Clinical features of primary Sjögren syndrome with purpura

doi:10.16150/j.1671-2870.2021.02.007

Abstract

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

CLC Number:

R541.7

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.

Figures/Tables 5

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

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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