儿童系统性红斑狼疮相关肺动脉高压七例并文献分析

Analysis and literature review of 7 cases of systemic lupus erythematosus associated pulmonary arterial hypertension in children

目的分析系统性红斑狼疮(systemic lupus erythematosus,SLE)合并肺动脉高压( pulmo-nary arterial hypertension,PAH)患儿的临床特点,以提高对该病的认识.方法对2017年1月至2018年12月我院诊断的7 例 SLE 合并 PAH 患儿进行回顾性分析.结果 SLE 合并 PAH 的发病率为5.2%.男女比例为1∶6,平均年龄是(11.43 ± 2.51)岁.PAH确诊距SLE发病的中位间隔期为2(1,5)个月.肺动脉收缩压平均(44.00 ± 11.08)mmHg(1 mmHg=0.133 kPa).有典型PAH临床表现者3例(42.8%).抗心磷脂抗体阳性1 例(25%),抗核抗体阳性5 例(71.4%),抗 dsDNA 抗体阳性6 例(85.7%),抗核小体抗体阳性4 例( 57.1%),抗组蛋白抗体阳性3 例(42.8%).N 端脑钠肽前体(N-terminal pro-brain natriuretic peptide,NT-pro BNP)升高100%,心包积液4例(57.1%).5例心功能Ⅰ/Ⅱ级患儿,经激素及免疫抑制剂治疗后,肺动脉收缩压下降.1例心功能Ⅲ级患儿在上述治疗基础上联合安立生坦靶向治疗,肺动脉收缩压下降.1例心功能Ⅳ级患儿,退院后死亡.结论 PAH是SLE的一个严重并发症,早期症状不典型,需要定期监测心脏彩色多普勒超声,早期诊断、早期治疗,以改善预后.

Objective To analyze the clinical features of pulmonary artery hypertension(PAH)in systemic lupus erythematosus(SLE).Methods Seven SLE patients with PAH were enrolled from January 2017 to December 2018.The clinical and laboratory features, treatment and prognosis of those seven patiens were evaluated retrospectively.Results Incidence of PAH was 5.2%.Ratio of male to female was 1∶6, with average age of(11.43±2.51)years.The median period from onset of SLE to diagnosis of PAH was 2(1, 5)months.Average pulmonary artery systolic pressure(PASP)was(44±11.08)mmHg(1 mmHg=0.133 kPa).Three patients(42.8%) had typical PAH clincal manifestation.Anticardiolipid antibody was positive in 1 patient(25.0%), antinuclear antibody was positive in 5 patients(71.4%).Anti-dsDNA antibody, anti-nucleosome antibody, anti-histone antibody were positive in 6(85.7%), 4(57.1%), 3(42.8%)patients respectively.N-terminal pro-brain natriuretic peptide(NT-pro BNP)was elevated in all 4 patiens(100%).Pericardial effusion was positive in 4 patiens(57.1%).PASP of 5 patients who were New York Heart Association(NYHA)Ⅰ/Ⅱ decreased after using glucocorticoids and immunosupressive agents.PASP of 1 patient who was NYHA Ⅲ decreased after combined with ambrisentan.One patient who was NYHA Ⅳ died after refusal of treatment.Conclusion PAH is one of severe complication of SLE.Early symptom is not typical.Ultrasound cardiogram is useful for early diagnosis and can improve the prognosis.