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Peripheral Neuropathies Associated With Primary Sjögren Syndrome: Immunologic Profiles of Nonataxic Sensory Neuropathy and Sensorimotor Neuropathy.
MedLine Citation:
PMID:  21358442     Owner:  NLM     Status:  Publisher    
We conducted this study to characterize the relationship between primary Sjögren syndrome (pSS)-associated peripheral neuropathy (PN) and markers of B-cell monoclonal proliferation and chronic activation.The cohort included 120 consecutive patients presenting with definite pSS according to the American-European Consensus Group criteria. Serum markers of chronic B-cell activation included autoantibodies and hypergammaglobulinemia. Markers of monoclonal B-cell proliferation included mixed cryoglobulin, monoclonal gammopathy, abnormal κ/λ free light chain (FLC) ratio, and B-cell non-Hodgkin lymphoma (B-NHL). Definite PN was present in 30 patients (25%) including 7 patients (23%) with sensorimotor neuropathy, 3 patients (10%) with ataxic sensory neuropathy, and 20 patients (67%) with nonataxic sensory neuropathy. Patients with a sensorimotor neuropathy differed from those without PN by higher rates of monoclonal B-cell proliferation markers, that is, mixed cryoglobulin (57% vs. 11%; p = 0.008), monoclonal gammopathy (71% vs. 17%; p = 0.004), higher FLC ratio (2.7 ± 1.5 vs. 1.7 ± 1.8; p = 0.024), and B-NHL (57% vs. 3%; p < 0.001). Patients with nonataxic sensory neuropathy were characterized by a higher age (57.5 ± 10.7 vs. 48.7 ± 14.3 years; p = 0.007), more frequent central nervous system (CNS) involvement (15% vs. 2%; p = 0.04) and a lower prevalence of chronic B-cell activation serum markers, that is, antinuclear antibodies (ANA) (60% vs. 90%; p = 0.003), anti-SSA (Ro) (40% vs. 72%; p = 0.009), anti-SSB (La) (15% vs. 41%; p = 0.039), rheumatoid factor (37% vs. 67%; p = 0.02), and hypergammaglobulinemia (35% vs. 64%; p = 0.023). In multivariate analysis, sensorimotor neuropathy was associated with the presence of B-NHL (odds ratio [OR], 39.0; p < 0.001), whereas nonataxic sensory neuropathy was associated with the presence of CNS involvement (OR, 17.0; p = 0.025) and ANA (OR, 0.07; p <0.001).In conclusion, we found that up to 25% of pSS patients presented with PN, predominantly sensory neuropathy. Distinctive immunologic profiles were found according to the type of SS-associated neuropathy: nonataxic sensory neuropathy was marked by a low prevalence of B-cell activation markers, and sensorimotor neuropathy was marked by a high prevalence of B-cell monoclonal proliferation markers.
Damien Sène; Moez Jallouli; Jean-Pascal Lefaucheur; David Saadoun; Nathalie Costedoat-Chalumeau; Thierry Maisonobe; Marie-Claude Diemert; Lucile Musset; Julien Haroche; Jean-Charles Piette; Zahir Amoura; Patrice Cacoub
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-2-22
Journal Detail:
Title:  Medicine     Volume:  -     ISSN:  1536-5964     ISO Abbreviation:  -     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-3-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
From Service de Médecine Interne (D. Sène, D. Saadoun, MJ, NCC, JH, JCP, ZA, PC), Laboratoire de Neuropathologie (TM), and Laboratoire d'Immunochimie (MCD, LM), AP-HP, Hôpital Pitié-Salpêtrière, and Université Pierre et Marie Curie-Paris 6, Paris; and Service de Physiologie-Explorations Fonctionnelles (JPL), AP-HP, Hôpital Henri Mondor, Créteil, France.
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