首发具肺损伤的系统性红斑狼疮患儿临床特点分析

Clinical characteristics of SLE with lung injury at onset in children

目的探讨首发即具狼疮性肺损伤的系统性红斑狼疮(SLE)患儿的临床特点及预后。方法回顾分析2011年1月至2016年10月收治的初诊SLE的159例患儿的临床资料,据胸部影像学分为狼疮性肺损伤组及无狼疮性肺损伤组,对比两组患儿的临床特点及预后。结果狼疮性肺损伤组89例(56.0%),其中仅37例有呼吸道症状,10例有肺部体征。胸部影像学以胸腔积液/胸膜炎,肺实质病变为主。狼疮性肺损伤组患儿SLE疾病活动指数评分(SLEDAI)及自身免疫性溶血性贫血、血尿、蛋白尿、低补体C 3比例均高于无狼疮性肺损伤组,差异均有统计学意义(P<0.05)。14例死亡患儿均来自狼疮性肺损伤组患儿,狼疮性肺损伤组死亡患儿比例高于无狼疮性肺损伤组(P<0.05)。结论SLE患儿首发易出现狼疮性肺损伤,但多无特异性表现,胸部影像学检查很重要;首发具狼疮性肺损伤者预后差。

Objective To investigate the clinical characteristics and prognosis of systemic lupus erythematosus(SLE)with lupus lung injury at the first onset in children.Method The clinical data of SLE in 159 children from January 2011 to October 2016 were retrospectively analyzed.According to chest imaging,the patients were divided into lupus lung injury group and non-lupus lung injury group.The clinical characteristics and prognosis of the two groups were compared.Results There were 89 cases(56.0%)in lupus lung injury group,of which only 37 had respiratory symptoms and 10 had pulmonary signs.Chest imaging showed mainly pleural effusion/pleurisy and lung parenchymal lesions.SLE disease activity index score(SLEDAI)and the proportion of autoimmune hemolytic anemia,hematuria,proteinuria and low complement C3 in lupus lung injury group were higher than those in non-lupus lung injury group,and the difference was statistically significant(P<0.05).All 14 death cases were from lupus lung injury group.The mortality rate of lupus pulmonary injury group was significantly higher than that of non-lupus pulmonary injury group(P<0.05).Conclusion SLE is prone to have lupus lung injury at the first onset in children,but there are no speci fic manifestations.Chest imaging is very important.SLE with lupus lung injury at the first onset in children has poor prognosis.