收稿日期: 2021-04-07
网络出版日期: 2022-06-28
基金资助
国家自然科学基金面上项目(81573037);国家自然科学基金面上项目(81872523);国家自然科学基金面上项目(82073432);国家临床重点专科建设项目(2012649);上海市科委医学引导类项目(134119a6100);上海申康医院发展中心临床创新三年行动计划(16CR3084B);上海交通大学医学院高峰学科—临床医学研究型医师(20172009)
Clinical features of primary Sjögren syndrome with purpura
Received date: 2021-04-07
Online published: 2022-06-28
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。
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 . DOI: 10.16150/j.1671-2870.2021.02.007
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
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