Primary Sjögren’s syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. In the course of pSS the internal organs may be involved and the symptoms may concern any system. Neurological disorders are one of the most common extraglandular manifestations of pSS. Available literature data estimate the prevalence of neurological symptoms as about 8.5–70% of patients diagnosed with pSS. The most common neurological complication of pSS is peripheral neuropathy, and in particular sensory polyneuropathy. Central nervous system involvement is much less common. There are also reports of various symptoms connected with damage to cranial nerves and the autonomic nervous system. A careful neurological evaluation, combined with neurophysiological tests, is recommended in patients with pSS. This review summarizes the neurological manifestations of pSS, their possible pathogenic mechanisms, diagnostic evaluation and potential treatment.
Objective The aim of this study was to evaluate: the prevalence, type of neuropathy and the relationship between the presence of autoantibodies and neuropathy development in patients with primary Sjögren syndrome (pSS). Methods 61 pSS patients underwent a complete neurological and electrophysiological examination as well as immunological tests including rheumatoid factor (RF) and autoantibodies as antinuclear antibodies, (ANA), anti-Ro/SSa, anti-La/SSB antibodies. Results The axonal loss or demyelination were found in 39 patients (63.9%). 29 (47.5%) subjects fulfilled both clinical and electrophysiological criteria of peripheral neuropathy of predominantly axonal type. Seropositivity to both anti-Ro and anti-La antibodies was more frequently found in patients with normal nerve conduction study. Seropositivity to anti-Ro alone was present in the majority of patients with axonal neuropathy (p<0.05). The presence of RF was associated with several electrodiagnostic signs of demyelination (p<0.01). The ANA titer showed no independent association with neuropathy. Conclusion Peripheral neuropathy is a frequent complication in patients with primary Sjögren syndrome. Seropositivity limited to anti-Ro is associated with increased risk of axonal neuropathy in comparison to seropositivity to both anti-Ro and anti-La antibodies. Seropositivity to RF may contribute to demyelination.
klinika i Poliklinika neuroortopedii i neurologii narodowego instytutu Geriatrii, reumatologii i rehabilitacji w Warszawie A-przygotowanie projektu badania, B-zbieranie danych, C-analiza statystyczna, D-interpretacja danych, E-przygotowanie maszynopisu, F-opracowanie piśmiennictwa, G-pozyskanie funduszy
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