Peripheral neuropathies in Sjögren syndrome: a new reappraisal
- Pantelis P Pavlakis1,
- Haralampos Alexopoulos1,
- Michael L Kosmidis1,
- Eleftherios Stamboulis2,
- John G Routsias1,
- Socrates J Tzartos3,
- Athanasios G Tzioufas1,
- Haralampos M Moutsopoulos1,
- Marinos C Dalakas1,4
- 1Department of Pathophysiology, Medical School, University of Athens, Athens, Greece
- 2Second Department of Neurology, Attikon Hospital, Medical School, University of Athens, Athens, Greece
- 3Department of Biochemistry, Hellenic Pasteur Institute, Athens, Greece
- 4Clinical Neurosciences, Neuromuscular Diseases, Imperial College London, London, UK
- Correspondence to Professor Marinos C Dalakas, Department of Pathophysiology, Medical School, Room 39, Building 16, University of Athens, 75 Mikras Asias Street, 11527 Athens, Greece;
- Received 24 June 2010
- Accepted 7 October 2010
- Published Online First 16 December 2010
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.
Competing interests None.
Ethics approval Ethics approval was provided by the University of Athens Medical School Ethics Committee.
Provenance and peer review Not commissioned; externally peer reviewed.