Objectives Sjögren's syndrome (SS) is a chronic inflammatory disease with an autoimmune background with possible complications from peripheral (PNS) and central nervous system (CNS). The aim of this study was to assess the prevalence and to describe the phenotype of peripheral neuropathies in patients with SS. Materials & Methods We studied fifty patients with primary Sjögren's syndrome for peripheral nervous system involvement. All patients underwent neurological and rheumatological examination followed by nerve conduction studies (NCS) of nine peripheral nerves. Results Thirty‐six patients (72%) fulfilled the criteria for the diagnosis of neuropathy. Carpal tunnel syndrome (54%) and axonal sensorimotor neuropathy (22%) were the most common. Neurological symptoms preceded the diagnosis of SS in eight patients. Conclusions Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible autoimmune causes of neuropathies because clinical manifestations of neuropathy may precede the development of other symptoms of the autoimmune disease.
Sjögren's syndrome (SS) is a chronic autoimmune disease with a wide spectrum of possible organ involvement. Peripheral (PNS) and central nervous system (CNS)-related symptoms may occur in the course of the disease. The aim of this study was to compare the health-related quality of life (HR-QOL) in SS patients with and without peripheral neuropathy. The study involved 50 patients with primary Sjögren's syndrome (pSS). All patients underwent neurological clinical examination followed by nerve conduction studies (NCS) and rheumatological examination. Thirty-six-item Short Form Health Survey (SF-36) was used for evaluating HR-QOL. To assess pSS activity, the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) were used. For the assessment of clinical disability due to peripheral neuropathy, the Overall Disability Sum Score scale (ODSS) was used. Additional evaluation of pain was performed with the use of the Visual Analogue Scale (VAS) and a semistructured interview. Twenty-three (46%) patients were diagnosed with peripheral neuropathy. The most common PNS manifestation was sensorimotor neuropathy (47%). Neurological symptoms preceded the diagnosis of pSS in eight patients. The following domains of the SF-36 form were significantly lower scored by patients with peripheral nervous system involvement: role-physical [0 (0-100) vs. 75 (0-100)], role-emotional [67 (0-100) vs. 100 (0-100)], vitality [40 (10-70) vs. 50 (20-75)], bodily pain [45 (10-75) vs. 55 (0-100)], and general health [20 (5-50) vs. 30 (0-50)] (p ≤ 0.05). Our study showed that peripheral neuropathy was a common organ-specific complication in SS patients. In pSS patients, coexisting neurological involvement with symptoms such as pain and physical disability may be responsible for diminished HR-QOL.
Background:Systemic manifestations are common in primary Sjögren’s syndrome (pSS) and play a major role in the prognosis. Neurological complications may affect both the peripheral (PNS) and central nervous system (CNS). The incidence varies from several to several dozen percent. The course of the disease and the severity of the symptoms may be mild and self-limiting or progressive, leading to permanent neurological deficits. It is worth remembering that the symptoms of nervous system involvement may precede the onset of symptoms of dryness, and diagnosis of pSS may be delayed after a certain duration of neurological symptoms.Objectives:The aim of the study was to determine the prevalence of PNS involvement among symptomatic and asymptomatic patients with the diagnosis of pSS in our University Clinical Centre.Methods:We studied a consecutive group of fifty unselected patients aged from 33 to 74 years (mean 55.8 years). All patients fulfilled the criteria for the diagnosis of pSS. Additional connective tissue diseases and diabetes were the exclusion criteria. Disease activity was evaluated according to the EULAR Sjögren’s syndrome disease activity index (ESSDAI). All patients underwent clinical neurological examination and nerve conduction studies (NCS) of nine peripheral nerves. For the classification of polyneuropathies the ESTEEM (European Standardized Telematic tool to Evaluate Electrodiagnostic Methods) guidelines were used. Clinical examination and nerve conduction study were performed and evaluated by one certified neurologist.Results:Of our 50 patients, 48 were female with a mean (±SD) age 53.6±10.5 years and mean disease duration 7.9±5.3 years. Two were male with mean age 43.7±25.8 years and mean disease duration 5.7±0.6 years. The mean age at diagnosis was 50.4±14 years. 23 (46%) patients fulfilled the criteria for the diagnosis of neuropathy. The most common PNS manifestation was sensorimotor neuropathy 11/23 (47%), mononeuropathy was present in 6/23 (26%) patients, pure axonal sensory neuropathy was present in 1/23 (4.3%) patient, axonal motor neuropathy in 1/23 (4.3%), SFN in 1/23 (4.3%) and cranial nerve involvement was present in 4/23 (17.4%) (one of the patients had both cranial and sensorimotor neuropathy). Neurological symptoms preceded the diagnosis of pSS in 8 (35%) of 23 PNS+ patients. The frequency of following symptoms and extraglandular manifestations was significantly higher in PNS+ compared to PNS- patients: salivary gland enlargement (74% vs 44% p<0,05), respiratory tract involvement (65% vs 37% p<0,05) and lymphadenopathy (61% vs 19% p<0,05). The mean ESSDAI in patients with and without neurological involvement was 7±6,82 and 4,7±4,58 respectively (p=0.245). In this subgroup the use of cyclophosphamide, due to extraglandular manifestations, was increased (p<0,05).Conclusion:We found that PNS involvement is a common extraglandular manifestation of pSS (46% in our group). Sensorimotor neuropathies were most frequent. Involvement of the PNS seems frequent but remains underestimated. NCS is a...
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