系统性红斑狼疮合并重度血小板减少的治疗反应与临床转归

Therapeutic response and outcomes of systemic lupus erythematosus complicated with severe thrombocytopenia

目的分析系统性红斑狼疮（SLE）合并重度血小板减少患者的临床特征、治疗反应及临床转归。方法回顾性分析2009年1月至2015年4月在北京大学第一医院风湿免疫科住院治疗的29例SLE合并重度血小板减少患者的临床资料。均采用大剂量糖皮质激素、免疫抑制剂等治疗物。依据治疗后的血小板计数将患者分为治疗反应好组（血小板计数≥80×10^9/L，19例）和治疗反应差组（血小板计数＜80×10^9/L，10例）。记录患者的临床表现、实验室检查资料、治疗反应以及随访结果。采用SLE疾病活动指数（SLEDAI）评价疾病活动度。结果治疗前2组血小板计数、血红蛋白、白细胞计数、抗双链DNA抗体滴度、抗nRNP抗体阳性、抗rRNP抗体阳性、抗SSA抗体阳性、抗SSB抗体阳性、抗心磷脂抗体阳性的患者比例以及补体C3、C4水平比较，差异均无统计学意义（均P＞0.05）。治疗后，治疗反应差组的血小板计数明显低于治疗反应好组［（27±13）×10^9/L比（148±58）×10^9/L］（P＜0.01）。2组出血发生率比较差异无统计学意义（P＞0.05），均以皮肤及黏膜出血为主。2组之间SLEDAI评分比较差异无统计学意义（P＞0.05）。住院期间治疗反应差组3例（30.0%）患者死亡，均出现严重感染；失访3例，余4例随访（10±9）个月，末次随访血小板计数为（145±124）×10^9/L，其中2例血小板计数正常。治疗反应好组失访5例（26.3%），余14例平均随访时间为（24±19）个月，末次随访血小板计数为（193±53）×10^9/L。结论SLE合并重度血小板减少患者严重出血并不多见，大部分患者对大剂量糖皮质激素治疗反应好，长期预后良好；治疗反应差的患者主要死亡原因为感染。

ObjectiveTo analyze clinical characteristics, therapeutic response and outcomes of systemic lupus erythematosus（SLE） complicated with severe thrombocytopenia. MethodsTwenty-nine SLE patients with severe thrombocytopenia in Peking University First Hospital from January 2009 to April 2015 were retrospectively analyzed. The patients were divided into good therapeutic response group（platelet count≥80×10^9/L, 19 cases） and poor therapeutic response group（platelet count〈80×10^9/L, 10 cases） after treament with consequence of glucocorticoid and immunosuppressive agents. Clinical characteristics, laboratory indexes, disease activity[SLE Disease Activity Index（SLEDAI）] and outcomes were recorded. ResultsPlatelet count, hemoglobin level, white blood cell count, anti-dsDNA antibody titer, anti-nRNP antibody positive rate, anti-rRNP antibody positive rate, anti-SSA antibody positive rate, anti-SSB antibody positive rate, anticardiolipin antibody positive rate and C3, C4 levels had no significant differences between groups before treatment（P〉0.05）. Platete count in poor therapeutic response group was significantly lower than that in good therapeutic response group after treatment[（27±13）×10^9/L vs（148±58）×10^9/L]（P〈0.01）. The incidence of bleeding had no significant difference between groups（P〉0.05）. The SLEDAI score had no significant difference between groups（P〉0.05）. There were 3 death（30.0%） caused by severe infection and 3 loss of follow-up（30.0%） in poor therapeutic response group; the mean follow-up time of 4 cases was （10±9） months; the mean platelet count in final follow-up was （145±124）×10^9/L, with 2 cases of normal platelet count. There were 5 loss of follow-up（26.3%） in good therapeutic response group; the mean follow-up time of 14 cases was （24±19） months; the mean platelet count in final follow-up was （193±53）×10^9/L. ConclusionssSevere hemorrhage is not common in SLE complicated with severe thrombocytopenia. Long-term therapeutic effect of glucocorticoid is good. The major cause of death is infection.