Clinical Scenario

This protocol addresses pulmonary hypertension (PH) that develops in the setting of interstitial lung disease (ILD) — a distinct and clinically challenging sub-population within PH secondary to lung disease. The coexistence of a fibrotic or inflammatory lung process with pulmonary vascular dysfunction requires a carefully tailored management strategy that differs meaningfully from other forms of PH.

Treatment Approach

Evidence supports consideration of an inhaled prostacyclin-pathway agent in appropriate patients with PH associated with ILD. Importantly, certain agents used in other PH contexts carry specific recommendations against their use in related ILD subtypes.

The complete agent selection, conditions of use, and which therapies to avoid in this population are detailed in the full structured regimen below.

References

DOI: 10.1093/eurheartj/ehac237

Inhaled treprostinil may be considered in patients with PH associated with ILD.
A phase 3 RCT (INCREASE) examined inhaled treprostinil at a target dose of 72 µg given four times daily in 326 patients with PH associated with ILD.
In patients with ILD and PH, inhaled treprostinil may be considered based on the findings from the INCREASE study, but further data are needed, especially on long-term outcomes.
The use of ambrisentan is not recommended in patients with PH associated with IPF.
The use of riociguat is not recommended in patients with PH associated with IIP.
At week 16, the placebo-corrected 6MWD improved by 31 m with inhaled treprostinil.
There were also improvements in NT-proBNP and clinical worsening events, the latter driven by a lower proportion of patients whose 6MWD declined by >15% from baseline.

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Treatment of Pulmonary Hypertension Secondary to Lung Disease in Interstitial Lung Disease

References