系统性红斑狼疮合并弥漫性肺泡出血：单中心54例临床回顾性分析

发布时间：2018-08-19 17:42

【摘要】：背景：弥漫性肺泡出血(DAH)是系统性红斑狼疮(SLE)肺部病变中相对罕见,而进展迅猛、死亡率较高的临床表型,是导致SLE患者死亡的重要原因之一,目前临床尚无切实有效的诊治规范。目的：分析SLE-DAH患者的临床特点、危险因素、预后相关因素,为临床诊治提供依据。方法：回顾北京协和医院自2004年1月至2015年1月间诊断为SLE-DAH的住院患者,同时满足以下标准即入组：(1)符合1997年ACR或2009年SLICC的SLE分类标准；(2)同时符合以下4条标准中的3条及以上：①肺部症状；②新发的肺部浸润影；③HGB下降至少15g/L；④支气管肺泡灌洗液呈血性或可见到含铁血黄色细胞；并可除外严重凝血系统疾病、严重的急性肺水肿、严重的肺栓塞等。同时以208例同期住院的SLE非DAH的患者作为对照进行病例对照研究。收集全部患者的临床表现、实验室检查、影像学表现的特征,采用SPSS 20.0采集和分析数据。连续变量比较采用独立样本t检验(其中符合方差齐性者直接用t检验,不符合者用近似t检验,不符合正态分布者用秩和检验),分类变量资料采用x2检验。P值0.05被认为有统计学意义。结果：(1)共纳入SLE-DAH患者54例,DAH在SLE患者中患病率为0.73%。其中女性49例(91%),平均年龄为(30.8±12.4)岁,平均病程为(16.9±33.6)月,1/3患者以DAH表现首发。平均SLEDAI评分为(19±7.9)；(2)SLE-DAH患者最常见的症状是呼吸困难(91%),其余依次为低氧血症(89%)、咳嗽(87%)发热(43例,80%),26%患者临床无咯血；血红蛋白平均下降(36.1±14.3)g/L,24小时内平均下降(13.4±10.8)g/L。最常见的肺外受累为肾脏受累(91%)；(3)54例SLE-DAH患者中53例接受了糖皮质激素治疗,37例(69%)接受了甲基泼尼松龙冲击治疗；(4) SLE-DAH患者院内病死率为20.4%；与院内存活组相比,死亡组患者在发病年龄、病程上存在统计学差异,同时合并感染、机械通气的比例更高,或与不良预后相关； (5)与SLE-NDAH患者相比,两组患者在病程、SLEDAI评分、院内病死率(20% vs 7%)上存在统计学差异,且SLE-DAH患者出现皮肤黏膜病变(70.4% vs 49.0%)、血液系统病变(87% vs 43.8%)、血小板减少(72.2% vs 25.5%)、肾脏病变(90.7% vs 62.9%)更为多见,抗ds-DNA抗体阳性率更高(55.6%vs 42.6%),以上均存在统计学差异(P0.05)；(6)随着临床认识的增加,SLE-DAH死亡率在下降(50% vs 10%,P0.05)结论：DAH是SLE的的严重并发症,临床表现可无咯血表现,应重视支气管镜检查的诊断价值。积极的糖皮质激素、免疫抑制剂可改善预后。发病年龄、病程合并感染、应用机械通气与不良预后相关。提高临床认识,可改善SLE-DAH的预后。
[Abstract]:Background: diffuse alveolar hemorrhage (DAH) is a relatively rare pulmonary lesion in systemic lupus erythematosus (SLE), which is characterized by rapid progression and high mortality, which is one of the important causes of death in patients with SLE. At present, there is no practical and effective diagnosis and treatment standard. Objective: to analyze the clinical features, risk factors and prognostic factors of SLE-DAH patients. Methods: the hospitalized patients diagnosed with SLE-DAH from January 2004 to January 2015 in Peking Union Hospital were reviewed and the following criteria were met: (1) according to the SLE classification criteria of ACR in 1997 or SLICC in 2009; (2) at the same time, 3 or more of the following 4 criteria were conformed to one or more of the following criteria. The newly developed lung infiltrating 3HGB decreased by at least 15 g / L 4 bronchoalveolar lavage fluid (BALF) with blood or iron-containing yellow cells. And may exclude the serious coagulation system disease, the serious acute pulmonary edema, the serious pulmonary embolism and so on. A case-control study was conducted with 208 non-DAH SLE patients hospitalized at the same time. The clinical, laboratory and imaging features of all patients were collected and analyzed by SPSS 20.0. The independent sample t test was used to compare the continuous variables (where the homogeneity of variance was used directly by t test, and the non-conformance was tested by approximate t test). Rank sum test was used for those who did not conform to normal distribution). The data of classified variables were analyzed by x2 test. P value 0.05 was considered to be statistically significant. Results: (1) the prevalence of SLE-DAH in 54 patients with SLE was 0.73. The mean age was (30.8 卤12.4) years old and the mean course of disease was (16.9 卤33.6) months / 1 / 3 of DAH in 49 women (91%). The most common symptoms in patients with SLE-DAH were dyspnea (91%), hypoxiaemia (89%), cough (87%), fever (43 cases, 80%) and hemoglobin (36.1 卤14.3) g / L in 24 hours (13.4 卤10.8) g / L, respectively. The average SLEDAI score was (19 卤7.9); (2. The most common symptoms were dyspnea (91%), followed by hypoxiaemia (89%), cough (87%), fever (43 cases 80%) and no hemoptysis (26%). The most common extrapulmonary involvement was kidney involvement (91%); (3), 53 of 54 SLE-DAH patients received glucocorticoid therapy, 37 (69%) received methylprednisolone shock therapy, (4) the nosocomial mortality of SLE-DAH patients was 20. 4%. In the death group, there were statistical differences in age and course of disease, and the incidence of infection and mechanical ventilation were higher, or associated with poor prognosis. (5) compared with SLE-NDAH patients, the two groups had a SLEDAI score in the course of the disease. There were significant differences in hospital mortality (20% vs 7%), and there were skin and mucosal lesions (70.4% vs 49.0%), hematological diseases (87% vs 43.8%), thrombocytopenia (72.2% vs 25.5%) and renal lesions (90.7% vs 62.9%) in patients with SLE-DAH. The positive rate of anti ds-DNA antibody was higher (55.6%vs 42.6%), and there was statistical difference (P0.05); (6). With the increase of clinical knowledge, the mortality of SLE-DAH decreased (50% vs 10%, P0.05). Conclusion the positive rate of ds-DNA is a serious complication of SLE, and no hemoptysis is found in the clinical manifestation. Attention should be paid to the diagnostic value of bronchoscopy. Active glucocorticoids, immunosuppressants can improve prognosis. Age of onset, course of disease with infection, mechanical ventilation and poor prognosis were related. Improving clinical knowledge can improve the prognosis of SLE-DAH.
【学位授予单位】：北京协和医学院
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R593.241

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