Autonomic dysfunction in primary Sjogren’s syndrome: a prospective cohort analysis of 154 Korean patients

Abstract

Background/AimsTo determine the prevalence of autonomic dysfunction among Korean patients with primary Sjogren’s syndrome (pSS) and its associations with the clinical features of pSS.MethodsWe analyzed 154 participants from the Korean Initiative of primary Sjogren’s Syndrome (KISS) as a prospective pSS cohort and 154 age- and sex-matched healthy controls. A standardized 5-minute, supine, resting heart rate variability (HRV) test was performed, and autonomic dysfunction was defined as standard deviation of normal-to-normal RR intervals (SDNN) < 30 ms in patients < 50 years old and SDNN < 20 ms in patients ≥ 50 years old. The associations between autonomic dysfunction and various clinical features of pSS were analyzed.ResultsThe overall autonomic activity in patients with pSS was significantly lower than that in controls. Autonomic dysfunction with the HRV test was observed in 35.7% of the KISS participants and was associated with a higher European League Against Rheumatism Sjogren’s Syndrome Patient Reported Index fatigue score (p = 0.024). Raynaud’s phenomenon was a more frequent clinical presentation in pSS patients with autonomic dysfunction than in those without autonomic dysfunction (29.4% and 14.4%, respectively; p = 0.048). Decreased parasympathetic activity was observed in 41.6% of pSS patients. No differences were found in the oral and ocular signs of pSS according to the decreased parasympathetic activity.ConclusionsIn Korean patients with pSS, decreased and imbalanced autonomic activity is prevalent and is associated with fatigue. However, an association between autonomic dysfunction and glandular manifestations was not detected.

Neurological and Inflammatory Manifestations in Sjögren’s Syndrome: The Role of the Kynurenine Metabolic Pathway

“…Moreover, CNS manifestations of SS may be associated with focal syndromes (such as multiple-sclerosis-like epilepsy, movement disorders, neuromyelitis optica, and pseudotumor) and diffuse syndromes (encephalitis, meningitis, cognitive dysfunction, psychiatric disorders). Acute or chronic myelopathy and inferior motor neuron disease may also occur [57,58,62,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89]. This high variability of EGM and responses to treatment in SS are not completely understood.…”

Autonomic Abnormalities in Patients With Primary Sjogren’s Syndrome – Preliminary Results

“…While some investigation, via cardiovascular autonomic reflex testing, inferred alterations in both parasympathetic and sympathetic function others observed parasympathetic dysfunction only or no alteration at all (Andonopoulos et al, 1998; Barendregt et al, 1999; Mandl et al, 2001, 2007; Niemela et al, 2003; Kovacs et al, 2004). Furthermore, conflicting data have also been produced by studies using spectral analysis of heart rate (HR) variability, a sensitive non-invasive method to detect early and subtle abnormalities in cardiovascular autonomic function (Niemela et al, 2000; Tumiati et al, 2000; Barendregt et al, 2002; Cai et al, 2008; Ng et al, 2012; Koh et al, 2017). Finally, no data are available on the baroreceptor control in these patients.…”

<p>Managing fatigue in patients with primary Sj&ouml;gren&rsquo;s syndrome: challenges and solutions</p>

“…PSS patients also present a range of other manifestations associated with fatigue. These include sleep disturbances,21–23 autonomic dysfunction,24,25 depression,11,13,15,26,27 dysfunctional or alexithymic psychological profile,28 neuroticism, and fibromyalgia 15. These complex associations and comorbidities require appropriate management in clinical practice based on a multi-disciplinary approach including rheumatologists and other health professionals.…”