PURPOSE: Patients with an acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have poor prognosis (67% 3-month mortality) and have no clinically significant benefit from medical interventions. Lung transplantation (LTX) is not routinely performed in those patients. This study compares clinical outcomes after LTX in patients with IPF with and without an acute exacerbation at the time of transplantation.
METHODS: Forty-one patients with IPF who underwent LTX at our institution between 2005 and 2009 were identified. Of these, fourteen were clinically identified as having AE-IPF at the time of hospitalization. Clinical data was reviewed, and the explanted lung tissue for the AE-IPF patients was evaluated by a single lung pathologist. Descriptive statistics for the two groups were compared using the Mann-Whitney test for and the Fisher's exact test. Survival data was evaluated using the log-rank test and Kaplan-Meier plots.
RESULTS: No significant differences in age, sex, ethnicity, percent smokers and percentage of patients with diabetes or coronary artery disease were seen. The patients with AE-IPF had significantly higher mean pulmonary artery pressures prior to transplantation (44.3 mmHg for AE-IPF, 27.0 mmHg for IPF, p = 0.002), and spent significantly more days in-hospital prior to transplantation (7.6 days for AE-IPF, 0.6 days for IPF, p = 0.004). No differences in the number of double lung vs. single lung transplants or in the CMV status of donors or recipients were observed between groups. Histological exam of explanted tissue showed diffuse alveolar damage on a usual 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). Ninety-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).
CONCLUSIONS: In this cohort, similar clinical outcomes were observed in patients undergoing lung transplantation for AE-IPF and IPF.
CLINICAL IMPLICATIONS: Lung transplantation may be a viable option in patients with AE-IPF.
DISCLOSURE: The following authors have nothing to disclose: Ali Mansour, Kiran Nair, Roberto Barrios, Ramesh Kesavan, Amit Parulekar, Matthias Loebe, Saverio La Francesca, Brian Bruckner, Scott Scheinin, George Noon, Pawel Kolodziejski, Justin Nguyen, Harish Seethamraju
No Product/Research Disclosure Information