Patients with an acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have extremely poor prognosis. Lung transplantation (LT) is not routinely performed in this group. Our study compares outcomes after LT in patients with IPF with and without an acute exacerbation at the time of LT. Of forty-one patients with IPF who underwent LT, fourteen were identified as having AE-IPF at the time of hospitalization. Descriptive statistics for the groups were compared using the Mann-Whitney test and the Fisher's exact test. Survival data were evaluated using the log-rank test and Kaplan-Meier plots. Patients with AE-IPF had higher mean pulmonary artery pressures prior to LT (44.3 mmHg for AE-IPF, 27.0 mmHg for IPF, p = 0.002), and spent more days in hospital prior to LT (7.6 days for AE-IPF, 0.6 days for IPF, p = 0.004). Histological exam of explanted tissue showed diffuse alveolar damage on a interstitial pneumonia background, fibroblastic foci, and honeycombing. Secondary causes for exacerbation such as cytopathologic changes or evidence of chronic thromboemboli were not seen. The median hospital stay was 14.5 days for AE-IPF patients and 13.0 days for IPF patients (p = 0.762). The median ICU stay was 7 days for AE-IPF patients and 9 days for the IPF patients (p = 0.516). Median follow-up was 18 months (0 – 64 months). 90-day survival was 85.7% for the AE-IPF patients and 81.5% for the IPF patients (p = 0.678). One-year survival was 78.6% for the AE-IPF and 77.8% for the IPF groups, respectively (p = 0.865). In this cohort, similar clinical outcomes were observed in patients undergoing LT for AE-IPF and IPF. LT may be a viable option in patients with AE-IPF.
Seethamraju et al. (Thu,) studied this question.