Idiopathic Pulmonary Fibrosis: The Epidemiology and Natural History of Disease

Idiopathic pulmonary fibrosis (IPF) was once thought to be a rare disease and has been classically described as a disease that progresses in a “relentless and often insidious manner.” However, recent epidemiologic studies have revealed the true burden of this disease on society and identified risk factors for the development of disease that may ultimately allow for not only disease prevention but also further insight into the pathobiology of this—as of yet—idiopathic disease. At the same time, recent cohort studies and clinical trials have better defined the natural history of this disease. In this manuscript, we will review recently acquired epidemiologic data and summarize the current understanding of the natural history of IPF.