Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Episodes of Acute exacerbation (AE) of Idiopathic Pulmonary fibrosis (IPF) are important events in the disease trajectory of IPF, associated with punctuated decline in lung function with significant mortality and morbidity associated with it. These episodes are idiosyncratic, and often unpredictable and may have triggers. Our diagnostic criteria for these events, etiology, pathogenesis, risk factors and management continue to evolve over the years, with limited availability of qualitative research data to help guide management. Outcome in general is poor with no well-defined therapy but prevention may be possible with use of Nintedanib. Our chapter aims to explore the contemporary knowledge of the key aspects of this disease entity.