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Primary Sjögren's Syndrome Associated Neuropathy

Published online by Cambridge University Press:  02 December 2014

Svein Ivar Mellgren ,
Lasse G Göransson  and
Roald Omdal
Svein Ivar Mellgren
Affiliation:
The Department of Neurology, Institute of Clinical Medicine, University of Tromsø and University Hospital of North Norway, Tromsø
Lasse G Göransson
Affiliation:
Department of Internal Medicine, Stavanger University Hospital, Stavanger and Institute of Internal Medicine, University of Bergen, Bergen, Norway
Roald Omdal
Affiliation:
Department of Internal Medicine, Stavanger University Hospital, Stavanger and Institute of Internal Medicine, University of Bergen, Bergen, Norway
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Abstract

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Primary Sjögren's syndrome (PSS) mainly affects exocrine glands and is clinically characterized by keratoconjunctivitis sicca and xerostomia. Among several possible extraglandular manifestations, involvement of the peripheral nervous system may occur with reported frequencies from 10% to 60%. Peripheral nerve manifestations constitute sensory neuropathy, including sensory ganglioneuronopathy, sensorimotor, including polyradiculoneuropathy and demyelinating neuropathy, motor neuropathy, multiple mononeuropathy, trigeminal and other cranial neuropathies, autonomic neuropathy, and mixed patterns of neuropathy. Knowledge of the neurological manifestations of PSS is hampered by evolving classification criteria of PSS over the years, and by use of highly selected patient populations on the basis of a primary neurological diagnosis. Sural nerve biopsy may show vascular or perivascular inflammation of small epineurial vessels (both arterioles and venules) and in some cases necrotizing vasculitis. Loss of myelinated nerve fibers is common and loss of small diameter nerve fibers occurs. Pathology in cases of sensory ganglioneuronopathy consists of loss of neuronal cell bodies and infiltration of T cells. Peripheral neuropathy in PSS often is refractory to treatment although newer biological agents may provide more effective treatment options. Current treatment strategies used in autoimmune neuropathies may be tried depending upon characteristics of the neuropathy and results obtained by a thorough clinical and laboratory investigation.

Résumé:

RÉSUMÉ:

Neuropathie associée à un syndrome de Sjön primaire. Le syndrome de Sjön primaire (SSP) atteint principalement les glandes exocrines. Il se caractérise au point de vue clinique par une kératoconjonctivite sèche et une xérostomie. Une atteinte du système nerveux périphérique est l'une des manifestations extraglandulaires possibles dont la fréquence rapportée varie de 10% à 60%. Les manifestations nerveuses périphériques comprennent une neuropathie sensitive, dont une ganglioneuronopathie sensitive, une neuropathie sensitivomotrice, dont une polyradiculoneuropathie et une neuropathie démyélinisante, une neuropathie motrice, de multiples mononeuropathies, des neuropathies du trijumeau et d'autres nerfs crâniens, des neuropathies autonomes et des tableaux mixtes de neuropathies. La connaissance des manifestations neurologiques est entravée par des critères de classification du SSP qui ont évolué au cours des ans et par l'observation de populations de patients choisis à cause d'un diagnostic neurologique primaire. La biopsie du nerf sural peut mettre en évidence une inflammation vasculaire ou périvasculaire des petits vaisseaux de l'épinèvre (tant les artérioles que les veinules) et dans certains cas, une vasculite nécrosante. On observe fréquemment une perte des fibres nerveuses myélinisées et parfois également une perte des fibres nerveuses de petit calibre. L'anatomopathologie chez les cas de ganglioneuronopathie révèle une perte des corps cellulaires neuronaux et une infiltration par des cellules T. La neuropathie périphérique du SSP est souvent réfractaire au traitement. Cependant, de nouveaux agents biologiques pourraient représenter des options de traitement plus efficaces. Les stratégies de traitement actuelles, utilisées pour traiter les neuropathies autoimmunes, peuvent être essayées, selon les caractéristiques de la neuropathie et les résultats d'une évaluation clinique et paraclinique méticuleuse.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 34 , Issue 3 , August 2007 , pp. 280 - 287
Copyright
Copyright © The Canadian Journal of Neurological 2007

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