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≥80×109/L,19例)和治疗反应差组(血小板计数＜80×109/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、CA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)×109/L比(148 ±58)×109/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)×109/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)×109/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.Objective To analyze clinical characteristics,therapeutic response and outcomes of systemic lupus erythematosus (SLE) complicated with severe thrombocytopenia.Methods Twenty-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 × 109/L,19 cases) and poor therapeutic response group (platelet count ＜ 80 × 109/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.Results Platelet 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) × 109/L vs(148 ±58) × 109/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) × 109/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) × 109/L.Conclusions Severe 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.国家自然科学基金(81373196)National Natural Science Foundation of China4546-5491