Yvan Jamilloux1, Laurent Magy2, Jean-François Hurtevent3, Guillaume Gondran4, Jérôme de Seze5, David Launay6, Kim H Ly4, Marc Lambert6, Eric Hachulla6, Pierre-Yves Hatron6, Elisabeth Vidal4, Anne-Laure Fauchais7. 1. Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France; EA 3842 - Department of Immunology, Limoges University, Limoges F-87042, France. Electronic address: yvanjamilloux@hotmail.com. 2. Department of Neurology, Limoges University Hospital, Limoges F-87042, France. 3. Department of Neurology, Lille University Hospital, Lille F-59037, France. 4. Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France. 5. Department of Neurology, Strasbourg University Hospital, Strasbourg F-67091, France. 6. Department of Internal Medicine, Lille University Hospital, Lille F-59037, France. 7. Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France; EA 3842 - Department of Immunology, Limoges University, Limoges F-87042, France.
Abstract
BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: In pSS, patients with sensorimotor neuropathies and pure sensory neuropathies have distinct extraglandular and immunological profiles.
BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: In pSS, patients with sensorimotor neuropathies and pure sensory neuropathies have distinct extraglandular and immunological profiles.
Authors: Sandrine Indart; Jacques Hugon; Pierre Jean Guillausseau; Alice Gilbert; Julien Dumurgier; Claire Paquet; Damien Sène Journal: Medicine (Baltimore) Date: 2017-04 Impact factor: 1.889