Pulmonary Therapy最新文献

{"title":"Climate Change and the Impact On Interstitial Lung Diseases.","authors":"Bineet Ahluwalia, Sheetu Singh","doi":"10.1007/s41030-026-00347-0","DOIUrl":"10.1007/s41030-026-00347-0","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review aims to summarize the latest evidence on how climate change has altered the environmental exposures and their influence on the epidemiology, pathophysiology, and outcomes of interstitial lung diseases (ILD). Rising global temperatures are exacerbating environmental threats (like heatwaves, floods, and dust storms) and worsening air quality. This burden disproportionately affects certain vulnerable groups, accelerating the decline of their ILD. Epigenetic modifications play a vital role in explaining the interaction between the environmental factors and development and progression of ILD. Establishment of strong policies is critical for both reducing the rate of climate change and implementing better adaptation strategies to protect the vulnerable group from its ongoing consequences.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"145-160"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146053500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advances in the Diagnosis and Treatment of Obstructive Sleep Apnea in Women.","authors":"Izolde Bouloukaki, Antonio Fabozzi, Esther Irene Schwarz, Sophia E Schiza","doi":"10.1007/s41030-026-00350-5","DOIUrl":"10.1007/s41030-026-00350-5","url":null,"abstract":"<p><p>Obstructive sleep apnea (OSA) in women is often underdiagnosed due to various and different symptoms, significant delay of referrals, sex-specific polysomnographic patterns that are usually not detected by standard severity indices from the home sleep apnea test, and limitations of current screening tools. Up to 75% of women with OSA remain undiagnosed, with relevant clinical and socioeconomic consequences. Women often report daytime fatigue, insomnia, depression, anxiety, and poor sleep quality rather than excessive daytime sleepiness or snoring, which may lead to fewer sleep clinic referrals. Additionally, the menstrual phase significantly influences symptom expression. Comorbidities also exhibit sex-based differences: OSA in premenopausal women is strongly linked to depression, metabolic syndrome, and polycystic ovary syndrome, while postmenopausal women with OSA reported hypertension and diabetes more frequently, leading to a greater cardiometabolic risk in postmenopausal women with OSA. The screening questionnaires showed numerous limitations in women due to the lack of items concerning symptoms. Women's typical polysomnographic pattern, especially in the premenopause period, is characterized by predominant hypopneas, mild OSA with prevalent rapid eye movement (REM)-OSA, respiratory effort-related arousals (RERAs), and low arousal threshold, highlighting the crucial role of sleep fragmentation evaluation, beyond the apnea-hypopnea index (AHI). New indices such as hypoxic burden, pulse wave amplitude drops index and arousal burden may provide more appropriate OSA severity classification and risk stratification in women.After a review of the literature, we proposed four women phenotypes, highlighting the heterogeneity of OSA in women and the key role of sex-tailored OSA management. From a therapeutic perspective, women differ in apnea-hypopnea index (PAP) compliance, required lower PAP levels for the same disease severity as men, and experience mask-related side effects. However, we have to mention that this is suspected to be biased due to significant lower number of women included in cohorts and even lower in randomized controlled trials (RCTs). Mandibular advancement devices (MADs) and endotype-based pharmacotherapy may be beneficial in women with mild OSA and low arousal threshold or low muscle responsiveness. Emerging evidence suggests that a sex-centered approach to screening, diagnosis, and treatment may reduce the clinical and socioeconomic burden of OSA in women in the future.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"181-199"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992857/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147309517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emerging Therapies in Pulmonary Fibrosis.","authors":"Hugh Etchingham-Coll, Ekrem Temizel, Neso Okezie-Enyioma, Nieve Martin, Punchalee Kaenmuang, Sean Coghlan, Paolo Spagnolo, Nazia Chaudhuri","doi":"10.1007/s41030-026-00349-y","DOIUrl":"10.1007/s41030-026-00349-y","url":null,"abstract":"<p><p>Interstitial lung diseases (ILD) are a heterogenous group of respiratory disorders with varying degrees of inflammation and fibrosis. Idiopathic pulmonary fibrosis (IPF), the commonest and most debilitating type of ILD, is a chronic, progressive disease of the respiratory system characterized by fibrosis of the alveolar interstitium. Subsequent, relentless decline in lung function leads to progressive breathlessness and respiratory failure. Treatment options for IPF have remained mostly unchanged in the last decade, with the availability of two antifibrotic therapies: pirfenidone and nintedanib. Recently, the US Food and Drugs Administration (FDA) approved nerandomilast for the management of IPF and progressive pulmonary fibrosis. Nintedanib is also globally approved for the treatment of progressive non-IPF ILDs. While these therapies have been shown to reduce the decline of lung function, they do not reverse existing lung damage or fully address the complex pathophysiology of pulmonary fibrosis (PF). Accordingly, research in the field has shifted to developing new therapies with improved efficacy and minimal adverse effects that directly target the intricate pathogenesis in PF with the aim of arresting or reversing the disease. This review article will set the scene by first describing the pathogenesis and prevalence of ILDs, followed by exploring the current and emerging therapies in the field.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"161-180"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992858/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147285087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fungal-Associated Endotypes as a Treatable Trait in Bronchiectasis.","authors":"Kai Xian Thng, Micheál Mac Aogáin, Sanjay H Chotirmall","doi":"10.1007/s41030-025-00339-6","DOIUrl":"10.1007/s41030-025-00339-6","url":null,"abstract":"<p><p>Emerging evidence demonstrates the evolving role of fungi in the pathophysiology and disease progression observed in bronchiectasis. Fungal-associated traits are linked to disease severity, exacerbation frequency and airway inflammation. Structural abnormalities and impaired mucociliary clearance, characteristic of bronchiectasis, predispose to fungal colonisation, with subsequent immunopathogenic responses dependent on underlying host immunity. The diagnosis of fungal infection remains challenging in clinical settings, owing to the limitations of existing diagnostic modalities; however, the development of culture-independent molecular techniques shows promise. The use of next-generation sequencing has significantly advanced our understanding of the fungal microbiome in bronchiectasis, identifying fungi that are challenging to culture. Integrative microbiomics further elucidates the intricate and dynamic role of fungi in relation to other microbial kingdoms, and across distant organs such as the gut, revealing important relationships with bacterial pathogens including Pseudomonas aeruginosa. Airway inflammatory profiling has shown fungal-associated inflammatory endotypes which may serve as treatable traits. Environmental influences on fungi and bronchiectasis-exacerbated by air pollution and climate change-underscore the key role of the exposome in fungal-associated endotypes in bronchiectasis. This review outlines the clinical significance of fungi in bronchiectasis, the current diagnostic and treatment challenges, and emerging fungal-associated endotypes in the context of environmental influence on disease.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"79-98"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992841/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145857475","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Hidden Burden of COMISA: Clinical Implications and Treatment Challenges.","authors":"Cristian Popovici, Paschalis Steiropoulos, Stefan Mihaicuta, Silvia Dumitru, Sofia Dragila, Andras Bikov, Alexandru Corlateanu","doi":"10.1007/s41030-025-00331-0","DOIUrl":"10.1007/s41030-025-00331-0","url":null,"abstract":"<p><strong>Background: </strong>Insomnia disorder and obstructive sleep apnea (OSA) are two of the most common sleep disorders in the general population. Their coexistence, referred to as comorbid insomnia and sleep apnea (COMISA), exacerbates nighttime disturbances, increases daytime dysfunction, and further diminishes overall quality of life. COMISA is also associated with elevated cardiovascular and mental health risks, including resistant hypertension, heart failure, stroke, depression, and anxiety. Despite its clinical importance, COMISA often remains underdiagnosed, and its management poses notable challenges.</p><p><strong>Objectives: </strong>This narrative review article aims to provide a comprehensive overview of the current understanding of COMISA, focusing on its definition, epidemiology, pathophysiological mechanisms, clinical presentation, diagnostic challenges and treatment approaches, including the diagnostic decision-making process and criteria for selecting appropriate therapeutic strategies.</p><p><strong>Methods: </strong>Literature review of published studies with different designs, including peer-reviewed observational studies, randomized controlled trials, meta-analyses, and international clinical guidelines, was conducted. Databases used included PubMed, Scopus, and Web of Science, and they covered epidemiological, clinical, and therapeutic topics focusing on COMISA. Emphasis was placed on the cardiovascular, psychological, and therapeutic outcomes, as reported in the cited literature.</p><p><strong>Results: </strong>Patients diagnosed with COMISA typically present with symptoms such as greater sleep fragmentation, prolonged sleep latency, persistent daytime fatigue, cognitive deficits, and elevated psychological distress, compared with individuals with insomnia or OSA alone. COMISA is also associated with a threefold increased risk of resistant hypertension and heightened cardiovascular mortality. Despite the fact that continuous positive airway pressure (CPAP) remains the cornerstone of OSA treatment, its effectiveness is often limited by comorbid insomnia, making combined cognitive behavioral therapy for insomnia (CBT-I) and CPAP interventions more promising, as well as emerging pharmacotherapies, such as dual orexin receptor antagonists. Patients with significant insomnia-related impairment or suboptimal CPAP adherence may benefit from these combined or alternative approaches.</p><p><strong>Conclusions: </strong>COMISA represents a complex clinical entity that is associated with impairments in clinical outcomes and quality of life, requiring a multidisciplinary, personalized approach to simultaneously address sleep-disordered breathing and insomnia symptoms. Early recognition, individualized treatment strategies, and long-term monitoring are essential to improve prognosis and therapeutic success in this high-risk population.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"25-38"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992872/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145496518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hyperbaric Oxygen Therapy and Its Physio-Mechanical Effects on Sleep Breathing Disorder: A Systematic Review.","authors":"Sy Duong-Quy, Tran V Hoc, Thai Nguyen-Duy, Tram Tang-Thi-Thao, Toi Nguyen-Van, Tuan Huynh-Anh, Trung Mai-Xuan, Phap Tran-Quang, Viet Nguyen-Ba, Bang Nguyen-Trong, Thu Nguyen-Ngoc-Phuong, Hai Doan-Ngoc, Giap Vu-Van, Nhung Nguyen-Viet, Franck Soyez, Francis Martin, Thomas Penzel, Clete Kushida, Timothy Craig","doi":"10.1007/s41030-025-00335-w","DOIUrl":"10.1007/s41030-025-00335-w","url":null,"abstract":"<p><strong>Introduction: </strong>Hyperbaric oxygen therapy (HBOT), traditionally used for decompression sickness and chronic wounds, has recently attracted interest for its potential role in sleep breathing disorders (SBD). Because sleep is highly sensitive to oxygen homeostasis, altered oxygenation can significantly disrupt sleep architecture and efficiency.</p><p><strong>Method: </strong>This review examines emerging evidence on HBOT's effects in modulating sleep physiology and alleviating major SBD phenotypes, including obstructive sleep apnea (OSA), central sleep apnea (CSA), and high-altitude-related breathing disorders. HBOT may exert therapeutic benefits through several mechanisms-reducing inflammation and oxidative stress, reversing tissue hypoxia, improving pulmonary function and oxygenation, enhancing neurocognitive function, modulating arousal threshold and loop gain, and influencing brain regions involved in sleep regulation. These physiological pathways provide a rationale for considering HBOT as an adjunctive or alternative therapy, especially for patient's intolerance of conventional treatments. This systematic review aims to synthesize existing evidence on the effects of HBOT in SBD, particularly OSA, CSA, and altitude-related sleep disturbances.</p><p><strong>Conclusion: </strong>Current studies offer promising but preliminary evidence supporting HBOT's role in selected SBD populations. However, heterogeneity in protocols, small sample sizes, and limited long-term follow-up constrain interpretation. Future multicenter trials should focus on optimizing treatment pressure, duration, and patient selection, while ensuring safety across vulnerable populations. Understanding the interactions among hyperoxia, neurophysiology, and sleep regulation could unlock novel therapeutic directions for refractory or comorbid SBD.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"39-55"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992743/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145638047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Misconceptions of Traits to Predict Response to Inhaled Corticosteroid and Bronchodilator Therapies in Asthma: A Narrative Review.","authors":"Guy Brusselle, Jodie Crawford, Peter G Gibson, David Leather, Alison Moore, John J Oppenheimer, Ian D Pavord, Marcus Stanaland, Emilio Pizzichini","doi":"10.1007/s41030-025-00323-0","DOIUrl":"10.1007/s41030-025-00323-0","url":null,"abstract":"<p><p>The \"treatable traits\" approach to asthma management has helped revolutionize severe asthma treatment with biologic therapy and includes using biomarkers to identify patients most likely to benefit from a specific treatment. The ability to understand which characteristics predict response to inhaled corticosteroid (ICS) or bronchodilator therapy in mild and moderate-to-severe asthma is also vital for physicians to provide treatment tailored to an individual's phenotype/endotype. Here, we identified studies of inhaled treatments in asthma exploring treatment outcomes based upon subgroups of baseline characteristics, including type 2 biomarkers, asthma attack history, baseline lung function, bronchodilator reversibility, patient age and age at asthma onset, body mass index, smoking status, sex, and ethnicity. We assessed the available evidence regarding the influence of each characteristic on lung function, asthma attacks or asthma control in patients with asthma following treatment with either ICS, ICS/long-acting β₂-agonist (LABA) therapy, or ICS/LABA/long-acting muscarinic antagonist therapy. Of all the characteristics examined, only type 2 biomarkers (blood eosinophil levels and fractional exhaled nitric oxide) appear to consistently predict treatment response, particularly regarding ICS. For all other characteristics, we found either evidence that baseline values are not predictive of response to inhaled treatment or mixed and inconclusive evidence requiring further investigation.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"57-78"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992740/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145649026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction: Dupilumab Versus Mepolizumab for COPD: Evaluating Efficacy Outcomes Using Placebo-Adjusted Indirect Treatment Comparison.","authors":"Surya P Bhatt, Nick Freemantle, Mena Soliman, Jigna Heble, Yann Cabon, Ernesto Mayen Herrera, Joe Yang, Yingxin Xu","doi":"10.1007/s41030-025-00332-z","DOIUrl":"10.1007/s41030-025-00332-z","url":null,"abstract":"","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"219"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145550314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Symptomatic Sarcoidosis Treated with Acthar^® Gel: Insights from a Physician-Reported Chart Review.","authors":"Amit Patel, Priyanka P Shanbhag, Destri R Evans, Kyle Hayes, Mary P Panaccio, Johanna Purcell, George J Wan","doi":"10.1007/s41030-026-00344-3","DOIUrl":"10.1007/s41030-026-00344-3","url":null,"abstract":"<p><strong>Introduction: </strong>Real-world data on evolving treatment patterns of Acthar Gel, a Food and Drug Administration-approved therapy for patients with symptomatic sarcoidosis, have remained limited. This study described the characteristics of patients with symptomatic sarcoidosis treated with Acthar Gel, medication utilization patterns, and physicians' assessments of the effects of Acthar Gel on patients' health status.</p><p><strong>Methods: </strong>A prospectively designed medical chart review study with a predefined protocol and analysis plan was conducted in November 2024, with data abstracted from patient records between April 2022 and November 2024. Eligible patients were aged ≥ 18 years, had symptomatic sarcoidosis, and had received Acthar Gel within ≤ 24 months.</p><p><strong>Results: </strong>On average, patients with symptomatic sarcoidosis were 47 years old; most were men (56%, 22/39) and African American (46%, 18/39). Common comorbidities included chronic joint disease, hypertension, and arthritis/osteoarthritis. Before receiving Acthar Gel, physicians reported that 56% (22/39) of patients had a fair-to-poor health status (average rating 3.4/5, where 1 = excellent and 5 = poor). Frequent symptoms were fatigue, joint pain, and muscle aches or weakness. Before initiating Acthar Gel, most patients with symptomatic sarcoidosis had previously received treatment with corticosteroids (82%, 32/39), immunosuppressive drugs (51%, 20/39), and biologic disease-modifying antirheumatic drugs (51%, 20/39). Based on physician assessment, 92% (36/39) of patients experienced improved health after Acthar Gel treatment. Improvements included reduced overall symptoms (69%, 25/36), improved physical function (47%, 17/36), decreased pain (44%, 16/36), improved fatigue (36%, 13/36), reduced corticosteroid use (33%, 12/36), improved lung functioning (25%, 9/36), and improved strength (22%, 8/36).</p><p><strong>Conclusions: </strong>Based on this chart review, Acthar Gel may represent a potential treatment option for selected patients with symptomatic sarcoidosis. In this study, among patients with symptomatic sarcoidosis treated with Acthar Gel, physicians documented reduced overall symptoms, improved physical function, decreased pain, improved fatigue, reduced corticosteroid use, improved lung functioning, and improved strength using prespecified assessments.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"385-395"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992844/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146143246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Healthcare Resource Utilization and Medical Costs in Pulmonary Arterial Hypertension Management in Japan: A Retrospective Study Using a Claims Database.","authors":"Atsushi Tajima, Yoko Arai, Takao Nakamura, Shigeru Tokita, Anna Watzker, Takumi Sugiyama, Noriaki Emoto","doi":"10.1007/s41030-026-00343-4","DOIUrl":"10.1007/s41030-026-00343-4","url":null,"abstract":"<p><strong>Introduction: </strong>To investigate the healthcare resource utilization (HCRU), medical costs in pulmonary arterial hypertension (PAH) patients in Japan, implementation of right heart catheterization (RHC), and treatment intensity, a retrospective observational cohort study using a nationwide health insurance claims database provided by JMDC Inc. was conducted.</p><p><strong>Methods: </strong>Patients with PAH, classified based on evidence of a PAH diagnosis and PAH-targeted drug prescription within the same calendar month, were identified from the database. Demographic and clinical characteristics, as well as the implementation of RHC during the baseline period and the type of PAH-targeted drugs prescribed for the first-line therapy, were described. Outcomes included HCRU and direct medical costs between March 1, 2018 and March 31, 2023.</p><p><strong>Results: </strong>A total of 405 patients with PAH were included in the analyses; 298 patients (73.6%) did not undergo RHC [RHC(-) cohort], and 107 patients (26.4%) underwent RHC [RHC(+) cohort] during the baseline period. Among the overall population, 59.3% received oral/inhaled monotherapy and 5.4% received parenteral prostacyclin analogue (PPA) combination therapy; 70.1% of the RHC(-) cohort received oral/inhaled monotherapy versus 29.0% in the RHC(+) cohort, 1.7% of the RHC(-) cohort received PPA combination therapy versus 15.9% in the RHC(+) cohort. Overall, the HCRU and the total medical costs tended to increase with treatment intensity in both RHC(-) and RHC(+) cohorts. The increase of the medical costs was mainly due to increase in costs related to hospitalization and prescription of PAH-targeted drugs.</p><p><strong>Conclusion: </strong>The results revealed that HCRU and medical costs were especially high in those who were treated intensively with PPA combination therapy at first-line. In terms of extensive HCRU and associated medical costs, the limitations of existing treatment strategies for PAH were noted. In addition, these results highlight the need for innovative treatments to reduce the considerable disease burden described in this study.</p>","PeriodicalId":20919,"journal":{"name":"Pulmonary Therapy","volume":" ","pages":"365-384"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992881/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146202335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}