Abstract
Purpose
This study was performed to compare the outcome of lung transplantation (LT) for idiopathic pleuroparenchymal fibroelastosis (IPPFE) with that of LT for idiopathic pulmonary fibrosis (IPF).
Methods
We reviewed, retrospectively, all adult patients who underwent LT for IPPFE or IPF in Japan between 1998 and 2018.
Results
There were 100 patients eligible for this study (31 with IPPFE and 69 with IPF). Patients with IPPFE tended to have a significantly lower body mass index (BMI) than those with IPF (median, 16.7 vs. 22.6 kg/m2, respectively; P < 0.01). However, Kaplan–Meier survival curves showed no significant difference in overall survival between the groups. The BMI did not increase in patients with IPPFE, even 1 year after LT (pretransplant, 16.5 ± 3.2 kg/m2 vs. 1 year post-transplant, 15.6 ± 2.5 kg/m2; P = 0.08). The percent predicted forced vital capacity (%FVC) 1 year after LT was significantly lower in the IPPFE group than in the IPF group (48.4% ± 19.5% vs. 68.6% ± 15.5%, respectively; P < 0.01).
Conclusions
Despite extrapulmonary problems such as a flat chest, low BMI, and associated restrictive impairment persisting in patients with IPPFE, patient survival after LT for IPPFE or IPF was equivalent.
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Abbreviations
- %DLco:
-
Percent predicted diffusing capacity of carbon monoxide
- %FEV1 :
-
Percent predicted forced expiratory volume in 1 s
- %FVC:
-
Percent predicted forced vital capacity
- APDT:
-
Anteroposterior diameter of the thoracic cage
- BMI:
-
Body mass index
- FEV1 :
-
Forced expiratory volume in 1 s
- FVC:
-
Forced vital capacity
- ICU:
-
Intensive care unit
- IIPs:
-
Idiopathic interstitial pneumonias
- IPF:
-
Idiopathic pulmonary fibrosis
- IPPFE:
-
Idiopathic pleuroparenchymal fibroelastosis
- LT:
-
Lung transplantation
- TDT:
-
Transverse diameter of the thoracic cage
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Acknowledgements
We thank the Japanese Society of Lung and Heart-Lung Transplantation for helping us to collect the patients’ data.
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Shiiya, H., Nakajima, J., Date, H. et al. Outcomes of lung transplantation for idiopathic pleuroparenchymal fibroelastosis. Surg Today 51, 1276–1284 (2021). https://doi.org/10.1007/s00595-021-02232-6
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DOI: https://doi.org/10.1007/s00595-021-02232-6