REVIEW - Primary Sjögren's syndrome associated neuropathy

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Can J Neurol Sci. 2007 Aug;34(3):280-7.

Primary Sjögren's syndrome associated neuropathy.

Mellgren SI, Göransson LG, Omdal R.

Department of Neurology (SIM), Institute of Clinical Medicine,

University of Tromsø and University Hospital of North Norway, Tromsø,

Norway.

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.

PMID: 17803024

http://www.ncbi.nlm.nih.gov/pubmed/17803024

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