中华临床免疫和变态反应杂志2017，Vol.11Issue(1)：6-13,8.DOI:10.3969/j.issn.1673-8705.2017.01.002
吡非尼酮治疗结缔组织病相关肺间质病变三例及文献复习
Pirfenidone for the Treatment of Connective Tissue Diseases Associated InterstitialLung Disease:A Report of Three Cases and Review of Literature
摘要
Abstract
Objective To explore the efficacy and safety of Pirfenidone for the treatment of connective tissue diseases(CTD) associated interstitial lung disease(ILD).Methods A report of three cases with CTD-ILD and literature were reviewed in this paper.Clinical features, efficacy, variables, and safety data were analyzed.Results There were 43 cases with CTD-ILD, including 40 cases collected through databases searching,such as Chinese databases(CNKI, CBM disc, Wanfang, and VIP)and English database (Pubmed, Embase) and 3 cases of this reports.Of all the 43 cases, there were 29 female cases (67.4%), and the average age was about 49.7 years old.Their primary diseases were dermatomyositis (n=31, 72.1%), systemic sclerosis (n=9, 20.9%), Sjogren`s syndrome (n=1), systemic lupus erythematosus (n=1), undifferentiated connective tissue disease (n=1),respectively.The primary disease duration ranged from 8 months to 21 years.Pirfenidone was administrated to all patients suffering from progressive respiratory dysfunction, and together with adequate treatment with glucocorticoid and immunosuppressant.We found that dyspnea were relieved, high resolution CT(HRCT) signs were stable and pulmonary function index [forced vital capacity(FVC)%, diffusion capacity for carbon monoxide of the lung(DLco)%, total lung capacity(TLC)%] were improved.No serious adverse events were evident during the whole treatment.Conclusion Based on our experience on pirfenidone for the management CTD associated ILD and the review of current literature, we herein can conclude that pirfenidone may be of some value in patients with CTD-ILD, especially for those with progressive respiratory dysfunction after adequate treatment of glucocorticoid and immunosuppressant.关键词
结缔组织疾病/肺间质病变/吡非尼酮/肺功能检测/文献复习Key words
connective tissue disease/interstitial lung disease/pirfenidone/pulmonary function test/literature review分类
医药卫生引用本文复制引用
邱明亮,姜楠,吴婵媛,王迁..吡非尼酮治疗结缔组织病相关肺间质病变三例及文献复习[J].中华临床免疫和变态反应杂志,2017,11(1):6-13,8.基金项目
国家自然科学基金(81471615) （81471615）
