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

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From diagnosis to treatment in interstitial lung disease
S55 Derivation and validation of a simple longitudinal score which strongly predicts mortality in interstitial lung disease (ild) associated pulmonary hypertension (ild-ph)
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  1. SRB Bax1,
  2. C Breedy1,
  3. K Dimopoulos1,
  4. A Kempny1,
  5. A Devaraj1,
  6. S Walsh2,
  7. J Joseph1,
  8. A Nair3,
  9. G Kier4,
  10. M Kokosi1,
  11. C Harries1,
  12. V Kouranos1,
  13. C McCabe1,
  14. W Li1,
  15. M Wilde5,
  16. AU Wells1,
  17. LC Price1,*,
  18. SJ Wort1,*
  1. 1Royal Brompton Hospital, London, UK
  2. 2Kings College Hospital Foundation Trust, London, UK
  3. 3Guy’s and St Thomas’ NHS Foundation Trust, London, UK
  4. 4Princess Alexandra Hospital, Brisbane, Australia
  5. 5Surrey and Sussex NHS Trust, London, UK
  6. *Joint final author

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.

Abstact S55 Figure 1
Abstact S55 Figure 1

Derivation cohort – ILD-PH prognostic score.

https://doi.org/10.1136/thoraxjnl-2017-210983.61

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