Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.
New Insights in Sjgren Syndrome Associated Peripheral Neuropathies: The Small Fibre Neuropathy and Its Clinical, Immunological and Neurophysiological Features from a Moncentre Cohort of 24 Patients.
Sene2, Damien, Lefaucheur1, Jean-Pascal, Haroche2, Julien, Saadoun2, David, Hervier2, Baptiste, Piette2, Jean-Charles, Amoura2, Zahir
Primary Sjögren syndrome (pSS) may account for 9 to 30% of causes of sensory small fibre neuropathies (SFN), clinically characterized by sensory painful symptoms mostly associated with normal clinical and electromyographic examinations. Nevertheless, the main features of SFN have never been evaluated in a large cohort of pSS-patients. The aim of this study was to analyze the clinical, immunological and neurophysiological features of pSS-associated SFN.
Patients and Methods:
24 consecutive pSS-patients (according to Americano-European group criteria consensus revised on 2002) presenting a definite SFN were included. The SFN was defined by the presence of sensory painful symptoms with a DN4 score >=4 and the evidence of abnormal nerve small fibre neurophysiological tests, including laser evoked potentials (LEP), quantitative sensory test (QST) and sensory skin reflex (SSR). The 24 included patients were compared to 89 SS-patients without peripheral neuropathy.
the 24 patients included 20 female (83%) and 4 men (17%) with a mean age of 64±10 years. For 21 patients (87.5%), SFN led to pSS diagnosis. SFN involved both upper and lower limbs for 19 patients (79%) and lower limbs alone for 5 patients (21%). The neurophysiological tests abnormalities included altered LEP in 21 patients (87.5%), abnormal QST in 21 patients (87.5%) and abnormal SSR in 13 patients (54.2%).
Compared to the 89 pSS-patients who did not present with a peripheral neuropathy, patients with a pSS-associated SFN were older at the time of pSS diagnosis (63±10.5 vs 48.5±14 years; P<10-4), and had more frequently a central nervous system involvement (25% vs 2%; P<10-4). They were featured by a lower frequency of serum B cell activation markers, i.e, less often positive ANA (58% vs 90%; P<10-3), anti-SSA (37.5% vs 73%; P=0.001), anti-SSB (12.5% vs 42%; P=0.008) and rheumatoid factor (37.5% vs 68%; P=0.008), and lower serum gammaglobulins levels (12.5±5 vs 16±7 mg/L; P=0.0045).
Our study reported for the first time the main features of SFN in pSS patients. Our results showed that patients with a pSS-associated SFN are characterized by an older age at SS diagnosis and a more frequent CNS involvement, and a distinctive immunological profile hallmarked by a lower frequency of serum B cell activation markers (ANA, anti-SSA, anti-SSB, rheumatoid factor, and gammaglobulins).
To cite this abstract, please use the following information:
Sene, Damien, Lefaucheur, Jean-Pascal, Haroche, Julien, Saadoun, David, Hervier, Baptiste, Piette, Jean-Charles, et al; New Insights in Sjgren Syndrome Associated Peripheral Neuropathies: The Small Fibre Neuropathy and Its Clinical, Immunological and Neurophysiological Features from a Moncentre Cohort of 24 Patients. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1908