The Diagnosis and Management Landscape of Idiopathic Pulmonary Fibrosis: An Australian Experience

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common form of the fibrosing idiopathic interstitial pneumonias of unknown aetiology. The condition is characterised by incessant and progressive lung function decline, leading to worsening symptoms of dyspnoea, impaired physical exercise capacity and poorer quality of life. The overall prognosis of IPF is poor with a median survival of merely 3-5 years. While the disease remains incurable, the advent of novel antifibrotic therapies of pirfenidone and nintedanib both reducing disease progression, herald a new dawn in the management of IPF. However, akin to its unknown aetiology, many questions regarding the diagnostic process and the true impact of antifibrotics on the management of IPF, remains unanswered

The main objective of this thesis is to further investigate components in the diagnostic process of IPF and to explore the effect of antifibrotics on IPF management, with a focus on the Australian experience. In Chapter Two, we sought consensus through a Delphi methodology, on the essential features of an interstitial lung disease multidisciplinary meeting (ILD MDM) which is currently considered the ‘gold standard’ for the diagnosis of ILD. Chapter Three explored the effect of nintedanib and pirfenidone on extracellular matrix components in IPF, and the role of monocyte count as a prognostic marker for IPF. In Chapter Four, we conducted a meta-analysis and systematic review of antifibrotics for the treatment of both IPF and other ILD with a progressive pulmonary fibrosis (PPF) phenotype. Chapter Five and Six describe the Australian experience on the use of antifibrotics for the treatment of IPF. Using data from the Australian IPF Registry, we studied the diagnostic process of IPF, prescription practices of antifibrotics and management strategies used for the treatment of IPF in Australia, while describing the experiences and sentiments of patients and physicians using antifibrotic therapies.