Ab0638 neurological involvement in patients with primary sjögren’s syndrome: a retrospective cross-sectional study

Annals of the Rheumatic Diseases(2023)

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Background Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease that primarily affects the exocrine glands and eventually leads to xerophthalmia and xerostomia [1] . Up to 75% of pSS patients may experience extraglandular involvement ranging from mild arthralgia to life-threatening vasculitis [2,3] . Neurological involvement, one of the most common extraglandular manifestations associated with pSS, is classified into two subgroups: peripheral nervous system (PNS) involvement and central nervous system (CNS) involvement. Objectives The aim of this study is to identify the types of PNS and CNS involvement in pSS patients, as well as to investigate any relationships between clinical and immunological features and neurological involvement. Methods We retrospectively assessed 2127 patients with an ICD-10 code for Sjögren recorded in hospital database.Among these patients, those meeting the pSS classification criteria and having neurological symptoms and an objective evaluation accordingly were enrolled.After comparing the patients with and without neurological involvement, peripheral and central involvement subtypes were also compared within themselves. Results A total of 199 pSS patients were enrolled and neurological involvement was found in 31.6%. Patients with neurological involvement had a higher frequency of Schirmer’s test, anti-Ro/SS-A and anti-La/SS-B positivity and presence of interstitial lung disease, articular involvement, lymphadenopathy, anemia and hypocomplementemia than patients without those. In multivariate regression analysis, only articular involvement had a higher risk for the development of neurologic involvement [OR 10.01 (4.18–23.97), p0.0001]. Among the patients with PNS, the frequency of anti-Ro/SS-A positivity, low C3 and Schirmer’s test positivity were statistically increased compared to those who were not in PNS (p=0.032, p=0.044, and p=0.029, respectively). In comparison to CNS involvement, patients with CNS involvement were younger, had a shorter disease duration, and had a higher frequency of anti-Ro/SS-A positivity than patients without those (p=0.041, p=0.027, and p=0.046, respectively). Conclusion In our study, it was shown that one third of the symptomatic pSS patients had objective neurological involvement. The presence of neurological symptoms should be considered, especially in patients with articular involvement in pSS. References [1]Moreira I, Teixeira F, Martins Silva A, Vasconcelos C, Farinha F, Santos E. Frequent involvement of central nervous system in primary Sjögren syndrome. Rheumatol Int. 2015;35(2):289-94. [2]Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A, et al. Primary Sjogren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford, England). 2014;53(5):839-44. [3]Fauchais AL, Magy L, Vidal E. Central and peripheral neurological complications of primary Sjögren’s syndrome. Presse medicale (Paris, France: 1983). 2012;41(9 Pt 2):e485-93. Table 1. Comparison of clinical and immunological features of pSS patients with and without neurologic involvement Without neurologic involvement, n=136 With neurologic involvement, n=63 p Female, n (% ) 131 (96.3) 58 (92.1) 0.201 Age, years, mean (SD ) 53.8 (10.8) 54.4 (13.1) 0.755 Age of diagnosis, years, mean (SD ) 48.6 (10.6) 48.8 (13.2) 0.911 Positive Schirmer’stest, n (% ) 107 (78.7) 58 (92.1) 0.020 Interstitial lung disease, n (% ) 5 (3.7) 8 (12.7) 0.017 Articular involvement, n (% ) 57 (41.9) 55 (87.3) <0.001 Positive anti-Ro/SS-A, n (% ) 77 (56.6) 46 (73.0) 0.021 Positive anti-La/SS-B, n (% ) 40 (29.4) 25 (39.7) 0.151 Low C3, n (% ) 11(8.1) 10 (15.9) 0.096 ANA: Antinuclear antibodies, Anti-SSA:Anti-Sjögren’s syndrome type A antibodies, AntiSSB: Anti-Sjögren’s syndrome type B antibodies Acknowledgements: NIL. Disclosure of Interests None Declared.
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sjögrens,neurological involvement,syndrome,ab0638,cross-sectional
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