BACKGROUND: The prevalence of peripheral neuropathy in patients with Sjögren 
syndrome remains unclear owing to conflicting results in the published series, 
with numbers ranging from 2% to over 60% of Sjögren syndrome patients. Whether 
peripheral neuropathy is a feature of the systemic or glandular disease or 
whether it is related to a circulating antineuronal antibody remains also 
uncertain.
METHODS: The authors reviewed the records of patients with primary Sjögren 
syndrome (pSS), fulfilling the Revised European-American Classification 
Criteria, seen in their department from 1992 to 2009. The patients with 
previously recorded neuropathic features were re-examined clinically and 
electrophysiologically. Other causes of polyneuropathy were excluded. The 
authors also searched for circulating antineural antibodies using 
immunofluorescence and western blot and for antibodies against muscarinic and 
nicotinic acetylcholine receptors as potential biomarkers.
RESULTS: 509 cases met the diagnostic criteria for pSS. Among these, 44 patients 
were recorded as having neuropathic symptoms. After completing the evaluation, 
however, only nine (1.8%) had polyneuropathy with objective clinical signs and 
abnormal electrophysiological findings. The neuropathy was axonal in all, in 
five pure sensory and in four sensorimotor. The patients with peripheral 
neuropathy had extraglandular manifestations such as palpable purpura and 
vasculitis. No evidence of antineural autoimmunity was found, and no candidate 
biomarkers were identified.
CONCLUSION: Polyneuropathy is a rare manifestation of pSS occurring in 1.8% of 
patients. In the majority of patients, it is a late event and frequently 
associated with systemic disease or risk factors for lymphoma development.