What is new in the treatment of interstitial lung diseases

Abstract

Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 pulmonary disorders characterized by lung architectural distortion, with variable amount of fibrotic and/or inflammatory lesions, and associated with significant morbidity and mortality. Pharmacological management is driven by lumping ILDs according to the presumptive predominant pathogenic mechanism, although the coexistence of inflammation and fibrosis may be challenging. In inflammation-predominant diseases, corticosteroids and immunomodulatory agents are the mainstay of treatment. Antifibrotics were initially launched in idiopathic pulmonary fibrosis (IPF). Recently, the concept of progressive pulmonary fibrosis (PPF) has been coined to describe fibrotic lung diseases other than IPF that develop, within a period of 1 year, a progressive disease behavior. The use of antifibrotics may decelerate PPF worsening, similar to their beneficial effects on IPF, and treatment with nintedanib is suggested in patients with PFF, when standard management for fibrotic ILD has failed. Despite the development of novel pharmacological agents and the establishment of non-pharmacological interventions with promising therapeutic potential, in clinical practice, the decisions regarding the management of patients with ILDs still remain highly complex. The integration of individually tailored therapeutic regimens as dictated by personalized medicine approaches is of paramount importance.