摘要目的 探讨肺移植治疗结缔组织病相关性肺间质病(CTD-ILD)的适应症、手术时机和预后、疗效.方法 回顾性分析2015年9月18日至2017年2月10日在无锡市人民医院经肺移植术治疗的CTD-ILD患者的临床资料,结合文献加以分析.结果 11例CTD-ILD患者经肺移植术评估,接受肺移植手术治疗.其中皮肌炎/多发性肌炎并间质性肺疾病(PM/DM-ILD)2例,类风湿性关节炎并间质性肺疾病(RA-ILD)4例,干燥综合征并间质性肺疾病(pSS-ILD)4例,系统性硬化并间质性肺疾病(SSc-ILD)1例.其中CTD-ILD伴有PAH患者6例.患者男5例,女6例,年龄37 ～70岁,平均年龄(52.73±9.53)岁,单肺移植7例,双肺移植4例,围手术期死亡3例,1例于术后第4天死于肺动脉栓塞,1例于术后第25天死于感染性休克、心功能衰竭,1例于术后第2天死于重度原发性移植物失功(PGD).其余患者随访至今(4～ 24个月),均未发生急慢性排斥反应等并发症,生存状况良好.结论 伴或不伴PAH的CTD-ILD患者行肺移植治疗后并未发现急慢性排斥反应、原发病复发等高风险的并发症,且存活率与其他病种来源的受者相当.

abstractsObjective To investigate the timing and complications of lung transplantation for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD).Method The clinical data of connective tissue-associated disease lung transplantation recipients from September,2015 to February,2017 were collected.Results 11 patients with CTD-ILD were evaluated by lung transplantation and were treated with lung transplantation.Including 2 cases dermatomyositis/polymyositis and interstitial lung disease (PM/DM-ILD),4 cases rheumatoid arthritis and interstitial lung disease (RA-ILD),4 cases of primary Sjgren's syndrome and interstitial lung disease (pSS-ILD),1 case of systemic sclerosis and interstitial lung disease (SSc-ILD).There are 6 cases are CTD-ILD-PAH patients of all.There were 5 males and 6 females,aged 37-70 years,with an average age of (52.73 ± 9.53) years.7 patients received single lung transplantation,4 patients received double lung transplantation.3 patients died in the early postoperative period,one for pulmonary embolism,one for septic shock and heart failure,one for severe primary graft failure (PGD).The remaining patients were followed up (4-24 months) with good survival,no acute and chronic rejection and other complications were observed.The survival rate was similar to that of other disease sources.Conclusion There is no recurrence,acute and chronic rejection and other complications were observed in CTD-ILD and/or PAH lung transplant recipients.And the survival rate was comparable to that of other disease sources.Lung transplantation is a safe and effective choice for selected CTD-ILD patients.