吗替麦考酚酯治疗结缔组织病相关肺动脉高压11例临床分析  

Reports and analyses of 11 cases of connective tissue diseases related pulmonary arterial hypertension treated by mycophenolate mofetil

作　　者：周俊飞[1] 温晓宏[1] 白羽[1] 刘媛[1] 付强[1] 郑毅[1] Zhou Junfei Wen Xiaohong Bai Yu Liu Yuan Fu Qiang Zheng Yi.(Department of Rheumatology, Beijing Chao-yang Hospital, Capital Medical University, Beijing 100020, China)

机构地区：[1]首都医科大学附属北京朝阳医院风湿免疫科,100020

出　　处：《中华风湿病学杂志》2017年第7期450-454,共5页Chinese Journal of Rheumatology

基　　金：北京市科学技术委员会资助项目（Z141107002514134）

摘　　要：目的探索免疫抑制类药物吗替麦考酚酯（MMF）在结缔组织病相关肺动脉高压（CTD—PAH）临床治疗中的作用。方法收集2014年1月至2016年6月在首都医科大学附属北京朝阳医院风湿免疫科住院并应用MMF治疗CTD-PAH的11例患者病历资料并进行分析。结果11例CTD-PAH患者中SLE相关肺动脉高压（SLE-PAH）7例，SSe相关肺动脉高压（SSc-PAH）2例，RA相关肺动脉高压（RA—PAH）1例，MCTD相关肺动脉高压（MCTD-PAH）1例。均为女性患者，平均年龄（40±14）岁，PAH平均病程为（34±35）个月。激素、MMF联合治疗共7例，激素、MMF、波生坦联合治疗1例，激素、MMF、西地那非联合治疗3例，患者临床症状缓解。9例患者随诊满1年，1例患者随诊7个月，1例患者随诊6个月，1年生存率为100％（9／9）。1例患者应用激素、MMF联合治疗并维持疾病稳定约111个月，后因胸闷症状加重，加用波生坦仍能够达到病情稳定。结论MMF可能对CTD-PAH的症状缓解及延长病情稳定有一定的作用。Objective To explore the efficacy of mycophenolate mofetil （MMF）, which is a kind of immuno-suppressant drugs, on the treatment of Connective tissue diseases-pulmonary arterial hypertension （CTD-PAH）. Methods Medical charts of eleven cases of hospitalized patients who were diagnosed as CTD- PAH and treated by MMF in Beijing Chaoyang Hospital affiliated to the Capital Medical University, from January 2014 to June 2016 were collected and analyzed. Results In the 11 cases of CTD-PAH, the systemic lupus erythematosus （SLE） related pulmonary hypertension （SLE-PAH） were 7, while the systemic selero- derma associated pulmonary hypertension （SSc-PAH） were 2, and rheumatoid arthritis related pulmonary hy- pertension （RA-PAH） was 1, and the mixed connective tissue disease related pulmonary hypertension （MCTD- PAH） was 1. All patients were women, and the average age was （40±14） years, and the average duration of PAH was （34 ±35） months. The combination therapy of corticosteriods and MMF was applied to 7 cases, meanwhile the therapy of corticosteriods, MMF and bosentan was used in 1 case, corticosteriods, MMF and sildenafil was prescribed for 3 cases, and symptoms of the patients alleviated. Except for one case having been followed up for 7 months and one for 6 months, 9 patients completed the 1-year follow-up, and the survival rate was 100%（9/9）. Notably, one patient, who had been alleviated for 111 months with therapy of corticosteriods and MMF, adopted the combination therapy of corticosteriods, MMF and bosentan for aggravated chest distress, and became stable eventually. Conclusion MMF may have therapeutic effects on inducing and even maintaining the stabilization of CTD-PAH.

关 键 词：肺动脉高压 肺性 结缔组织病 吗替麦考酚酯 

分 类 号：R544.1[医药卫生—心血管疾病] R593.2[医药卫生—内科学]