Rapid Inpatient Uptitration of Inhaled Treprostinil in PH-ILD Patients with Severe Phenotype

Document Type

Article

Publication Title

Advances in Respiratory Medicine

Abstract

Highlights: What are the main findings? Rapid inpatient uptitration of inhaled treprostinil allowed all six severe PH-ILD patients to reach therapeutic dosing within one week. The protocol was well tolerated and associated with early hemodynamic improvement without dose interruptions or 90-day readmissions. What are the implications of the main findings? Accelerated inpatient uptitration may offer a safe and practical strategy for high-risk PH-ILD patients who require faster therapeutic optimization. This approach provides a protocol for future prospective studies evaluating early, aggressive prostacyclin escalation in severe PH-ILD. Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a progressive condition with limited treatment options and associated with high mortality rates. Inhaled treprostinil (iTre) is the only approved therapy for PH-ILD and has been shown to improve exercise capacity and delay disease progression. However, the conventional outpatient titration schedule requires 8–16 weeks to achieve therapeutic dosing, which may delay clinical benefit in those with advanced disease. We conducted a retrospective study of six patients with severe PH-ILD admitted to a tertiary academic center for initiation of iTre using a rapid inpatient uptitration protocol. iTre was started at 3 breaths four times daily (QID) and increased by 2 additional breaths every 12–24 h as tolerated, aiming for ≥9–12 breaths QID within one week under close monitoring. All six patients achieved target dosing without dose reduction or interruption. At three-month follow-up, mean pulmonary artery pressure decreased from 42 ± 5.5 to 35.2 ± 4.5 mmHg, pulmonary vascular resistance from 8.0 ± 1.2 to 6.0 ± 0.9 WU, and cardiac index increased from 2.05 ± 0.13 to 2.15 ± 0.12 L/min/m2. No readmissions occurred within 90 days. This study demonstrates that rapid inpatient uptitration of iTre in severe PH-ILD is feasible and well-tolerated, with preliminary evidence of short-term hemodynamic improvement.

DOI

10.3390/arm94010007

Publication Date

2-1-2026

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