Shelsey W Johnson, Farbod N Rahaghi, Raul San Jose Estépar
{"title":"The clinical relevance of chest computed tomography imaging in pulmonary hypertension associated with lung diseases.","authors":"Shelsey W Johnson, Farbod N Rahaghi, Raul San Jose Estépar","doi":"10.1097/MCP.0000000000001282","DOIUrl":"10.1097/MCP.0000000000001282","url":null,"abstract":"<p><strong>Purpose of review: </strong>The purpose of this review is to describe the use of chest computed tomography (CT) data to expand sub-phenotyping of pulmonary hypertension associated with chronic lung disease (Group 3 pulmonary hypertension) and inform identification of effective treatment.</p><p><strong>Recent findings: </strong>In the last 5 years, breakthroughs in clinical pulmonary hypertension research highlight the importance of chest CT data to inform disease trajectory and mortality risk. In presumed idiopathic pulmonary arterial hypertension (Group 1 pulmonary hypertension), a Group 1 'lung phenotype' with abnormal CT chest findings such as emphysema and fibrosis, experiences a mortality risk similar to that of Group 3 disease. Post hoc analyses of Group 3 pulmonary hypertension clinical trials highlight CT abnormalities to inform adverse treatment response, with implications for additional failed clinical trials to date. In fact, pulmonary hypertension guidelines emphasize the critical role for acquisition of standardized high-resolution CT data at pulmonary hypertension diagnosis and clinical trial enrollment.</p><p><strong>Summary: </strong>Chest CT imaging is critical in the clinical management of pulmonary hypertension associated with chronic lung disease. Future research will not only incorporate CT data into Group 3 pulmonary hypertension phenotyping research but also consider treatment effect visualization on the lung parenchyma and pulmonary arterial vasculature as a novel clinical trial endpoint.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"365-371"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13270433/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147834062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Association between upper airway obstruction and pulmonary hypertension in children with Down syndrome.","authors":"Kevin Guy, Amal Isaiah","doi":"10.1097/MCP.0000000000001287","DOIUrl":"10.1097/MCP.0000000000001287","url":null,"abstract":"<p><strong>Purpose of review: </strong>Down syndrome (DS) is the most common nonlethal chromosomal aneuploidy, affecting 1 in 700 live births. Pulmonary hypertension (PH) occurs in approximately 25% of children with DS and contributes to a 10% mortality rate within 3 years of diagnosis. Despite obstructive sleep apnea (OSA) affecting up to 80% of children with DS, the specific contribution of upper airway obstruction to PH in this population remains poorly characterized. This review synthesizes current evidence to address that gap.</p><p><strong>Recent findings: </strong>Children with DS develop multilevel upper airway obstruction due to craniofacial dysmorphology, relative macroglossia, hypotonia, and reduced peripheral chemosensitivity. OSA-driven chronic hypoxemia promotes pulmonary vascular remodeling, with 87% of recurrent PH cases classified as WHO Group III. Echocardiographic diagnosis is limited by chronic lung disease. NT-proBNP is the most reliable biomarker in DS. Adenotonsillectomy reduces apnea severity, though evidence of a reduction in mean pulmonary arterial pressure in DS is lacking. Endothelin receptor antagonists show functional benefit, while sildenafil appears less effective in DS-specific analyses.</p><p><strong>Summary: </strong>No DS-specific PH staging system exists, representing a critical gap. Future research should quantify the impact of airway interventions on pulmonary hemodynamics, develop DS-tailored biomarkers, and optimize pharmacologic regimens in this molecularly distinct population.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"386-393"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13294707/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148027906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nonsteroid treatment options in (pulmonary) sarcoidosis. When to consider and why?","authors":"Paolo Spagnolo, Jelle Miedema","doi":"10.1097/MCP.0000000000001293","DOIUrl":"10.1097/MCP.0000000000001293","url":null,"abstract":"<p><strong>Purpose of review: </strong>Sarcoidosis requires treatment in 30-50% of cases. According to current guidelines, corticosteroids (CS) are the initial drugs of choice, particularly for patients with major organ involvement or when rapid symptom relief is required. However, CS use is associated with substantial toxicity even at low dose. Here, we discuss the evidence that argues against the traditional CS-centred model of sarcoidosis treatment.</p><p><strong>Recent findings: </strong>A recent prospective trial of patients with pulmonary sarcoidosis showed that high-dose (40 mg) prednisolone was not superior to a lower dose (20 mg) in improving outcomes or health-related quality of life and was associated with similar adverse effects. In addition, in patients with pulmonary sarcoidosis, initial treatment with methotrexate has been shown to be noninferior to prednisone with regard to the change from baseline to week 24 in the percentage of the predicted forced vital capacity (FVC).</p><p><strong>Summary: </strong>The appreciation of CS adverse effects coupled with the observation that methotrexate is as effective as prednisone as initial treatment in pulmonary sarcoidosis supports a paradigm shift in the treatment of pulmonary sarcoidosis that moves away from CS. Differences in the side-effect profile between methotrexate and prednisone may inform shared decision making by providers and patients about the appropriate treatment approach.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"463-470"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13566476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148375011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Brandon Budhram, Sébastien Bonnet, Jason Weatherald
{"title":"Promising signalling pathways for the treatment of pulmonary arterial hypertension.","authors":"Brandon Budhram, Sébastien Bonnet, Jason Weatherald","doi":"10.1097/MCP.0000000000001278","DOIUrl":"10.1097/MCP.0000000000001278","url":null,"abstract":"<p><strong>Purpose of review: </strong>Improved understanding of the complex and interconnected mechanisms driving pulmonary arterial hypertension (PAH) has expanded therapeutic development beyond the traditional vasodilator pathways. This review summarizes recently established and emerging signalling pathways that may influence the next generation of targeted PAH therapies.</p><p><strong>Recent findings: </strong>The transforming growth factor-β (TGF-β) superfamily has emerged as a fourth major therapeutic pathway. Therapies that target this pathway, such as the activin signalling inhibitor sotatercept, have demonstrated significant clinical and hemodynamic benefits in large randomized clinical trials. Additional promising strategies focus on receptor tyrosine kinases - particularly platelet-derived growth factor receptor signalling - as well as hypoxia-related and metabolic reprogramming pathways. Growing evidence also supports the role of immune dysregulation, hormonal and neurohormonal signalling, and epigenetic, genetic, and cell-cycle abnormalities in the development of PAH.</p><p><strong>Summary: </strong>The therapeutic landscape in PAH is shifting toward mechanism-based therapies with the potential to modify disease progression. Continued translational research and upcoming clinical trials will be essential to define appropriate patient selection, optimize therapeutic strategies, and determine the short- and long-term benefit of these therapies on clinical and survival outcomes.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"410-423"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Molecular mechanisms of environmental risk factors for interstitial lung disease.","authors":"Gillian C Goobie","doi":"10.1097/MCP.0000000000001286","DOIUrl":"10.1097/MCP.0000000000001286","url":null,"abstract":"<p><strong>Purpose of review: </strong>Interstitial lung disease (ILD) comprises a diverse group of conditions characterized by lung inflammation and fibrosis. Cumulative lifetime exposures (i.e. the exposome) contribute to ILD onset and progression by interacting with genetic susceptibility and influencing multiple molecular pathways. This review summarizes current evidence evaluating how environmental exposures interact across the genome, epigenome, transcriptome, proteome, metabolome, and microbiome to drive ILD pathogenesis.</p><p><strong>Recent findings: </strong>Environmental exposures, including air pollution, influence ILD risk through interactions with genetic factors that modify disease susceptibility. Epigenetic mechanisms, particularly DNA methylation, reflect key pathways through which exposures may contribute to ILD onset and progression and serve as sensitive biomarkers of environmental injury. Exposure-associated molecular alterations can be detected across multiple omic layers, including transcriptomic, proteomic, and metabolomic profiles. In parallel, exposures like cigarette smoking, silica, and air pollution influence the respiratory microbiome, with potential downstream effects on immune responses and fibrogenesis. Integrating these findings highlights environmentally-sensitive pathways that may represent novel targets for therapeutic modulation.</p><p><strong>Summary: </strong>Integration of exposomic and multiomic molecular frameworks offers new opportunities to improve ILD risk stratification, prognostication, and precision therapeutic development, while also strengthening our mechanistic understanding of environmentally-mediated disease.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"432-438"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148027953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sabina A Guler, Yet H Khor, Anne E Holland, Magnus Ekström
{"title":"Oxygen therapy in interstitial lung disease - navigating benefit and burden.","authors":"Sabina A Guler, Yet H Khor, Anne E Holland, Magnus Ekström","doi":"10.1097/MCP.0000000000001288","DOIUrl":"10.1097/MCP.0000000000001288","url":null,"abstract":"<p><strong>Purpose of review: </strong>Oxygen therapy is a key component of interstitial lung disease (ILD) management. This review summarizes the current evidence and explores how to balance its benefits and burdens in people with ILD.</p><p><strong>Recent findings: </strong>Hypoxemia frequently occurs in ILD and is associated with worse prognosis, but the extent to which oxygen therapy modifies outcomes remains uncertain. Observational findings suggest home oxygen therapy may reduce acute exacerbations and hospitalizations in people with ILD with a life-expectancy of more than 1 year. Ambulatory oxygen can improve symptoms and health-related quality of life in some patients; however, recent evidence indicates that portable oxygen concentrators may not improve daily oxygenation, symptoms, or physical activity. In patients with isolated exertional or nocturnal desaturation or significant respiratory symptoms with hypoxemia, careful consideration of individual benefit versus burden is essential within a shared decision-making framework. High-flow oxygen therapy is effective for acute respiratory failure, but its role in palliative care, pulmonary rehabilitation, and especially in the home environment, are areas of ongoing investigation.</p><p><strong>Summary: </strong>Oxygen therapy can support symptom relief in ILD, but its impact on long-term outcomes is unclear. High-quality evidence remains sparse, and advances in oxygen delivery technologies are needed to improve effectiveness while minimizing burden.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"456-462"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13566398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148216744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Oscar van der Have, Rachel K Hopper, Rebecca J Kameny, Karin Tran-Lundmark
{"title":"Recent advances in the clinical management of end-stage pediatric pulmonary hypertension.","authors":"Oscar van der Have, Rachel K Hopper, Rebecca J Kameny, Karin Tran-Lundmark","doi":"10.1097/MCP.0000000000001283","DOIUrl":"10.1097/MCP.0000000000001283","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review aims to give an overview of recent advances in the clinical management of children <18 years of age with end-stage pulmonary hypertension refractory to conventional medical therapy.</p><p><strong>Recent findings/summary: </strong>The increased awareness and characterization of pediatric pulmonary vascular disease has amounted more data toward creating an accurate, and comprehensive, risk prediction tool. This underscores the importance of serial outpatient re-assessment, as early consideration of advanced pharmaceutical and interventional therapies, as well as lung transplantation, improves outcomes in children. Despite the emergence and approval of new therapies against pulmonary hypertension in adults, pediatric-specific complications have not yet been adequately explored. It is, however, encouraging that several trials are ongoing to address this issue. Interventional strategies to unload the right ventricle are increasingly being utilized, although evidence remains scarce in this area. Extra-corporeal membrane oxygenation may, in addition to being a bridge to lung transplantation in well-selected patients, be considered as a bridge to catheter-based or surgical interventions. Management of pediatric right ventricular failure in the setting of advanced pulmonary hypertension is a truly complex clinical challenge at the intersection of cardiac, vascular and respiratory physiology and requires careful integration of invasive hemodynamic assessment, pulmonary vasodilator therapy, ventilatory strategy, and timely consideration of mechanical circulatory support.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"402-409"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147970963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Florence Jeny, Sophie Brun, Stéphane Tran Ba, Yurdagül Uzunhan
{"title":"Chronic pulmonary aspergillosis and sarcoidosis.","authors":"Florence Jeny, Sophie Brun, Stéphane Tran Ba, Yurdagül Uzunhan","doi":"10.1097/MCP.0000000000001299","DOIUrl":"10.1097/MCP.0000000000001299","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review addresses the intersection between chronic pulmonary aspergillosis (CPA) and sarcoidosis, a clinically important but relatively understudied association. While few new sarcoidosis-specific data have emerged, recent advances in CPA research across broader underlying conditions (particularly structural lung diseases) provide updated insights into diagnosis, antifungal therapy, and management strategies that can be extrapolated to sarcoidosis.</p><p><strong>Recent findings: </strong>CPA affects ~2% of sarcoidosis patients in tertiary cohorts and arises from combined structural lung damage, immune dysfunction, and environmental exposure. Diagnosis relies on integrated imaging, Aspergillus-specific immunoglobulin G, and new microbiological tools. Recent data refine therapeutic strategies regarding triazole selection, treatment duration, and salvage therapies. Management of haemoptysis relies on bronchial artery embolization, with emerging adjuncts such as local antifungal therapies and endobronchial valves. In addition, new epidemiological data have better characterized severe forms of CPA and refined estimates of mortality, highlighting a substantial disease burden. Serological markers may help predict relapse, and updated consensus criteria standardize response assessment.</p><p><strong>Summary: </strong>CPA in sarcoidosis reflects advanced fibrocystic disease with complex host-pathogen interactions. Early recognition, optimized antifungal therapy, careful adjustment of immunosuppression, and structured haemoptysis management are central to care. Prospective sarcoidosis-specific studies are still needed to refine long-term strategies.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"508-517"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
James Tadjkarimi, Evelyn Lynn, Sujal Desai, Vasileios Kouranos
{"title":"Recent updates in advanced pulmonary sarcoidosis.","authors":"James Tadjkarimi, Evelyn Lynn, Sujal Desai, Vasileios Kouranos","doi":"10.1097/MCP.0000000000001295","DOIUrl":"10.1097/MCP.0000000000001295","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review describes recent developments in the phenotyping, monitoring, and treatment of advanced pulmonary sarcoidosis (APS), highlighting the positive strides made to recognise and treat this severe form of disease.</p><p><strong>Recent findings: </strong>Key to this is the use of recently described high-resolution chest computed tomography (HRCT)-based phenotyping, the prognostic use of fibrosis extent coupled with pulmonary function tests, early echocardiography, and the increasing application of FDG-PET CT as a functional marker of inflammatory burden. Treatment paradigms have changed, with a shift from excessive corticosteroid use to steroid-sparing agents with less cumulative toxicity. Sarcoidosis-associated pulmonary hypertension (SAPH) is a well described lethal complication of APS with multiple potential underlying pathogenetic mechanisms. Directed therapies are dependent on the underlying mechanism but often vasodilators would be trialled given the multifactorial cause of the disease once SAPH has been confirmed.</p><p><strong>Summary: </strong>APS is used to describe end-stage sequelae of pulmonary sarcoidosis, including established fibrotic lung disease with or without active inflammation and sarcoidosis-associated pulmonary hypertension. These manifestations are associated with significant morbidity and mortality, underscoring the need for early assessment, multidisciplinary involvement and ongoing vigilance.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"491-499"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148497259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Functional testing for interstitial lung disease in 2026.","authors":"Leona Dowman, Emma Marshall, Yet H Khor","doi":"10.1097/MCP.0000000000001285","DOIUrl":"10.1097/MCP.0000000000001285","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review synthesizes the role of functional testing in the care of interstitial lung disease (ILD), evaluating the clinical utility of current approaches and discussing innovative strategies that may enhance patient monitoring and management.</p><p><strong>Recent findings: </strong>The 6-min walk test (6MWT) remains the most studied and comprehensive assessment capturing functional and prognostic information, as well as outcomes related to interventions and treatments, in people with ILD. Step tests and sit-to-stand tests offer space-efficient alternatives with clinically relevant functional and prognostic insights. Current approaches are limited by accessibility and the ability to reflect patients' real-world performance. Advances in digital health and remote monitoring offer new opportunities. Physical activity trackers are widely available with step count and physical activity levels being associated with mortality, and are increasing used to evaluate effectiveness of pharmacological and nonpharmacological interventions. Smart home technologies is an emerging approach with potential for monitoring functional status, although evidence is limited.</p><p><strong>Summary: </strong>Functional performance in people with ILD can be assessed using a range of approaches. With further research, remote functional testing, physical activity monitoring, and smart home technologies are poised to optimize the assessment of functional status in people with ILD.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"448-455"},"PeriodicalIF":2.7,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147970973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}