INTRODUCTION: Acute exacerbation of idiopathic pulmonary fibrosis (IPF) is defined by an acute worsening of the respiratory status without any identified cause.

STATE OF THE ART: IPF is the most frequent type of chronic interstitial pneumonia. In general, its course is a progressive worsening with a median survival at 3years after diagnosis. Acute exacerbation is now recognized as a severe complication of IPF. It develops most often within less than 1 month and is characterized by a worsening of dyspnoea associated with the occurrence of new images on chest radiograph. Its diagnosis requires the exclusion of an identified cause for acute deterioration such as pulmonary embolism, bronchopulmonary infection, left heart failure or pneumothorax. The treatment of acute exacerbations of IPF is not well standardized and even though isolated cases of therapeutic success have been reported, its prognosis remains poor. In the most severe cases, mechanical ventilation is generally considered to be ineffective, thus leading most often to a conservative management strategy with no transfer to ICU. However, this attitude is now being questioned since it is now potentially possible to perform an urgent lung transplantation in some patients at least in several countries, including France.

CONCLUSION: Acute exacerbation of IPF is a severe complication of IPF, but its optimal management is not yet clearly defined.