Multi-dimensional scores to predict mortality in patients with idiopathic pulmonary fibrosis undergoing lung transplantation assessment.
The heterogeneous progression of idiopathic pulmonary fibrosis (IPF) makes prognostication difficult and contributes to high mortality on the waitlist for lung transplantation (LTx). Multi-dimensional scores (Composite Physiologic index [CPI], [Gender-Age-Physiology [GAP]; RIsk Stratification scorE [RISE]) demonstrated enhanced predictive power towards outcome in IPF. The lung allocation score (LAS) is a multi-dimensional tool commonly used to stratify patients assessed for LTx. We sought to investigate whether IPF-specific multi-dimensional scores predict mortality in patients with IPF assessed for LTx.



The study included 302 patients with IPF who underwent a LTx assessment (2003-2014). Multi-dimensional scores were calculated. The primary outcome was 12-month mortality after assessment. LTx was considered as competing event in all analyses.



At the end of the observation period, there were 134 transplants, 63 deaths, and 105 patients were alive without LTx. Multi-dimensional scores predicted mortality with accuracy similar to LAS, and superior to that of individual variables: area under the curve (AUC) for LAS was 0.78 (sensitivity 71%, specificity 86%); CPI 0.75 (sensitivity 67%, specificity 82%); GAP 0.67 (sensitivity 59%, specificity 74%); RISE 0.78 (sensitivity 71%, specificity 84%). A separate analysis conducted only in patients actively listed for LTx (n = 247; 50 deaths) yielded similar results.



In patients with IPF assessed for LTx as well as in those actually listed, multi-dimensional scores predict mortality better than individual variables, and with accuracy similar to the LAS. If validated, multi-dimensional scores may serve as inexpensive tools to guide decisions on the timing of referral and listing for LTx.
Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Jolene H
JH
Fisher
King Abdulaziz University, Department of Medicine, Jeddah, Saudi Arabia; Division of Respirology, Department of Medicine, Western University, London, Ontario, Canada.
Faris
F
Al-Hejaili
Division of Thoracic Radiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada.
Sonja
S
Kandel
Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Alim
A
Hirji
Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Shane
S
Shapera
Division of Respirology, Department of Medicine, Western University, London, Ontario, Canada. Electronic address: marco.mura@lhsc.on.ca.
Marco
M
Mura
D000368:Aged; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery*; D015994:Incidence; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000401:mortality / Q000503:physiopathology*; D016040:Lung Transplantation / Q000009:adverse effects / Q000379:methods*; D008297:Male; D008875:Middle Aged; D063305:Organ Dysfunction Scores; D017063:Outcome Assessment, Health Care; D011237:Predictive Value of Tests; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D013534:Survival; D014797:Vital Capacity / Q000502:physiology; D014850:Waiting Lists; D000070857:Walk Test / Q000379:methods
Idiopathic pulmonary fibrosis; Interstitial lung disease; Lung transplantation; Survival