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Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome
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.
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“Other autoantibodies with a proposed aetiological role in POTS include antibodies against nicotinic acetylcholine-receptor in autonomic ganglia [26] and Sj€ ogren autoantibodies [41,49], detected in POTS through antibody screening programmes.”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [49]
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Cited by
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Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management
Fedorowski, A. (2019) · Journal of Internal Medicine · doi:10.1111/joim.12852 · Cited by 352
Vernino, S., Bourne, K. M., Stiles, L. E., Grubb, B. P., Fedorowski, A., Stewart, J. M., Arnold, A. C., Pace, L. A., Axelsson, J., Boris, J. R., et al. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102828 · Cited by 304
Self-citationAutoimmunity in postural orthostatic tachycardia syndrome: Current understanding
Vernino, S., & Stiles, L. E. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.04.005 · Cited by 107
Evaluation of postural tachycardia syndrome (POTS)
Goodman, B. P. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.04.004 · Cited by 77
Self-citation
Metadata
- Authors
- Goodman, B. P., Crepeau, A., Dhawan, P. S., Khoury, J. A., & Harris, L. A.
- Affiliations
- Department of Neurology
- Mayo Clinic (US)
- Corresponding author
- Goodman, Brent P.
- Crepeau, Amy
- Khoury, Julie A.
- Venue
- The Neurologist
- Published
- 2017-07
- Volume
- 22
- Issue
- 4
- Pages
- 127-130
- ISSN
- 1074-7931 (ISSN-L)
- OpenAlex
- W2691795141
- Cited by
- 43
- References
- 0
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 5 · 2023: 5 · 2022: 4 · 2021: 8 · 2020: 11 · 2019: 3 · 2018: 5
- FWCI
- 5.34
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Neurologist (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Physiology (medical) › Salivary Gland Disorders and Functions
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Keywords
- Sjögren's syndrome, autonomic dysfunction, orthostatic hypotension, esophageal motility disorders, gastrointestinal dysfunction, genitourinary dysfunction, immunomodulatory therapy, syncope
- MeSH terms
- Adolescent, Adult, Aged, Autonomic Nervous System Diseases, Female, Humans, Male, Middle Aged, Sjogren's Syndrome, Young Adult
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