Autonomic symptoms are common and are associated with overall symptom burden and disease activity in primary Sjögren's syndrome

Abstract

ObjectivesTo determine the prevalence of autonomic dysfunction (dysautonomia) among patients with primary Sjögren's syndrome (PSS) and the relationships between dysautonomia and other clinical features of PSS.MethodsMulticentre, prospective, cross-sectional study of a UK cohort of 317 patients with clinically well-characterised PSS. Symptoms of autonomic dysfunction were assessed using a validated instrument, the Composite Autonomic Symptom Scale (COMPASS). The data were compared with an age- and sex-matched cohort of 317 community controls. The relationships between symptoms of dysautonomia and various clinical features of PSS were analysed using regression analysis.ResultsCOMPASS scores were significantly higher in patients with PSS than in age- and sex-matched community controls (median (IQR) 35.5 (20.9–46.0) vs 14.8 (4.4–30.2), p<0.0001). Nearly 55% of patients (vs 20% of community controls, p<0.0001) had a COMPASS score >32.5, a cut-off value indicative of autonomic dysfunction. Furthermore, the COMPASS total score correlated independently with EULAR Sjögren's Syndrome Patient Reported Index (a composite measure of the overall burden of symptoms experienced by patients with PSS) (β=0.38, p<0.001) and disease activity measured using the EULAR Sjögren's Syndrome Disease Activity Index (β=0.13, p<0.009).ConclusionsAutonomic symptoms are common among patients with PSS and may contribute to the overall burden of symptoms and link with systemic disease activity.

The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren’s Syndrome Diagnosis

“…Renal involvement is rare, occurring in less than 10% of cases [16] and most commonly involving renal tubular acidosis. Autonomic symptoms may affect 50% of individuals with SS [17]. Clinical pulmonary involvement occurs in 20-30% of patients with SS and is associated with a four-fold increase in 10-year mortality [18].…”

Bilateral Tonic Pupils as the Initial Manifestation of Sjögren’s Syndrome

“…4 The association of autonomic dysfunction and primary Sjö gren's syndrome has been established in previous studies. 5 Newton et al's 2012 study of 317 patients with primary Sjögren's syndrome demonstrated a 55% prevalence of dysautonomia, predominantly orthostatic hypotension. 5 However, very few cases of tonic pupils have been reported in Sjö gren's syndrome.…”

“…5 Newton et al's 2012 study of 317 patients with primary Sjögren's syndrome demonstrated a 55% prevalence of dysautonomia, predominantly orthostatic hypotension. 5 However, very few cases of tonic pupils have been reported in Sjö gren's syndrome. 4,[6][7][8][9][10][11][12] Our cases demonstrate bilateral tonic pupils as the initial manifestation of Sjö gren's-associated autonomic neuropathy/neuronopathy.…”

Peripheral nervous system in primary Sjögren’s syndrome

“…Większość badaczy zgadza się ze stwierdzeniem, że przeciwciała przeciw receptorowi muskarynowemu typu 3 dla acetylocholiny są odpowiedzialne za zaburzenia prawidłowego funkcjonowania autonomicznego układu nerwowego, a zwłaszcza części przywspółczulnej u chorych z PSS [20].…”

“…Ten ostatni objaw jest spowodowany zapaleniem neuronów zwoju rzęskowego [29]. Wykazano ponadto, że dysfunkcja układu autonomicznego [20]. Leczenie jest zwykle objawowe; jeśli przebiega z innymi typami neuropatii, stosuje się leczenie skojarzone, które jest wskazane w danym typie uszkodzenia obwodowego układu nerwowego.…”