中国当代儿科杂志2017，Vol.19Issue(6)：658-662,5.DOI:10.7499/j.issn.1008-8830.2017.06.009
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
李冀 1马菁苒 1孙之星 1姜静婧 1董艳青 1王迁 2宋红梅1
作者信息
- 1. 中国医学科学院北京协和医院 儿科,北京 100730
- 2. 中国医学科学院北京协和医院 风湿免疫科,北京 100730
- 折叠
摘要
Abstract
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.关键词
系统性红斑狼疮/肺高压/雷诺现象/儿童Key words
Systemic lupus erythematosus/Pulmonary hypertension/Raynaud's phenomenon/Child引用本文复制引用
李冀,马菁苒,孙之星,姜静婧,董艳青,王迁,宋红梅..儿童系统性红斑狼疮合并肺高压15例临床分析[J].中国当代儿科杂志,2017,19(6):658-662,5.
