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中国系统性红斑狼疮研究协作组(CSTAR)登记研究:血小板减少症对中国系统性红斑狼疮患者的临床意义。

Chinese SLE Treatment and Research group (CSTAR) registry: Clinical significance of thrombocytopenia in Chinese patients with systemic lupus erythematosus.

机构信息

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Department of Rheumatology, Jiangsu Provincial People's Hospital, Nanjing, Jiangsu, China.

出版信息

PLoS One. 2019 Nov 20;14(11):e0225516. doi: 10.1371/journal.pone.0225516. eCollection 2019.

Abstract

OBJECTIVES

To investigate the prevalence, clinical characteristics, and prognosis of thrombocytopenia (TP) in Chinese patients with systemic lupus erythematosus (SLE).

METHODS

The study was conducted based on the Chinese SLE Treatment and Research group (CSTAR) registry. Thrombocytopenia was defined as the platelet count<100,000/mm3 at enrollment. Severe thrombocytopenia was defined as the platelet count<50,000/mm3. The prevalence of SLE-related TP, the associations of thrombocytopenia with demographic data, organ involvements, laboratory findings, disease activity, damage, and mortality were investigated.

RESULTS

Of 2104 patients with SLE, 342 patients (16.3%) were diagnosed with thrombocytopenia. The prevalence of neuropsychiatric SLE, vasculitis, myositis, nephritis, mucocutaneous lesions, pleuritis, fever, leukocytopenia and hypocomplementemia were significantly higher in patients with thrombocytopenia (p<0.05). SLE disease activity index (SLEDAI) was significantly higher in patients with thrombocytopenia (p<0.05). Multivariate analysis showed that leukocytopenia (OR = 2.644), lupus nephritis (OR = 1.539), hypocomplementemia (OR = 1.497) and elevated SLEDAI (OR = 1.318) were independently associated with thrombocytopenia (p<0.05). Long disease duration (OR = 1.006) was an independent risk factor of severe thrombocytopenia, while anti-rRNP (OR = 0.208) was an independent protective factor of severe thrombocytopenia (p<0.05). Long disease duration was an independent risk factor of mortality in patients with thrombocytopenia (RR = 1.006). The 6-year survival of patients with thrombocytopenia was significantly lower than patients without thrombocytopenia (88.2% vs. 95.5%).

CONCLUSIONS

Thrombocytopenia was a common manifestation of SLE and was associated with leukocytopenia, nephritis and severe disease activity. Severe thrombocytopenia tended to occur in long-term and relatively inactive SLE. Patients with SLE-related thrombocytopenia has a decreased long-term survival rate. Long disease duration was an independent risk factor of mortality in patients with thrombocytopenia.

摘要

目的

研究中国系统性红斑狼疮(SLE)患者血小板减少症(TP)的患病率、临床特征和预后。

方法

本研究基于中国系统性红斑狼疮研究协作组(CSTAR)的登记数据。血小板减少症定义为纳入时血小板计数<100,000/mm3。严重血小板减少症定义为血小板计数<50,000/mm3。研究调查了 SLE 相关 TP 的患病率,血小板减少症与人口统计学数据、器官受累、实验室检查、疾病活动度、损伤和死亡率的相关性。

结果

在 2104 例 SLE 患者中,342 例(16.3%)诊断为血小板减少症。血小板减少症患者的神经精神性 SLE、血管炎、肌炎、肾炎、黏膜皮肤病变、胸膜炎、发热、白细胞减少和低补体血症的患病率显著更高(p<0.05)。血小板减少症患者的 SLE 疾病活动指数(SLEDAI)显著更高(p<0.05)。多变量分析显示,白细胞减少症(OR=2.644)、狼疮肾炎(OR=1.539)、低补体血症(OR=1.497)和升高的 SLEDAI(OR=1.318)与血小板减少症独立相关(p<0.05)。疾病病程较长(OR=1.006)是严重血小板减少症的独立危险因素,而抗 rRNP(OR=0.208)是严重血小板减少症的独立保护因素(p<0.05)。疾病病程较长是血小板减少症患者死亡的独立危险因素(RR=1.006)。血小板减少症患者的 6 年生存率明显低于无血小板减少症患者(88.2% vs. 95.5%)。

结论

血小板减少症是 SLE 的常见表现,与白细胞减少症、肾炎和严重疾病活动度相关。严重血小板减少症倾向于发生在长期且相对不活跃的 SLE 中。SLE 相关血小板减少症患者的长期生存率降低。疾病病程较长是血小板减少症患者死亡的独立危险因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6634/6867648/118fbb003e23/pone.0225516.g001.jpg

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