Peripheral neuropathies in Sjögren's syndrome: a new reappraisal.

Pantelis P Pavlakis (1) , Haralampos Alexopoulos (1) , Michael L Kosmidis (1) , Eleftherios Stamboulis (2) , John G Routsias (1) , Socrates J Tzartos (3) , Athanasios G Tzioufas (1) , Haralampos M Moutsopoulos (1) , Marinos C Dalakas (4)

1 Department of Pathophysiology, Medical School, University of Athens
2 2nd Department of Neurology, Medical School, University of Athens
3 Institut Pasteur Hellénique
4 Imperial College London

Background The prevalence of peripheral neuropathy in patients with Sjögren's syndrome remains unclear due to conflicting results in the published series, with numbers ranging from 2 to over 60% of Sjögren's syndrome patients. Whether peripheral neuropathy is a feature of the systemic or glandular disease or if it is related to a circulating anti-neuronal antibody, remains also uncertain. Methods We reviewed the records of patients with primary Sjögren's syndrome (pSS), fulfilling the Revised European - American Classification Criteria, seen in our department from 1992 to 2009. The patients with previously recorded neuropathic features were re-examined by us clinically and electrophysiologically. Other causes of polyneuropathy were excluded. We also searched for circulating anti-neural 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 them, 44 patients were recorded as having neuropathic symptoms. After completing our evaluation however, only 9 (1,8%). of them had polyneuropathy with objective clinical signs and abnormal electrophysiological findings. The neuropathy was axonal in all; in 5 was pure sensory and in 4 sensorimotor. The patients with peripheral neuropathy had extraglandular manifestations such as palpable purpura and vasculitis. No evidence of anti-neural 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.

Mots clés: NEUROPATHY; RHEUMATOLOGY

Pantelis P Pavlakis, Haralampos Alexopoulos, Michael L Kosmidis, Eleftherios Stamboulis, John G Routsias, et al.. Peripheral neuropathies in Sjögren's syndrome: a new reappraisal.. Journal of Neurology, Neurosurgery and Psychiatry, 2010, 82 (7), pp.798. ⟨10.1136/jnnp.2010.222109⟩. ⟨hal-00600751⟩