title: Sjögren Syndrome-Associated Small Fiber Neuropathy: Characterization From a Prospective Series of 40 Cases.
abstract: We conducted the current study to analyze the clinical, immunologic, and neurophysiologic features of primary Sjögren syndrome (pSS)-associated sensory small fiber neuropathies (SFNs). Forty consecutive pSS patients with SFN were included. SFN was defined by the presence of suggestive sensory painful symptoms with normal nerve conduction studies and abnormal neurophysiologic tests for small nerve fibers or a low intraepidermal nerve fiber density at skin biopsy. Included patients were compared to 100 pSS patients without peripheral neuropathy.SFN patients were mainly female (92.5%). Age at pSS diagnosis was 55.3 ± 13.1 years, and at SFN diagnosis, 58.9 ± 11.8 years, with a median time to SFN diagnosis after symptom onset of 3.4 years. Clinical symptoms included burning pains (90%), numbness (87.5%), tingling (82.5%), pins and needles (72.5%), electric discharges (70%), and allodynia (55%). Dysautonomia included vasomotor symptoms (66%) and hyperhidrosis (47%). Abnormal neurophysiologic tests included laser evoked potentials (97.5%), thermal quantitative sensory testing (67.5%), and sympathetic skin reflex (40%). A skin biopsy revealed low intraepidermal nerve fiber density in 76% of the 17 tested patients.Compared to the 100 pSS patients without peripheral neuropathy, the 40 pSS-SFN patients were older at pSS diagnosis (55.3 ± 13.1 vs. 49.5 ± 14.9 yr; p = 0.03), and more often had xerostomia (97.5% vs. 81%; p = 0.01) and arthralgia (82.5% vs. 65.0%; p = 0.04). Immunologically, they were characterized by a lower prevalence of serum B-cell activation markers, that is, antinuclear antibodies (65% vs. 85%; p = 0.01), anti-SSA (42.5% vs. 71%; p = 0.002), and anti-SSB (17.5% vs. 39%; p = 0.017); rheumatoid factor (32.5% vs. 66%; p = 0.0005); and hypergammaglobulinemia (35% vs. 62%; p = 0.005).In conclusion, we report the main features of SFN in patients with pSS, the first such study to our knowledge. Our results show that patients with pSS-associated SFN are characterized by an older age at pSS diagnosis and a distinctive immunologic profile hallmarked by a lower frequency of serum B-cell activation markers.
authors: From the Service de Médecine Interne 2, AP-HP, Hôpital Lariboisière, Université Paris Diderot-Paris 7, Paris (D. Sène, PJG); Service de Médecine Interne 2, AP-HP, Hôpital Pitié-Salpêtrière, Paris, Université, Pierre et Marie Curie-Paris 6, Paris (PC, JH, D. Saadoun, ZA); Centre de Référence des Maladies Neuromusculaire Garches-Necker-Mondor-Hendaye, AP-HP, Hôpital Henri Mondor; INSERM U955, Equipe 10, Université Paris Est-Créteil, Créteil (FJA); and Service de Neurologie (AC) and Service de Physiologie-Explorations Fonctionnelles (JPL), APHP, Hôpital Henri Mondor,Créteil, Université Paris Est-Créteil, Créteil; France.
authors: Damien
authors: D
authors: Sène
authors: Patrice
authors: P
authors: Cacoub
authors: François-Jérôme
authors: FJ
authors: Authier
authors: Julien
authors: J
authors: Haroche
authors: Alain
authors: A
authors: Créange
authors: David
authors: D
authors: Saadoun
authors: Zahir
authors: Z
authors: Amoura
authors: Pierre-Jean
authors: PJ
authors: Guillausseau
authors: Jean-Pascal
authors: JP
authors: Lefaucheur
references: Brain. 2013 Jun;136(Pt 6):1857-67
references: 23474848
references: Neurophysiol Clin. 2012 Jun;42(4):199-206
references: 22632868
references: Pain. 2011 Feb;152(2):410-418
references: 21185650
references: Brain. 2005 Nov;128(Pt 11):2518-34
references: 16049042
references: J Neurol Neurosurg Psychiatry. 2006 Aug;77(8):967-9
references: 16844955
references: Medicine (Baltimore). 2008 Jul;87(4):210-219
references: 18626304
references: J Neurol. 2001 Jul;248(7):577-84
references: 11517999
references: Muscle Nerve. 2003 Nov;28(5):553-60
references: 14571456
references: Eur J Neurol. 2010 Jul;17(7):903-12, e44-9
references: 20642627
references: Clin Neurophysiol. 2010 Aug;121(8):1256-66
references: 20347388
references: Eur J Neurol. 2004 Mar;11(3):153-62
references: 15009162
references: Neurology. 1999 Nov 10;53(8):1641-7
references: 10563606
references: Neurology. 2012 May 22;78(21):1635-43
references: 22539570
references: Eur J Pain. 2009 Aug;13(7):711-8
references: 18789872
references: Clin Rheumatol. 2011 Apr;30(4):485-90
references: 20393864
references: Brain. 2004 Jul;127(Pt 7):1593-605
references: 15128619
references: Ann Neurol. 2012 Jan;71(1):26-39
references: 21698661
references: Lepr Rev. 2007 Dec;78(4):369-80
references: 18309711
references: Rev Med Interne. 2010 Oct;31(10):677-84
references: 20851508
references: J Peripher Nerv Syst. 2010 Mar;15(1):57-62
references: 20433606
references: Mod Rheumatol. 2009;19(4):437-40
references: 19458906
references: Neurology. 2005 Sep 27;65(6):925-7
references: 16186536
references: Neurology. 2009 Jan 13;72(2):177-84
references: 19056667
references: Rev Med Interne. 2011 Mar;32(3):142-8
references: 20943291
references: Neurophysiol Clin. 1996;26(4):202-15
references: 8975110
references: Arch Neurol. 2006 Oct;63(10):1410-3
references: 17030656
references: Presse Med. 2012 Jun;41(6 Pt 1):575-9
references: 22197096
references: Neurology. 1997 Apr;48(4):855-62
references: 9109867
references: Pain. 2005 Mar;114(1-2):29-36
references: 15733628
references: Autoimmun Rev. 2010 Jul;9(9):595-9
references: 20457283
references: Brain. 2008 Jul;131(Pt 7):1912-25
references: 18524793
references: J Neurol. 2008 Aug;255(8):1197-202
references: 18574618
references: Medicine (Baltimore). 2002 Jul;81(4):270-80
references: 12169882
references: Medicine (Baltimore). 2011 Mar;90(2):133-138
references: 21358442
references: J Neurol Neurosurg Psychiatry. 2004 Mar;75(3):417-22
references: 14966158
references: J Neurol Sci. 2009 Apr 15;279(1-2):57-61
references: 19168191
references: Muscle Nerve. 2012 Jan;45(1):86-91
references: 22190313
references: Muscle Nerve. 2006 May;33(5):672-6
references: 16453296
references: J Neurol Neurosurg Psychiatry. 2008 Feb;79(2):163-9
references: 17911181
references: Clin Chem. 1992 Jun;38(6):798-802
references: 1597004
references: Neurophysiol Clin. 2002 Apr;32(2):91-8
references: 12035490
references: Lancet. 2005 Jul 23-29;366(9482):321-31
references: 16039337
references: Eur J Neurol. 2005 Oct;12(10):747-58
references: 16190912
references: J Neurol. 2011 Oct;258(10):1852-64
references: 21472496
references: Arch Neurol. 2006 Nov;63(11):1612-5
references: 17101831
references: Diabetes Metab Res Rev. 2010 Feb;26(2):100-6
references: 19943330
references: Clin Rheumatol. 1990 Mar;9(1 Suppl 1):123-30
references: 2203591
references: Clin Investig. 1994 Nov;72(11):822-9
references: 7894206
references: Diabetes Care. 2010 Oct;33(10):2285-93
references: 20876709
references: Rheumatology (Oxford). 1999 Mar;38(3):245-53
references: 10325663
references: Ann Rheum Dis. 2002 Jun;61(6):554-8
references: 12006334
references: J Neurol Sci. 2012 Aug 15;319(1-2):139-46
references: 22658898
references: Diabetes Care. 2010 Dec;33(12):2654-9
references: 20841612
references: J Neurol Neurosurg Psychiatry. 1994 Aug;57(8):983-6
references: 8057125
references: J Neurol. 2013 Jun;260(6):1497-503
references: 23306657
references: J Neurol. 2009 Jul;256(7):1067-75
references: 19252773
references: Ann Rheum Dis. 2001 Sep;60(9):876-81
references: 11502615
references: Muscle Nerve. 2008 Jan;37(1):50-60
references: 17763459
references: Arthritis Rheum. 2002 Mar;46(3):741-7
references: 11920410
references: Eur J Neurol. 2010 Jan;17(1):67-72
references: 19614969
references: J Auton Nerv Syst. 1999 Jan 15;75(1):70-6
references: 9935271