Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis

¹ Division of Pulmonary and Critical Care Medicine
² Dept of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN

^* To whom correspondence should be addressed. E-mail: daniels.craig{at}mayo.edu.

	Abstract

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease that commonly results in respiratory failure and death. However, causes of death in these patients have not been fully defined.

We reviewed clinical records and pathologic findings of 42 consecutive patients with IPF who underwent autopsy at Mayo Clinic, Rochester MN over a 9-year period from January 1996 to December 2004.

Median age at autopsy was 74 years (range, 46 to 98 years) and included 25 men (60%). Thirty-one patients (74%) died in hospital. Immediate causes of death were respiratory, (64%), cardiovascular, (21%), or non-cardiopulmonary (14%). Acute exacerbation of IPF was the most common immediate cause of death (29%). Pneumonia, aspiration, and drug-induced lung disease were other causes of respiratory deaths. Evidence of pulmonary hypertension was present at autopsy in 19 patients (45%) and was the immediate cause of death in 2 of these. The immediate cause of death was clinically unsuspected in 5 patients (12%) and IPF was diagnosed postmortem in 9 patients (21%).

The majority of patients with IPF undergoing autopsy died from respiratory causes. Acute exacerbation of IPF was the most common cause of death and death from gradual progression of IPF was less common.

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