{"doi":"10.1183/13993003.02414-2022","title":"Long-term inhaled treprostinil for pulmonary hypertension due to interstitial lung disease: INCREASE open-label extension study","abstract":"Introduction The 16-week randomised, placebo-controlled INCREASE trial (RCT) met its primary end-point by improving 6-min walk distance (6MWD) in patients receiving inhaled treprostinil for pulmonary hypertension due to interstitial lung disease (PH-ILD). The open-label extension (OLE) evaluated long-term effects of inhaled treprostinil in PH-ILD. Methods Of 258 eligible patients, 242 enrolled in the INCREASE OLE and received inhaled treprostinil. Assessments included 6MWD, pulmonary function testing, N-terminal pro-brain natriuretic peptide (NT-proBNP), quality of life and adverse events. Hospitalisations, exacerbations of underlying lung disease and death were recorded. Results At INCREASE OLE baseline, patients had a median age of 70 years and a mean 6MWD of 274.2 m; 52.1% were male. For the overall population, the mean 6MWD at week 52 was 279.1 m and the mean change from INCREASE RCT baseline was 3.5 m (22.1 m for the prior inhaled treprostinil arm and −19.5 m for the prior placebo arm); the median NT-proBNP decreased from 389 pg·mL −1 at RCT baseline to 359 pg·mL −1 at week 64; and the absolute (% predicted) mean forced vital capacity change from RCT baseline to week 64 was 51 mL (2.8%). Patients who received inhaled treprostinil versus placebo in the RCT had a 31% lower relative risk of exacerbation of underlying lung disease in the OLE (hazard ratio 0.69 (95% CI 0.49–0.97); p=0.03). Adverse events leading to drug discontinuation occurred in 54 (22.3%) patients. Conclusions These results support the long-term safety and efficacy of inhaled treprostinil in patients with PH-ILD, and are consistent with the results observed in the INCREASE RCT.","journal":"European Respiratory Journal","year":2023,"id":318050,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":69,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.8183,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1025037,"name":"Ricardo Restrepo‐Jaramillo","orcid":null,"position":1,"is_corresponding":false},{"id":394463,"name":"Thenappan Thenappan","orcid":"0000-0002-6210-0467","position":2,"is_corresponding":false},{"id":1025038,"name":"Peter A. Engel","orcid":null,"position":3,"is_corresponding":false},{"id":1025039,"name":"ABUBAKR A BAJWA","orcid":null,"position":4,"is_corresponding":false},{"id":681851,"name":"Ashwin Ravichandran","orcid":"0000-0003-2571-4432","position":5,"is_corresponding":false},{"id":335558,"name":"Jeremy Feldman","orcid":null,"position":6,"is_corresponding":false},{"id":476888,"name":"Amy Hajari Case","orcid":"0000-0001-8663-2022","position":7,"is_corresponding":false},{"id":468979,"name":"Rahul Argula","orcid":"0000-0002-6184-4093","position":8,"is_corresponding":false},{"id":103438,"name":"Victor F. Tapson","orcid":"0000-0001-9708-1457","position":9,"is_corresponding":false},{"id":869649,"name":"Peter Smith","orcid":"0000-0002-2051-7774","position":10,"is_corresponding":false},{"id":869646,"name":"C. Q. Deng","orcid":"0000-0002-5641-4101","position":11,"is_corresponding":false},{"id":869648,"name":"Eric Shen","orcid":"0000-0001-5621-6570","position":12,"is_corresponding":false},{"id":552234,"name":"Steven D. Nathan","orcid":"0000-0002-6270-1617","position":13,"is_corresponding":false},{"id":377391,"name":"Aaron B. Waxman","orcid":"0000-0001-7797-0361","position":0,"is_corresponding":true}],"reference_count":27,"raw_metadata":null,"created_at":"2026-07-19T01:06:55.884740Z","pmid":"37080567","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}