Sjogren's syndrome (SS) is a chronic organ-specific autoimmune disease mainly involving exocrine glands such as lacrimal and salivary glands. SS may also involve central and peripheral nervous system with variable prevalence due to differences in diagnostic criteria and in time length to reach diagnosis. Clinical features of the central nervous involvement share similarities with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), two major neuroimmune disorders. SS may even coexist with MS or NMOSD. Sensory neuropathy, chronic polyradiculoneuropathy, cranial neuropathies as well as small fibre neuropathy are the main manifestations of the peripheral nervous system involvement. The pathogenic mechanism underlying neuro-SS is unclear even though molecular mimicry and epitope spreading have been hypothesized for central nervous involvement, whereas vasculitis with or without direct damage to nerve could account for peripheral nervous involvement. Treatment is mainly based on immunosuppressive therapies requiring a close cooperation between neurologists and rheumatologists to achieve the best management.
Annunziata, P. (2019). Neuroinflammation and Sjogren’s Syndrome. In Neuroimmune diseases (pp. 699-709). GEWERBESTRASSE 11, CHAM, CH-6330, SWITZERLAND : SPRINGER INTERNATIONAL PUBLISHING AG [10.1007/978-3-030-19515-1_23].
Neuroinflammation and Sjogren’s Syndrome
Annunziata, Pasquale
2019-01-01
Abstract
Sjogren's syndrome (SS) is a chronic organ-specific autoimmune disease mainly involving exocrine glands such as lacrimal and salivary glands. SS may also involve central and peripheral nervous system with variable prevalence due to differences in diagnostic criteria and in time length to reach diagnosis. Clinical features of the central nervous involvement share similarities with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), two major neuroimmune disorders. SS may even coexist with MS or NMOSD. Sensory neuropathy, chronic polyradiculoneuropathy, cranial neuropathies as well as small fibre neuropathy are the main manifestations of the peripheral nervous system involvement. The pathogenic mechanism underlying neuro-SS is unclear even though molecular mimicry and epitope spreading have been hypothesized for central nervous involvement, whereas vasculitis with or without direct damage to nerve could account for peripheral nervous involvement. Treatment is mainly based on immunosuppressive therapies requiring a close cooperation between neurologists and rheumatologists to achieve the best management.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
https://hdl.handle.net/11365/1107200
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