+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Primary Sjögren's syndrome associated neuropathy



Primary Sjögren's syndrome associated neuropathy



Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques 34(3): 280-287



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.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 055173394

Download citation: RISBibTeXText

PMID: 17803024

DOI: 10.1017/s0317167100006697


Related references

Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine 90(2): 133-138, 2011

Cytokine concentrations in stimulated whole saliva among patients with primary Sjögren's syndrome, secondary Sjögren's syndrome, and patients with primary Sjögren's syndrome receiving varying doses of interferon for symptomatic treatment of the condition: a preliminary study. Clinical Oral Investigations 5(2): 133-135, 2001

Peripheral neuropathy associated with primary Sjogren's syndrome. Journal of Neurology Neurosurgery and Psychiatry 57(8): 983-986, 1994

Sensory neuropathy and primary Sjögren syndrome. Medicina Clinica 90(6): 266-267, 1988

Peripheral neuropathy in primary sjogren's syndrome. Neurology 39(3): 390-394, 1989

Chemokine saliva levels in patients with primary Sjögren's syndrome, associated Sjögren's syndrome, pre-clinical Sjögren's syndrome and systemic autoimmune diseases. Rheumatology 50(7): 1288-1292, 2011

Unilateral sensory neuropathy of the trigeminal nerve as the leading symptom of primary Sjögren syndrome ("sicca syndrome"). Der Nervenarzt 58(5): 322-325, 1987

Autonomic cardiovascular neuropathy in primary Sjogren's syndrome. Rheumatology International 15(3): 127-129, 1995

Small fibre neuropathy in primary Sjögren syndrome. La Revue de Medecine Interne 32(3): 142-148, 2011

Optic neuropathy in a child with primary Sjögren's syndrome. Revue du Rhumatisme 65(5): 355-357, 1998