Objective To assess the diagnostic outcome of patients with an idiopathic neuropathy that were referred to a specialist Sjögren's clinic.
Background In about 15% of patients with a chronic axonal polyneuropathy a cause cannot be found following standard investigations. Primary Sjögren'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 Sjögren'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.