Elsevier

La Presse Médicale

Volume 41, Issue 9, Part 2, September 2012, Pages e485-e493
La Presse Médicale

Quarterly Medical Review
Central and peripheral neurological complications of primary Sjögren's syndrome

https://doi.org/10.1016/j.lpm.2012.06.002Get rights and content

Summary

Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. Clinically significant neurologic manifestations affect approximately 20% of patients and may be the first manifestation of the disease in at least 25% of the cases. The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability.

Section snippets

Epidemiology and subtypes

Primary SS is associated with several different types of neuropathies in approximately 2 to 20% of patients [7], [8], [10], [11], [12], [14], [15], [16].

Indeed, the clinical spectrum of peripheral neuropathies encountered in pSS is wide, and sensory neuropathies are the most commonly reported [7], [8], [10], [11], [12], [13], [14], [15], [16]. These pSS-related sensory neuropathies are represented by distal, length-dependent axonal sensory neuropathy and sensory ataxic neuronopathy involving

Small fiber neuropathy and primary Sjögren's syndrome

The prevalence of small fiber neuropathy in pSS is actually unknown; however, the systematic DN4 screening of neuropathic pain in pSS is positive at least in 10 to 15% of the cases [8], [40], with abnormal electromyographic studies in 45% of the cases only. Hence, a small fiber involvement could be suspected in 5 to 8% of pSS patients [8], [16], [41]. pSS patients with SFN exhibited chronic neuropathic pain (i.e. burning sensation, prickling, dysesthesia, allodynia). Neuropathic pain involved

Epidemiology and subtypes

CNS involvement in pSS was initially controversial and seems to occur in 1 to 60% of patients depending on heterogeneous inclusion criteria [1], [3], [4], [5], [6], [29]. However, more recent reports emphasize the involvement of the CNS, often mimicking the clinical symptoms of primary progressive or relapsing-remitting multiple sclerosis although the association of pSS and multiple sclerosis is possible. Hence, pSS appears to be a great imitator of multiple sclerosis, involving the CNS more

Conclusion

Neurological complications affect 20% of pSS patients and may be inaugural. Both peripheral and central involvement could be related to a vasculitis process. Hence, systematic research of complement activation and cryoglobulinemia must be systematic in pSS patients suspect from neurologic manifestations. However, no characteristic immunogical profile has been associated with pSS neurologic manifestations.

The DN4 questionnaire may improve early detection of pSS-related neuropathies. Axonal

Disclosure of interest

the authors declare that they have no conflicts of interest concerning this article.

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