IDIOPATHIC AXONAL NEUROPATHY AND PRIMARY SJöGREN'S SYNDROME

Abstract

Objective: To assess the diagnostic outcome of patients with an idiopathic neuropathy that were referred to a specialist Sjogren's clinic., Background: In about 15% of patients with a chronic axonal polyneuropathy a cause cannot be found following standard investigations. Primary Sjogren's Syndrome (PSS) has been associated with a variety of peripheral neuropathies, particularly dorsal root ganglioneuronopathy and small fibre neuropathy., Method: We retrospectively reviewed the clinicopathological records of thirty consecutive patients with undiagnosed neuropathies referred from a specialist peripheral nerve clinic to a regional Sjogren's clinic for further investigation., Results: Ten (33%) of the thirty patients were confirmed on lip biopsy to have PSS. 9/10 patients had a sensory/sensorimotor axonal neuropathy and 1/10 had a dorsal root ganglioneuronopathy. Three cases (30%) were positive for anti-nuclear antibodies at titres of 1:160 but all were negative for anti Ro/La antibodies. 4/10 had a normal Schirmer's test., Six (60%) of the PSS cases had dysaesthetic symptoms and four (40%) complained of ataxia. Eight (80%) patients had distal sensory impairment of pinprick and/or vibration with absent ankle jerks., Conclusions: PSS is a common association in otherwise undiagnosed sensory axonal neuropathy. Salivary gland biopsy should be considered in all patients with an unidentified cause for sensory neuropathy and sicca symptoms., (C) 2014 BMJ Publishing Group Ltd