Brainstem auditory evoked potentials in patients with primary Sjögren’s syndrome without central nervous system involvement

Waliszewska‐Prosół, Marta; Sebastian, Agata; Wiland, Piotr; Budrewićz, Sławomir; Dziadkowiak, Edyta; Ejma, Maria

doi:10.1007/s10067-020-05344-5

Cited by 4 publications

(2 citation statements)

References 27 publications

(31 reference statements)

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“…The evidence in the literature supports the fact that Sjögren’s syndrome can cause minor cognitive dysfunction associated with the duration and severity of the symptoms of the disease, without manifestations of mental disorders or even central nervous system involvement [ 31 ]. This could be attributed to bioelectrical dysfunction in an ongoing long-term autoimmune process that can affect other sensory systems, such as the auditory pathway [ 32 ]. Therefore, neuropathy should be considered as a possible factor compromising the smell function in patients diagnosed with SS.…”

Section: Discussionmentioning

confidence: 99%

Smell Dysfunction in Patients with Primary Sjögren’s Syndrome: Impact on Quality of Life

Al‐Ezzi

Khan

Tappuni

2023

JCM

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Objectives: Patients with primary Sjögren’s syndrome (pSS) often report smell and taste disturbances. However, the correlation between smell impairment and mucosal dryness is not well understood. The objectives of this study were to investigate the following: (1) the prevalence of smell hypofunction in patients with SS; (2) the impact of smell hypofunction on their quality of life (QoL); (3) whether the patients’ smell is correlated with xerostomia; and (4) whether the patients’ smell is affected by taste hypofunction, disease duration, age, smoking or self-reported neuropathy. Methodology: An ethically approved cross-sectional study was conducted on 65 female patients with SS and 62 sex-matched healthy controls. Their smell was assessed using the University of Pennsylvania Smell Identification Test. Their taste acuity was assessed using the Taste Strips Test. A visual analogue scale was used for the self-assessment of smell and taste functions. Xerostomia was assessed by the salivary flow rate, clinical oral dryness score and the Xerostomia Inventory. The patients’ QoL and mental health well-being were assessed using validated questionnaires. Results: In the SS group, the patients’ smell function was impaired in 27/65 patients compared with the controls (15/62, p < 0.05), and it did not correlate with the severity of xerostomia, taste acuity (r = 0.05, p = 0.6) or self-reported nasal dryness (r = −0.02, p = 0.7). In the patients’ group, smell hypofunction was not correlated with disease duration (β = 0.1, 95% CI = −0.07–0.1) or smoking (β = −0.02, 95% CI = −8–7). Age was not correlated with the smell function in the patients’ group (β = −0.1, p = 0.5) but was correlated significantly with smell in the healthy participants’ group (β = −0.3, p = 0.02). Neuropathy affected 81.2% of the patients’ group. Their QoL and mental health well-being were not affected by smell hypofunction. Conclusion: Smell hypofunction appears to be a clinical manifestation in patients with SS, but it does not seem to be associated with the severity of mucosal dryness or with taste disturbance.

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Section: Discussionmentioning

confidence: 99%

Smell Dysfunction in Patients with Primary Sjögren’s Syndrome: Impact on Quality of Life

Al‐Ezzi

Khan

Tappuni

2023

JCM

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“…On the other hand, central nervous system involvement is very rare in pSS and difficult to detect due to non-uniform definition and variable imaging features. In this setting, abnormalities of brainstem auditory evoked potentials, a test employed to detect central nervous system involvement, were recorded in about 16% of pSS patients (56) Prevalence of extra-glandular manifestations, ESSDAI scores and antibody status were similar between the two groups. Interestingly, radiographic sacroiliitis was reported in 65% of patients, among which 47% had bilateral involvement associated with anterior syndesmophytes (26%) and presence of HLA-B27 (13%).…”

Section: From Pathogenesis To Traditional and Novel Biomarkers In Pssmentioning

confidence: 90%

Sjögren's syndrome: one year in review 2022

Manfrè

Chatzis

Cafaro

et al. 2022

Clinical and Experimental Rheumatology

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Primary Sjögren's syndrome (pSS) is a complex disabling systemic autoimmune disorder. The hallmark of pSS is the T-cell-mediated hyperactivation of B-cells, evolving from asymptomatic conditions to systemic complications and lymphoma development. On tissue level, the typical feature is the lymphocytic infiltration of the salivary gland by B-, T-and antigen presenting cells, as mirrored by the diagnostic cornerstone role of minor salivary gland (MSG) biopsy. B-cells show multiple possible roles in disease pathogenesis, from autoantibody production, to antigen presentation, and cytokine production. B-cells hyperactivation is supported by genetic risk factors, T-cell dependent and independent mechanisms, and the presence of different pathogenic B-cell subsets must be reminded. Many aspects have been investigated in the last year regarding genetic and epigenetics, B-and T-cell role in pSS pathogenesis, their interaction with salivary gland epithelial cells (SGECs) and in their direct or indirect use as biomarkers and predictors of disease development, activity, and lymphomagenesis.In this review, following the others of this series, we will summarise the most recent literature on pSS pathogenesis and clinical features focusing in particular on new insights into pSS molecular stratification and therapeutic advances in the era of precision medicine.

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Neurologic involvement in seronegative primary Sjögren’s syndrome with positive minor salivary gland biopsy: a single-center experience

et al. 2023

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ObjectiveTo assess the demographics, neurologic manifestations, comorbidities, and treatment of patients with seronegative primary Sjögren’s syndrome (pSS).Patients and methodsWe conducted a retrospective chart review on patients with seronegative pSS evaluated by a neurologist at the University of Utah Health between January 2010 and October 2018. The diagnosis was based on characteristic symptoms, positive minor salivary gland biopsy according to the American-European Consensus Group 2002 criteria, and seronegative antibody status.ResultsOf 45 patients who met the study criteria, 42 (93.3%) were Caucasian, and 38 (84.4%) were female. The patients’ mean age at diagnosis was 47.8 ± 12.6 (range 13–71) years. Paresthesia, numbness and dizziness, and headache were noted in 40 (88.9%), 39 (86.7%), and 36 patients (80.0%), respectively. Thirty-four patients underwent brain magnetic resonance imaging. Of these, 18 (52.9%) showed scattered nonspecific periventricular and subcortical cerebral white matter T2/fluid-attenuated inversion recovery hyperintense foci. Twenty-nine patients (64.4%) presented to the neurology clinic prior to pSS diagnosis, and the median delay in diagnosis from the first neurology clinic visit was 5 (interquartile ranges 2.0–20.5) months. Migraine and depression were the most common comorbidities in 31 patients (68.9%). Thirty-six patients received at least one immunotherapy, and 39 were on at least one medication for neuropathic pain.ConclusionPatients often display various nonspecific neurological symptoms. Clinicians should express a high degree of skepticism regarding seronegative pSS and consider minor salivary gland biopsy to avoid delaying diagnosis, as undertreatment can affect patients’ quality of life.

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Brainstem auditory evoked potentials in patients with primary Sjögren’s syndrome without central nervous system involvement

Cited by 4 publications

References 27 publications

Smell Dysfunction in Patients with Primary Sjögren’s Syndrome: Impact on Quality of Life

Smell Dysfunction in Patients with Primary Sjögren’s Syndrome: Impact on Quality of Life

Sjögren's syndrome: one year in review 2022

Neurologic involvement in seronegative primary Sjögren’s syndrome with positive minor salivary gland biopsy: a single-center experience

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