Background/Purpose:

The course of primary Sjögren’s syndrome (pSS) may be characterized by extra-glandular and potentially life-threatening manifestations. Among these, neurological involvement represents a clinical challenge due to its heterogeneous clinical presentation and diagnostic complexity. Indeed, reported prevalence of central (C) and peripheral (P) nervous system (NS) involvement is high variable due to lack of unified definition and diagnostic tools. Moreover, the association between disease clinical and serological manifestations and SN involvement remains unclear. Aim of this study was to evaluate the prevalence of neurological involvement in a pSS cohort and to investigate factors associated with this risk.

Methods: Data of a cohort of pSS patients fulfilling the 2002 AEGC criteria and enrolled in an Italian multicenter database (the Italian Group of SS Study-GRISS) were retrospectively analyzed. Disease clinical and serologic data were sistematically collected with specific analysis of CNS and PNS involvement. Clinical and serologic features associated with SN involvement were investigated. The Shapiro–Wilk test was used to assess the normal distribution of variables. The chi‐squared test and the Mann–Whitney U‐test were used for comparisons of categorical variables and non‐normally distributed continuous variables, respectively. Separate multivariate logistic regression analysis was performed.

Results:

The cohort comprised 1.707 patients (1.629 F, 78 M). Mean age at diagnosis was 52±14 and mean disease duration 6±7 years. Prevalence of NS involvement was 4.5% (78/1.707). PNS manifestations were recorded in 61 (3,6%), including 29 sensorimotor neuropathy, 17 pure sensory neuropathy, 8 sensory or sensorimotor polyneuropathy, 6 small-fiber neuropathy and 1 mononeuritis multiplex. CNS manifestations were recorded in 13 (0,8%), including 4 cerebral vasculitis, 3 trasverse myelitis, 2 otpic neuritis, 2 cranial nerve involvement and 1 multiple sclerosis-like syndrome. No diagnosis was available in 4 patients. Male sex was associated with higher risk of NS involvement (10% M vs 4% F, p= 0.025). Patients with NS involvement showed significant higher frequency of articular involvement (76% vs 62%), purpura (24% vs 7%), other organ involvement (96% vs 38%) lymphoma (9% vs 4%), low complement (42% vs 19%), leukopenia (39% vs 24%) and cryoglobulins (20% vs 4%). Median salivary gland focus score was higher in patients without NS involvement (p=0.001). At multivariate analysis, male sex (OR=2,7; 95% CI 1,1, 6), other organ involvement (OR=15; 4,5, 50,6), low complement (OR=2; 1,2, 3,5) and cryoglobulins (OR=3; 1,4, 6,3) were independently associated with NS involvement risk. Higher focus score was associated with lower risk (OR=0.7; 0,5, 0,9).

Conclusion:

Results of this multicenter study highlight that, although not frequent, NS involvement in pSS is protean and mainly characterizing male sex. The association with specific clinical and serologic disease features suggests that vasculitis may exert a prominent role in its pathogenesis. Awareness of these factors may help in predict neurological complications in different subgroups of patients and provide insight into better therapeutic approach.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/neurological-involvement-in-a-cohort-of-primary-sjogrens-syndrome-patients-results-of-a-multicenter-italian-study/