Idiopathic pulmonary fibrosis-A challenging disease with new horizons and changing therapeutic landscape.

Abstract

To discuss the pathophysiological hypotheses of IPF with a view to summarise the data on pharmacological aspects of treatment of this fibrotic Interstitial Lung Disease. Furthermore, the adverse effects are briefly discussed for the currently available and licenced anti-fibrotic agents. The data were obtained from the Randomised Controlled Trials and scientific studies published in English literature. The manuscript is kept brief to provide an overview of pathophysiological and pharmacological interplay involved in IPF and it was aimed not to be exhaustive so an update is provided on the aspect of pharmacotherapeutics for physicians involved in managing patients with IPF. Two pharmacological agents Pirfenidone and Nintedanib are discussed with the evidence backing up for the rationale of these drugs to slow the disease progression and potentially improve mortality in this disease with a dismal prognosis. The drugs are associated with adverse events and a careful consideration to balance the efficacy with quality of life of individual patient should be considered before commencing these medications. We are seeing real-world data on the value of these anti-fibrotic agents and there is further evidence of them to be efficacious in severe disease and even in the cohort with significant progression over 6-12 months period. The landscape of IPF management has seen a paradigm shift in the last decade form anti-inflammatory to anti-fibrotic approach and with evidence of slowing disease progression. We are likely to improve IPF management in the near future with multi-modality and personalised therapy.