Severe sensory neuronopathy responsive to infliximab in primary Sjögren’s syndrome
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Sjögren’s syndrome (SS) is an autoimmune disease characterized by mononuclear infiltration and destruction of exocrine glands.1 SS can be primary or may occur in association with other autoimmune disorders. Neurologic complications of primary SS mainly affect the peripheral nervous system, with an incidence ranging from 10 to 50%.1 Several forms of peripheral nerve injury occur, the most common being trigeminal sensory neuropathy, symmetric distal sensory or sensorimotor neuropathy, and pure sensory neuronopathy. No definitive treatment is available.1
The events that lead to autoimmune responses in SS have not been established. Genetic, endocrine, and viral factors are involved in the pathogenesis.1 Laboratory and clinical evidence suggest that proinflammatory cytokines, particularly tumor necrosis factor α (TNFα), may have a role in SS.2 Interleukin (IL)-1, IL-6, and TNFα have neurotoxic effects.3,4⇓ We describe a patient with sensory neuronopathy occurring in association with primary SS. Treatment of …
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