Abstract
Primary Sjögren’s syndrome (pSS) is an autoimmune disease which primarily affects the exocrine glands, but can also affect other organs, including the nervous system. Many studies have reported evidence of autonomic nervous system (ANS) dysfunction in pSS which may contribute to a wide range of symptoms and functional burden. Symptoms of ANS dysfunction are common and widespread among patients with pSS and are associated with other features of the disease, particularly fatigue. Accumulating data on the inter-relationship between the ANS and the immune system via the vagus nerve have been reported. Vagus nerve stimulation (VNS) has also been associated with improvement in fatigue in patients with pSS. Taken together, these data suggest that the ANS may be a potential treatment target for pSS, in particularly those with fatigue being a predominant symptom. Future research to dissect the link between the ANS, immune dysregulation and clinical manifestations in pSS and to evaluate the potential of VNS as a therapy for pSS is warranted.
Highlights
Primary Sjögren’s syndrome is an autoimmune disease which primarily affects the exocrine glands, other organs including the nervous system can be affected
autonomic nervous system (ANS) dysfunction may result in alterations in the regulation of heart rate (HR), blood pressure (BP), baroreceptor sensitivity, heart rate variability (HRV) and blood pressure variability (BPV)
Of note, reduced HRV is a strong and independent predictor of a cardiac event in the general population [32], the HRV test reports better sensitivity and reproducibility compared to reflex tests [33] whilst correlating well with autonomic dysfunction [34]
Summary
Primary Sjögren’s syndrome (pSS) is an autoimmune disease which primarily affects the exocrine glands, other organs including the nervous system can be affected. ANS dysfunction may result in alterations in the regulation of heart rate (HR), blood pressure (BP), baroreceptor sensitivity, heart rate variability (HRV) and blood pressure variability (BPV) Cardiovascular reflex tests, such as measurements of HR or BP following deep breathing or a Valsalva manoeuvre, changes from lying to standing (tilt table test) can give insight into the autonomic function of the body. The investigators reported significant differences in autonomic reflexes such as finger skin blood flow, deep breathing, and orthostatic blood pressure testing in pSS patients compared to healthy controls suggesting dysfunction of both sympathetic and parasympathetic function. They further reported a significant decrease in orthostatic systolic and diastolic BP readings in 46 pSS patients compared to 56 age-matched controls [26]. Brunetta et al investigated the cardiovascular autonomic function of 19 patients with pSS by studying elements of the electrocardiogram (ECG), using RR variability alongside systolic pressure variability to create a low-frequency
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