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Review
. 2023 Dec;81(12):1077-1083.
doi: 10.1055/s-0043-1777105. Epub 2023 Dec 29.

Sjogren's syndrome: a neurological perspective

Orlando Grazianni Povoas Barsottini  1 , Marianna Pinheiro Moraes de Moraes  1 , Pedro Henrique Almeida Fraiman  1 , Victor Hugo Rocha Marussi  2 , Alexandre Wagner Silva de Souza  3   4 , Pedro Braga Neto  5   6 , Mariana Spitz  7
Affiliations
Review

Sjogren's syndrome: a neurological perspective

Orlando Grazianni Povoas Barsottini et al. Arq Neuropsiquiatr. 2023 Dec.

Abstract
in English, Portuguese

Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.

A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.

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Conflict of interest statement

There is no conflict of interest to declare.

Figures

Figure 1
Figure 1
Step-by-Step Diagnosis of Sjogren's syndrome. Adapted from Shiboski et al. Abbreviations: CNS, central nervous system; ESSDAI, EULAR Sjogren's syndrome disease activity index; PNS, peripheral nervous system.
Figure 2
Figure 2
Neuroimaging findings of SS. (A, B) Axial and Sagittal T2WI showing extensive central spinal cord lesion (arrowheads) in a patient with NMOSD secondary to SS. (C), Axial T2WI revealing focal posterior lesion (thick arrow) in cervical spinal cord. (D) Coronal 3D STIR SPACE MIP (maximum intensity projection) displaying diffuse thickening of brachial plexus (hollow arrows) as well as diffuse enlargement within microcysts in parotid glands, a finding typical of SSj (hollow arrowheads). (E, F) showing brainstem and left optic radiation (thin arrows) enhancing lesions in two different patients.
Figure 3
Figure 3
SS treatment. (a). Oral hygiene, knowledge about SjS and what symptoms require medical attention; (b). Appropriate immunization prior to immunosuppression; (c). Hormonal contraception is contraindicated in the case of antiphospholipid antibodies or a history of thrombosis. Abbreviations: AQP4 Ab, anti-aquaporin 4 antibody; CyA, ciclosporin A; CyC, cyclophosphamide [pulses 0.5 g/15 day (maximum six pulses)]; Ecu, eculizumab (doses 1–4: 900mg IV qWeek for first 4 weeks, followed by dose 5, 1200mg IV 1 week later, then 1200 mg IV q2Weeks); GC, glucocorticoids; HCQ, hydroxychloroquine (200mg/day); IVIg, intravenous immunoglobulins (0.4–2 g/kg 5 days); MOG, myelin oligodendrocyte glycoprotein; NMOSD, neuromyelitis optica spectrum disorder; NSAIDs, non-steroidal anti-inflammatory drugs (no longer than 7–10 days); PEX, plasma exchanges; RA, rheumatoid arthritis; RTX, rituximab [1 g/15 days (x2)]. Adapted from Ramos-Casals M, et al.
See this image and copyright information in PMC

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