Initial presentation of idiopathic pulmonary fibrosis as an acute exacerbation

SummaryTwo cases presenting as an acute exacerbation of idiopathic pulmonary fibrosis (IPF) are presented. Both patients had good response to corticosteroids and exhibited the underlying pathology of organizing pneumonia superimposed on usual interstitial pneumonia. The challenges with diagnosing an IPF exacerbation in patients with previously unknown IPF are discussed. The impact of underlying histopathology during an exacerbation on patient outcomes is also discussed.