Nathan SD, Flaherty KR, Glassberg MK, et al. - Among a variety of disorders included in the interstitial lung diseases, many are characterized by fibrotic changes (fILD). Idiopathic pulmonary fibrosis is identified as the most common fILD. Frequently, pulmonary hypertension (PH) complicates fILD and is linked with impaired functional capability, lower physical activity and significantly decreased life expectancy. In this work, results from the first cohort of a phase 2b/3 trial with pulsed iNO in patients with fILD-PH are reported. Individuals in cohort 1 were administered either iNO 30 mcg/kg IBW/hr (iNO30) or placebo for 8 weeks of blinded treatment; then transitioning of individuals was done to open label extension (OLE) on iNO30 followed by dose escalation to iNO45 then iNO75. Twenty-three patients were randomized to iNO 30 and 18 to placebo. A clinically and statistically significant benefit in moderate/vigorous physical activity and clinically significant benefit in overall activity were observed among individuals receiving iNO 30. In the OLE, researchers identified higher doses of iNO as safe and well-tolerated while showing maintenance in activity parameters.