Nathan SD, Flaherty KR, Glassberg MK, et al. - Given that a variety of disorders, many of which are characterized by fibrotic changes (fILD), are incorporated in the interstitial lung diseases, and fILD is often complicated by pulmonary hypertension (PH), so, researchers describe findings from the first cohort of a phase 2b/3 trial with pulsed inhaled nitric oxide (iNO) in patients with fILD-PH. In a random manner, the individuals in cohort 1 were assigned to iNO 30 μg/kg ideal body weight/hr (iNO30) or placebo for 8 weeks of blinded treatment; individuals then transitioned to open-label extension (OLE) on iNO30 followed by dose escalation to iNO45 then iNO75. Findings revealed that clinically and statistically significant benefit in moderate/vigorous physical activity as well as clinically significant benefit in overall activity was afforded by treatment with iNO30. In the OLE, the safety as well as the good tolerability of higher doses of iNO was also evident along with maintenance in activity parameters.