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  • Updated 04.01.2021
  • Released 06.28.2006
  • Expires For CME 04.01.2024

Sjogren syndrome: neurologic complications

Introduction

Overview

Neurologic involvement is a systemic manifestation of primary Sjögren syndrome that may involve the central, peripheral, and autonomous nervous systems. The frequency of neurologic involvement is relatively unknown, although data from the Big Data Sjögren Syndrome Cohort reported a frequency of 6.3% for peripheral neuropathies and 1.9% for central nervous system, according to The European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) definitions (97). Neurologic involvement often precedes the typical glandular manifestations of Sjögren syndrome and has a wide spectrum of manifestations and underlying neuropathologic mechanisms, making the diagnosis and approach to treatment a difficult challenge that requires a multidisciplinary approach. The diagnosis and treatment of these manifestations always must be coordinated by neurologists. The level of evidence for treatment efficacy of the main drugs used for managing neuroSjogren is limited.

Key points

• Sjögren syndrome is a common systemic autoimmune disease, mainly diagnosed in women aged 30 to 50 years old, that is manifested by sicca symptoms and organ-specific systemic involvement.

• Neurologic symptoms occur in 18% to 45% of patients with Sjögren syndrome due to involvement of cranial nerves (Bell palsy, trigeminal neuralgia, diplopia), peripheral nerves (sensorimotor neuropathies), and the central nervous system.

• Sjögren syndrome can mimic the symptoms and imaging features of multiple sclerosis.

• Sjögren syndrome is pathogenically closely associated with neuromyelitis optica, a demyelinating disease of the central nervous system caused by antibodies to aquaporin-4 (NMO IgG).

• A high index of suspicion is required given the pleomorphic manifestations and the fact that neurologic symptoms often precede the clinical diagnosis of Sjögren syndrome.

Historical note and terminology

In 1933, Henrik Sjögren described the association of keratoconjunctivitis sicca (filamentary keratitis) with arthritis (113). Morgan and Castleman noted the histopathological commonality between the keratitis described by Sjögren and the glandular enlargement described by Mikulicz (79; 76). By 1973, the term “Sjögren syndrome” became widely accepted as these disorders were considered variants of the same process (71). The name Gougerot-Sjögren syndrome is commonly used in the French literature (25) given that Henry Gougerot first reported, in Paris, the typical symptoms of xerostomia and xerophthalmia due to atrophy of salivary and lachrymal glands (39).

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