# Development of a Tool to Assess the Severity of Pulmonary Hypertension in Patients with Interstitial Lung Disease: A Guide to Assist Therapeutic Choices

## TL;DR

A new scoring tool was developed to assess the severity of pulmonary hypertension in interstitial lung disease patients, helping guide treatment decisions and improve care.

## Contribution

The novel contribution is the creation of a PH-ILD Severity score integrating clinical parameters to guide therapy and lung transplant evaluation.

## Key findings

- A PH-ILD Severity score of 3 or higher had an AUC of 0.831 for predicting clinical worsening.
- The score integrates WHO FC, CI, TAPSE, and PVR to triage patients for different treatment options.
- The tool can help determine which patients may benefit from inhaled medications or lung transplant evaluation.

## Abstract

What are the main findings?
We created such a scoring tool to guide PH-specific therapy in PH-ILD patients using subjective and objective information (WHO FC, CI, TAPSE, PVR).A score of 3 or greater in the PH-ILD Severity score yielded an AUC of 0.831 for the composite endpoint of clinical worsening.

We created such a scoring tool to guide PH-specific therapy in PH-ILD patients using subjective and objective information (WHO FC, CI, TAPSE, PVR).

A score of 3 or greater in the PH-ILD Severity score yielded an AUC of 0.831 for the composite endpoint of clinical worsening.

What is the implication of the main finding?
Similarly to the way that risk assessment tools can guide subsequent steps in therapy of PH patients, this PH-ILD Severity score will triage patients who may benefit from inhaled medications, who may require parenteral prostacyclin therapy, and who should be considered for expedited lung transplant evaluation.

Similarly to the way that risk assessment tools can guide subsequent steps in therapy of PH patients, this PH-ILD Severity score will triage patients who may benefit from inhaled medications, who may require parenteral prostacyclin therapy, and who should be considered for expedited lung transplant evaluation.

Background: Pulmonary hypertension (PH) is a frequent complication in patients with interstitial lung disease (ILD); its occurrence results in significant morbidity and mortality. Currently approved treatment options for PH-ILD include inhaled prostacyclin therapy, although this approach may be insufficient in patients who have developed simultaneous right ventricular failure. Moreover, there is no available treatment algorithm regarding the optimal therapy and timing of lung transplant referral for PH-ILD patients based on disease severity. Design/Methods: In this study, we created such a tool to guide PH-specific therapy in PH-ILD patients, especially as further treatment strategies are developed. We developed a 4-point PH-ILD Severity score that integrated both subjective and objective information (WHO FC, CI, TAPSE, PVR) from retrospective analysis of 57 PH-ILD patients. Results: A score of 3 or greater in the PH-ILD Severity score yielded an AUC of 0.831 (p < 0.001) for the composite endpoint of clinical worsening (hospitalization due to a cardiopulmonary indication; decrease in 6 min walk distance by >15% at 2 consecutive visits; all-cause mortality; lung transplantation). Conclusions: Further confirmation and evolution of this PH-ILD Severity score will assist in the development of optimal treatment plans in ILD patients diagnosed with concomitant PH.