Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: a post-hoc analysis of the INCREASE study

Nathan, Steven D.; Waxman, Aaron B.; Rajagopal, Sudarshan; Case, Amy Hajari; Johri, Shilpa; DuBrock, Hilary M.; Zerda, David J. De La; Sahay, Sandeep; King, Christopher S.; Melendres‐Groves, Lana; Smith, Peter; Shen, Eric; Edwards, Lisa; Nelsen, Andrew C.; Tapson, Victor F.

doi:10.1016/s2213-2600(21)00165-x

Cited by 76 publications

(82 citation statements)

References 23 publications

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“…Post hoc analysis found a difference in change in FVC per cent predicted of 1.8% (0.2% to 3.4%; P=0.028), favoring inhaled treprostinil over placebo, by week 16. 128 Notably, this study also found that the largest treatment effect occurred in patients with IPF. Based on these data, a phase 3 randomized, double blind, placebo controlled study began in April 2021 to evaluate the safety and efficacy of inhaled treprostinil in people with IPF, with change in FVC as the primary outcome measure (NCT04708782).…”

Section: Inhaled Treprostinilsupporting

confidence: 54%

Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Liu

Budinger

Dematte

2022

BMJ

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Similarly to idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases can develop progressive pulmonary fibrosis (PPF) characterized by declining lung function, a poor response to immunomodulatory therapies, and early mortality. The pathophysiology of disordered lung repair involves common downstream pathways that lead to pulmonary fibrosis in both IPF and PPF. The antifibrotic drugs, such as nintedanib, are indicated for the treatment of IPF and PPF, and new therapies are being evaluated in clinical trials. Clinical, radiographic, and molecular biomarkers are needed to identify patients with PPF and subgroups of patients likely to respond to specific therapies. This article reviews the evidence supporting the use of specific therapies in patients with IPF and PPF, discusses agents being considered in clinical trials, and considers potential biomarkers based on disease pathogenesis that might be used to provide a personalized approach to care.

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Section: Inhaled Treprostinilsupporting

confidence: 54%

Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Liu

Budinger

Dematte

2022

BMJ

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“…There is growing interest in treating pulmonary vascular disease in patients with IPF. A post hoc analysis showed inhaled treprostinil, a prostacyclin analogue, which has been demonstrated to increase exercise and lung function in patients with IPF 71. Our NMA highlights the potential for sildenafil in the future of IPF, but more study needs to be completed to clearly support its role in managing this disease.…”

Section: Discussionmentioning

confidence: 84%

Medical treatments for idiopathic pulmonary fibrosis: a systematic review and network meta-analysis

Pitre

Mah

Helmeczi

et al. 2022

Thorax

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BackgroundIdiopathic pulmonary fibrosis (IPF) is a respiratory disorder with a poor prognosis. Our objective is to assess the comparative effectiveness of 22 approved or studied IPF drug treatments.MethodsWe searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials and clinicaltrials.gov from inception to 2 April 2021. We included randomised controlled trials (RCTs) for adult patients with IPF receiving one or more of 22 drug treatments. Pairs of reviewers independently identified randomised trials that compared one or more of the target medical treatments in patients with IPF. We assessed the certainty of evidence using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach for network meta-analysis. We calculated pooled relative risk (RR) ratios and presented direct or network estimates with 95% credibility intervals (95% CI), within the GRADE framework.ResultsWe identified 48 (10 326 patients) eligible studies for analysis. Nintedanib [RR 0.69 (0.44 to 1.1), pirfenidone [RR 0.63 (0.37 to 1.09); direct estimate), and sildenafil [RR (0.44 (0.16 to 1.09)] probably reduce mortality (all moderate certainty). Nintedanib (2.92% (1.51 to 4.14)), nintedanib+sildenafil (157 mL (–88.35 to 411.12)), pirfenidone (2.47% (–0.1 to 5)), pamrevlumab (4.3% (0.5 to 8.1)) and pentraxin (2.74% (1 to 4.83)) probably reduce decline of overall forced vital capacity (all moderate certainty). Only sildenafil probably reduces acute exacerbation and hospitalisations (moderate certainty). Corticosteroids+azathioprine+N-acetylcysteine increased risk of serious adverse events versus placebo (high certainty).Conclusion and relevanceFuture guidelines should consider sildenafil for IPF and further research needs to be done on promising IPF treatments such as pamrevlumab and pentraxin as phase 3 trials are completed.

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“…Participants with IIPs on inhaled treprostinil had numerically fewer exacerbations. 227 These results, along with preclinical data suggesting that treprostinil has direct antifibrotic effects on the lung, 233,234 have led to the ongoing TETON trial of IIP without PH (REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04708782). In another post hoc analysis of INCREASE, treatment with inhaled treprostinil resulted in decreased likelihood of disease progression defined as either ≥5% decline in 6MWD, ≥10% decline in forced vital capacity, acute exacerbation, cardiopulmonary hospitalization, lung transplantation, or death (22 versus 36% multiple progression events in treatment versus placebo groups, respectively; P =0.005).…”

Section: Pulmonary Vasodilatorsmentioning

confidence: 99%

Group 3 Pulmonary Hypertension: From Bench to Bedside

Singh

Dorfmüller

Shlobin

et al. 2022

Circulation Research

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Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent approval of the first pulmonary arterial hypertension therapy for the treatment of Group 3 PH related to interstitial lung disease represents an encouraging advancement. This review focuses on molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, the right ventricular dysfunction observed in this population and clinical trial data that inform the use of pulmonary vasodilators in Group 3 PH.

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Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: a post-hoc analysis of the INCREASE study

Cited by 76 publications

References 23 publications

Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Medical treatments for idiopathic pulmonary fibrosis: a systematic review and network meta-analysis

Group 3 Pulmonary Hypertension: From Bench to Bedside

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