A specific phenotype of interstitial lung disease—progressive pulmonary fibrosis / 中国医师杂志

ABSTRACT

The diagnosis and treatment of interstitial lung diseases (ILD) are difficult, due to the complicated classification and the unknown etiology. In the scope of ILD, in addition to idiopathic pulmonary fibrosis (IPF), there are some diseases still progressing after active treatment, the degree of fibrosis continues to aggravate, lung function continues to deteriorate and the early mortality rate rises. Their prognosis are similar to IPF. We collectively refer to these diseases with similar progression as progressive fibrosing interstitial lung disease (PF-ILD). PF-ILD is a new concept which was put forward in recent years. However, there is no clear consensus to guide the diagnosis and treatment of such diseases. This review comprehensively elaborates the definition, epidemiology, diagnosis, treatment, prognosis and future research directions of PF-ILD, which will help us better understand and manage PF-ILD in the future.