系统性红斑狼疮合并弥漫性肺泡出血患者临床特征分析

Clinical characteristics of systemic lupus erythematosus complicated with diffuse alveolar hemorrhage

目的分析系统性红斑狼疮(systemic lupus erythematosus,SLE)合并弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH)患者临床资料,探讨其临床特征及预后。方法SLE患者660例,其中13例合并DAH者为合并DAH组,随机选取39例未合并DAH的SLE患者为未合并DAH组。比较2组入院治疗期间呼吸困难、发热、咯血、肾脏损害、低氧血症等发生率;比较2组入院次日血清C反应蛋白、血红蛋白、补体C3、补体C4、血清白蛋白水平,CD3^(-)CD19^(+)B淋巴细胞比率及SLE疾病活动度评分(SLE disease activity index,SLEDAI)等。出院后随访6~140个月,比较合并DAH组生存患者与死亡患者入院治疗期间血浆置换、机械通气、糖皮质激素冲击治疗比率。结果合并DAH组呼吸困难(92.3%)、发热(92.3%)、咯血(100.0%)、肾脏损害(100.0%)、血管炎症(69.2%)、低氧血症(84.6%)发生率及血清C反应蛋白水平[(7.71±1.53)mg/L]、SLEDAI评分[(10.3±1.2)分]、CD3^(-)CD19^(+)B淋巴细胞比率[(19.13±10.22)%]均高于未合并DAH组[12.8%、25.6%、17.9%、43.6%、23.1%、5.1%、(6.31±1.63)mg/L、(9.0±1.9)分、(11.45±7.54)%](P<0.05),血红蛋白[(78.10±10.22)g/L]、补体C3[(0.23±0.19)g/L]、补体C4[(0.06±0.07)g/L]、血清白蛋白[(20.13±6.35)g/L]水平均低于未合并DAH组[(104.15±12.14)、(0.54±0.19)、(0.19±0.14)、(26.34±5.45)g/L](P<0.05),面部皮疹、关节炎、口腔溃疡、雷诺现象、神经系统损害、间质性肺炎、肺动脉高压发生率,抗双链DNA抗体、抗Sm抗体、抗核糖体P蛋白抗体、抗心磷脂抗体阳性率及CD3^(+)、CD3^(+)CD4^(+)、CD3^(+)CD8^(+)T细胞淋巴比率与未合并DAH组比较差异均无统计学意义(P>0.05)。合并DAH组13例患者均采用大剂量糖皮质激素治疗,其中10例治疗第1~3天采用糖皮质激素冲击治疗;部分患者联合血浆置换、静脉血液透析、机械通气治疗。随访6~140个月,合并DAH组死亡8例,生存5例,生存患者入院治疗期间血浆置换(100.0%)、糖皮质激素冲击治疗(100.0%)比率均高于死亡患者(12.5%、62.5%)(P<0.05),机械通气比率(60.0%)低于死亡患者(75.0%)(P<0.05),病程及入院时血红蛋白水平与死亡患者比较差异均无统计学意义(P>0.05)。结论SLE合并DAH患者多并发肾脏损害、呼吸困难、咯血、血管炎症,血清C反应蛋白、CD3^(-)CD19^(+)B淋巴细胞比率、SLEDAI评分升高,血红蛋白、补体C3、补体C4、血清白蛋白水平降低,糖皮质激素冲击治疗和血浆置换可改善其预后。

Objective To analyze the clinical data of patients with systemic lupus erythematosus(SLE)complicated with diffuse alveolar hemorrhage(DAH),and to investigate the clinical characteristics and prognosis.Methods In 660 SLE patients,13 patients were complicated with DAH(DAH group)and another 39 patients without DAH were randomly selected(non-DAH group).The incidences of dyspnea,fever,hemoptysis,renal involvement and hypoxemia during hospitalization were compared between two groups.The levels of C-reactive protein,hemoglobin,complement C3,complement C4,serum albumin,CD3^(-)CD19^(+)B lymphocyte ratio and SLE disease activity index(SLEDAI)score were detected on the second day after admission and compared between two groups.All patients were followed up for 6 to 140 months after discharge.The percentages of plasma exchange,mechanical ventilation and glucocorticoid shock therapy during treatment were compared between the survived patients and the died patients in DAH group.Results The incidences of dyspnea,fever,hemoptysis,renal involvement,vascular inflammation,hypoxemia,C-reactive protein,SLEDAI score,and CD3^(-)CD19^(+)T lymphocyte ratio were higher in DAH group(92.3%,92.3%,100.0%,100.0%,69.2%,84.6%,(7.71±1.53)mg/L,10.3±1.2,(19.13±10.22)%)than those in non-DAH group(12.8%,25.6%,17.9%,43.6%,23.1%,(6.31±1.63)mg/L,9.0±1.9,(11.45±7.54)%)(P<0.05).The levels of hemoglobin,C3,C4 and serum albumin were lower in DAH group((78.10±10.22),(0.23±0.19),(0.06±0.07),(20.13±6.35)g/L)than those in non-DAH group((104.15±12.14),(0.54±0.19),(0.19±0.14),(26.34±5.45)g/L)(P<0.05).There were no significant differences in the incidences of facial skin rashes,arthritis,oral ulcer,Renault phenomenon,nervous system damage,interstitial pneumonia and pulmonary hypertension,in the positive rates of anti-double stranded DNA antibody,anti-Sm antibody,anti-ribosomal P protein antibody and anti-cardiolipin antibody,and in the levels of CD3^(+)and CD3^(+)CD4^(+)as well as the CD3^(+)CD8^(+)T lymphocyte ratio between two groups(P>0.05).DAH group received high-dose glucocorticoid therapy,and 10 of them received glucocorticoid shock therapy in the first 3 days of treatment,while some patients were treated with plasma exchange,intravenous hemodialysis and mechanical ventilation.During 6-140 months of follow-up,8 patients died and 5 survived in DAH group.The percentages of plasma exchange and glucocorticoid shock therapy were higher in the survived patients(100.0%,100.0%)than those in the died patients(12.5%,62.5%)(P<0.05),and the percentage of mechanical ventilation was lower in the survived patients(60.0%)than that in the died patients(75.0%)(P<0.05).The course of disease and the hemoglobin level showed no significant differences between the survived and died patients(P>0.05).Conclusion SLE patients complicated with DAH often develop renal damage,dyspnea,hemoptysis and vasculitis,and their levels of hemoglobin,complement C3,complement C4 and serum albumin decrease,while the level of C-reactive protein,CD3^(-)CD19^(+)B lymphocyte ratio and SLEDAI score increase.Glucocorticoid shock therapy and plasma exchange could improve the prognosis.