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原发性干燥综合征合并轻至重度血小板减少症的临床及实验室特征

Clinical and laboratory features of primary Sjögren's syndrome complicated with mild to severe thrombocytopenia.

作者信息

Wu Jian, Chang Xin, Zhang Jian, Liu Cuiping, Liu Mingxing, Chen Weichang

机构信息

Department of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou, China.

Jiangsu Institute of Clinical Immunology & Jiangsu Key Laboratory of Clinical Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China.

出版信息

Ann Transl Med. 2022 Mar;10(6):300. doi: 10.21037/atm-22-162.

Abstract

BACKGROUND

Patients with thrombocytopenia accompanied by positive Ro/SS-A and/or La/SS-B autoantibodies have a possible diagnosis of Sjögren's syndrome (SS). Owing to its low prevalence, large-sample controlled studies on thrombocytopenia in primary SS (pSS) are scarce. Thus, this study aimed to investigate the clinical and laboratory characteristics of pSS complicated with mild to severe thrombocytopenia, and compared them with pSS patients without thrombocytopenia.

METHODS

This medical records review study analyzed the demographic data, clinical manifestations, laboratory examinations, and other results of 88 patients diagnosed with pSS between March 2007 and March 2018 in the Department of Rheumatology of The First Affiliated Hospital of Soochow University. A platelet (PLT) count of peripheral blood below 50×10/L (≤50×10/L) was regarded as mild to severe thrombocytopenia.

RESULTS

Of the 88 pSS patients, 43 developed mild to severe thrombocytopenia (thrombocytopenia group) and 45 had no thrombocytopenia (control group). No significant difference was found in the levels of autoantibodies and inflammatory markers between the thrombocytopenia group and the control group. Dry mouth (P<0.01) and dry eyes (P<0.01) were not frequently observed in the thrombocytopenia group, but the level of complement C4 dropped significantly (P<0.05). In contrast, the control group was more likely to have leukopenia (P=0.01) and interstitial lung disease (P<0.01).

CONCLUSIONS

In pSS patients with mild to severe thrombocytopenia, the incidence of xerostomia, xerophthalmia, and lung involvement was markedly reduced. Knowledge about the features of pSS associated with thrombocytopenia will lead to earlier and better diagnosis and treatment.

摘要

背景

血小板减少症患者伴有Ro/SS - A和/或La/SS - B自身抗体阳性,可能诊断为干燥综合征(SS)。由于其患病率较低,关于原发性干燥综合征(pSS)中血小板减少症的大样本对照研究较少。因此,本研究旨在调查合并轻至重度血小板减少症的pSS患者的临床和实验室特征,并将其与无血小板减少症的pSS患者进行比较。

方法

本病历回顾性研究分析了2007年3月至2018年3月期间在苏州大学附属第一医院风湿科确诊为pSS的88例患者的人口统计学数据、临床表现、实验室检查及其他结果。外周血血小板(PLT)计数低于50×10⁹/L(≤50×10⁹/L)被视为轻至重度血小板减少症。

结果

88例pSS患者中,43例发生轻至重度血小板减少症(血小板减少症组),45例无血小板减少症(对照组)。血小板减少症组与对照组之间自身抗体和炎症标志物水平无显著差异。血小板减少症组口干(P<0.01)和干眼(P<0.01)的发生率较低,但补体C4水平显著下降(P<0.05)。相比之下,对照组更易出现白细胞减少(P = 0.01)和间质性肺病(P<0.01)。

结论

在合并轻至重度血小板减少症的pSS患者中,口干、干眼和肺部受累的发生率明显降低。了解与血小板减少症相关的pSS特征将有助于早期更好地诊断和治疗。

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