居艳娟++郭迪斌++周序锋++孙剑芳

[摘要]目的 探讨糖皮质激素、环磷酰胺联合西地那非治疗结缔组织病合并肺动脉高压（CTD-PAH）的效果。方法 选取本院2015年12月～2016年2月收治的CTD-PAH患者70例作为研究对象，运用数字随机法将其分为两组，每组35例。对照组给予单纯西地那非治疗，观察组在对照组的基础上给予环磷酰胺、糖皮质激素联合治疗。统计两组患者的临床疗效、肺动脉收缩压水平以及不良反应。结果 观察组的总有效率为97.14%，显著高于对照组的80.00%（χ2=5.081，P=0.024）；肺动脉收缩压水平明显低于对照组（P<0.05），6 min步行距离显著长于对照组（P<0.05）；观察组的不良反应发生率为5.71%，明显低于对照组的22.86%（χ2=4.200，P=0.040）。结论 糖皮质激素、环磷酰胺联合西地那非治疗CTD-PAH可有效改善肺动脉高压水平，效果显著。

[关键词]肺动脉高压；结缔组织病；西地那非；糖皮质激素

[中图分类号] R593.2 [文献标识码] A [文章编号] 1674-4721（2017）08（b）-0123-03

Effect of Glucocorticoid，Cyclophosphamide Combined with Sildenafil in treatment of connective tissue disease complicated with pulmonary hypertension （CTD-PAH）

JU Yan-juan1 GUO Di-bin1 ZHOU Xu-feng2▲ SUN Jian-fang1

1.Department of Rheumatology and Immunology，the First Hospital Affiliated to Gannan Medical College in Jiangxi Province，Ganzhou 341000；2.The Second Department of Heart，the First Hospital Affiliated to Gannan Medical College in Jiangxi Province，Ganzhou 341000

[Abstract]Objective To investigate the effect of Glucocorticoid，Cyclophosphamide Combined with Sildenafil in treatment of connective tissue disease complicated with pulmonary hypertension （CTD-PAH）.Methods 70 patients with CTD-PAH treated in our hospital from December 2015 to February 2016 were selected as the study objects，and they were divided into two groups according to the number random method，and there were 35 cases in each group.The control group was treated with Sildenafil alone，and the observation group was given combined Cyclophosphamide and Glucocorticoids therapy on the basis of the control group.Clinical effect，pulmonary artery systolic pressure level and adverse reactions were observed in the two groups.Results Total effective rate of the observation group was 97.14%，which was significantly higher than that of the control group （80.00%） （χ2=5.081，P=0.024）.Systolic pulmonary arterial pressure level was significantly lower than that of the control group （P<0.05），and walking distance of 6 min was significantly longer than that in the control group （P<0.05）.The incidence of adverse reactions in the observation was 5.71%，which was significantly lower than of that of the control group （22.86%） （χ2=4.200，P=0.040）.Conclusion Glucocorticoid，Cyclophosphamide Combined with Sildenafil treatment of connective tissue disease complicated with pulmonary hypertension （CTD-PAH）

[Keywords]Pulmonary hypertension；Connective tissue disease；Sildenafil；Glucocorticoid

結缔组织病（connective tissue disease，CTD）病情可累及多个器官，其病情发展受多种因素影响，发病机制未明。肺动脉高压（pulmonary hypertension，PAH）可导致患者右心衰竭甚至死亡，是CTD的常见并发症[1-2]。相关研究显示[3-4]，西地那非与糖皮质激素、环磷酰胺联合可改善CTD-PAH患者的血流动力学情况，使患者病情得到有效控制，得到专业人士认可。本研究选取70例CTD-PAH患者作为研究对象，观察此病的临床治疗情况，报道如下。endprint

1资料与方法

1.1一般资料

選取本院风湿免疫科于2015年12月～2016年2月进行诊治的70例CTD-PAH患者（包括系统性红斑狼疮、系统性硬化症、混合性CTD、原发性干燥综合征、皮肌炎、多肌炎、未分化CTD和白塞病）作为研究对象。纳入标准：①两组病例均符合相应的美国风湿病学会（ACR）或相应的国际分类诊断标准[5]；②运用超声心动图测得肺动脉收缩压>40 mmHg即可诊断PAH，临床表现为呼吸困难、乏力、昏厥以及胸痛等；③……