SLE合并弥漫性肺泡出血3例临床分析并文献复习

Systemic lupus erythematosus complicated with diffuse alveolar hemorrhage: a report of three cases and literature review

目的探讨SLE合并弥漫性肺泡出血(DAH)的临床特点及治疗方法。方法对3例SLE-DAH患者的临床资料进行分析,并以"弥漫性肺泡出血""肺出血""系统性红斑狼疮"或"diffuse alveolar hemorrhage""pulmonary hemorrhage""systemic lupus erythematosus"为检索词,对2013年1月至2018年7月中国期刊全文数据库、万方数据知识服务平台、维普中文科技期刊数据库、PubMed收录的论文进行检索,收集并分析检索到的SLE-DAH患者临床资料。结果 3例均为女性患者,年龄分别为23、19、25岁,住院期间均有间断性干咳、发热、呼吸困难、肾损伤、血红蛋白下降、抗核抗体及抗双链DNA抗体阳性、低氧血症、低补体血症,其中2例有咯血、血小板下降、CRP升高、ESR升高、抗心磷脂抗体阳性,SLE病情活动指数分别为18、15、18分,在入院初期的影像学均表现为新发的弥漫性、大片状高密度浸润影,经大剂量糖皮质激素联合环磷酰胺及Ig冲击治疗后肺部阴影均明显吸收,随访2～3年均未见复发。文献检索到临床资料比较完整的SLE-DAH患者共115例,其临床表现常见咯血(44%)、血小板减少(47%)、抗双链DNA抗体阳性(69%)、低补体血症(76%)、肾损伤(61%),治疗上主要使用糖皮质激素冲击(43%)、Ig冲击(29%)、环磷酰胺(59%),病死率为67%(77/115)。结论 DAH是SLE少见而危重的并发症,临床表现无特异性,可同时存在多器官损伤,早期诊断、积极治疗可有效控制病情。

Objective To investigate the clinical characteristics and treatment of systemic lupus erythematosus (SLE) complicated with diffuse alveolar hemorrhage (DAH). Methods Clinical data of 3 patients with SLE-DAH were analyzed. Literature review was conducted from January 2013 to July 2018 in the CNKI, Wanfang Data, Chongqing VIP and PubMed databases by using the keywords of “diffuse alveolar hemorrhage”,“ pulmonary hemorrhage” and“ systemic lupus erythematosus” in both English and Chinese. Clinical data of the retrieved SLE-DAH patients were collected and analyzed. Results Three female patients, aged 23, 19 and 25 years, were retrieved. During hospitalization, they presented with intermittent dry cough, fever, dyspnea, renal damage, hemoglobin decline, positive for antinuclear antibody and antidsDNA antibody, hypoxemia and hypocomplementemia. Among them, 2 patients experienced hemoptysis, thrombocytopenia, C-reactive protein (CRP) elevation, increased erythrocyte sedimentation rate (ESR) and positive for anticardiolipin antibody. The SLEDAI for three patients was 18, 15 and 18, respectively. The imaging findings upon admission revealed new diffuse, large mass-like high-density infiltration shadow in lungs, which were significantly absorbed after high-dose glucocorticoid (hormone) combined with cyclophosphamide and Ig shock therapy. The pulmonary shadow did not recur during 2-3 year follow-up. A total of 115 SLE-DAH patients with relatively complete clinical data were retrieved from the literature review. Clinical manifestations mainly included hemoptysis (44%), thrombocytopenia (47%), positive for antidsDNA( 69%), hypocomplementemia( 76%) and renal damage( 61%). Hormonal shock therapy( 43%), Ig shock therapy (29%), cyclophosphamide (59%) were mainly adopted. Among 115 patients, 77 patients died( 67%). Conclusions DAH is a rare and critical complication of SLE. DAF is characterized with nonspecific clinical manifestations. Early diagnosis and active treatment can effectively control the progression of DAH.