The incidence and prevalence of idiopathic pulmonary fibrosis (IPF) are increasing globally. This trend predates the COVID-19 pandemic.

Although once considered a rare disorder, IPF may lose this distinction soon. Adjusted prevalence rates in North America are 2.40 to 2.98 per 10,000 persons. The world’s highest prevalence occurs in South Korea, 4.51 per 10,000 persons.

IPF is an irreversible condition in which an abnormal extracellular matrix disrupts the normal lung function and seems to involve a series of micro-injuries to the aging alveolar epithelium which triggers fibrogenic growth factors, producing myofibroblasts that build up extracellular matrices.

Risk favors for IPF are older age, smoking, Caucasian race, lower body mass index, exposure to particulate matter, working around livestock, and genetic predispositions. The role of COVID-19 and other chronic viral factors in current IPF epidemiology is unclear.

Treatments are largely supportive care as IPF is both progressive and irreversible. Lung transplant may be considered in appropriate patients.