Neuro-Sjogren: Peripheral Neuropathy With Limb Weakness in Sjogren's Syndrome

Objective: Sjogren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine characteristics of Sjogren's syndrome as a cause for severe neuropathy with limb weakness. Methods: One hundred and eighty four patients with polyneuropathy associated with limb weakness underwent routine diagnostics including investigations for Sjogren's syndrome. Forty-four patients with Sjogren's syndrome (ACR-EULAR classification criteria) and severe neuropathy were identified. Results: Sjogren's syndrome was found at a median age of 63 years and the gender distribution showed a balanced female-male ratio of 1 :1 . Anti-SSA(Ro) antibodies were detected in 48% while seronegative patients were diagnosed with Sjogren's syndrome based on sialadenitis on minor salivary gland biopsy with a focus score >= 1. The majority of patients (93%) were diagnosed with Sjogren's syndrome after neurological symptoms appeared. Limbs were symmetrically involved in 84% of patients (57% tetraparesis, 27% paraparesis). Sensory function was not affected in 11% of patients indicating that Sjogren's syndrome associated neuropathy can present as a pure motor syndrome. Electrophysiological measurements did not reveal pathognomonic findings (23% demyelinating pattern, 36% axonal pattern, 41% both demyelinating and axonal damage signs). More than half of our patients fulfilled the European Federation of Neurological Societies (EFNS) diagnostic criteria for CIDP indicating that distinction between Neuro-Sjogren and other causes of neuropathy such as CIDP is challenging. Interpretation: Our findings show that severe neuropathy with limb weakness is often associated with Sjogren's syndrome. This is of great importance in identifying and understanding the causes of immune mediated polyneuropathy.