Abstract: We conducted the current study to analyze the clinical, immunologic, and neurophysiologic features of primary Sj?gren 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.
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机译：摘要：我们进行了当前的研究，以分析原发性干燥综合征（pSS）相关的感觉小纤维神经病（SFNs）的临床，免疫学和神经生理学特征。包括40例SSF的连续pSS患者。 SFN定义为存在提示的感觉性疼痛症状，并具有正常的神经传导研究和皮肤活检时神经纤维细小或表皮内神经纤维密度低的异常神经生理学检查。将纳入的患者与没有周围神经病变的100例pSS患者进行比较。 SFN患者主要为女性（92.5％）。 pSS诊断的年龄为55.3±13.1岁，SFN诊断的年龄为58.9±11.8岁，症状发作后SFN诊断的中位时间为3.4年。临床症状包括灼痛（90％），麻木（87.5％），刺痛（82.5％），针和针（72.5％），放电（70％）和异常性疼痛（55％）。自主神经异常包括血管舒缩症状（66％）和多汗症（47％）。异常的神经生理学检查包括激光诱发电位（97.5％），热定量感觉检查（67.5％）和交感性皮肤反射（40％）。皮肤活检显示17名受测患者中有76％的表皮内神经纤维密度低。与100例无周围神经病变的pSS患者相比，40例pSS-SFN患者在pSS诊断时年龄更大（55.3±13.1比49.5±14.9岁; p = 0.03），口干症的发生率更高（97.5％比81％; p = 0.03）。 p = 0.01）和关节痛（82.5％比65.0％; p = 0.04）。在免疫学上，它们的特征是血清B细胞活化标记物的患病率较低，即抗核抗体（65％比85％; p = 0.01），抗SSA抗体（42.5％比71％; p = 0.002） ，以及抗SSB（分别为17.5％和39％; p = 0.017）;类风湿因子（32.5％vs. 66％; p = 0.0005）;和高球蛋白血症（35％比62％; p = 0.005）。总之，我们报道了pSS患者SSF的主要特征，这是我们所知的第一个此类研究。我们的结果表明，与pSS相关的SFN患者的特征是在pSS诊断时年龄较大，并且具有独特的免疫学特征，其特征在于较低的血清B细胞活化标记物频率。
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