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原发性干燥综合征相关继发性免疫性血小板减少症的临床特征

Clinical Characteristics of Secondary Immune Thrombocytopenia Associated With Primary Sjögren's Syndrome.

作者信息

Dai Fan, Yang Guomei, Rao Peishi, Wu Puqi, Chen Rongjuan, Sun Yuechi, Peng Yun, Qian Hongyan, Wang Bin, Chen Shiju, Liu Yuan, Shi Guixiu

机构信息

Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, China.

School of Medicine, Xiamen University, Xiamen, China.

出版信息

Front Med (Lausanne). 2020 Apr 17;7:138. doi: 10.3389/fmed.2020.00138. eCollection 2020.

Abstract

Clinical characteristics of immune thrombocytopenia (ITP) associated with primary Sjögren's syndrome (pSS) have not been clearly defined. This study aimed to evaluate the prevalence and clinical characteristics of secondary ITP in patients with pSS. 291 pSS patients in our hospital were retrospectively analyzed. Clinical manifestations and laboratory findings were compared between pSS patients with and without ITP. The prevalence of secondary ITP in pSS patients was 12.03%. Compared to pSS patients without ITP, pSS patients with ITP were younger and had higher disease activity. The prevalence of interstitial lung diseases (ILD) was significantly lower in pSS patients with ITP (30.43 vs. 54.95%; = 0.029), and it was the same with arthritis (17.14 vs. 3.9.11%; = 0.014) and dry eye (33.33 vs. 54.17%, = 0.027) compared with those without ITP. Serum creatinine level was lower in pSS patients with ITP ( = 0.009), while positivity of anti-histone autoantibodies was higher in pSS patients with ITP ( = 0.025). This study is an initial report describing clinical features of ITP in pSS. The lower incidence of ILD and arthritis among pSS patients with ITP indicated potential active roles of platelets in the pathogenesis of fibrosis or inflammatory arthritis, which may open the way for further experimental and clinical work.

摘要

原发性干燥综合征(pSS)相关免疫性血小板减少症(ITP)的临床特征尚未明确界定。本研究旨在评估pSS患者继发性ITP的患病率及临床特征。对我院291例pSS患者进行回顾性分析。比较了合并ITP和未合并ITP的pSS患者的临床表现及实验室检查结果。pSS患者继发性ITP的患病率为12.03%。与未合并ITP的pSS患者相比,合并ITP的pSS患者更年轻,疾病活动度更高。合并ITP的pSS患者间质性肺疾病(ILD)的患病率显著更低(30.43%对54.95%;P = 0.029),与未合并ITP的患者相比,关节炎(17.14%对39.11%;P = 0.014)和干眼症(33.33%对54.17%,P = 0.027)的患病率情况相同。合并ITP的pSS患者血清肌酐水平更低(P = 0.009),而抗组蛋白自身抗体阳性率在合并ITP的pSS患者中更高(P = 0.025)。本研究是描述pSS中ITP临床特征的初步报告。合并ITP的pSS患者中ILD和关节炎发病率较低,提示血小板在纤维化或炎性关节炎发病机制中可能发挥积极作用,这可能为进一步的实验和临床工作开辟道路。

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