特发性肺纤维化的治疗与进展

特发性肺纤维化（IPF）是指原因不明并以普通型问质性肺炎（UIP）为特征性病理改变的一种慢性炎症性间质性肺疾病。病理改变主要表现为弥漫性肺泡炎、肺泡单位结构紊乱和肺纤维化。主要症状为进行性干咳、呼吸困难，常由运动诱发或加重；伴有发绀、杵状指；胸片示两肺中下野弥漫性间质浸润，部分肺容积缩小；肺功能呈限制性通气障碍和弥散功能下降以及动脉低氧血症。目前临床上还缺乏有效治疗手段。近年来有关肺纤维化治疗及研究进展如糖皮质激素的治疗、免疫抑制剂的应用：秋水仙碱、环磷酰胺、硫唑嘌呤等；目前正在研究实验的治疗药物：吡非尼酮、干扰素r1b、干扰素B、卡托普利、角质化细胞生长因子、白介素-1、肿瘤坏死因子等。

Development of the Treatment of Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis(IPF) is a chronic phlogistic interstitial lung disease,which causes remain unknown.It's pathology characteristics are the same as usual interstitial pneumonia: diffuse alveolus inflammatory,disorder of a alveolar unit structure and pulmonary fibrosis.The principal symptoms are described as dry cough and decompensation that are caused and aggravated by physical movement,accompanied by cyanosed and pestle-like fingers.X-ray manifestation showed that diffuse interstitial pneumonia invaded middle and lower parts of the lungs,lung cubage diminished,lung function appeared restrictively aerated obstruction and lung diffuse function declined,hypoxemia was also manifested in the X-ray.There is no effective way to cure this disease yet.This paper gives a brief introduction of the development of the therapy and study in recent years,for instance,cortex hormone therapy and the application of Immunosuppressant.