【摘要】：目的探討系統(tǒng)性紅斑狼瘡(SLE)合并肺高壓(PH)患兒的臨床和實驗室檢查特點、診斷治療及預后。方法選取住院診斷SLE合并PH(SLE-PH)的兒童患者15例,對其臨床癥狀、實驗室檢查、超聲心動圖特點、SLE病情活動指標和治療轉歸等進行回顧性分析。結果 15例SLE-PH患兒中,從SLE確診到PH診斷的間隔時間中位數(shù)為0.1年(范圍:0~6.5年)。除PH相關癥狀外,40%的患兒合并雷諾現(xiàn)象。反映SLE疾病活動度的指標(如補體C3、C4、ESR水平及抗ds DNA陽性率)在PH輕-中度組與重度組間比較差異無統(tǒng)計學意義。13例患兒接受糖皮質激素及免疫抑制劑治療,2例同時接受PH靶向藥物等治療。診斷PH后,中位隨訪時間8.0年(范圍:0.5~18.1年),期間2例患兒死亡,其心功能為Ⅲ~Ⅳ級;余13例病情平穩(wěn)。結論雷諾現(xiàn)象是SLE-PH患兒常見的臨床表現(xiàn)。PH輕重程度與SLE疾病活動度無明顯關聯(lián),應重視SLE患兒肺動脈壓篩查。早期診斷、早期治療有利于改善患兒預后。
[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，

本文編號：2454520