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 language | English (US) |
|---|---|
| Pages (from-to) | 127-130 |
| Number of pages | 4 |
| Journal | Neurologist |
| Volume | 22 |
| Issue number | 4 |
| DOIs | |
| State | Published - Jan 1 2017 |
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Keywords
- autonomic nervous system
- autonomic neuropathy
- orthostatic hypotension
- postural orthostatic tachycardia syndrome
- Sjögren syndrome
ASJC Scopus subject areas
- Clinical Neurology
Cite this
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 journal › Article
}
TY - JOUR
T1 - Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome
AU - Goodman, Brent P.
AU - Crepeau, Amy
AU - Dhawan, Priya S.
AU - Khoury, Julie A.
AU - Harris, Lucinda A.
PY - 2017/1/1
Y1 - 2017/1/1
N2 - 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.
AB - 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.
KW - autonomic nervous system
KW - autonomic neuropathy
KW - orthostatic hypotension
KW - postural orthostatic tachycardia syndrome
KW - Sjögren syndrome
UR - http://www.scopus.com/inward/record.url?scp=85021796272&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85021796272&partnerID=8YFLogxK
U2 - 10.1097/NRL.0000000000000134
DO - 10.1097/NRL.0000000000000134
M3 - Article
C2 - 28644253
AN - SCOPUS:85021796272
VL - 22
SP - 127
EP - 130
JO - Neurologist
JF - Neurologist
SN - 1074-7931
IS - 4
ER -