Pragmatic Real-world Approach to Treat Diffuse Parenchymal Lung Disease in Logistically Constraint Situations: A Proposition

Understanding the etiology and the tempo of progression with inexorable and self-perpetuating fibrosis [identification of progressive-fibrotic (PF) phenotype of interstitial lung disease (ILD) (PF-ILD)] can help to decide the treatment of diffuse parenchymal lung disease (DPLD) in the real-world practice. An evidence-supported pragmatic approach has been forwarded for such circumstances.

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

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.