系统性红斑狼疮合并弥漫性肺泡出血34例临床分析  

Clinical analysis of 34 cases with diffuse alveolar hemorrhage in systemic lupus erythematosus

作　　者：黄子宸 王荷郡 赵铖[1] 郑乐婷[1] 文静[1] 陈战瑞[1] 雷玲[1] 

机构地区：[1]广西医科大学第一附属医院风湿免疫科,南宁530021 [2]河南中医药大学第一附属医院,郑州450003

出　　处：《实用医学杂志》2018年第1期100-102,106,共4页The Journal of Practical Medicine

基　　金：广西医科大学青年科学基金项目(编号:GXMUYSF201306);广西教育厅课题(自筹经费)项目(编号:201106LX112)

摘　　要：目的总结系统性红斑狼疮(SLE)合并弥漫性肺泡出血(DAH)的临床特点和治疗效果。方法回顾分析广西医科大学第一附属医院2006年1-12月确诊的34例SLE并发DAH患者的临床特点、诊治经过和转归。结果 SLE合并DAH发病率为0.39%,年龄19(IQR 11.75~32)岁,病程6(2~15.75)个月,主要表现为咳嗽、呼吸困难及发热。SLE疾病活动指数(SLEDAI)评分为19.5(16~25)分;抗双链DNA抗体定量24.35(2.89~129.25);补体C3 0.38(0.26~0.84)g/L。预后:总体病死率73.5%,经风湿免疫科治疗52.9%。影响病死率的治疗有CTX(OR=0.059,95%CI 0.006~0.554,P=0.013)及免疫抑制剂联合大于常规剂量激素治疗(OR=0.059,95%CI 0.006~0.554,P=0.013)。结论 SLE合并DAH症状常不典型,表现咳嗽、呼吸困难及发热等,影像学及支气管镜检查可以辅助诊断,容易发生肺部感染,病死率高;早期诊断及应用CTX联合大于常规剂量激素治疗原发病可降低病死率。Objective To identify the clinical characteristics and therapeutic effect of systemic lupus ery- thematosus （SLE） patients with diffuse alveolar hemorrhage （DAH）. Methods Clinical characteristcs, diagnosis, treatment and outcome in 34 patients hospitalized in The First Affiliated Hospital of Guangxi Medical University from January 2006 to December 2016 were retrospectively analyzed. Results The incidence of DAH involvement of SLE was about 0.39 %. Median age was 19 （interquartile range （IQR） 11.75 - 32） years. The duration of SLE before clinical DAH was 6 months （IQR 2 - 15.75 ） months. The main clinical manifestations of DAH were cough, dyspnea and fever. The SLE disease activity index （SLEDAI） score was 19.5 （16 - 25 ）points and anti-dsDNA antibody titer elevated markedly （ 38.2% ）. The overall mortality rate was 73.5% , however patients who chose department of rheumatism have lower mortality （52.9%）. And treatment of CTX was associated with mortality （ OR = 0.059, 95% CI 0.006 - 0.554, P = 0.013 ） , as well as steroids and immunosuppressant combination therapy. Conclusions The clinical symptoms of SLE with DAH is often atypical. There is manifestation of cough, fever, dyspnea and etc. Imaging and broncoseopy can assist the diagnosis and its prone to the pulmonary infection and high mortality.Early diagnosis and application of CTX combined with conventional dose of hormone theraphy can in early diagnosis and reduce the mortality.

关 键 词：系统性红斑狼疮 弥漫性肺泡出血 

分 类 号：R563[医药卫生—呼吸系统] R593.241[医药卫生—内科学]