Patients who receive lung transplants during an acute exacerbation of idiopathic pulmonary fibrosis (IPF) have a significantly lower survival rate compared with patients who have stable IPF, according to a recent study published in CHEST.

A total of 89 patients with IPF were listed for lung transplantation; 52 of whom received transplants during stable IPF and 37 of whom were hospitalized because of an acute exacerbation of IPF. Of these 37 patients, 28 received transplants and 9 died before transplantation. Researchers evaluated survival rates, Lung Allocation Scores (LAS), and hospitalization data.

Patients diagnosed with IPF who received a lung transplant were followed for 3 years post transplantation. Compared with individuals who had stable IPF, those with an acute exacerbation of IPF had a significantly shorter survival, especially at the 3 year follow-up (Kaplan-Meier survival curves, 90% vs 60%; P =.0001). Compared with stable IPF, the hazard ratio for dying after lung transplantation during an acute exacerbation of IPF was 5.00 (95% CI, 1.92-13.04; P =.001). An LAS of 80 as a cutoff for patient illness severity yielded a similar hazard ratio (5.7 times higher in patients with LAS >80 compared with patients with LAS <80; 95% CI, 2.33-14.0; P <.0005).

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The causes of death in patients with acute exacerbations of IPF were mainly due to chronic illnesses and organ failure, which were likely related to the prolonged complications from decreased lung function.

In addition, because most of the study patients received lung transplants before anti-fibrotic agents were routinely prescribed, the researchers could not determine the benefit of these medications on transplant outcomes.

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In conclusion, patients with acute exacerbations of IPF and very high LAS during transplantation have lower survival rates and therefore, “may not experience the post transplantation survival advantage expected from lung transplantation.”


Dotan Y, Vaidy A, Shapiro W, et al. Effect of acute exacerbation of idiopathic pulmonary fibrosis on lung transplantation outcome [published online July 2, 2018]. CHEST.  doi:10.1016/j.chest.2018.06.027