我国西藏地区(高原)系统性红斑狼疮患者临床及免疫学特征分析

doi:10.19723/j.issn.1671-167X.2018.06.011

摘要/Abstract

摘要：

目的: 探讨我国西藏地区系统性红斑狼疮(systemic lupus erythematosus,SLE)患者发病特点、受累器官、免疫学特点。方法: 回顾性研究西藏自治区人民医院风湿免疫科2014年5月至2016年4月住院的SLE患者共70例,随机抽取北京大学人民医院风湿免疫科SLE数据库中120例年龄及性别匹配的住院SLE患者作为对照组,对比高原与平原地区SLE患者临床特点、受累器官、免疫学特点。结果: 西藏地区SLE患者男女比例1 :10.7,平原患者男女比例1 :11.0;高原患者发病年龄(33.2±11.4)岁,平原患者发病年龄(35.3±13.2)岁,两组发病年龄相当。首发临床症状方面,高原地区SLE患者以关节炎(78.6%)、脱发(55.7%)、颧部红斑(48.6%)为常见,其中关节炎、脱发发生率明显高于平原组(P<0.05);高原地区SLE患者70%出现血液系统受累,与平原地区类似;狼疮肾炎及狼疮脑病发生率均明显低于平原组(38.6% vs. 56.7%, 2.9% vs. 17.5%, P<0.05)。血清学方面,高原地区抗双链DNA(double-stranded DNA,dsDNA)抗体阳性率为57.1%,抗Smith(Sm)抗体(55.7%)、抗干燥综合征A(Sj?gren syndrome A, SSA)抗体(74.3%)、抗干燥综合征B(Sj?gren syndrome B, SSB)抗体(41.4%)及抗u1核糖核蛋白(u1-ribosenuclear protein,u1RNP)抗体(45.7%)阳性率均显著高于平原地区患者(P<0.05);分别有61.4%、38.6%的高原SLE患者出现补体C3、C4减低,显著低于平原患者(P<0.05)。SLE疾病活动度指数(SLE disease activity index,SLEDAI)评分在两组间差异无统计学意义(高原组为12.19±5.58,平原组为12.69±7.28)。此外,西藏地区SLE患者合并陈旧结核或者活动结核病13例(18.6%),慢性乙型肝炎或携带者7例(10%)。高原组SLE患者血清25羟维生素D3(25-dihydroxy-vitamin D3, 25-OH-VD3)减低者少于平原组(76.7% vs. 90.0%,P=0.046),血清25-OH-VD3水平高原组为(31.14±18.74) nmol/L,平原组为(26.91±14.27) nmol/L,两组间差异无统计学意义(P=0.16)。结论: 西藏地区SLE患者首发临床症状以关节炎、脱发、颧部红斑最为常见,其中关节炎、脱发发生率显著高于平原地区;狼疮肾炎及狼疮脑病发生率明显低于平原;多种自身抗体的阳性率显著高于平原地区。在年龄、性别构成、SLEDAI评分方面与平原地区类似,血清25-OH-VD3减低者平原组多于高原组,但25-OH-VD3水平在两组间无明显差异。

关键词: 红斑狼疮, 系统性, 西藏, 生物学标记, 症状和体征

Abstract:

Objective: To describe the clinical, immunological characteristics and organ involvement of patients with systemic lupus erythematosus (SLE) in Tibet plateau, China.Methods:We retrospectively investigated 70 patients admitted in the Tibet Autonomous Region People’s Hospital between May 2014 and April 2016. In the study, 120 hospitalized patients with SLE from the Department of Rheumatology and Immunology of the Peking University People’s Hospital were randomly selected as the control (plain) group. The major organ involvement, clinical and immunological characteristics were compared between the two groups.Results:The female to male ratio of Tibet plateau group was 10.7, while the correspon-ding ratio of plain group was 11.0. The mean age at disease diagnosis was (32.21±11.40) and (35.38±13.25) years, respectively. the most common initial manifestations of SLE were arthritis (78.6%), alopecia (55.7%) and malar rash (48.6%) in Tibet plateau group, the prevalence of arthritis and alopecia was significantly higher than in plain group (P<0.05). The incidence of neuropsychiatric and kidney involvement was significantly lower in Tibet plateau group compared with plain group (P<0.05). As for the serological manifestations, the positivity of anti-double-stranded DNA (dsDNA) (57.1%), anti-Smith (Sm) antibody (55.7%), anti-Sj?gren syndrome A (SSA) antibody (72.3%), anti-Sj?gren syndrome B (SSB) antibody (41.4%) and anti-u1-ribosenuclear protein (u1RNP) antibody (45.7%) was significantly higher in Tibet plateau group (P<0.05). While the incidence of low serum complement C3 (61.4%), C4 (38.6%) less frequent in Tibet plateau group. Mean SLE disease activity index (SLEDAI) score was similar in the Tibet plateau group (12.18±5.58) and plain group (12.69±7.28). Moreover, there were 13 (18.6%) SLE patients suffering from tuberculosis and 7 (10%) SLE patients infected with hepatitis B virus in Tibet plateau group. The number of recent-onset SLE patients with lower 25-dihydroxy-vitamin D3 (25-OH-VD3) in Tibet plateau group was fewer than that in the plain group (76.7% vs. 90.0%, P=0.046). Serum 25-OH-VD3 levels in Tibet plateau plateau group were (31.14±18.74) nmol/L, those in plain group were (26.91±14.27) nmol/L, and the difference was not significant.Conclusion:The age, gender and SLEDAI scores in Tibet plateau group was similar to those in plain group. But there are significant differences in clinical manifestations, distributions of antibodies and immunological changes between Tibet plateau group and plain group. The patients with lower serum 25-OH-VD3 levels were more in plain group than in Tibet plateau group, while there was no signi-ficant difference in the 25-OH-VD3 level between the two groups.

Key words: Lupus erythematosus, systemic, Tibet, Biological markers, Symptoms and signs

中图分类号:

R593.241

杨娇,姚海红,莫晓冬,罗增,白玛央金. 我国西藏地区(高原)系统性红斑狼疮患者临床及免疫学特征分析[J]. 北京大学学报（医学版）, 2018, 50(6): 1004-1008.

Jiao YANG,Hai-hong YAO,Xiao-dong MO,Zeng LUO,yang-jin Bai-ma. Clinical and immunological characteristics of patients with systemic lupus erythematosus in Tibet plateau, China[J]. Journal of Peking University(Health Sciences), 2018, 50(6): 1004-1008.

图/表 5

表1

西藏高原地区与平原地区SLE患者一般情况比较"

General situation	Tibet plateau group (n=70)	Plain group (n=120)	P
Female, n(%)	64 (91.4)	110 (91.7)	0.955
Onset age/years, $x -$ ±s	33.21±11.40	35.38±13.25	0.213
SLEDAI score, $x -$ ±s	12.19±5.58	12.69±7.28	0.596

表1

表2

表3

表4

图1

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Clinical features	Tibet plateau group (n=70)	Plain group (n=120)	P
Malar rash	34 (48.6)	65 (54.2)	0.456
Arthritis	55 (78.6)	45 (37.5)	<0.001
Hair loss	39 (55.7)	38 (31.7)	0.001
Fever	17 (27.3)	36 (30.0)	0.397
Oral ulcers	17 (27.3)	19 (15.8)	0.152
Raynaud phenomenon	4 (5.7)	15 (12.5)	0.133

Items	Tibet plateau group (n=70)	Plain group (n=120)	P
Blood system	49 (70.0)	76 (63.3)	0.350
Urinary system
Lupus nephritis	27 (38.6)	68 (56.7)	0.016
Cystitis	0	1 (0.8)	>0.990
Retinal vasculitis	2 (4.3)	4 (3.3)	>0.990
Neuropsychiatric lupus	2 (2.9)	21 (17.5)	0.003
Respiratory system
Interstitial lung disease/ Pulmonary fibrosis	6 (8.6)	16 (13.3)	0.322
Valvular hemorrhage	3 (4.3)	4 (3.3)	0.709
Cardiovascular system
Valvular heart disease	0	2 (1.7)	>0.992
Pulmonary hypertension	6 (8.6)	15 (12.5)	0.405
Digestive system
Autoimmune pancreatitis	2 (2.9)	2 (1.7)	0.623
Mesenteric vasculitis	2 (2.9)	4 (3.3)	>0.989
Liver injury	3 (4.3)	12 (10.0)	0.163
Myositis	2 (2.9)	10 (8.3)	0.131

Items	Tibet plateau group (n=70)	Plain group (n=120)	P
ANA+	66(94.3)	112(93.3)	0.795
Anti-dsDNA+	40(57.1)	48(40.0)	0.022
Anti-Sm+	39(55.7)	10(8.3)	<0.001
Anti-SSA+	52(74.3)	40(33.3)	<0.001
Anti-SSB+	29(41.4)	7(5.8)	<0.001
Anti-u1RNP+	32(45.7)	24(20.0)	<0.001
ACL+	16(22.9)	33(27.5)	0.480
C3↓	43(61.4)	105(87.5)	<0.001
C4↓	27(38.6)	92(76.7)	<0.001