Petitpierre N, Beigelman C, Letovanec I, Nicod L P, Lazor R
CHUV, Lausanne.
Rev Med Suisse. 2014 Nov 19;10(451):2208-10, 2212-3.
Idiopathic pulmonary fibrosis (IPF) is the most frequent of the idiopathic interstitial pneumonias. It is a progressive disorderwith a poor prognosis. Its diagnosis requires the careful exclusion of potential causes, and a pattern of usual interstitial pneumonia at high-resolution computed tomography or video-assisted surgical lung biopsy. Several recent randomized trials have profoundly modified the therapeutic management of IPF. The combination of prednisone and azathioprine, often prescribed until recently, has been shown to be harmful and is no longer indicated. N-acetylcystein, also used in the past decade, failed to show an efficacy. However, two new antifibrotic drugs, pirfenidone and nintedanib, have for the first time proven effective in slowing disease progression.
特发性肺纤维化(IPF)是特发性间质性肺炎中最常见的一种。它是一种进行性疾病,预后较差。其诊断需要仔细排除潜在病因,并在高分辨率计算机断层扫描或电视辅助手术肺活检中呈现普通型间质性肺炎的影像特征。最近的几项随机试验深刻改变了IPF的治疗管理方式。过去常用的泼尼松和硫唑嘌呤联合用药已被证明有害,不再推荐使用。过去十年中使用的N-乙酰半胱氨酸也未显示出疗效。然而,两种新型抗纤维化药物吡非尼酮和尼达尼布首次被证明可有效减缓疾病进展。