儿童系统性红斑狼疮合并肺高压15例临床分析

A clinical analysis of 15 children with systemic lupus erythematosus accompanied by pulmonary hypertension

目的探讨系统性红斑狼疮(SLE)合并肺高压(PH)患儿的临床和实验室检查特点、诊断治疗及预后。方法选取住院诊断SLE合并PH(SLE-PH)的儿童患者15例,对其临床症状、实验室检查、超声心动图特点、SLE病情活动指标和治疗转归等进行回顾性分析。结果 15例SLE-PH患儿中,从SLE确诊到PH诊断的间隔时间中位数为0.1年(范围:0~6.5年)。除PH相关症状外,40%的患儿合并雷诺现象。反映SLE疾病活动度的指标(如补体C3、C4、ESR水平及抗ds DNA阳性率)在PH轻-中度组与重度组间比较差异无统计学意义。13例患儿接受糖皮质激素及免疫抑制剂治疗,2例同时接受PH靶向药物等治疗。诊断PH后,中位随访时间8.0年(范围:0.5~18.1年),期间2例患儿死亡,其心功能为Ⅲ~Ⅳ级;余13例病情平稳。结论雷诺现象是SLE-PH患儿常见的临床表现。PH轻重程度与SLE疾病活动度无明显关联,应重视SLE患儿肺动脉压筛查。早期诊断、早期治疗有利于改善患儿预后。

Objective To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus （SLE） accompanied by pulmonary hypertension （PH）. Methods The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. Results Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0.1 year （range： 0-6.5 years）. Aside from PH-related symptoms, Raynaud＇s phenomenon was observed in 6 （40%） of the 15 patients. There was no significant difference in SLE disease activity （evaluated by complements 3 and 4 levels, erythrocyte sedimentation rate, and positive rate of anti-double-stranded DNA） between patients with mild-to-moderate PH and those with severe PH （P〈0.05）. As for treatment, 13 patients received immunosuppressive therapy with glucocorticoids, and among them 2 patients received PH-targeted therapy. During a median follow-up of 8.0 years （range： 0.5-18.1 years） since the diagnosis of PH, 2 deaths were noted with class Ⅲ or IV cardiac function （World Health Organization）, while the other patients were in a stable condition. Conclusions Raynaud＇s phenomenon is a common clinical manifestation in children with SLE accompanied by pulmonary hypertension （PH）. PH severity is not significantly associated with SLE disease activity, and thus greater focus should be placed upon early screening of pulmonary arterial pressure in SLE patients. Early diagnosis and early treatment can improve the prognosis of children with SLE.