系统性红斑狼疮继发难治性血小板减少的临床特征分析

Clinical characteristics of refractory thrombocytopenia secondary to systemic lupus erythematosus

目的 分析SLE患者合并难治性血小板减少(RLTP)的临床特征及其影响因素.方法 回顾性分析2015年1月至2018年6月经苏州大学附属第一医院风湿免疫科门诊和住院部诊断为SLE合并血小板减少的患者113例,收集患者的病史及实验室检查,分为难治组(RLTP组,25例)和非难治性组(NRLTP,88例),采用t检验、Mann-Whitney检验及χ^2检验方法比较分析2组临床表现、血常规、生化及免疫学等指标,进一步采用非条件Logistic回归分析影响RLTP患者预后的因素以及Kaplan-Meier生存曲线分析RLTP患者的累积生存率.结果 与NRLTP患者比较,RLTP患者的病程更长[72(30,120)个月与38.5(8.5,93)个月,H=-2.401,P=0.016)],神经系统损害(28%与7%,χ^2=8.58,P=0.016)比例更高、出血风险更高[(4.6±1.7)与(3.8±1.3),t=2.548,P=0.012]及病死率更高(8%与0,χ^2=7.167,P<0.01);同时RLTP组抗GP Ⅰ b/Ⅸ阳性率显著高于NRLTP组(27%与4%,χ^2=8.647,P<0.01);采用非条件多因素Logistic回归分析显示,抗GP Ⅰ b/Ⅸ阳性是RLTP的主要影响因素之一.采用Kaplan-Meier生存曲线分析结果显示RLTP组的累积生存率较NLTP组明显降低(χ^2=7.909,P<0.01).结论 RLTP病程长、易合并神经系统损害及抗GPIb/IX阳性率高并且出血风险高及预后差,应早期识别该类患者,调整治疗策略,改善患者预后.

Objective To explore the clinical characteristics and influencing factors of refractory lupus thrombocytopenia(RLTP)secondary to systemic lupus erythematosus(SLE).Methods A retrospective analysis of 113 patients with thrombocytopenia secondary to SLE in the outpatient and inpatient Department of Rheumatology of the First Affiliated Hospital of Soochow University from January 2015 to June 2018 was carried out.The medical record and laboratory tests of patients were collected,and they were divided them into the refractory group(RLTP,n=25)and non-refractory group(NRLTP,n=88).The clinical manifestations,blood count,biochemical and immunological test of the two groups were analyzed and compared.All data were analyzed by t-test,Mann-Whitney test,χ2 test,Logistic regression analysis and Kaplan-Meier survival analysis.Results Compared with NRLTP patients,RLTP patients had longer disease course[72(30,120)months vs 38.5(8.5,93)months,H=-2.401,P=0.016),nervous system damage(28%vs 7%,χ2=8.58,P=0.016),higher bleeding risk[(4.6±1.7)vs(3.8±1.3),t=2.548,P=0.012]and higher mortality rate(8%vs 0,χ2=7.167,P<0.01).Meanwhile,the positive rate of anti-GPⅠb/Ⅸin RLTP group was significantly higher than that in NRLTP group(27%vs 4%,χ2=8.647,P<0.01).Further unconditional multivariate logistic regression analysis showed that anti-GPⅠb/Ⅸpositive was one of the main influencing factors of RLTP.Kaplan-Meier survival curve analysis revealed that the cumulative survival rate of RLTP group was significantly lower than that of NLTP group(χ2=7.909,P<0.01).Conclusion RLTP has a long course of disease,prone to nervous system impairment and positive anti-GPⅠb/Ⅸantibody,and has a high risk of bleeding.It is necessary to identify these patients early,adjust treatment strategies and improve the prognosis of patients.