Nathan SD, Flaherty KR, Glassberg MK, et al. - Given that interstitial lung diseases incorporate a variety of disorders, many of which are recognized by fibrotic changes (fILD), and fILD is often complicated with pulmonary hypertension (PH), and considering that there is a lack of proven treatment for patients suffering from fILD-PH, researchers describe findings from the first cohort of a phase 2b/3 trial with pulsed inhaled nitric oxide (iNO) among patients experiencing fILD-PH. Random assignment of individuals in cohort 1 was done to iNO 30 μg/kg ideal body weight/hr (iNO30) or placebo for 8 weeks of blinded treatment; participants then transitioned to open-label extension (OLE) on iNO30 followed by dose escalation to iNO45 then iNO75. In this study, therapy with iNO30 showed clinically as well as statistically significant advantage in moderate/vigorous physical activity and clinically significant advantage in overall activity. In the OLE, higher doses of iNO were also identified to be safe and have good tolerability while demonstrating maintenance in activity parameters.