特发性肺纤维化IPF_培训课件.ppt

* * IPF 的 治 疗 （三）大环内酯抗生素 红霉素、罗红霉素、克拉霉素、阿齐霉素等能抑制间质炎症很多环节，可静滴，也可口服。 对COP患者激素可并用红霉素长期治疗。 （四）抗氧化剂 N—乙酰半胱氨酸（富露施）400—600mg，3/日；VitE等。 * * 治疗失败者可考虑肺移植治疗. 晚期药物治疗无效者为施行肺移植的主要对象 据报告，全世界已有500例以上IPF患者成功地进行了单侧肺移植 移植后1年存活率约为60%～80%。存活者肺功能均明显改善，移植肺未见再有纤维化发生 晚期病人单肺移植5年存活率50%--60% * * 预后 IPF预后差,平均生存期3年?如果不进行肺移植，IPF病人平均存活 率 2--4年，轻者最长12年；重者2年。 虽可治疗，但难以控制病程的进展。 患者呼吸困难的严重程度和病情进展的速度因人而异。 * * Better Prognosis in Concordant NSIP vs Concordant UIP or Discordant UIP/NSIP 12% of cases Monaghan H, et al. Chest. 2004;125:522-526. 100 75 50 25 0 = Concordant UIP (n = 25) = Discordant UIP–NSIP (n = 8) = Concordant NSIP (n = 31) 60 48 36 24 12 0 Time (months) Percentage Survival * * 思考题 1、何谓弥漫性实质性肺病？ 2、简述IPF的肺功能特点。 3、IPF胸部影像学检查有何特点？ 4、试述无肺活检资料IPF的诊断标准？ * * * Progression of IPF: Acute Exacerbation vs Slow Decline The traditional view of IPF progression involved a slow and steady decline in respiratory function as illustrated by this chart. According to this view, the progressive decline in lung function ultimately leads to respiratory failure and death. * Progression of IPF: Acute Exacerbation vs Slow Decline Emerging evidence suggests that IPF may involve multiple injuries or “hits” to the lung over a period of time, and these hits lead to acute exacerbations that result in periods of more rapid decline in lung function. In other words, this step theory of IPF progression posits that IPF involves many subclinical events with superimposed acute exacerbations that lead to functional decline. These acute exacerbations can be mild or severe and lead to a precipitous decline in respiratory function, respiratory failure, and death. * Epidemiology of IPF This slide reiterates that the incidence and prevalence of IPF increases with age, and that the disease is more common in men than women. Because IPF is a chronic disease that is almost uniformly fatal, the ratio of the prevalence to the incidence can provide a crude indication of the duration of survival after diagnosis.