Sjögren Syndrome-Associated Small Fiber Neuropathy: Characterization From a Prospective Series of 40 Cases

Damien Sène; Patrice Cacoub; François-Jérôme Authier; Julien Haroche; Alain Créange; David Saadoun; Zahir Amoura; Pierre-Jean Guillausseau; Jean-Pascal Lefaucheur

doi:10.1097/MD.0000000000000005

Sjögren Syndrome-Associated Small Fiber Neuropathy: Characterization From a Prospective Series of 40 Cases

Medicine (Baltimore). 2013 Sep;92(5):e10-e18. doi: 10.1097/MD.0000000000000005.

Authors

Damien Sène¹, Patrice Cacoub, François-Jérôme Authier, Julien Haroche, Alain Créange, David Saadoun, Zahir Amoura, Pierre-Jean Guillausseau, Jean-Pascal Lefaucheur

Affiliation

¹ 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.

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.