干燥综合征神经系统损害的临床特征及危险因素
Clinical features and risk factors of neurological involvement in Sjögren's syndrome.
机构信息
Rheumatology Department, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
Rheumatology Department, Ruian People's Hospital, Wenzhou, China.
出版信息
BMC Neurosci. 2018 Apr 27;19(1):26. doi: 10.1186/s12868-018-0427-y.
BACKGROUND
To investigated distinct manifestations of Sjögren's syndrome (SS) patients with neurological complications and the potential risk factors associated with neurological complications in SS, and to produce a disease evaluation and neurological involvement prediction for SS.
METHODS
566 patients who fulfilled the 2002 classification criteria for SS from the Rheumatology Department of the First Affiliated Hospital of Wenzhou Medical University were included in the cross-sectional study. Clinical, immunological and histological characteristics were surveyed, and potential risk factors for neurological complications were examined by multivariate analysis.
RESULTS
Among 566 SS patients, 184 (32.5%) patients had neurological involvement, with more than 10% got limbs pain, limbs numbness and cerebral infarction, respectively. Of these 184 SS patients with neurological complications, secondary SS (sSS) patients had a higher prevalence of peripheral nervous system (PNS) involvement than primary SS (pSS) patients (31.1 vs. 19%). And sSS patients showed higher total ESSPRI score and higher prevalence of xerostomia and low C3, C4 levels with more liver, articular involvement and saliva gland atrophy, and more severe lymphocyte infiltration in salivary glands than pSS patients. As for the specific factors associated with neurological involvement, low C3 level were found to be significant in pSS or sSS patients who were younger 50 year old, and ANA positivity, cardiac involvement, saliva gland atrophy were demonstrated to be associated in elder pSS patients. And xerophthalmia was found to be associated in sSS patients.
CONCLUSION
Low complement (C3) levels, xerophthalmia, ANA positive, cardiac involvement and labial salivary gland histological result were good ways to predict neurological complications in different subgroups of SS, which might provide insight into better clinical decision-making, especially at early stages of the disease.
背景
本研究旨在探讨伴有神经并发症的干燥综合征（SS）患者的不同表现，以及 SS 患者发生神经并发症的相关潜在危险因素，从而为 SS 患者进行疾病评估和神经受累预测。
方法
本横断面研究纳入了温州医科大学附属第一医院风湿免疫科 2002 年 SS 分类标准的 566 例患者。通过多变量分析调查了临床、免疫和组织学特征，并研究了神经并发症的潜在危险因素。
结果
在 566 例 SS 患者中，有 184 例（32.5%）患者存在神经受累，分别有超过 10%的患者出现四肢疼痛、四肢麻木和脑梗死。在这 184 例 SS 合并神经并发症的患者中，继发性 SS（sSS）患者的周围神经系统（PNS）受累发生率高于原发性 SS（pSS）患者（31.1% vs. 19%）。sSS 患者的 ESSPRI 总分更高，口干和低 C3、C4 发生率更高，肝、关节受累和唾液腺萎缩更多，唾液腺淋巴细胞浸润更严重。至于与神经受累相关的具体因素，在年龄<50 岁的 pSS 或 sSS 患者中，低 C3 水平与神经受累显著相关，而在年龄较大的 pSS 患者中，ANA 阳性、心脏受累、唾液腺萎缩与神经受累相关。在 sSS 患者中，眼干与神经受累相关。
结论
低补体（C3）水平、眼干、ANA 阳性、心脏受累和唇腺组织学结果是预测不同 SS 亚组神经并发症的良好方法，这可能为更好的临床决策提供依据，尤其是在疾病早期。
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