S55 Derivation and validation of a simple longitudinal score which strongly predicts mortality in interstitial lung disease (ild) associated pulmonary hypertension (ild-ph). (15th November 2017)

• Record Type: Journal Article
• Authors: Bax, SRB; Breedy, C; Dimopoulos, K; Kempny, A; Devaraj, A; Walsh, S; Joseph, J; Nair, A; Kier, G; Kokosi, M; Harries, C; Kouranos, V; McCabe, C; Li, W; Wilde, M; Wells, AU; Price, LC; Wort, SJ
• Is Part Of: Thorax. Volume 72(2017)Supplement 3
• Page Start: A35
• Page End: A36
• Publication Date: 2017-11-15
• Subjects: Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2017-210983.61
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract : Introduction: Pulmonary hypertension commonly occurs in ILD, and is a malignant prognostic factor. Predicting mortality in this group remains problematic. We hypothesised that a combination of baseline demographics and longitudinal change in PFT's and the biomarker brain-natriuretic peptide (BNP) would predict mortality in ILD-PH. Methods: Demographics, ILD subtype, PFTs, echocardiogram, and CTs were reviewed in consecutive patients undergoing right heart catheterisation (RHC) for suspected ILD-PH. Predictors of prognosis were evaluated in their ability to predict mortality using Cox proportional hazard analysis. A prognostic model was developed and tested in a derivation cohort and tested in a separate validation cohort. Results: 180 patients with confirmed PH formed the derivation cohort (mean pulmonary arterial pressure (mPAP) at RHC 37±9 mmHg; 50% male). At baseline, the strongest predictor of mortality was the underlying ILD diagnosis, with idiopathic pulmonary fibrosis or chronic hypersensitivity pneumonitis strongly associated with mortality (hazard ratio (HR):3.58, p<0.001). A relative decline in forced vital capacity (FVC) of 10% at 12–24 months after RHC predicted mortality (HR:3.20, p=0.001), and an increase in BNP at 12–24 months was also associated with mortality (HR:2.27, p=0.005). A prognostic model combining baseline and longitudinal change risk stratified patients into very-high risk, high-risk and moderate risk groups. In the derivation cohort, the high-risk group had a HR of 2.20 (p=0.01), and the very high-risk group a HR of 4.40 (p=0.001). 50 patients with confirmed PH made up the validation cohort (mPAP 37±9 mmHg; 46% male). The high-risk group had a HR of 3.60 (p=0.01) and the very high-risk group a HR of 8.17 (p<0.001). Conclusion: A simple prognostic score using longitudinal change in FVC and BNP powerfully predicts mortality in ILD-PH, and could be used to prognosticate and help prioritise precious organ allocation in this challenging population.