系统性红斑狼疮合并弥漫性肺泡出血13例分析

Systemic lupus erythematosus with diffuse alveolar hemorrhage: analysis of 13 cases

目的探讨系统性红斑狼疮(SLE)合并弥漫性肺泡出血(DAH)患者的临床特征及治疗经验。方法回顾性分析空军军医大学第二附属医院2015年7月至2021年1月,SLE合并DAH的住院患者13例的临床资料、实验室检查、影像学资料、治疗及转归等数据。结果13例患者中男性1例,女性12例,入院年龄(52.4±13.0)岁,SLE发病年龄(32.0±10.0)岁,病程(5.9±5.8)年。患者入院SLE疾病活动度评分(SLEDAI)为(10.3±1.2)分。其突出临床表现是呼吸困难(92.3%)、发热(92.3%)和咯血(100.0%)。13例患者均表现为肾脏受累,9例出现血管炎。13例均接受大剂量肾上腺糖皮质激素治疗,部分患者接受血浆置换和持续静脉血液透析联合治疗。住院期间死亡或放弃治疗及出院1周内死亡者统计为死亡;存活患者均出院后每月门诊复诊,随访1年后仍存活定义为存活。最终存活5例(38.5%),死亡8例(61.5%),存活组和死亡组之间血浆置换率比较差异有统计学意义(100.0%vs 12.5%,P<0.01)。结论SLE合并DAH的患者常伴有肾脏损害、呼吸困难、咯血、血管炎及低补体、低白蛋白及高SLEDAI评分的特征,且病死率高,积极的激素冲击和血浆置换可取得较好效果。

Objective To investigate the clinical characteristics and treatment of systemic lupus erythematosus(SLE) complicated with diffuse alveolar hemorrhage(DAH). Methods A retrospective analysis was performed on the clinical data, laboratory examination, imaging features, treatment and outcome of 13 inpatients with SLE complicated with DAH in the Second Affiliated Hospital of Air Force Military Medical University from July 2015 to January 2021. Results Among 1 male patient and 12 female patients, the average age for admission was(52.4±13.0) years old, the average onset age of SLE was(32.0±10.0) years old, and the average duration of disease was(5.9±5.8) years. In all patients, mean SLE disease activity index(SLEDAI) was 10.3±1.2, and the prominent clinical manifestations were dyspnea(92.3%), fever(92.3%), hemoptysis(100.0%) and renal involvement(100.0%). All patients were treated with high-dose adrenal glucocorticoid, and some patients received plasma exchange and continuous intravenous hemodialysis. Five patients(38.5%) survived during 1 year follow-up, and 8 patients(61.5%) died and giving up treatment during hospitalization or within 1 week after discharge. There was a significant difference in therapeutic plasma exchange between survival group and death group(100.0% vs 12.5%, P<0.01). Conclusion SLE-DAH patients are often accompanied by renal damage, dyspnea, hemoptysis, vasculitis, low complement levels, low albumin levels, high SLEDAI score and high mortality. High-dose glucocorticoid pulse therapy and plasma exchange can get good results for the patients.