{"title":"Elexacaftor-tezacaftor-ivacaftor use after solid organ transplant.","authors":"Siddhartha G Kapnadak, Kathleen J Ramos","doi":"10.1097/MCP.0000000000001110","DOIUrl":"10.1097/MCP.0000000000001110","url":null,"abstract":"<p><strong>Purpose of review: </strong>In 2019, the United States Food and Drug Administration approved a breakthrough therapeutic for cystic fibrosis, elexacaftor-tezacaftor-ivacaftor (ETI), because of its profound effect on lung function in large phase III clinical trials. ETI acts directly on the dysfunctional protein that causes the systemic manifestations of cystic fibrosis and also leads to improvement in nonpulmonary symptoms of cystic fibrosis. Transplant recipients were excluded from the pivotal clinical trials of ETI but may stand to benefit from correction of the underlying protein defect. Drug interactions between the three drugs in ETI and immunosuppression medications are one of the primary concerns about using ETI after transplant. No rigorous studies exist to assess the safety of ETI in transplant recipients.</p><p><strong>Recent findings: </strong>Multiple recent publications describe the use of ETI after solid organ transplantation, including lung and nonlung transplants, and the real-world evidence for drug interactions between ETI and immunosuppression medications. In nonlung transplant recipients, the pulmonary benefits of ETI have been confirmed, but adverse events occur and may have implications for their transplanted organ (e.g. liver biopsy in the setting of elevated transaminases). Lung transplant recipients may have higher rates of ETI discontinuation than nontransplant recipients given a lack of direct pulmonary benefit and frequency of side effects. Drug interactions have not been difficult to manage, with most studies reporting variable rates of mild to moderate increased tacrolimus levels after initiation of ETI.</p><p><strong>Summary: </strong>Limited data exist to support the use of ETI after solid organ transplantation and further research is warranted. Given the unknown risks and benefits, case by case consideration of ETI use is indicated when extra-pulmonary manifestations are present in lung transplant recipients with cystic fibrosis. Given the proven benefit in cystic fibrosis lung disease, benefits likely outweigh the risks of ETI for nonlung solid organ transplant recipients.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"660-666"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11611685/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141855055","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}
Nicholas Avdimiretz, Kieran Halloran, Christian Benden
{"title":"Outcomes of lung transplantation in cystic fibrosis.","authors":"Nicholas Avdimiretz, Kieran Halloran, Christian Benden","doi":"10.1097/MCP.0000000000001108","DOIUrl":"10.1097/MCP.0000000000001108","url":null,"abstract":"<p><strong>Purpose of review: </strong>Lung transplantation (LTX) has transformed care for people with cystic fibrosis (pwCF) suffering from advanced cystic fibrosis lung disease (ACFLD), and it has evolved into an accepted therapy for patients with ACFLD across all ages. We review cystic fibrosis as a major indication for LTX, particularly highlighting outcomes including survival, a changing landscape over time, and factors affecting sequelae following LTX in cystic fibrosis.</p><p><strong>Recent findings: </strong>Although some populations such as those undergoing lung retransplantation exhibit inferior posttransplant outcomes, LTX for pwCF provides an excellent long-term survival that has significantly improved over time, likely due to specialized cystic fibrosis center care and recognition of common comorbidities in pwCF post-LTX. There are gaps in post-LTX outcomes for pwCF, including that identified between Canada and the United States, and that seen in adolescents - both of which are likely multifactorial. In particular, the revolution in cystic fibrosis medical therapy with CFTR modulator therapy has resulted in a dramatic decline in programs performing LTX for cystic fibrosis. How durable this effect will remains to be seen.</p><p><strong>Summary: </strong>Overall, LTX remains a well accepted ultimate therapy option in patients with ACFLD if compatible with the individual's goals of care, offering an improved quality of life and maximization of overall survival.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"646-651"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141855057","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}
Krish Dodani, Lucía Pinilla, Manuel Sánchez-de-la-Torre
{"title":"Predictors and markers of the cardiovascular impact of obstructive sleep apnoea.","authors":"Krish Dodani, Lucía Pinilla, Manuel Sánchez-de-la-Torre","doi":"10.1097/MCP.0000000000001118","DOIUrl":"10.1097/MCP.0000000000001118","url":null,"abstract":"<p><strong>Purpose of review: </strong>Obstructive sleep apnoea (OSA) is the most common form of sleep-disordered breathing and has been linked to cardiovascular health. However, some of the findings supporting this are controversial. These discrepancies might be a result of heterogeneity among OSA patients, and thus, additional information would be required to better stratify OSA patients according to cardiovascular risk. In this review, we aim to discuss the potential of biomarkers to fulfil this role.</p><p><strong>Recent findings: </strong>Randomized controlled trials have been unable to confirm whether OSA treatment with continuous positive airway pressure (CPAP) has a positive effect on cardiovascular outcomes. Emerging physiology-based metrics of OSA seem to be more suitable for identifying patients at higher risk of cardiovascular disease and predicting the effects of CPAP outcomes on cardiovascular health. Similarly, blood-based molecular markers have gained attention in this context over the last few years.</p><p><strong>Summary: </strong>Accurate cardiovascular risk stratification and appropriate treatment allocation for OSA patients remain challenging. However, significant efforts are being made to develop novel tools to address these important issues.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"613-620"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072315","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}
Serena Scarpelli, Valentina Alfonsi, Luigi De Gennaro
{"title":"Exploring the role of dreams: insights from recent studies.","authors":"Serena Scarpelli, Valentina Alfonsi, Luigi De Gennaro","doi":"10.1097/MCP.0000000000001112","DOIUrl":"10.1097/MCP.0000000000001112","url":null,"abstract":"<p><strong>Purpose of review: </strong>Dreaming has only recently become a topic of scientific research. This review updates current findings on dream studies, emphasizing recent research on the neural mechanisms of dreaming. Additionally, it summarizes new evidence on the functional role of dreams, including insights from studies on dreams and nightmares during the coronavirus disease 2019 (COVID-19) pandemic.</p><p><strong>Recent findings: </strong>Recent advances on the neural basis of mental activity during sleep have shifted towards dream-related phenomena, such as dream experiences in relation to parasomnias and hypnagogic hallucinations. Although some findings are consistent with the main models explaining dream recall (i.e., continuity hypothesis; activation hypothesis), some results contrast with the role of parieto-occipital region in dream experience. Moreover, recent findings - related to COVID-19 pandemic - underlined that dream experiences could express emotion regulation processes as well as provide a simulation of reality to prepare for future dangerous or social interactions.</p><p><strong>Summary: </strong>Overall, we highlighted the intricate interplay between brain regions in dreaming and suggest that dreams serve multiple functions, from reflecting waking-life experiences to simulating adaptive responses to threats. Understanding the neural bases and functions of dreaming can provide valuable insights into human mental health, nevertheless, further research is needed.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"583-588"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141901225","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":"Pulmonary vascular diseases at high altitude - is it safe to live in the mountains?","authors":"Anna Titz, Rodrigo Hoyos, Silvia Ulrich","doi":"10.1097/MCP.0000000000001092","DOIUrl":"10.1097/MCP.0000000000001092","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review addresses the concern of the health effects associated with high-altitude living and chronic hypoxia with a focus on pulmonary hypertension. With an increasing global population residing at high altitudes, understanding these effects is crucial for public health interventions and clinical management.</p><p><strong>Recent findings: </strong>Recent literature on the long-term effects of high-altitude residence and chronic hypoxia is comprehensively summarized. Key themes include the mechanisms of hypoxic pulmonary vasoconstriction, the development of pulmonary hypertension, and challenges in distinguishing altitude-related pulmonary hypertension and classical pulmonary vascular diseases, as found at a low altitude.</p><p><strong>Summary: </strong>The findings emphasize the need for research in high-altitude communities to unravel the risks of pulmonary hypertension and pulmonary vascular diseases. Clinically, early and tailored management for symptomatic individuals residing at high altitudes are crucial, as well as access to advanced therapies as proposed by guidelines for pulmonary vascular disease. Moreover, identifying gaps in knowledge underscores the necessity for continued research to improve understanding and clinical outcomes in high-altitude pulmonary vascular diseases.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"459-463"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11343446/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733663","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":"Palliative care in advanced pulmonary fibrosis.","authors":"Meena Kalluri","doi":"10.1097/MCP.0000000000001093","DOIUrl":"10.1097/MCP.0000000000001093","url":null,"abstract":"<p><strong>Purpose of review: </strong>Updated clinical practice guidelines for pulmonary fibrosis recommend early integrated palliative care using a primary palliative approach. Clinicians need to be aware of the various palliative interventions in order to implement guidelines' recommendations. This review provides an update on evidence-based palliative therapies.</p><p><strong>Recent findings: </strong>Literature review indicates early integration of palliative care in pulmonary fibrosis is feasible and meets patient needs. Key components of a primary palliative approach include comprehensive symptoms and needs screening, systematic symptom management using nonpharmacologic interventions, supplemental oxygen and opioids for dyspnea and cough. Patient-centered communication is essential for successful integration of palliative care. Early, iterative advance care planning in clinic to understand patient goals, values and preferences for current and future care, improves patient care and satisfaction. Prioritizing caregiver inclusion in clinics can address their needs. Collaborating with a multidisciplinary allied team facilitates integration of palliative care and supports patients throughout the disease course. Different models of palliative care delivery exist and can be adapted for local use. The use of artificial intelligence algorithms and tools may improve care and continuity.</p><p><strong>Summary: </strong>Clinicians must develop competency in palliative care. Organizational and policy support is essential to enable seamless integration of palliative care throughout the care continuum.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"530-539"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141497384","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":"Novel interventions on the tricuspid valve: how to consider the pulmonary circulation?","authors":"Roland Wensel, Christian Opitz","doi":"10.1097/MCP.0000000000001101","DOIUrl":"10.1097/MCP.0000000000001101","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review addresses treatment options for moderate to severe tricuspid valve regurgitation and the importance of right ventricular function and the pulmonary circulation.</p><p><strong>Recent findings: </strong>Several interventional treatment options for severe tricuspid regurgitation have been developed including transcatheter edge-to-edge repair, annuloplasty and valve replacement. So far, transcatheter edge-to-edge repair is most frequently used with procedural success rates of more than 95% and improvements in functional and quality of life parameters for up to 2 years. Right ventricular function as well as pulmonary artery pressure and resistance levels are important outcome predictors. Mean pulmonary artery pressure more than 30 mmHg, transpulmonary gradient more than 17 mmHg and right ventricular to pulmonary artery coupling ratio less than 0.406 indicate poor outcome.</p><p><strong>Summary: </strong>Despite the remarkable safety of interventional treatment of severe tricuspid regurgitation right ventricular dysfunction and abnormal pulmonary hemodynamics are important determinants of procedural success and clinical outcome.Complete hemodynamic work-up should be an integral part of prerepair assessment although validated data predicting outcome are limited.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":"30 5","pages":"444-450"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141901249","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}
Roberta Eufrasia Ledda, Cristina Marrocchio, Nicola Sverzellati
{"title":"Progress in the radiologic diagnosis of idiopathic pulmonary fibrosis.","authors":"Roberta Eufrasia Ledda, Cristina Marrocchio, Nicola Sverzellati","doi":"10.1097/MCP.0000000000001086","DOIUrl":"10.1097/MCP.0000000000001086","url":null,"abstract":"<p><strong>Purpose of review: </strong>To discuss the most recent applications of radiological imaging, from conventional to quantitative, in the setting of idiopathic pulmonary fibrosis (IPF) diagnosis.</p><p><strong>Recent findings: </strong>In this article, current concepts on radiological diagnosis of IPF, from high-resolution computed tomography (CT) to other imaging modalities, are reviewed. In a separate section, advances in quantitative CT and development of novel imaging biomarkers, as well as current limitations and future research trends, are described.</p><p><strong>Summary: </strong>Radiological imaging in IPF, particularly quantitative CT, is an evolving field which holds promise in the future to allow for an increasingly accurate disease assessment and prognostication of IPF patients. However, further standardization and validation studies of alternative imaging applications and quantitative biomarkers are needed.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"500-507"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141418213","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}
Raphaël Borie, Ibrahima Ba, Marie-Pierre Debray, Caroline Kannengiesser, Bruno Crestani
{"title":"Syndromic genetic causes of pulmonary fibrosis.","authors":"Raphaël Borie, Ibrahima Ba, Marie-Pierre Debray, Caroline Kannengiesser, Bruno Crestani","doi":"10.1097/MCP.0000000000001088","DOIUrl":"10.1097/MCP.0000000000001088","url":null,"abstract":"<p><strong>Purpose of review: </strong>The identification of extra-pulmonary symptoms plays a crucial role in diagnosing interstitial lung disease (ILD). These symptoms not only indicate autoimmune diseases but also hint at potential genetic disorders, suggesting a potential overlap between genetic and autoimmune origins.</p><p><strong>Recent findings: </strong>Genetic factors contributing to ILD are predominantly associated with telomere (TRG) and surfactant-related genes. While surfactant-related gene mutations typically manifest with pulmonary involvement alone, TRG mutations were initially linked to syndromic forms of pulmonary fibrosis, known as telomeropathies, which may involve hematological and hepatic manifestations with variable penetrance. Recognizing extra-pulmonary signs indicative of telomeropathy should prompt the analysis of TRG mutations, the most common genetic cause of familial pulmonary fibrosis. Additionally, various genetic diseases causing ILD, such as alveolar proteinosis, alveolar hemorrhage, or unclassifiable pulmonary fibrosis, often present as part of syndromes that include hepatic, hematological, or skin disorders.</p><p><strong>Summary: </strong>This review explores the main genetic conditions identified over the past two decades.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":" ","pages":"473-483"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141418214","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":"Evidence from recent clinical trials in fibrotic interstitial lung diseases.","authors":"Vincent Cottin, Claudia Valenzuela","doi":"10.1097/MCP.0000000000001089","DOIUrl":"10.1097/MCP.0000000000001089","url":null,"abstract":"<p><strong>Purpose of review: </strong>Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrosing interstitial lung diseases. It is mirrored by progressive pulmonary fibrosis (PPF), an umbrella term which characterizes disease behavior of various fibrotic interstitial lung diseases with irreversible progression, accounting for loss of lung function, exercise intolerance and respiratory failure leading to early mortality. Pirfenidone and nintedanib halve the decline in lung function but do not halt disease progression.</p><p><strong>Recent findings: </strong>Since the publication in 2014 of pivotal pirfenidone and nintedanib studies, a number of clinical trials were conducted, many of them did not reach their primary endpoints. In IPF, promising phase 2 trials were followed by large phase 3 trials that did not confirm a favorable efficacy to tolerability favorable profile, including those with ziritaxestat, an autotaxin-1 inhibitor, zinpentraxin-alpha (human recombinant pentraxin-2), and the monoclonal antibody pamrevlumab targeting connective tissue growth factor. Nevertheless, newer compounds that hold promise are currently being evaluated in phase 3 or phase 2b randomized controlled trials, including: nerandomilast, a preferential phosphodiesterase 4B inhibitor; admilparant, a lysophosphatidic acid receptor antagonist; inhaled treprostinil, a prostacyclin agonist; and bexotegrast, a dual-selective inhibitor of αvβ6 and αvβ1 integrins. Nerandomilast, admilparant, inhaled treprostinil, and inhaled AP01 (pirfenidone), are currently studied in patients with PPF.</p><p><strong>Summary: </strong>Despite recent frustrating negative results, there is a growing portfolio of candidate drugs developed in both IPF and PPF.</p>","PeriodicalId":11090,"journal":{"name":"Current Opinion in Pulmonary Medicine","volume":"30 5","pages":"484-493"},"PeriodicalIF":2.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141901227","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}