原发性干燥综合征重度血小板减少患者的临床及免疫学特点分析

王英.原发性干燥综合征重度血小板减少患者的临床及免疫学特点分析[J].内科急危重症杂志,2022,28(2):103-107

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DOI：10.11768/nkjwzzzz20220204

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基金项目:国家自然科学基金资助项目(No:82171779)

作者	单位	E-mail
王英	厦门大学附属第一医院	gshi@xmu.edu.cn

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中文摘要:

目的：探讨原发性干燥综合征 (pSS)伴重度血小板减少患者的临床及免疫学特点、疾病活动度情况及其相关性。方法：回顾性分析 2014年1月至2020年1月厦门大学附属第一医院风湿免疫科住院的281例pSS患者的临床表现、实验室检查、疾病活动度及转归。pSS合并血小板减少48例，其中26例为重度血小板减少（PLT＜30 ×109/L），血小板正常的pSS 患者233例，比较并分析重度血小板减少与血小板正常pSS患者的临床资料。结果：重度血小板减少发生率9.3%。重度血小板减少组贫血、皮疹、脾大、高球蛋白血症的发生率明显高于血小板正常组，抗SSA抗体、抗ACA抗体、抗AHA抗体阳性率明显高于血小板正常组（P均＜0.05），疾病活动度亦明显增高（P＜0.05）, 且血小板减少程度与疾病活动度呈负相关，差异有统计学意义(r=-0.32，P=0.026)。结论：pSS伴重度血小板减少的发生率约9.3%,可能与抗SSA抗体和脾肿大等因素有关，其皮疹、高球蛋白血症阳性率、疾病活动度明显增高，且疾病活动度ESSDAI评分与血小板减少程度呈负相关。

英文摘要:

Objective: To investigate the clinical and immunological characteristics, disease activity and correlation of primary Sjgren syndrome (pSS) with severe thrombocytopenia. Methods: The clinical manifestations, laboratory examination, disease activity and outcome of 281 pSS patients hospitalized in Department of Rheumatology and Clinical Immunology, the First Affiliated Hospital of Xiamen University from January 2014 to January 2020 were retrospectively analyzed. A total of 48 patients were complicated with thrombocytopenia, including 26 cases of severe thrombocytopenia (platelet<30×109/L). The clinical data of 233 pSS patients without thrombocytopenia were compared and analyzed. Results: The incidence of severe thrombocytopenia was 9.3%. As compared with the non-thrombocytopenia group, the incidence of anemia, rash, splenomegasis and hyperglobulinemia in the severe thrombocytopenia group was significantly increased (P<0.05), and the positive rates of anti-SSA antibody, anti-ACA antibody and anti-AHA antibody were significantly higher than those in the normal platelet group (P<0.05). The level of disease activity was significantly increased (P<0.05), and negatively correlated with the degree of thrombocytopenia and the level of disease activity(r =-0.32, P=0.026). Conclusion: The incidence of pSS with severe thrombocytopenia was about 9.3%, which may be related to anti-SSA antibody and splenomegasis, etc. The rash, positive rate of hyperglobulinemia and disease activity were significantly increased, and EULAR Sjgren syndrome disease activity index score was negatively correlated with the degree of thrombocytopenia.