Curr Opin Pulm Med [JOURNAL]
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200 papers
Current hot topics in pulmonary hypertension.
Zeder K, Kovacs G
Curr Opin Pulm Med · 2025 Sep · PMID 40767088 · Publisher ↗
Abstract loading — click title to view on PubMed.
Echocardiographic evaluation of the pulmonary circulation and right ventricular functional unit during exercise and its clinical value.
John T, Roschinsky T, Douschan P
Curr Opin Pulm Med · 2025 Sep · PMID 40698754 · Publisher ↗
PURPOSE OF REVIEW: In this review, we provide an overview of the echocardiographic evaluation of the pulmonary circulation - right ventricular functional unit during exercise and its clinical value. RECENT FINDINGS: An i...
PURPOSE OF REVIEW: In this review, we provide an overview of the echocardiographic evaluation of the pulmonary circulation - right ventricular functional unit during exercise and its clinical value. RECENT FINDINGS: An increased understanding on the physiological response of pulmonary hemodynamics during exercise and evidence of the impact of an abnormal mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope on survival led to the reintroduction of exercise pulmonary hypertension (EPH), defined as a mPAP/CO slope >3 mmHg/l/min, in the current European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines for pulmonary hypertension. Additionally, noninvasive exercise echocardiography may enable easy and readily available assessment of pulmonary exercise hemodynamics. SUMMARY: Impaired pulmonary exercise hemodynamics are considered early hemodynamic signs of cardiopulmonary disease. In order to diagnose EPH, exercise right heart catheterization (RHC) is needed, which is invasive, highly cost- and staff intensive and reserved to expert centers. Noninvasive surrogates derived from exercise echocardiography might be an appropriate alternative to invasive RHC.
Deciphering sarcoidosis immunopathogenesis through systems biology.
Jbeli AH, Crouser ED, Bhargava M
Curr Opin Pulm Med · 2025 Sep · PMID 40671562 · Publisher ↗
PURPOSE OF REVIEW: Sarcoidosis is a complex, multisystem disease characterized by granulomatous inflammation and variable clinical outcomes. Its pathogenesis and progression are driven by intricate biological interaction...
PURPOSE OF REVIEW: Sarcoidosis is a complex, multisystem disease characterized by granulomatous inflammation and variable clinical outcomes. Its pathogenesis and progression are driven by intricate biological interactions, involving a complex interplay between patient-specific factors such as genetic background, sex, and environmental exposures, as well as epigenetic modifications that regulate gene expression and protein levels. These interconnected layers ultimately drive immune response to yet unidentified trigger(s), culminating in granuloma formation and, in some cases, with an aberrant repair response leading to irreversible organ dysfunction in some cases. In this review, we aim to synthesize recent multiomics research that unravels the underlying biological networks, offering a systems-level understanding of sarcoidosis. RECENT FINDINGS: Recent studies have identified several potential robust biomarkers, including microRNAs, CD14, LBP, HBEGF, eNAMPT, and ANG-2, while also highlighting the central role of the PI3K/AKT pathway in immune activation. Additionally, new noninvasive methods, such as extracellular vesicle profiling, have emerged as promising alternatives to traditional tissue biopsies. SUMMARY: We highlight recent findings from transcriptomics, epigenomics, and proteomics. These studies illuminate key molecular pathways that may be crucial in sarcoidosis pathogenesis, offering promising opportunities to identify novel therapeutic targets that could transform clinical management and improve patient outcomes.
PROMs in sarcoidosis - what to use for systemic disease and individual organs and why?
Ravaglia C
Curr Opin Pulm Med · 2025 Sep · PMID 40671555 · Publisher ↗
PURPOSE OF REVIEW: This review provides a critical evaluation of patient-reported outcome measures (PROMs) in sarcoidosis, focusing on their use in assessing systemic disease and organ-specific involvement. We aim to sum...
PURPOSE OF REVIEW: This review provides a critical evaluation of patient-reported outcome measures (PROMs) in sarcoidosis, focusing on their use in assessing systemic disease and organ-specific involvement. We aim to summarize current tools, identify evidence gaps, and explore the evolving role of PROMs in both clinical research and practice. RECENT FINDINGS: generic instruments such as the SF-36, EQ-5D, and PROMIS have been widely applied in sarcoidosis cohorts, yet their lack of disease specificity limits their interpretability and responsiveness. Sarcoidosis-specific tools [including the Sarcoidosis Health Questionnaire (SHQ), the King's Sarcoidosis Questionnaire (KSQ), and the Fatigue Assessment Scale (FAS)] offer greater construct validity and capture symptoms more relevant to patient experience. On the other hand, PROMs for cardiac, neurologic, ocular, and hepatic involvement remain underdeveloped. PROMs are increasingly incorporated into clinical trials but are rarely used in routine care, partly due to challenges in implementation, interpretation, and integration into workflows. Technological innovations such as computer-adaptive testing and ePROMs offer promising solutions. SUMMARY: PROMs are essential for capturing the subjective burden of sarcoidosis, particularly in domains poorly reflected by physiologic measures. Further work is needed to expand validation across phenotypes, develop organ-specific tools, and embed PROMs into clinical decision-making and regulatory frameworks.
Lung ultrasound as a screening tool for interstitial lung disease in patients with rheumatoid arthritis: state of the art.
Otaola M, Davidsen JR
Curr Opin Pulm Med · 2025 Sep · PMID 40643573 · Publisher ↗
PURPOSE OF REVIEW: This review describes the properties and examines available evidence supporting LUS as a screening tool for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). ILD is a common a...
PURPOSE OF REVIEW: This review describes the properties and examines available evidence supporting LUS as a screening tool for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). ILD is a common and serious complication of RA, associated with high mortality rates, especially when diagnosed late. Despite its high prevalence and significant prognostic impact, the need for and approach to systematic screening for lung involvement in RA remain unclear and are not recommended in current guidelines. While high-resolution computed tomography (HRCT) is the gold standard for diagnosing ILD, it cannot be frequently repeated due to limitations in the availability, cost, and radiation exposure. RECENT FINDINGS: Lung ultrasound (LUS) has emerged as a potential noninvasive, accurate, low-cost, and nonionizing alternative for detecting ILD, offering high sensitivity and negative-predictive value (NPV) compared to HRCT. Key LUS findings indicative of ILD include B-line artefacts (BLA) and pleural irregularity. Evidence supporting the performance and applicability of LUS in diagnosing RA-ILD continues to grow. SUMMARY: LUS has shown a good performance in ILD detection. Further research and standardization efforts are needed to fully integrate LUS into routine RA screening protocols.
Moving past multidisciplinary discussions and Gender-Age-Physiology model: precision medicine through biological phenotyping in interstitial lung disease.
Maddali MV, Oldham JM, Moor CC
Curr Opin Pulm Med · 2025 Sep · PMID 40643570 · Full text
PURPOSE OF REVIEW: Interstitial lung disease (ILD) presents significant diagnostic and therapeutic challenges due to underlying biological heterogeneity and variable clinical course. Traditional diagnostic and prognostic...
PURPOSE OF REVIEW: Interstitial lung disease (ILD) presents significant diagnostic and therapeutic challenges due to underlying biological heterogeneity and variable clinical course. Traditional diagnostic and prognostic tools are limited in their ability to capture this heterogeneity or guide personalized treatment. Advances in biological phenotyping have set the stage for precision medicine in ILD, improving diagnosis, prognosis, and therapeutic decision-making in ILD. This review highlights recent advances in biological phenotyping technologies and their potential to reshape ILD care. RECENT FINDINGS: Emerging evidence supports the use of genomic, transcriptomic, and proteomic, and metabolomic biomarkers to identify distinct ILD subgroups with prognostic or therapeutic relevance. Several biomarkers are being evaluated prospectively, including TOLLIP genotype and eNose technology. Machine learning enables the integration of high-dimensional multiomics data, offering insights beyond what single biomarkers can provide. SUMMARY: Precision medicine in ILD is advancing rapidly and holds promise for more individualized care. Future efforts should prioritize multimodal integration, prospective validation across diverse ILD subtypes, and translating research into clinical practice. Continued innovation and collaboration will be essential to fully realize the potential of precision medicine in transforming ILD care and research.
Unraveling mechanistic insights through interstitial lung disease multiomics.
Menon AA, Ghosh AJ
Curr Opin Pulm Med · 2025 Sep · PMID 40620193 · Publisher ↗
PURPOSE OF REVIEW: Interstitial lung disease (ILD) and consequent pulmonary fibrosis are associated with significant morbidity and mortality with limited treatment options. There are more than 200 different etiologies th...
PURPOSE OF REVIEW: Interstitial lung disease (ILD) and consequent pulmonary fibrosis are associated with significant morbidity and mortality with limited treatment options. There are more than 200 different etiologies that can lead to ILD. As a result, diagnostic accuracy and delay, prognostication, and treatment responses are still rife with challenges. The integration of bioinformatics with clinical practice is gaining momentum, evolving from a research tool to a practical resource with potential applications at the bedside. Work in this field has opened avenues into the pursuit of precision medicine in ILD. RECENT FINDINGS: Across various 'omics-based technologies, numerous studies highlight the potential of using molecular data to disentangle the complex processes that lead to pulmonary fibrosis. Recent studies point toward integrating signals across domains to filter out noise and identify true signals. However, there is still a need for functional work to connect the high-dimensional signals to the biology underlying pulmonary fibrosis. SUMMARY: Pursuing a multiomic approach across multiple domains in ILD holds promise for better biomarkers, clinical trial enrichment, and developing a deeper understanding of disease pathology.
Precision medicine in pulmonary hypertension: how close are we today?
Maron BA
Curr Opin Pulm Med · 2025 Sep · PMID 40620185 · Publisher ↗
PURPOSE OF REVIEW: Pulmonary hypertension (PH) is a specific but heterogeneous disease defined foremost by elevated pulmonary artery pressure, typically occurring due to pulmonary vascular fibroproliferative, plexigenic,...
PURPOSE OF REVIEW: Pulmonary hypertension (PH) is a specific but heterogeneous disease defined foremost by elevated pulmonary artery pressure, typically occurring due to pulmonary vascular fibroproliferative, plexigenic, or thrombotic remodelling. The heterogenous clinical and pathobiological basis of PH poses challenges and opportunities for optimizing treatment alignment to individual patients. RECENT FINDINGS: Advancing precision medicine through personalized treatment pathways in PH is particularly timely owing to persistent morbidity and shortened lifespan reported for patients despite an expanding armamentarium of pharmacotherapeutics, particularly for pulmonary arterial hypertension. Accomplishing this goal successfully has benefited from efforts that optimize clinical phenotyping, establishing reticulotypes that represent the phenotypic consequences of functionally essential pathogenic molecular events, and build greater insight on treatment response variability observed in randomized clinical trials. SUMMARY: Although as a scientific field PH remains early in the precision medicine journey, wider availability and lower cost of high throughput -omics platforms, and increasingly sophisticated analytical methodologies introduces optimism that clinically actionable strategies that improve patient-treatment alignment can be realized in the near-term.
Small fibre neuropathy and sarcoidosis, target for diagnosis and treatment?
Atkins CP
Curr Opin Pulm Med · 2025 Sep · PMID 40620177 · Publisher ↗
PURPOSE OF REVIEW: To update the reader on recent evidence relating to symptoms, diagnostic strategies and management options for small-fibre neuropathy (SFN) in sarcoidosis. Learning points from recent studies are prese...
PURPOSE OF REVIEW: To update the reader on recent evidence relating to symptoms, diagnostic strategies and management options for small-fibre neuropathy (SFN) in sarcoidosis. Learning points from recent studies are presented in the context of previous research forming the basis of our knowledge of SFN in patients with sarcoidosis. RECENT FINDINGS: Recent studies have shown overlap of SFN and other symptoms beyond pain in patients with sarcoidosis. Where SFN exists, determining the optimal diagnostic modalities is challenging; there have been developments in how forms of temperature threshold testing should be performed, as well as whether this test may miss patchy involvement by SFN in sarcoidosis. To aid identification of SFN in sarcoidosis, questionnaires has been developed specifically looking at sarcoidosis-associated SFN, attempting to capture the patients with patchy or intermittent SFN symptoms. Standard systemic management for sarcoidosis is ineffective for SFN, though infliximab shows promise and is increasingly being utilised as a treatment option. SUMMARY: Few studies have been published in the last two years but there have been developments that have progressed our knowledge. This review collates the latest research alongside prior work, aiming to help summarise diagnosis and management strategies for this problematic manifestation of sarcoidosis.
Artificial intelligence in cardiac sarcoidosis: ECG, Echo, CPET and MRI.
Umeojiako WI, Lüscher T, Sharma R
Curr Opin Pulm Med · 2025 Sep · PMID 40620172 · Publisher ↗
PURPOSE OF REVIEW: Cardiac sarcoidosis is a form of inflammatory cardiomyopathy that varies in its clinical presentation. It is associated with significant clinical complications such as high-degree atrioventricular bloc...
PURPOSE OF REVIEW: Cardiac sarcoidosis is a form of inflammatory cardiomyopathy that varies in its clinical presentation. It is associated with significant clinical complications such as high-degree atrioventricular block, ventricular tachycardia, heart failure and sudden cardiac death. It is challenging to diagnose clinically, and its increasing detection rate may represent increasing awareness of the disease by clinicians as well as a rising incidence. Prompt diagnosis and risk stratification reduces morbidity and mortality from cardiac sarcoidosis. Noninvasive diagnostic modalities such as ECG, echocardiography, PET/computed tomography (PET/CT) and cardiac MRI (cMRI) are increasingly playing important roles in cardiac sarcoidosis diagnosis. Artificial intelligence driven applications are increasingly being applied to these diagnostic modalities to improve the detection of cardiac sarcoidosis. RECENT FINDINGS: Review of the recent literature suggests artificial intelligence based algorithms in PET/CT and cMRIs can predict cardiac sarcoidosis as accurately as trained experts, however, there are few published studies on artificial intelligence based algorithms in ECG and echocardiography. SUMMARY: The impressive advances in artificial intelligence have the potential to transform patient screening in cardiac sarcoidosis, aid prompt diagnosis and appropriate risk stratification and change clinical practice.
New therapeutic options in sarcoidosis.
Nunes H, Hindré R, Jeny F
Curr Opin Pulm Med · 2025 Sep · PMID 40620171 · Publisher ↗
PURPOSE OF REVIEW: Corticosteroids remain the cornerstone of sarcoidosis treatment but are associated with significant toxicities and impaired quality of life. Second-line and third-line treatments include hydroxychloroq...
PURPOSE OF REVIEW: Corticosteroids remain the cornerstone of sarcoidosis treatment but are associated with significant toxicities and impaired quality of life. Second-line and third-line treatments include hydroxychloroquine or immunosuppressants (methotrexate, azathioprine, leflunomide and mycophenolate mofetil), and anti-TNF-α agents (infliximab and adalimumab), respectively. The latest identification of significant key cellular pathways in sarcoidosis pathogenesis has led to the development of new therapeutic strategies described in this review. RECENT FINDINGS: JAK inhibitors, mainly tofacitinib, exhibited encouraging results in case reports, small retrospective series, and two prospective open-label trials for skin and pulmonary sarcoidosis. mTOR inhibitors demonstrated efficacity in one cross-over study on skin involvement. Promising findings were obtained with efzofitimod, an inhibitor of neuropilin-2 receptor, in a pilot study on pulmonary sarcoidosis, which needs to be confirmed. Other drugs with potentially relevant mechanisms of action have been used in case reports or small series or are under investigation: CTLA-4 inhibitors, IL-6 inhibitors, NLRP3 inflammasome inhibitors, GM-CSF inhibitors, PDE-4 inhibitors, and statins. There is very little data available on antifibrotic agents for fibrotic pulmonary sarcoidosis. SUMMARY: Despite many challenges, well designed studies are warranted to investigate new therapeutic options in sarcoidosis, particularly in patients with refractory forms or unacceptable side-effects with third-line treatment.
Important negative trials in pulmonary hypertension.
Cullivan S, McCourt L, Gaine S
Curr Opin Pulm Med · 2025 Sep · PMID 40620169 · Publisher ↗
PURPOSE OF REVIEW: The pathobiology of pulmonary arterial hypertension (PAH) is complex and has been characterized by aberrant signalling in diverse pathways, many of which have been explored in recent studies. While som...
PURPOSE OF REVIEW: The pathobiology of pulmonary arterial hypertension (PAH) is complex and has been characterized by aberrant signalling in diverse pathways, many of which have been explored in recent studies. While some of these studies have demonstrated negative results, nonetheless they provide valuable insights into these drugs and the disease. RECENT FINDINGS: The focus of this article is to provide an overview of recent negative trials in pulmonary hypertension, with a specific focus on PAH. These include recent studies exploring the use of tocilizumab, selonsertib, rodatristat ethyl, anastrazole and dry powder inhalation of imatinib (AV-101). SUMMARY: This article provides an overview of the key learning points from these studies and reinforces the importance of the publication of all trials, irrespective of outcome, so that we can learn from negative studies and prioritize promising therapies and treatment pathways.
Rare pulmonary diseases and pulmonary hypertension.
Tagariello F, Elia D, Harari SA
Curr Opin Pulm Med · 2025 Sep · PMID 40575830 · Publisher ↗
PURPOSE OF REVIEW: Pulmonary hypertension (PH) is a significant complication of various lung diseases, including rare conditions such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH)....
PURPOSE OF REVIEW: Pulmonary hypertension (PH) is a significant complication of various lung diseases, including rare conditions such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH). This review explores the pathophysiology, diagnostic challenges, and therapeutic strategies for managing PH in these conditions, emphasizing recent findings and gaps in knowledge. RECENT FINDINGS: In LAM, PH primarily results from parenchymal destruction and hypoxic vasoconstriction rather than direct vascular involvement, leading to its reclassification from Group 5 to Group 3 PH. Sirolimus, an mTOR inhibitor, has demonstrated benefits in stabilizing lung function and may indirectly reduce pulmonary pressures, though direct effects remain unproven. In PLCH, PH is often disproportionate to lung function impairment, suggesting a distinct pulmonary vasculopathy. Histopathologic studies reveal extensive vascular remodeling, including features of pulmonary veno-occlusive disease. Case reports suggest potential benefits of PH-specific therapies such as endothelin receptor antagonists and phosphodiesterase-5 inhibitors, but their use requires caution due to the risk of worsening gas exchange. SUMMARY: PH in LAM and PLCH is uncommon but clinically relevant, particularly in advanced disease. While emerging therapies show promise, further research is needed to optimize management and improve patient outcomes.
Precision medicine in rheumatoid arthritis-associated interstitial lung disease: current evidence and future directions.
Matson SM
Curr Opin Pulm Med · 2025 Sep · PMID 40548519 · Full text
PURPOSE OF REVIEW: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) leads to poor survival, on average only 3-5 years from time of diagnosis. Despite its clinical impact, evidence-based treatment approa...
PURPOSE OF REVIEW: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) leads to poor survival, on average only 3-5 years from time of diagnosis. Despite its clinical impact, evidence-based treatment approaches remain limited, creating urgent clinical uncertainty about optimal management strategies. This review examines emerging precision medicine approaches that may guide more effective, individualized treatment decisions. RECENT FINDINGS: Current treatment paradigms based on radiologic patterns lack empirical validation, with recent evidence suggesting radiologic features poorly predict immunomodulatory response. Advances in RA joint precision medicine using synovial biopsy and RNA sequencing have identified molecular endotypes predicting differential treatment response, establishing a framework that could be applied to RA-ILD. Emerging biomarkers including leukocyte telomere length, circulating proteomics, and lung-based systems biology show promise for identifying RA-ILD molecular heterogeneity and guiding treatment selection. SUMMARY: Progress in RA-ILD precision medicine requires multimodal approaches integrating molecular phenotyping with targeted therapeutic trials. Lessons from RA joint precision medicine suggest accessing the disease compartment directly through bronchoscopy may provide crucial information beyond peripheral biomarkers. Prospective biomarker-stratified trials are urgently needed to overcome clinical equipoise and improve outcomes for this challenging condition.
ANCA associated pulmonary fibrosis: vasculitis or not vasculitis.
Codes-Méndez H, Castillo D, Moya-Alvarado P … +2 more , Giménez-Palleiro A, Castellvi I
Curr Opin Pulm Med · 2025 Sep · PMID 40548517 · Publisher ↗
PURPOSE OF REVIEW: The intersection of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represents a complex and increasingly recognized clinical challenge. This...
PURPOSE OF REVIEW: The intersection of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represents a complex and increasingly recognized clinical challenge. This review aims to summarize current understanding, highlight diagnostic and therapeutic approaches, and identify key gaps in the literature regarding ANCA-associated ILD. RECENT FINDINGS: ANCA positivity-particularly MPO-ANCA- is increasingly identified in patients with fibrotic ILD, even in the absence of systemic vasculitis. This overlap raises questions about disease classification and management, especially as radiologic patterns such as usual interstitial pneumonia (UIP) appear to predict prognosis. Immunosuppressive therapy remains the mainstay of treatment, though its role varies depending on the presence of systemic features and lung fibrosis. Emerging biomarkers, and the potential role of antifibrotic agents offer promising avenues for improved monitoring and therapy. SUMMARY: ANCA-ILD represents a heterogeneous and underexplored disease spectrum that challenges existing classification systems. A multidisciplinary approach is critical, and prospective studies are urgently needed to redefine diagnostic criteria and guide treatment strategies in order to improve clinical outcomes.
Current concepts in the pathogenesis and clinical management of lymphangioleiomyomatosis.
O'Malley D, Gupta N, McCarthy C
Curr Opin Pulm Med · 2025 Sep · PMID 40501369 · Publisher ↗
PURPOSE OF REVIEW: Lymphangioleiomyomatosis (LAM) is a systemic, low-grade, metastasizing neoplasm that predominantly affects women. This review demonstrates recent progression in this rare disease, from improved underst...
PURPOSE OF REVIEW: Lymphangioleiomyomatosis (LAM) is a systemic, low-grade, metastasizing neoplasm that predominantly affects women. This review demonstrates recent progression in this rare disease, from improved understanding of pathogenesis, to novel treatments. We provide an overview of recent advances that are shaping the future of LAM diagnostic and treatment approaches. RECENT FINDINGS: Understanding the role of hormonal pathways, immune system interactions, mechanistic target of rapamycin (mTOR) signalling inhibition and the LAM microenvironment is creating opportunities for combination therapies with curative potential. Evidence supporting the uterine origin of LAM cells has renewed interest in hormonal signalling pathways, while potential immune evasion mechanisms of LAM cells are under investigation. More complete blockade of mTOR pathways by newer generation agents, as well as research into the crosstalk between LAM cells and their surroundings is informing development of novel combination therapies with disease modifying potential. Biomarker identification beyond vascular endothelial growth factor D (VEGF-D) is essential for diagnostic, prognostic and treatment decision making. Cellular mapping using single-cell RNA sequencing and spatial transcriptomics, as well as integration of artificial intelligence into diagnostic and therapeutic development pathways, has the potential to significantly advance our understanding of this rare disease. SUMMARY: LAM research demonstrates how sustained investigation in rare disease can advance from preclinical mechanistic insights to targeted treatments. Understanding the role of hormonal pathways, immune system interactions, mTOR signalling inhibition and the microenvironment is creating opportunities for combination therapies with curative potential. Advancements in technology, including single cell analysis, spatial transcriptomics and artificial intelligence are accelerating the discover of biomarkers and therapeutic targets, positioning LAM for significant clinical advances in the coming years.
Pulmonary veno-occlusive disease: a paradigm of diagnosis and therapeutic challenges in pulmonary hypertension.
Aguado B, Grynblat J, Budhram B … +8 more , Ghigna MR, Boucly A, Antigny F, Jaïs X, Sitbon O, Savale L, Humbert M, Montani D
Curr Opin Pulm Med · 2025 Sep · PMID 40471666 · Publisher ↗
PURPOSE OF REVIEW: Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening form of precapillary pulmonary hypertension. This review aims to outline its genetic and environmental risk factors, highlight key...
PURPOSE OF REVIEW: Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening form of precapillary pulmonary hypertension. This review aims to outline its genetic and environmental risk factors, highlight key diagnostic challenges, and discuss current treatment options. RECENT FINDINGS: PVOD can occur sporadically or as a hereditary autosomal recessive condition with biallelic eukaryotic translation initiation factor 2 alpha kinase 4 ( EIF2AK4) mutations, leading to nearly complete disease penetrance. Known risk factors include specific drug/toxin and environmental exposures, such as mitomycin C and trichloroethylene, respectively. PVOD is characterized by progressive pulmonary venous and capillary remodelling, severe hypoxemia, and right ventricular failure. Diagnosis remains difficult due to overlapping features with pulmonary arterial hypertension (PAH), but high-resolution computed tomography (HRCT) findings, low lung diffusion capacity for carbon monoxide (DLCO), and genetic testing can aid differentiation. Initiation of PAH-approved drugs in patients with PVOD requires careful consideration due to limited evidence of long-term clinical benefits and the high risk of developing pulmonary oedema in this population. Lung transplantation remains the only curative treatment, with posttransplant survival rates comparable to idiopathic PAH. SUMMARY: PVOD is a progressive and fatal disease requiring early recognition and specific management. Due to its poor prognosis and lack of effective medical therapies, early referral for lung transplantation is crucial. Advances in genetic and molecular research may lead to novel treatment strategies.
Editorial: Integrating the general pulmonologist in the continuum of lung transplantation: a primer on current issues in referral, selection, and posttransplant care.
Lehr CJ, Patterson C
Curr Opin Pulm Med · 2025 Jul · PMID 40434813 · Publisher ↗
Abstract loading — click title to view on PubMed.
The search for peripheral tolerance in lung transplantation.
Snyder ME, McDyer JF
Curr Opin Pulm Med · 2025 Jul · PMID 40396535 · Publisher ↗
PURPOSE OF REVIEW: Median survival after lung transplantation is 5.7 years, which lags behind other solid organ transplants, such as heart, liver, and kidney. The major barrier to long-term survival in lung transplant re...
PURPOSE OF REVIEW: Median survival after lung transplantation is 5.7 years, which lags behind other solid organ transplants, such as heart, liver, and kidney. The major barrier to long-term survival in lung transplant recipients is chronic lung allograft dysfunction (CLAD). This review discusses the challenge of CLAD as a barrier to tolerance and identifies key areas in the field that require further development. RECENT FINDINGS: CLAD is a heterogenous disease in its kinetics of onset and severity and remains a clinical diagnosis of exclusion, based on a decline in allograft function. While acute cellular rejection and antibody-mediated rejection are major risk-factors for CLAD, other barriers to long-term allograft acceptance are aspiration and primary graft dysfunction. However infections, particularly respiratory viral infections and Cytomegalovirus (CMV) remain the most significant risks for CLAD. Additionally, the lung transplant field is limited by a lack of molecular diagnostic assays for CLAD. Further, new targets are needed for precision immunosuppression, and more studies are needed to develop novel interventions to extend allograft acceptance. SUMMARY: This review discusses new lines of study to address important unmet needs necessary to extend lung allograft acceptance. Other studies, such as tandem lung transplant and bone marrow transplant in select patients with primary immunodeficiency may provide additional lessons on how to potentially establish tolerance. However, tolerance in lung transplant is extremely rare, and further studies are needed to pursue this ultimate goal.
A comprehensive review of benign tumors in the lung.
Sarkar A, Husnain SMN, Harris K
Curr Opin Pulm Med · 2025 Jul · PMID 40366026 · Publisher ↗
PURPOSE OF REVIEW: The purpose of this review is to provide clinicians a comprehensive overview of the epidemiology, clinical symptoms, radiological features, pathological features, and management recommendations for the...
PURPOSE OF REVIEW: The purpose of this review is to provide clinicians a comprehensive overview of the epidemiology, clinical symptoms, radiological features, pathological features, and management recommendations for the vast majority of benign lung tumors. Benign lung tumors are very rare with incidence ranging from 1 in 1000 to 1 in 1 million. Despite not being malignant, certain benign tumors carry significant morbidity and mortality along with diagnostic challenges. RECENT FINDINGS: Advancements in genomic sequencing have led to discovery of mutations in particular benign lung tumors. Improved genotyping have aided the diagnosis of certain tumors and the identification of lung lesions with malignant transformation potential. Genomic understanding has also led to targeted therapy for tumors with significant morbidity. SUMMARY: Despite radiographic and pathologic advances in understanding benign lung tumors, the paucity of cases continues to impact management recommendations and early detection. Global collaborative initiatives in compiling and analyzing cases are essential for stronger evidence based management recommendations.
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