Immunological profiles determine neurological involvement in Sjögren's syndrome

•Neurological manifestations affect 20% of patients with primary Sjögren's syndrome.•Systemic and immunological profiles distinguish subgroups of neuroSjögren patients.•NeuroSjögren is associated with increased steroid/immunosuppressant treatments.

BackgroundUp 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.Methods420 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.ResultsWithin 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).ConclusionsIn pSS, patients with sensorimotor neuropathies and pure sensory neuropathies have distinct extraglandular and immunological profiles.