Abstract

Background  Neurological and psychiatric manifestations occur in patients with primary Sjögren’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 Sjögren’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.

Keywords
Neurological manifestations; Psychiatric disease; Cognitive impairments; Peripheral nervous system; Central nervous system; Sjögren’s syndrome; Sjögren’s disease