伴皮肤紫癜的原发性干燥综合征患者的临床特征分析

doi:10.16150/j.1671-2870.2021.02.007

摘要/Abstract

摘要：

目的:分析伴皮肤紫癜的原发性干燥综合征（primary Sjögren syndrome, pSS）患者的临床特征。方法:回顾性分析2017年1月至2020年1月间上海交通大学医学院附属瑞金医院皮肤科收治的101例pSS患者,根据其是否伴皮肤紫癜分为紫癜组（n=15）和非紫癜组（n=86）,比较2组患者的临床资料和实验室指标。结果:101例pSS患者中有15例（14.9%）伴有皮肤紫癜,其中12例为高球蛋白血症紫癜,其余3例（20%）分别为冷球蛋白血症紫癜、血小板减少性紫癜和色素性紫癜性皮病。4例pSS患者在出现口干、眼干等典型的腺体症状之前即出现皮肤紫癜,另外11例患者在腺体症状出现之后出现皮肤紫癜。与非紫癜组相比,紫癜组患者的发病年龄早[（41.6±13.5）岁比（51.7±11.4）岁, P=0.003],淋巴瘤发生率高（2/15比0, P=0.011）。紫癜组患者外周血中的类风湿因子（P=0.002）、免疫球蛋白（immunoglobulin,Ig）G（P<0.001）、丙种球蛋白（P=0.001）、Ig轻链κ（P<0.001）和Ig轻链λ（P=0.015）水平及红细胞沉降率（P=0.019）均显著高于非紫癜组,而其补体C₃（P=0.011）、C₄（P=0.021）水平则低于非紫癜组。结论:本研究中紫癜是pSS患者最常见的皮肤表现,且不同个体间具有较大的异质性。对于因皮肤紫癜就诊的患者,皮肤科医师应考虑到pSS的可能。伴皮肤紫癜的pSS患者发病早,反映疾病活动度的实验室指标升高明显,淋巴瘤发生率高,需对此类患者进行更密切的随访。

关键词: 原发性干燥综合征, 皮肤表现, 紫癜, 淋巴瘤

Abstract:

Objective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The patients were divided into 2 groups: pSS with purpura (purpura group, n=15) and without purpura (non-purpura group, n=86). The clinical presentations and laboratory data were compared between the 2 groups. Results: Of the 15 patients with manifestation of purpura (14.9%), 12(80%) were diagnosed as hypergammaglobulinemia purpura and the other 3(20%) were cryoglobulinemia purpura, thrombocytopenic purpura, and pigmented purpuric dermatosis, respectively. Four cases developed purpura prior to the glandular symptoms of dry mouth and eyes, and 11 cases had purpura after presentations of glandular symptoms. Com-paring with the non-purpura group, the pSS patients with purpura group had earlier onset age [(41.6±13.5) years vs. (51.7±11.4) years, P=0.003], higher incidence of lymphoma (2/15 vs. 0, P=0.011); higher serum levels of rheumatoid factor (RF) (P=0.002), erythrocyte sedimentation rate (ESR) (P=0.019), immunoglobulin (Ig) G (P<0.001), γ-globulin (P=0.001), free Ig light (κ) chains (P<0.001), free Ig heavy (λ) chains (P=0.015), and lower levels of compliment components C₃ (P=0.011) and C₄ (P=0.021). Conclusions: As a common and heterogeneous dermatological manifestation of pSS patients revealed in this study, the diagnosis of pSS should be considered in patients presenting purpura. The pSS patients with purpura with early onset and abnormal laboratory parameters have increased risk of lymphoma and demanded further monitoring.

Key words: Primary Sjögren syndrome, Cutaneous manifestation, Purpura, Lymphoma

中图分类号:

R541.7

赵茜, 赵肖庆, 刁立诚, 孙菲, 郑捷, 朱雪梅, 曹华. 伴皮肤紫癜的原发性干燥综合征患者的临床特征分析[J]. 诊断学理论与实践, 2021, 20(02): 155-160.

ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua. Clinical features of primary Sjögren syndrome with purpura[J]. Journal of Diagnostics Concepts & Practice, 2021, 20(02): 155-160.

图/表 5

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伴皮肤紫癜的pSS患者的临床特征

编号	年龄（岁）	性别	发病年龄（岁）	病程（年）	首发症状	腺体症状	抗SSA 抗体	抗SSB 抗体	抗核抗体	唇腺活检	皮肤活检	紫癜类型	皮损分布	系统症状
1	58	女	50	8	风团	无	+	+	+	+	真皮小血管周围少量淋巴细胞浸润,少量红细胞外渗	高球蛋白血症紫癜	下肢	肺间质病变、淋巴瘤
2	39	女	34	5	口干、眼干	口干、眼干	+	-	+	/	/	高球蛋白血症紫癜	下肢	关节痛、肌痛、肾小管性酸中毒
3	33	女	25	8	紫癜	口干、眼干	+	+	+	+	/	高球蛋白血症紫癜	下肢	无
4	66	女	58	8	紫癜	无	-	-	-	+	/	高球蛋白血症紫癜	下肢	无
5	49	女	43	6	乏力	口干、眼干	+	-	+	+	/	高球蛋白血症紫癜	下肢	乏力
6	35	女	28	7	口干	口干	+	+	+	/	/	高球蛋白血症紫癜	下肢	无
7	66	女	57	9	血小板减少	口干、眼干	+	-	+	/	/	血小板减少性紫癜	全身	血细胞减少
8	17	女	12	5	紫癜	无	+	+	+	+	真皮部分小血管周围淋巴细胞及个别中性粒细胞浸润	高球蛋白血症紫癜	下肢	无
9	48	女	41	7	口干、眼干	口干、眼干	,	-	+	+	/	高球蛋白血症紫癜	下肢	肺间质病变
10	43	女	38	5	口干	口干	+	+	+	+	真皮浅层小血管周围少量淋巴细胞浸润	高球蛋白血症紫癜	下肢	无
11	47	女	40	7	口干、紫癜	口干、眼干	+	+	+	-	/	高球蛋白血症紫癜	下肢	血细胞减少
12	44	女	41	3	口干、眼干、乏力	口干、眼干	-	-	-	+	/	高球蛋白血症紫癜	下肢	乏力
13	51	女	41	10	口干、眼干	口干、眼干	+	+	+	/	/	高球蛋白血症紫癜	下肢	关节痛、肺间质病变
14	70	女	62	8	关节痛	无	-	-	-	+	/	色素性紫癜性皮病	下肢	关节痛、肌痛
15	60	女	54	6	口干	口干	-	-	-	+	白细胞破碎性血管炎	冷球蛋白血症紫癜	四肢	关节痛、淋巴瘤

表2

表3

参考文献 17

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参数	紫癜组(n=15)	非紫癜组(n=86)	P值
发病年龄（岁）	41.6±13.5	51.7±11.4	0.003
病程（年）	7.0(5.0~8.0)	4.0(1.0~8.0)	0.050
女性[例(%)]	15(100%)	81(94.2%)	1.000

组别	紫癜组(n=15)	非紫癜组(n=86)	P值
RF (U/mL)	344.0(27.5~396.8)	10.0(10.0~40.5)	0.002
C反应蛋白(mg/dL)	0.34(0.21~0.66)	0.28(0.17~0.89)	0.610
红细胞沉降率(mm/h)	44.5(13.0~87.0)	15.0(10.0~34.0)	0.019
IgG (mg/dL)	2 500.8±816.4	1 549.3±640.1	<0.001
IgA (mg/dL)	330.1±170.0	291.0±123.3	0.308
IgM (mg/dL)	80.0(62.0~143.3)	122.0(72.5~167.0)	0.128
血清丙种球蛋白(%)	34.4(28.9~39.9)	22.5(18.3~25.7)	0.001
补体C₃ (mg/dL)	67.6±16.5	85.0±22.3	0.011
补体C₄ (mg/dL)	11.5(8.5~20.5)	18.0(14.5~23.0)	0.021
铁蛋白 (μg/L)	111.1±98.2	237.2±295.9	0.270
Ig轻链κ (g/L)	19.4±5.6	12.3±5.5	<0.001
Ig轻链λ (g/L)	11.1±4.7	6.7±2.3	0.015
抗SSA抗体阳性(%)	66.7	53.7	0.351
抗SSB抗体阳性(%)	46.7	39.0	0.579
抗核抗体阳性(%)	73.3	69.5	1.000
唇腺活检阳性(%)	88.9	94.5	0.463