Central and peripheral neurological complications of primary Sjögren's syndrome.

Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. Clinically significant neurologic manifestations affect approximately 20% of patients and may be the first manifestation of the disease in at least 25% of the cases. The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability.

Fauchais AL ，Magy L ，Vidal E 《-》

The kaleidoscope of neurological manifestations in primary Sjögren's syndrome.

Alunno A ，Carubbi F ，Bartoloni E ，Cipriani P ，Giacomelli R ，Gerli R ... -  《-》

Immunological profiles determine neurological involvement in Sjögren's syndrome.

Up to 68% of patients with primary Sjögren's syndrome (pSS) undergo neurological complications, and evidence for distinct immunological subgroups is emerging. We sought to determine systemic and immunological profiles associated with neurological manifestations. 420 patients fulfilling the 2002 American-European pSS criteria were retrospectively analyzed. Neurological manifestations were diagnosed through clinical, biological, electrophysiological, and imaging findings. Biographical, clinical, and laboratory data were compared. Within 93 (22%) patients with neurological manifestations, peripheral and central nervous systems were involved in 66% and 44%, respectively. Raynaud's phenomenon, cutaneous vasculitis, renal involvement, and cryoglobulinemia were associated with sensorimotor neuropathy and mononeuritis multiplex (p<0.05). Conversely, pure sensory neuropathy occurred without extraglandular manifestation, and without anti-Ro/SSA antibodies (p<0.05). All neurological manifestations were associated with increased use of corticosteroids and immunosuppressive drugs (p<0.05). In pSS, patients with sensorimotor neuropathies and pure sensory neuropathies have distinct extraglandular and immunological profiles.

Jamilloux Y ，Magy L ，Hurtevent JF ，Gondran G ，de Seze J ，Launay D ，Ly KH ，Lambert M ，Hachulla E ，Hatron PY ，Vidal E ，Fauchais AL ... -  《-》

[Neurologic manifestations of primary Gougerot-Sjögren syndrome].

Lafitte C 《REVUE NEUROLOGIQUE》

[Central nervous system involvement in primary Sjögren syndrome].

Ramírez M ，Ramos-Casals M ，Graus F 《-》