Pulmonary hypertension associated with Interstitial Lung Diseases (PH-ILD) is a severe and rare pulmonary health disorder characterized by elevated blood pressure in the pulmonary arteries supplying blood to the lungs. PH-ILD occurs in the context of pre-existing interstitial lung diseases, such as idiopathic pulmonary fibrosis, and can lead to increased strain on the right side of the heart.

As per DelveInsight’s Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) – Market Insight, Epidemiology, and Market Forecast – 2032 Report, it is estimated that 170,000 diagnosed prevalent cases of PH-ILD exist in the 7MM. Current treatment for PH-ILD involves the use of drugs like bosentan (an endothelin receptor antagonist), sildenafil (a phosphodiesterase-5 inhibitor), and epoprostenol (a prostacyclin analog) to relieve pulmonary vascular constriction and enhance lung blood flow, ultimately reducing elevated pulmonary artery pressure.

The growing recognition of the need for therapies that aim to reduce fibrosis and cure PH-ILD in patients has led to some targeted actions. Treprostinil palmitil inhalation powder (TPIP) is being developed by Insmed Inc. and is posed to become a promising treatment for pulmonary hypertension associated with interstitial lung disease (PH-ILD) and pulmonary arterial hypertension (PAH). The inhaled formulation is derived from treprostinil palmitil and offers a unique product profile tailored to the needs of patients.

At the European Respiratory Society International Congress, Italy, a poster titled “Safety and tolerability of inhaled treprostinil with phosphodiesterase-5 inhibitors in patients with pulmonary hypertension due to interstitial lung disease” was presented by Insmed, which detailed the results related to the therapy in PH-ILD. The poster presented the effect of adding PDE5i with inhaled treprostinil.

The INCREASE study’s positive results led to FDA approval of inhaled treprostinil for PH-ILD, but using sildenafil, a PDE5i, in these patients without controlled trial evidence posed a clinical challenge.

Data from a trial comparing inhaled treprostinil with a PDE5 inhibitor and inhaled treprostinil alone was presented at the ERS conference. Specifically, the PDE5 inhibitor group exhibited higher body weights, mean pulmonary artery pressures, and pulmonary vascular resistance values. Additionally, more individuals in this group had idiopathic interstitial pneumonia, while fewer had connective tissue disease-associated interstitial lung disease.

Despite these differences, both groups reached a median dose of inhaled treprostinil equivalent to 12 breaths administered four times daily by Week 48. This suggests that adding a PDE5 inhibitor did not impact the treprostinil dosing significantly.

In summary, the INCREASE study and its open-label extension have underscored the potential of inhaled treprostinil as a valuable treatment option for PH-ILD. However, whether adding PDE5 to this regimen provides additional benefits or potential risks warrants further investigation, as evidenced by the post hoc analysis. These findings contribute to the ongoing efforts to refine PH-ILD management and enhance the quality of life for those affected by this challenging condition.

According to DelveInsight’s Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) – Market Insight, Epidemiology, and Market Forecast – 2032, the PH-ILD market in the 7MM is estimated to be valued at USD 1,300 million in 2022.  The market is expected to make considerable gains with the evolving landscape of PH-ILD treatment and the growing focus on addressing the needs of its patient population.