amy ; Dhawan, Priya S. ; Khoury, Julie A. ; Harris, Lucinda A. / Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome. In: Neurologist. 2017 ; Vol. 22, No. 4. pp. 127-130.
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