Recommendations on neurologic, cognitive, and psychiatric manifestations in patients with Sjögren’s disease by the Brazilian Society of Rheumatology

Abstract Background Neurological and psychiatric manifestations occur in patients with primary Sjogren’s disease (SjD) with a wide-ranging clinical presentation, affecting quality of life, social participation, and prognosis. Despite this, neither central nor peripheral neurological symptoms are systematically evaluated in the context of autoimmunity or identified as manifestations of SjD. The EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI) covers only part of them in the neurological domain. Methods We performed a systematic review of the diagnosis and prevalence of central, peripheral, and autonomic nervous system manifestations in primary SjD, following the recommendations proposed by the Cochrane Collaboration Handbook. Observational studies were included when their main issue was the diagnosis and the prevalence of the manifestations individually. We employed a generalized linear mixed model (GLMM) method with a random-effects model, and the results were computed using logit transformation, implemented through the ‘meta’ and ‘metafor’ packages in the R software (version 3.6.1). To present these recommendations, agreement among experts was investigated using the Delphi method in in-person meetings. Results We propose ten recommendations regarding the investigation and management of neurological involvement in SjD that had 100% agreement among participants. Conclusion These recommendations add to the literature on the clinical care of patients with SjD.