原发性干燥综合征患者合并周围神经病变的临床及实验室特点分析

doi:10.16150/j.1671-2870.2019.01.010

摘要/Abstract

摘要： 目的了解原发性干燥综合征（primary Sjögren′s syndrome, pSS）患者合并周围神经（peripheral nervous system）病变的临床特点，探讨pSS合并周围神经病变的危险因素。方法回顾性分析2015年1月至2017年9月，同济大学附属同济医院风湿免疫科收治且符合2002年美欧协作组提出的干燥综合征分类标准的132例pSS患者。通过临床症状、神经系统查体及肌电图检查明确患者有无神经系统损害，进而将其分为pSS合并周围神经系统病变（primary Sjögren′s syndrome with peripheral nervous system, pSS-PNS）组与未合并周围神经系统病变（primary Sjögren′s syndrome not with peripheral nervous system, pSS-nPNS）组，并比较2组的临床表现、相应检查、实验室特征。结果 132例pSS患者中男性12例，女性120例，男女之比为1∶10，其中21例患者合并有周围神经系统受累(21/132，15.9%)，pSS-PNS与pSS-nPNS组间的性别构成、年龄、起病年龄及病程差异无统计学意义。与pSS-nPNS相比， pSS-PNS组的关节肿痛较为常见（71.4%比44.1%，P=0.02），且CD8水平明显下降[(21.25±13.11) %与（26.65±9.44）%，P=0.02]，CD4/CD8比值明显升高（2.80±1.45比1.91±1.12，P=0.04）。pSS-PNS组的欧洲抗风湿病联盟干燥综合征疾病活动度指数（European League Against Rheumatism Sjögren′s Syndrome Disease Activity Index，ESSDAI）较pSS-nPNS组明显增高[(4.71±0.46)分比（2.80±0.17）分，P<0.001]，且ESSDAI（OR=1.836，95%CI 1.169~2.883，P=0.008）是pSS合并周围神经病变的独立危险因素。结论 pSS-PNS患者的临床表现多样，疾病活动度高，高疾病活动度为pSS-PNS的独立危险因素。

关键词: 原发性干燥综合征, 周围神经病变, 肌电图, CD4/CD8

Abstract:

Objective: To investigate the clinical manifestations, electromyographic abnormalities and laboratory characteristics of primary Sjögren′s syndrome (pSS) complicated with peripheral neuropathy and to analyze the risk factor of pSS complicated with peripheral neuropathy. Methods: A retrospective analysis was performed on 132 pSS patients who met the 2002 American-European Consensus Group criteria for pSS at Tongji Hospital from January 2015 to September 2017. The patients were categorized into group with peripheral neuropathy (pSS-PNS) and group without peripheral neuropathy (pSS-nPNS). The clinical manifestations, corresponding examinations and laboratory characteristics of the two groups were compared. The data were analyzed by t test, χ² test and logistic regression analysis. Results: A total of 132 patients with pSS were enrolled in this study, including 12 males and 120 females. The ratio of male to female was 1∶10. Among them, 21 patients suffered from peripheral nervous system involvement and the prevalence rate was 15.9%(21/132). There were no significant differences in gender composition, age, onset age and course between the two groups. Compared with the group without peripheral neuropathy, joint swelling and pain (71.4% and 44.1%, P=0.02) were more common in the group with peripheral neuropathy, and CD8 [(21.25±13.11)% and（26.65±9.44）%, P=0.02] decreased significantly, ratio of CD4/CD8(2.80±1.45 vs 1.91±1.12, P=0.04) increased significantly. ESSDAI (European League Against Rheumatism Sjögren′s Syndrome Disease Activity Index) score [(4.71±0.46) and (2.80±0.17) ] was significantly higher in pSS-PNS group. ESSDAI (OR=1.836, 95%CI 1.169-2.883, P=0.008) was an independent risk factor for pSS with peripheral neuropathy. Conclusions: The clinical manifestations of pSS complicated with peripheral neuropathy are diverse, and the disease activity is high. High disease activity is an independent risk factor for Sjögren′s syndrome complicated with peripheral neuropathy.

Key words: Primary Sjögren′s syndrome, Peripheral nervous system lesions, Electromyography, CD4/CD8

中图分类号:

R593.2

王艳青, 黄琬雪, 梁媛媛, 吴珍珍, 王璇, 汤建平. 原发性干燥综合征患者合并周围神经病变的临床及实验室特点分析[J]. 诊断学理论与实践, 2019, 18(1): 51-55.

WANG Yanqing, HUANG Wanxue, LIANG Yuanyuan, WU Zhenzhen, WANG Xuan, TANG Jianping. Clinical and laboratory analysis of primary Sjögren′s syndrome complicated with neurological lesions[J]. Journal of Diagnostics Concepts & Practice, 2019, 18(1): 51-55.

图/表 5

表1

表2

表3

表4

pSS-PNS组与pSS-nPNS组实验室检查比较[n（%），$\bar{x}$±s]

组别		抗SSA抗体（+）	抗SSB抗体（+）		抗SSA抗体及抗SSB抗体（+）		抗核抗体颗粒型（+）		抗核抗体均质型（+）	抗核抗体着丝点（+）		唾液腺 ECT		血红蛋白（g/L）		血小板（×10⁹）		白细胞（×10⁹）
pSS-PNS		16(76.1)	5(23.8)		5(23.8)		11(52.3)		1(4.7)	6(28.5)		19(90.4)		115.76±14.48		161.9±44.30		5.17±1.30
pSS-nPNS		84(75.6)	33(29.7)		32(29.7)		64(57.6)		3(2.7)	12(10.8)		89(80.1)		118.03±14.35		188.2±60.81		5.05±1.98
Z值		0.96	0.64		0.58		0.65		0.61	0.03		0.26		-0.54^*		-2.00^*		-1.40^*
P值		1.00	0.77		0.83		0.84		1.00	0.06		0.41		0.59		0.04		0.16
组别	红细胞（×10¹²）			淋巴细胞（×10⁹）		中性粒细胞（×10⁹）		C反应蛋白（mg/L）			红细胞沉降率（mm/h）		CD3(%)		CD4(%)		CD8(%)		CD4/CD8
pSS-PNS	3.93±0.46			1.84±0.71		2.76±0.88		161.95±44.30			5.17±1.30		71.6±13.84		47.7±14.36		21.25±13.11		2.80±1.45
pSS-nPNS	3.94±0.45			1.83±0.71		3.39±4.98		188.20±60.81			5.05±1.98		71.3±12.06		42.3±11.51		26.65±9.44		1.91±1.12
Z值	-0.27			-1.66		-0.15		-0.70			-0.29		-0.56		-1.04		-2.30		-2.02
P值	0.78			0.09		0.88		0.48			0.76		0.57		0.29		0.02		0.04
组别	CD19(%)			补体C₃(g/L)		补体C₄(g/L)		总补体(g/L)			IgA(g/L)		IgM(g/L)		IgG(g/L)		IgE(g/L)		IgG4(g/L)
pSS-PNS	12.0±10.17			0.91±0.22		0.24±0.15		35.20±16.35			2.75±1.13		1.10±0.90		14.92±4.79		122.5±220.2		0.46±0.61
pSS-nPNS	12.85±8.00			0.97±0.23		0.20±0.08		36.34±10.74			2.85±2.44		1.25±0.80		15.29±6.60		78.40±145.3		0.51±0.45
Z值	-0.57			-0.43		-1.19		-0.42			-0.64		-1.44		-0.15		0.53		-0.72
P值	0.56			0.66		0.23		0.67			0.52		0.15		0.88		0.59		0.46
别	肿瘤坏死因子α(μg/L)			IL-2R(ng/L)		IL-6(ng/L)		IL-8(ng/L)			RF(U/mL)		抗CCP (U/mL)		抗双链 DNA抗体
pSS-PNS	10.5±8.2			390±129.8		1.65±1.69		24.65±24.72			13.19±60.44		11.93±13.47		37.9±33.04
pSS-nPNS	9.1±6.3			382±172.2		2.21±2.17		27.50±34.86			8.33±20.87		48.6±228.17		75.6±78.39
Z值	-0.37			-0.22		-0.64		-0.72			-1.90		-0.02		0.41
P值	0.75			0.82		0.52		0.46			0.05		0.98		0.40

表4

表5

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组别	年龄（岁）	发病年龄（年）	发病病程（个月）	住院时间（d）
pSS-PNS	57.56±14.45	49.7±13.86	97.20±101.04	8.67±2.85
pSS-nPNS	62.86±9.83	52.5±10.57	123.4±115.55	8.12±2.76
Z值	-1.80	-0.78	-1.20	-0.80
P值	0.07	0.43	0.22	0.44

肌电图表现	例数（n）
上肢神经损害	10
下肢神经损害	12
单神经病变	10
多神经病变	11
运动神经损害为主	13
感觉神经损害为主	8

组别	眼干	口干	皮疹	雷诺表现	口腔溃疡	龋齿	关节肿痛	肢体麻木	甲状腺累及	间质性肺炎
pSS-PNS	17（80.9）	19（90.4）	4（19）	4（19）	7（33.3）	14（66.6）	15（71.4）	13（61.9）	2（9.5）	8（38.0）
pSS-nPNS	76（68.4）	89（80.1）	26（23.4）	19（17.1）	21（18.9）	111（60.3）	49（44.1）	29（26.1）	16（14.1）	27（24.3）
χ²值	1.32	1.26	0.19	0.05	0.25	0.3	5.26	10.42	0.36	1.72
P值	0.31	0.36	0.78	0.76	0.61	0.63	0.02	0.001	0.73	0.19

变量	B	S.E.	Wals	OR值	P值	95% CI
ESSDAI	0.608	0.230	6.959	1.836	0.008	1.169~2.883
血小板计数	-0.005	0.007	0.442	0.995	0.506	0.982~1.009
CD8	-0.051	0.055	0.868	0.950	0.352	0.853~1.058
CD4/CD8	0.428	0.412	1.078	1.533	0.299	0.684~3.437
关节肿痛	-0.306	0.825	0.137	0.736	0.711	0.146~3.713