Peripheral Neuropathy In Primary Sjögren’s Syndrome (P4.123)

2014 
Objectives: To study the clinical features of peripheral neuropathy in PSS and to evaluate the mean interval between the appearance of peripheral neuropathy and the final diagnosis of PSS. Background: Primary Sjogren’s syndrome (PSS) is a chronic inflammatory autoimmune disease that mainly affects exocrine glands leading to sicca syndrome. The neurologic involvement seems to be the most significant extraglandular manifestation of PSS, and peripheral neuropathy may precede the sicca syndrome in many patients. Methods: Twenty five consecutive patients with peripheral neuropathy associated with PSS were retrospectively studied. All patients fulfilled the American-European Classification Criteria for Sjogren Syndrome revised in 2002. Results: All 25 patients were female with ages between 34 and 82 years (mean age of 52,48 ± 16,16 years). The mean disease duration interval ranged from 3 to 180 months (mean 52,80 ± 45,59 months). Twenty patients (80%) presented with small fiber polyneuropathy (SFP), three patients (12%) with polygangliononeuropathy (PGNP) and the last two patients (8%) with sensory-motor polyneuropathy (SMP). Antibodies to Ro/SSA and neuropathic pain (not simultaneously) were detected in 100% of patients with SMP and with PGNP and in 90% of patients with SFP. Sensory nerve conduction was abnormal in 50% of patients with SFP. We didn’t find any case of plexopathy. Sural nerve biopsy was performed in 23 patients and showed microvasculitis in 100% of patients with SMP, in 77,78% of patients with SFP and in 0% of cases of PGNP (p = 0,020).Conclusion: Peripheral neuropathy may precede sicca syndrome and be the first manifestation of PSS. The recognition of PSS in patients with peripheral neuropathy is important mainly in those with involvement of small fibers. A screening for primary Sjogren’s syndrome must be made systematically in patients that develop peripheral neuropathy with the objective of early diagnosis and treatment. Disclosure: Dr. Leijoto has nothing to disclose. Dr. Nascimento has nothing to disclose. Dr. Prado has nothing to disclose. Dr. Kosac has nothing to disclose. Dr. Bittar has nothing to disclose. Dr. Quintanilha has nothing to disclose. Dr. Nogueira has nothing to disclose. Dr. Vianna has nothing to disclose.
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