Introduction: Neurologic manifestations in primary Sjogren's Syndrome (pSS) are characterized by a heterogeneity of clinical manifestations. In clinical practice, physicians are challenged with the absence of diagnostic criteria and the lack of clinical trials to support treatment. In this article, we will review the epidemiology, clinical and immunological characterization, diagnosis, and treatment of neurologic events in pSS.
Areas covered: This narrative review provides an overview of the neurologic manifestations described in PSS, as well as complementary investigations and treatments reported. Articles were selected from PubMed searches conducted between December 2021 and February 2022.
Expert opinion: Epidemiology and clinical features of neurologic manifestations are derived from different cohort studies. Our understanding of pathophysiology of neurologic manifestations in pSS has significantly increased in the past few years, especially regarding PNS. However, there are still many knowledge gaps on therapeutics. The few available data on therapy rely upon small case series, from experiences with other autoimmune diseases, such as systemic lupus erythematosus or expert opinion. There is an urgent need for well-designed clinical trials.
Keywords: Primary Sjogren's Syndrome; cognitive impairment; magnetic resonance imaging; neuropsychiatric manifestations; peripheral nervous system.