Exacerbations in Idiopathic Pulmonary Fibrosis Triggered by Pulmonary and Nonpulmonary Surgery: A Case Series and Comprehensive Review of the Literature

Abstract

Acute exacerbations (AE) of idiopathic pulmonary fibrosis (IPF) are well recognized in the progression of this uniformly fatal disease. Surgical lung biopsy and lung resection may initiate these acute events leading to a rapid deterioration and permanent decline in lung function. Our aim is to discuss the role of pulmonary and nonpulmonary surgery as a precipitating factor and to review the literature on the nature, course, and outcomes of acute exacerbations in the context of surgical interventions. This study consisted of a retrospective case series of patients at the Johns Hopkins Hospital who experienced acute exacerbation following a surgical procedure. Patients included in the case series suffered from aggravation of dyspnea within 1 month after surgical intervention, with new infiltrates on imaging. There was no other more likely cause after diagnostic evaluation. A comprehensive review of the current literature pertaining to AEs of IPF in the context of a surgical intervention was performed. In a series of four patients from Johns Hopkins Hospital with AE in IPF, two of three patients who underwent video-assisted thoracoscopic surgery (VATS) lung biopsy had a fatal outcome. The fourth patient survived an AE after a total knee replacement but had a fatal outcome after a subsequent coronary artery bypass graft surgery. We found no report in the literature of AE in an IPF patient who underwent nonpulmonary surgery. Acute exacerbations of IPF can occur postoperatively after both pulmonary and nonpulmonary surgery and are associated with a high mortality rate. As a next step, a prospective multicenter clinical study of patients with IPF undergoing both pulmonary and nonpulmonary surgeries would allow the identification of perioperative risk factors in the development of AE of IPF.