特发性肺纤维化的临床研究进展

Advances in clinic study of idiopathic pulmonary fibrosis

特发性肺纤维化（IPF）是一种慢性进行性发展的并以普通性间质性肺炎为特征性病理改变的一种间质性肺疾病.新的指南中IPF定义为病因不明的疾病,但2011指南指出了IPF可能的高危因素,比如吸烟、环境及遗传因素等.IPF患者在临床症状出现后,大多死于呼吸衰竭,中位生存期只有2～5年.目前并没有药物能够明确改善患者的预后,本文强调临床检查血气分析、肺功能、凝血及血常规、6分钟步行试验、高分辨率CT对IPF的诊断意义,并通过检查了解疾病发展情况,从而对疾病做出综合评估,掌握常见的IPF并发症,做出合适的临床处理意见.

Idiopathic pulmonary fibrosis （IPF） is a specific form of chronic,progressive interstitial lung disease,and associated with the histopathologic pattern of usual interstitial pneumonia.IPF is defined as a disease of unknown etiology in the new guidelines,but the 2011 guide points to the possible risk factors for IPF,such as smoking,environmental and genetic factors.Most patients die of progressive respiratory failure within 2-5 mean survival years of the onset of symptoms.So far,no drug can clearly improve prognosis of patients.This paper emphasizes the role of clinical examination of blood gas analysis,pulmonary function,coagulation and blood routine,six-minute walk test,high resolution CT.By these,we may not only understand the development of disease to make a comprehensive assessment of the disease,but also master the common complications of IPF to make a suitable clinical treatment advice.