Abstract
BackgroundTo investigated distinct manifestations of Sjögren’s syndrome (SS) patients with neurological complications and the potential risk factors associated with neurological complications in SS, and to produce a disease evaluation and neurological involvement prediction for SS.Methods566 patients who fulfilled the 2002 classification criteria for SS from the Rheumatology Department of the First Affiliated Hospital of Wenzhou Medical University were included in the cross-sectional study. Clinical, immunological and histological characteristics were surveyed, and potential risk factors for neurological complications were examined by multivariate analysis.ResultsAmong 566 SS patients, 184 (32.5%) patients had neurological involvement, with more than 10% got limbs pain, limbs numbness and cerebral infarction, respectively. Of these 184 SS patients with neurological complications, secondary SS (sSS) patients had a higher prevalence of peripheral nervous system (PNS) involvement than primary SS (pSS) patients (31.1 vs. 19%). And sSS patients showed higher total ESSPRI score and higher prevalence of xerostomia and low C3, C4 levels with more liver, articular involvement and saliva gland atrophy, and more severe lymphocyte infiltration in salivary glands than pSS patients. As for the specific factors associated with neurological involvement, low C3 level were found to be significant in pSS or sSS patients who were younger 50 year old, and ANA positivity, cardiac involvement, saliva gland atrophy were demonstrated to be associated in elder pSS patients. And xerophthalmia was found to be associated in sSS patients.ConclusionLow complement (C3) levels, xerophthalmia, ANA positive, cardiac involvement and labial salivary gland histological result were good ways to predict neurological complications in different subgroups of SS, which might provide insight into better clinical decision-making, especially at early stages of the disease.
Highlights
To investigated distinct manifestations of Sjögren’s syndrome (SS) patients with neurological complications and the potential risk factors associated with neurological complications in SS, and to produce a disease evaluation and neurological involvement prediction for SS
The study design conformed to current National Health and Family Planning Commission of China ethical standards, with written informed consent provided by all patients. secondary SS (sSS) patients were diagnosed with systemic lupus erythematosus [11], systemic sclerosis [12], rheumatoid arthritis [13], inflammatory myopathies [14], mixed connective tissue disease [15], or primary biliary cirrhosis [16] according to existing classification criteria used in clinical practice
No significant difference in total ESSPRI scores was found between the two groups. primary SS (pSS) patients with neurological involvement showed a higher prevalence of antinuclear antibodies (ANA) antibody and hypergammaglobulinemia, but reduced level of C3 (P < 0.05)
Summary
To investigated distinct manifestations of Sjögren’s syndrome (SS) patients with neurological complications and the potential risk factors associated with neurological complications in SS, and to produce a disease evaluation and neurological involvement prediction for SS. It’s documented that 10–20% of SS patients had peripheral nervous system (PNS) involvement [7]. The prevalence of central nervous system (CNS) manifestations of SS ranged from 2.5 to 60% [8], which may due to the lack of unified definition of CNS involvement in SS [9, 10]. The aim of this study was to evaluate the prevalence and symptoms of neurological complications in a population of patients with SS and to investigate the potential risk factors for neurological involvement to facilitate a comprehensive disease evaluation and prognosis prediction for Chinese SS patients
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