Background Clinical evidence of Primary Sjögren’s Syndrome (pSS) includes sicca symptoms, autoantibodies, physical exam evidence of keratoconjunctivitis sicca, and reduced salivary output. Patients with pSS may also present with peripheral neuropathy (PN), and the contribution of pSS to the underlying pathology may be under-recognized. There are limited data on the diagnosis and treatment of patients with pSS complicated by peripheral neuropathy.
Objectives To evaluate the demographic, clinical, laboratory and serological data of patients with a (+) MSGB with pSS with and without associated peripheral neuropathy (PN)
Methods We reviewed 214 records of patients who had a MSGB for a clinical suspicion of SS, in the Division of Rheumatology at the University of Michigan, between 1993 and 2011. Eighteen patients with other autoimmune/neoplastic processes were excluded. Data were collected including demographics, sicca symptoms, extra-glandular manifestations, histopathology, serology and clinical laboratory values. Peripheral neuropathies were documented and classified based on patterns of electrodiagnostic studies (EMG/NC). The protocol was approved by the University of Michigan Institutional Review Board. Statistical analysis included the descriptive frequencies, and correlations parametric and nonparametric. A two-tailed value of p<0.05 was taken to indicate statistical significance.
Results Of the 196 patients evaluated for SS, forty three met the American-European Consensus Group (AECG) criteria for pSS. Thirteen had peripheral neuropathy. Of them, seven with (+) and one with (-) MSGB met the AECG-criteria for pSS 8/43 (18.6%), and with a mean age (SD) of 49 (±8.9) years. Five were women and all were Caucasian. Elapsed time between neuropathic symptoms and confirmatory diagnosis of PN with EM/NC or nerve biopsy was 24.6 (±26) months. Time from presentation of peripheral neuropathy symptoms to pSS diagnosis was 15 (±6.8) months. Time from symptoms to prednisone treatment was 9.9 (±10.5) months and immunosuppressive therapy onset was 17.8 (±14.1) months. Diagnosis of pSS antedated only two cases. Of all 43 patients with pSS, xerophthalmia correlated inversely with neuropathies r= -0.466 (p=0.001). In patients with neuropathies, the frequency of clinical features were xerophthalmia 5 patients, xerostomia 7, fatigue 8, Raynaud’s phenomenon (RP) 3, other CNS findings 2, vasculitis 2 and fibromyalgia 3. Two cases with (+) MSGB, one with and the other without diagnosis of pSS, had negative tests for autoantibodies. The patient with confirmed pSS received chemotherapy, but the other patient did not.
Conclusions Minor salivary gland biopsy may be helpful in assessment of patients with peripheral neuropathy and pSS. Concomitant symptoms of fatigue, Raynaud’s phenomenon, and a positive ANA may be helpful in suggesting an underlying contribution from pSS in patients with peripheral neuropathy. Patients with pSS may have limited sicca symptoms at the time of presentation with neuropathy symptoms, and this may contribute to a delay in diagnosis and treatment. In patients with negative serology but positive MSGB decision management should be taken according to the clinical judgment.
Disclosure of Interest R. Peredo Employee of: University of Michigan. Nothing to Declare., A. Impens Employee of: Midwestern University. Nothing to declare, K. Phillips Employee of: University of Michigan. Nothing to Declare.
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