title: Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy.
abstract: 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.
authors.affiliation: 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.
authors.forename: Damien
authors.initials: D
authors.lastname: Sène
authors.forename: Moez
authors.initials: M
authors.lastname: Jallouli
authors.forename: Jean-Pascal
authors.initials: JP
authors.lastname: Lefaucheur
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Thierry
authors.initials: T
authors.lastname: Maisonobe
authors.forename: Marie-Claude
authors.initials: MC
authors.lastname: Diemert
authors.forename: Lucile
authors.initials: L
authors.lastname: Musset
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000328:Adult; D000367:Age Factors; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D015415:Biomarkers / Q000097:blood; D049109:Cell Proliferation; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology / Q000276:immunology*; D012737:Sex Factors; D012859:Sjogren's Syndrome / Q000150:complications / Q000276:immunology*
chemical_list: D015415:Biomarkers