Advances in management of pulmonary fibrosis

Abstract

Pulmonary fibrosis care, affecting both idiopathic pulmonary fibrosis and other forms of interstitial lung disease (ILD) characterised by fibrosis, has transformed with a range of innovations that affect the diagnosis, treatment and prognosis of this condition. Pharmacotherapeutic options have expanded, with increased indications for the application of effective antifibrotic therapy in non-IPF progressive pulmonary fibrosis as a solo treatment or combined with immunosuppression, emerging evidence for immunomodulatory therapy including biologic agents and greater access to clinical trials. The diagnostic approach to unclassifiable ILD now includes transbronchial lung cryobiopsy, a less invasive method to obtain histopathology with reduced morbidity and mortality compared to surgical lung biopsy. A multidisciplinary approach optimises the care of people with ILD and includes non-pharmacological management, addressing significant comorbidities, symptom care and advanced care planning. This review will summarise recent updates in pulmonary fibrosis management.

Keywords: antifibrotic; cryobiopsy; interstitial lung disease; pulmonary fibrosis.