Abstract
Objective: Sjögren'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 Sjögren'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 Sjögren's syndrome. Forty-four patients with Sjögren's syndrome (ACR-EULAR classification criteria) and severe neuropathy were identified.Results: Sjögren'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 Sjögren's syndrome based on sialadenitis on minor salivary gland biopsy with a focus score ≥1. The majority of patients (93%) were diagnosed with Sjögren'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 Sjögren'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-Sjögren and other causes of neuropathy such as CIDP is challenging.Interpretation: Our findings show that severe neuropathy with limb weakness is often associated with Sjögren's syndrome. This is of great importance in identifying and understanding the causes of immune mediated polyneuropathy.
Highlights
Sjögren’s syndrome has been described as an inflammatory disease of salivary and lacrimal glands characterized by typical sicca symptoms of dry mouth and eyes and lymphocytic infiltration of glandular tissues [1]
At presentation to our department, 5 patients (11%) received primary workup leading to the diagnosis of peripheral neuropathy and Sjögren’s syndrome
This study facilitates the recognition of Sjögren’s syndrome as a cause for severe neuropathy with limb weakness, which favorably responds to immunosuppressive therapy
Summary
Sjögren’s syndrome has been described as an inflammatory disease of salivary and lacrimal glands characterized by typical sicca symptoms of dry mouth and eyes and lymphocytic infiltration of glandular tissues [1]. Inflammation of the nervous system represents another complication of Sjögren’s syndrome. Motor impairment due to neuropathy has not been widely recognized until now in patients with Sjögren’s syndrome [3, 4]. This severe complication of Sjögren’s syndrome with fulminant development of limb weakness but favorable outcome after immunosuppressive therapy was described in several case reports [5,6,7,8,9]. Patients presenting with limb weakness as a main complaint of polyneuropathy are predominantly treated in neurological departments, where a potentially causal Sjögren’s syndrome may not be routinely investigated. In order to define this entity in detail, we assessed the clinical picture of Sjögren’s syndrome patients with neuropathy and motor dysfunction to facilitate diagnostic approaches and treatment eligibility
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