ArticleJACC. Asia2022
Changes in the Characteristics and Initial Treatments of Pulmonary Hypertension Between 2008 and 2020 in Japan.
Article in JACC. Asia, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed.
- Efficacy, Safety, and Pharmacokinetics of Macitentan in Japanese Pediatric Patients With Pulmonary Arterial Hypertension: Prospective, Multicenter, Open-Label Study.Circulation reports · 2026Article
- Clinical Practice of Pulmonary Arterial Hypertension (PAH) and Potential Barriers to Intensive Treatment Among PAH-Specialized and Non-PAH-Specialized Centers in Japan.Pulmonary circulation · 2026Article
- Healthcare Resource Utilization and Medical Costs in Pulmonary Arterial Hypertension Management in Japan: A Retrospective Study Using a Claims Database.Pulmonary therapy · 2026Article
- Phase 3, Open-Label Multicenter Study of Sotatercept in Japanese Participants With Pulmonary Arterial Hypertension.JACC. Asia · 2026Article
- Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.Biomolecules · 2026Review
- Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry.Advances in therapy · 2025Article
- Recent advances in bioactive peptides from fermented plant-based foods and their bioactivities.Food chemistry: X · 2025Review
- Sex differences in pulmonary (arterial) hypertension: does it matter?Current opinion in pulmonary medicine · 2025Review
- Diagnostic Delays and Quality of Life in Japanese Patients with Pulmonary Hypertension: A Nationwide Survey.Pulmonary therapy · 2025Article
- Treatment algorithm for pulmonary arterial hypertension.The European respiratory journal · 2024Review
- Switching from Beraprost to Selexipag in the Treatment of Pulmonary Arterial Hypertension: Insights from a Phase IV Study of the Japanese Registry (The EXCEL Study: EXChange from bEraprost to seLexipag Study).Pharmaceuticals (Basel, Switzerland) · 2024Article
- Patient and Therapeutic Profiles of Pulmonary Hypertension in Chronic Lung Diseases in Japan: A Cohort Study Using a Claims Database.Pulmonary therapy · 2024Article
- Comparative Treatment Persistence and Adherence to Endothelin Receptor Antagonists Among Patients with Pulmonary Arterial Hypertension in Japan: A Real-World Administrative Claims Database Study.Pulmonary therapy · 2023Article
- Real-world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database.Pulmonary circulation · 2023Article
- SATISFY-JP, a phase II multicenter open-label study on Satralizumab, an anti-IL-6 receptor antibody, use for the treatment of pulmonary arterial hypertension in patients with an immune-responsive-phenotype: Study protocol.Pulmonary circulation · 2023Article
- Clinical Management and Outcomes of Patients With Portopulmonary Hypertension Enrolled in the Japanese Multicenter Registry.Circulation reports · 2022Article
- Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan.JGH open : an open access journal of gastroenterology and hepatology · 2022Article
- Pulmonary Arterial Hypertension Management in Asia: State of the Art From Japan, and Next Steps.JACC. Asia · 2022Article
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Authors and funding
13 authors.
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Abstract
Background: Pulmonary arterial hypertension (PAH) is a rare, progressive disease. The treatment landscape for PAH in Japan has evolved considerably in recent years, but there is limited knowledge of the changes in treatment practices or patient characteristics. Objectives: The aim of this study was to evaluate the changes in characteristics and initial treatments for PAH in Japan over time. Methods: This study used data from the Japan Pulmonary Hypertension Registry (JAPHR) to compare patient characteristics and treatment practices between 2008-2015 (n = 316) and 2016-2020 (n = 315). Results: The mean ± standard deviation age at diagnosis increased from 47.9 ± 16.7 years in 2008-2015 to 52.7 ± 16.9 years in 2016-2020. The mean pulmonary arterial pressure decreased from 45.4 ± 15.0 to 38.6 ± 13.1 mm Hg. Idiopathic/hereditary PAH was the most common etiology in both periods (50.0% and 51.1%, respectively). The proportion of patients prescribed oral/inhaled combination therapies increased from 47.8% to 57.5%. Oral/inhaled combination therapies were frequently prescribed to patients with congenital heart disease-related PAH (81.8%). There was no significant trend in prescribing practices based on French low-risk criteria: among patients with 0, 1, 2, 3, or 4 criteria, 53.8%, 68.8%, 52.8%, 66.7%, and 39.4% were prescribed oral/inhaled combination therapies, and 0%, 16.7%, 27.0%, 17.3%, and 15.2% were prescribed oral/inhaled monotherapies. Macitentan, tadalafil, selexipag, and epoprostenol were the most frequently prescribed drugs. Conclusions: The severity of PAH decreased over time in Japan. Oral/inhaled combination therapies were generally preferred. Physicians generally prescribed therapies after considering the patients' hemodynamics and clinical severity. (Japan Pulmonary Hypertension Registry [JAPHR]; UMIN000026680).
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