Clinical analysis of the neurological involvement in primary Sjögren′s syndrome

Abstract

Objective To analyze the clinical and laboratory manifestations of primary Sjogren′s syndrom (pSS) with neurological involvement. Methods One hundred and forty eight patients fulfilling the 2002 American-European pSS classification criteria were retrospectively analyzed. Neurological manifestations were diagnosed based on the clinical, biological, electrophysiological, and imaging findings. Biographical, clinical, and laboratory data were compared between patients with and without neurological manifestations. Statistical methods used were Mann-Whitney U test, Chi-square test and Fisher exact probability. Results The prevalence of neurological involvement in pSS was 20.3% (30/148), and the incidence of peripheral neur-opathy, the central neuropathy and combination of the central neuropathy with peripheral neuropathy were 10.1%(15/148), 9.5%(14/148) and 0.7%(1/148), respectively. The clinical spectrum of peripheral neuropathies encountered in Sjogren′s syndrome (SS) patients varied, with the pure sensory neuropathies being the most common, followed by sensorimotor neurophathies. Motor neuron disease was the most common type of central neurophathies. Compared with those without neurological manifestations, the duration of peripheral nerve system/central nerve system (PNS/CNS)-pSS patients was relatively short [(55±76) months vs (100±108) months, Z=-2.682, P<0.05], and the antinuclear antibody (ANA) titer and RF titer were lower [(234±248) vs (377±339), Z=-2.008, P<0.05; (126±279) U/ml vs (359±1 445) U/ml, Z=-2.243, P<0.05]. In PNS/CNS-pSS patients, the most common clinical manifestations included numbness(50%), pain (23%), and muscle weakness (63%). Conclusion The prevalence of neurological involvement in pSS is high. The duration is relatively short and the disease activity is high, but the disease features are atypical and may be neglected by rheumatologists. Key words: Sjogren′s syndrome; Neurologic manifestations; Peripheral nerve system; Central nerve system; Clinical features