{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:41:08Z","timestamp":1759970468742,"version":"build-2065373602"},"reference-count":10,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T00:00:00Z","timestamp":1737504000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background: Systemic sclerosis-associated pulmonary hypertension (SSc-PH) is widely recognized as the most severe subtype of connective tissue disease-associated pulmonary hypertension (CTD-PH), particularly in patients with complicating factors such as interstitial lung disease (ILD) and biventricular failure. This condition is associated with the poorest clinical outcomes among PH subtypes, presenting significant challenges in both management and prognosis. Despite the use of conventional therapies, including intravenous administration of epoprostenol, a promising prostacyclin analogue, treatment outcomes for SSc-PH remain suboptimal. While epoprostenol has demonstrated efficacy in reducing pulmonary arterial pressures, its clinical application is often constrained by the risk of ventilation\u2013perfusion (V-Q) mismatch, particularly at higher doses. Case presentation: We report the case of a 73-year-old woman with SSc-PH complicated by ILD, who experienced progressive hemodynamic deterioration despite receiving optimized therapy with intravenous epoprostenol. Efforts to escalate the dose of epoprostenol were limited by the development of severe V-Q mismatch, precluding further dose increases. In light of these challenges, inhaled treprostinil was introduced as an adjunctive therapy. There were significant improvements in her pulmonary hypertension and hemodynamic parameters, ultimately allowing the discontinuation of intravenous dobutamine and stabilization of her hemodynamics, as well as her respiratory function, exercise capacity, and quality of life. Conclusions: This case highlights the potential clinical utility of combining inhaled treprostinil with intravenous epoprostenol for the treatment of SSc-PH in patients with concurrent ILD. By addressing the limitations associated with high-dose intravenous prostacyclin therapy, this combination approach may represent a promising therapeutic strategy for improving outcomes in this difficult-to-treat patient population. Further investigation is warranted to establish the efficacy and feasibility of this combination therapy in larger cohorts of patients with SSc-PH and associated ILD.<\/jats:p>","DOI":"10.3390\/medicina61020184","type":"journal-article","created":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T05:50:53Z","timestamp":1737525053000},"page":"184","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Efficacy of Inhaled Treprostinil in a Patient with Systemic Sclerosis-Associated Pulmonary Hypertension and Interstitial Lung Diseases Refractory to Conventional Intravenous Epoprostenol"],"prefix":"10.3390","volume":"61","author":[{"given":"Yuki","family":"Hida","sequence":"first","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7294-7637","authenticated-orcid":false,"given":"Teruhiko","family":"Imamura","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4543-596X","authenticated-orcid":false,"given":"Ryuichi","family":"Ushijima","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichiro","family":"Kinugawa","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1055\/s-0039-1685214","article-title":"Pulmonary hypertension associated with connective tissue disease","volume":"40","author":"Fayed","year":"2019","journal-title":"Semin. Respir. Critical Care Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3043","DOI":"10.1002\/art.22069","article-title":"Clinical Differences Between Idiopathic and Scleroderma-Related Pulmonary Hypertension","volume":"54","author":"Fisher","year":"2006","journal-title":"Arthritis Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/art.41512","article-title":"Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated with Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated with Interstitial Lung Disease","volume":"73","author":"Chauvelot","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). 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