Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease that commonly results in respiratory failure and death. However, the cause of death in these patients has not previously been fully defined. The current study reviews the clinical records and pathological findings of 42 consecutive patients with IPF who underwent a post mortem at the Mayo Clinic (Rochester, MN, USA) over a 9-yr period, from January 1996 to December 2004. The median (range) age at post mortem for the patients was 74 (46-98 yrs) yrs, which included 25 (60%) males. A total of 31 (74%) patients died in the hospital. The immediate causes of death were reported as: respiratory (64%), cardiovascular (21%), or noncardiopulmonary (14%). Acute exacerbation of IPF was the most common immediate cause of death (29%). Pneumonia, aspiration and drug-induced lung disease were identified as other causes of respiratory death. Evidence of pulmonary hypertension was present in the post mortem of 19 (45%) patients and was the immediate cause of death in two of these patients. The immediate cause of death was clinically unsuspected in five (12%) patients and IPF was diagnosed post mortem in nine (21%) patients. The majority of patients with idiopathic pulmonary fibrosis who had undergone a post mortem were found to have died from respiratory causes. Acute exacerbation of idiopathic pulmonary fibrosis was found to be the most common cause of death whilst death from the gradual progression of idiopathic pulmonary fibrosis was found to be less common. Copyright©ERS Journals Ltd 2008.