儿童系统性红斑狼疮合并肺高压15例临床分析
发布时间：2019-04-08 10:56
【摘要】：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。
[Abstract]:Objective to investigate the clinical and laboratory features, diagnosis, treatment and prognosis of patients with systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH). Methods 15 children with SLE complicated with PH (SLE-PH) were enrolled in this study. The clinical symptoms, laboratory examination, echocardiographic features, SLE activity index and treatment outcome were analyzed retrospectively. Results the median interval between diagnosis of SLE and diagnosis of PH was 0.1 years (range: 0 ~ 6.5 years) in 15 patients with SLE-PH. In addition to PH-related symptoms, 40% of the children complicated with Renault phenomenon. There was no significant difference in the activity of SLE disease (such as complement C3, C4, ESR level and anti-ds DNA positive rate) between mild and moderate PH group and severe group. 13 children were treated with glucocorticoid and immunosuppressive agent. Two cases were treated with PH targeted drugs at the same time. After the diagnosis of PH, the median follow-up time was 8.0 years (range: 0.5-18.1 years), during which 2 cases died, the cardiac function was grade 鈪，
本文编号：2454520
[Abstract]:Objective to investigate the clinical and laboratory features, diagnosis, treatment and prognosis of patients with systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH). Methods 15 children with SLE complicated with PH (SLE-PH) were enrolled in this study. The clinical symptoms, laboratory examination, echocardiographic features, SLE activity index and treatment outcome were analyzed retrospectively. Results the median interval between diagnosis of SLE and diagnosis of PH was 0.1 years (range: 0 ~ 6.5 years) in 15 patients with SLE-PH. In addition to PH-related symptoms, 40% of the children complicated with Renault phenomenon. There was no significant difference in the activity of SLE disease (such as complement C3, C4, ESR level and anti-ds DNA positive rate) between mild and moderate PH group and severe group. 13 children were treated with glucocorticoid and immunosuppressive agent. Two cases were treated with PH targeted drugs at the same time. After the diagnosis of PH, the median follow-up time was 8.0 years (range: 0.5-18.1 years), during which 2 cases died, the cardiac function was grade 鈪，
本文编号：2454520
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