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Curr Opin Pulm Med [JOURNAL]

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Anesthesia strategies to minimize lung atelectasis in peripheral bronchoscopy.

Peralta AR, Debiane LG

Curr Opin Pulm Med · 2026 Jan · PMID 41065573 · Publisher ↗

PURPOSE OF REVIEW: Peripheral bronchoscopy has become a widely adopted, minimally invasive modality for the diagnosis of peripheral lung lesions. However, its diagnostic yield remains limited in part due to intra-procedu... PURPOSE OF REVIEW: Peripheral bronchoscopy has become a widely adopted, minimally invasive modality for the diagnosis of peripheral lung lesions. However, its diagnostic yield remains limited in part due to intra-procedural atelectasis - a frequently overlooked phenomenon that obscures lesions and exacerbates computed tomography (CT)-to-body divergence. This review highlights the prevalence and clinical impact of atelectasis during navigational bronchoscopy and presents anesthesia and ventilation strategies to mitigate its occurrence. RECENT FINDINGS: Emerging data from trials such as I-LOCATE, ventilatory strategy to prevent atelectasis (VESPA), and lung navigation ventilation protocol (LNVP) studies show that atelectasis develops early and frequently during bronchoscopy, particularly in dependent lung zones and patients with high BMI. Strategies to reduce atelectasis include high tidal volumes, optimized positive end-expiratory pressure, reduced FiO 2 , and recruitment maneuvers. Dedicated ventilation protocols like VESPA and LNVP have significantly reduced both incidence and severity of atelectasis, improved lesion visibility, and demonstrated safety. Apneic breath-hold techniques further enhance image quality and lesion targeting. SUMMARY: Atelectasis is a modifiable barrier to diagnostic success in peripheral bronchoscopy. Proactive pre and intra-procedural planning, implementation of structured ventilation protocols, and close collaboration with anesthesia are essential. Future research should focus on protocol standardization, novel imaging synchronization techniques, and validation of atelectasis grading tools.

Intraprocedural real-time imaging and tissue sampling in peripheral bronchoscopy.

Senyei G, Shaller BD, Di Felice C … +1 more , Sethi S

Curr Opin Pulm Med · 2026 Jan · PMID 41065564 · Publisher ↗

PURPOSE OF REVIEW: Peripheral lung lesions remain a prevalent and diagnostically challenging clinical entity. While computed tomography (CT)-guided transthoracic needle aspiration has been the gold-standard for tissue di... PURPOSE OF REVIEW: Peripheral lung lesions remain a prevalent and diagnostically challenging clinical entity. While computed tomography (CT)-guided transthoracic needle aspiration has been the gold-standard for tissue diagnosis, recent advances in technology have led to an increase in the ability of navigational bronchoscopy to successfully reach these lesions.However, despite successful bronchoscopic navigation based on preprocedural CT, diagnostic yield remains low if the proceduralist is unable to identify and correct CT-to-body divergence or confirm tool-in-lesion while sampling the lesion.The use of improved intraprocedural imaging techniques and biopsy tools have provided new techniques to correct positioning and sample lesions with greater diagnostic yield. RECENT FINDINGS: Integration of axial imaging in the form of fixed and mobile cone beam CT, digital tomosynthesis, and augmented fluoroscopy into bronchoscopic procedures have led to improved diagnostic accuracy. These advances improve peripheral bronchoscopy through identification and compensation of navigational errors relative to changes in real-time lesion location and confirmation of biopsy instrument location within the lesion. SUMMARY: Real-time imaging that is capable of identifying and correcting for CT-to-body divergence is vital to the increased diagnostic accuracy of peripheral bronchoscopic procedures.

Lung transplantation for chronic obstructive pulmonary disease patients: an overview.

Naamani TI, Verplancke V, Verleden GM

Curr Opin Pulm Med · 2026 Mar · PMID 41065562 · Publisher ↗

PURPOSE OF REVIEW: To provide an overview of current indications for lung transplantation (LTx) in COPD patients, to describe the different transplantation options, to compare the outcome of COPD and alpha1-antitrypsin d... PURPOSE OF REVIEW: To provide an overview of current indications for lung transplantation (LTx) in COPD patients, to describe the different transplantation options, to compare the outcome of COPD and alpha1-antitrypsin deficiency (AATD) patients versus non-AATD COPD patients and to discuss the possible complications, also specifically related to COPD, AATD patients and the transplantation procedure. RECENT FINDINGS: Some 30-50% of all lung LTx worldwide are performed in COPD patients, with the majority being operated via double lung transplantation (DLTx). Unilateral lung transplantation (SLTx) remains an option, depending on the donor availability and the center's experience. The mean survival after LTx for COPD remains somewhat lower compared to other underlying diseases, especially after SLTx, which may lead to specific complications such as native lung hyperinflation and development of a native lung cancer. SUMMARY: LTx for end-stage COPD remains an accepted treatment modality in selected patients, which increases the QOL and the survival. The global 5-year survival is around 60%; somewhat better for AATD, compared to non-AATD COPD and after DLTx compared to SLTx. The best procedure of choice remains a matter for further discussion, although most centers prefer to perform DLTx, certainly in patients with underlying AATD.

The rapidly changing paradigms for the diagnosis and treatment of cystic fibrosis, bronchiectasis, and primary ciliary dyskinesia.

Metersky ML, Conrad DJ, Shapiro AJ

Curr Opin Pulm Med · 2025 Nov · PMID 41037285 · Publisher ↗

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Understanding idiopathic hypersomnia: diagnosis, pathophysiology, and management.

Shahzadi M, Monderer R, Thorpy MJ

Curr Opin Pulm Med · 2025 Nov · PMID 40990641 · Publisher ↗

PURPOSE OF REVIEW: Idiopathic hypersomnia is a chronic and often disabling sleep disorder characterized by excessive daytime sleepiness despite adequate or prolonged nighttime sleep. With recent advances in diagnosis and... PURPOSE OF REVIEW: Idiopathic hypersomnia is a chronic and often disabling sleep disorder characterized by excessive daytime sleepiness despite adequate or prolonged nighttime sleep. With recent advances in diagnosis and treatment, this review is timely in addressing evolving approaches to understanding and managing idiopathic hypersomnia, a condition that remains underrecognized and frequently misdiagnosed. RECENT FINDINGS: Idiopathic hypersomnia is clinically distinct from narcolepsy, lacking REM-related features such as cataplexy and hypnagogic hallucinations. Until recently, treatment options for idiopathic hypersomnia were limited and often off-label. The 2021 FDA approval of low-sodium oxybate (LXB) marked the first medication specifically indicated for idiopathic hypersomnia. Additional agents such as modafinil, pitolisant, and traditional stimulants are used off-label with varying efficacy. Ongoing research is exploring promising treatments, including orexin-2 receptor agonists (e.g. ALKS 2680, ORX-750), serdexmethylphenidate, and flumazenil, which offer new hope for personalized management. SUMMARY: Growing insights into the pathophysiology and clinical features of idiopathic hypersomnia have led to improved diagnostic clarity and therapeutic innovation. These developments carry significant implications for clinical practice, offering hope for better symptom control and quality of life in affected individuals. Ongoing research is crucial for refining treatment strategies and deepening our understanding of this complex condition.

Primary ciliary dyskinesia phenotypes and correlation with genotype.

Horani A, Wee W, Omran H … +1 more , Ferkol T

Curr Opin Pulm Med · 2025 Nov · PMID 40948093 · Full text

PURPOSE OF REVIEW: Primary ciliary dyskinesia is a rare, inherited disease, and over 60 genes have been linked to motile ciliopathies. During the past quarter century, our understanding of the complex genetics and biolog... PURPOSE OF REVIEW: Primary ciliary dyskinesia is a rare, inherited disease, and over 60 genes have been linked to motile ciliopathies. During the past quarter century, our understanding of the complex genetics and biological function of motile cilia has greatly advanced. RECENT FINDINGS: Our growing knowledge of genetics and pathophysiology of primary ciliary dyskinesia has yielded insights into novel clinical features and genotype-phenotype relationships in motile ciliopathies. Children with biallelic CCDC39 or CCDC40 mutations have greater lung disease, related to both cilia motility-dependent and motility-independent effects. Pathogenic variants in genes involved in cilia generation, like CCNO , are also associated with more severe lung disease. Conversely, people who have defects in other genes, like DHAH11 and RSPH1 , have less severe lung disease, possibly related to residual ciliary motility. Finally, a growing number of primary ciliopathies are associated with abnormal motile cilia ultrastructure and function, and specific pathogenic variants can lead to distinct clinical presentations, best illustrated by structure-function studies in TUBB4B . SUMMARY: These findings have yielded new insights into the clinical heterogeneity of motile ciliopathies, thus broadening their clinical spectrum. Additional research to elucidate the underlying pathophysiology in these overlapping conditions is warranted.

Challenges and opportunities for drug development in rare pulmonary diseases like cystic fibrosis: an industry perspective.

Sellers ZM, Cohen AH

Curr Opin Pulm Med · 2025 Nov · PMID 40916973 · Full text

PURPOSE OF REVIEW: There is a critical need for new therapies addressing the high unmet needs of individuals with rare lung diseases. This review examines the challenges industry sponsors face in developing therapeutic p... PURPOSE OF REVIEW: There is a critical need for new therapies addressing the high unmet needs of individuals with rare lung diseases. This review examines the challenges industry sponsors face in developing therapeutic products for rare lung diseases, using cystic fibrosis as an example. RECENT FINDINGS: Since the development of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, the drug development landscape for cystic fibrosis has changed. New challenges include defining success in an era of small molecule CFTR modulators, recruitment from a small, ultra-rare population, limited experience with novel trial designs and biomarkers, and fluctuations in funding opportunities. SUMMARY: While challenges to drug development in rare lung disease, including cystic fibrosis, these challenges also present opportunities for innovation amongst industry sponsors, researchers, foundations/advocacy groups, regulators, and funders. Through collaborative partnerships, we can achieve our collective goal of improving the quality and length of lives of those suffering from rare lung diseases.

Primary ciliary dyskinesia: clinical manifestations and current diagnostic approaches.

Reklow RJ, Weir MJ, Dell SD

Curr Opin Pulm Med · 2025 Nov · PMID 40916971 · Publisher ↗

PURPOSE OF REVIEW: This review summarizes the clinical symptoms of primary ciliary dyskinesia (PCD) beginning at birth and current approaches for confirming diagnosis. Strengths and limitations of innovative adjunctive t... PURPOSE OF REVIEW: This review summarizes the clinical symptoms of primary ciliary dyskinesia (PCD) beginning at birth and current approaches for confirming diagnosis. Strengths and limitations of innovative adjunctive tests to improve detection are discussed, ultimately highlighting the importance of PCD expert networks to develop standardized guidelines and develop a standalone diagnostic tool. RECENT FINDINGS: PCD is underdiagnosed globally, reflecting overall awareness of this disease and limitations of diagnostic approaches. Over 50 disease-causing genes have been characterized, yet more are discovered each year. No single test can detect all PCD cases, therefore further research is needed to improve clinical options for diagnosis. SUMMARY: PCD is a genetic ciliopathy with serious health complications and impacts on quality of life. Clinical manifestation can vary significantly between individuals, which can delay diagnosis and negatively affect patient outcomes. Current diagnostic tests for PCD require significant resources and training to interpret, and the best-available tests may miss up to 30% of cases. Further work facilitated by expert collaborative networks will be instrumental to develop novel, enhanced diagnostic tools and ultimately improve outcomes for patients.

New anti-infective approaches to treat airway infections in persons with cystic fibrosis and bronchiectasis.

Massey J, Haidar G, Shields RK … +1 more , Van Tyne D

Curr Opin Pulm Med · 2025 Nov · PMID 40916970 · Publisher ↗

PURPOSE OF REVIEW: Cystic fibrosis (CF) and non-CF bronchiectasis can predispose patients to airway infections that are difficult to treat. The purpose of this review is to discuss recently developed anti-infectives whic... PURPOSE OF REVIEW: Cystic fibrosis (CF) and non-CF bronchiectasis can predispose patients to airway infections that are difficult to treat. The purpose of this review is to discuss recently developed anti-infectives which show promise in treating these infections. RECENT FINDINGS: The microbiology underlying respiratory tract infections in persons with CF (pwCF) and non-CF bronchiectasis is complex. Both traditional and nontraditional anti-infective approaches have recently been discovered and/or are actively being studied for the treatment of airway infections. Traditional antibiotics, including small molecules/compounds/formulations, and nontraditional methods, such as monoclonal antibodies and bacteriophages, have shown promise in their ability to treat airway infections in case studies, case series, and/or clinical trials. SUMMARY: Several new approaches are currently being developed to better manage airway infections associated with both CF and non-CF bronchiectasis. While many of these new therapies are promising, more studies are needed to assess their safety and efficacy.

Bronchiectasis evaluation 2025: pediatric and adult perspectives.

Tolle J, O'Connor M

Curr Opin Pulm Med · 2025 Nov · PMID 40916968 · Publisher ↗

PURPOSE OF REVIEW: There is a significant overlap between the diagnostic evaluation for adult and pediatric patients with bronchiectasis; however, also important age-specific unique considerations. This review focuses on... PURPOSE OF REVIEW: There is a significant overlap between the diagnostic evaluation for adult and pediatric patients with bronchiectasis; however, also important age-specific unique considerations. This review focuses on these specific considerations. RECENT FINDINGS: Bronchiectasis refers to the radiographic evidence of dilation of distal and proximal bronchi secondary to chronic infection and inflammation. Bronchiectasis can be suspected on plain chest radiograph but is confirmed and detailed through computed tomography (CT) imaging. Several different measures and descriptions of the radiographic findings of bronchiectasis exist, but the most common is a bronchial diameter equal to or greater than an adjacent blood vessel. Consideration for the presence of bronchiectasis begins with recognition of clinical symptoms of suppurative lung disease including persistent sputum producing cough and recurrent respiratory infections. Bronchiectasis etiologies include inherited forms, such as cystic fibrosis and primary ciliary dyskinesia, as well as secondary forms including chronic aspiration as well as certain infections, and immunodeficiency. Up to 40% remain idiopathic even after a comprehensive evaluation. SUMMARY: It is important to start a bronchiectasis evaluation with a broad differential, but secondary testing should focus on etiologies specific to the patient. A thoughtful combination of testing is often required to arrive at an etiology. Patients with bronchiectasis require ongoing monitoring including longitudinal follow-up of respiratory cultures, lung function testing, and repeat CT imaging.

Obstructive sleep apnea and the gut microbiota - mechanisms and opportunities.

Alcock J

Curr Opin Pulm Med · 2025 Nov · PMID 40916965 · Publisher ↗

PURPOSE OF REVIEW: The relationship between obstructive sleep apnea (OSA) and the gut microbiota is increasingly recognized, yet the involvement of specific microbial taxa and the direction of causality remain unclear. T... PURPOSE OF REVIEW: The relationship between obstructive sleep apnea (OSA) and the gut microbiota is increasingly recognized, yet the involvement of specific microbial taxa and the direction of causality remain unclear. This review synthesizes current evidence linking gut dysbiosis with disordered sleep, with a focus on OSA and its associated complications. RECENT FINDINGS: Studies of alpha and beta microbial diversity in OSA patients, sampled at different sites, have had inconsistent results. Members of the genus Fusobacterium and family Lachnospiraceae are enriched in some studies of OSA and have been linked with gut barrier permeability and complications, such as hypertension. OSA treatments, including positive airway pressure and GLP-1 agonists, have varying effects on the microbiota. SUMMARY: Microbiota-targeted therapies may reduce OSA-related complications, but these potential treatments require additional well designed trials to clarify the bidirectional relationship between gut microbes and sleep-related health.

Obstructive sleep apnea in pregnancy: emerging insights into maternal and fetal outcomes.

Ansari S, Buntak V, Bourjeily G

Curr Opin Pulm Med · 2025 Nov · PMID 40916951 · Publisher ↗

PURPOSE OF REVIEW: Sleep disordered breathing (SDB) encompasses a spectrum of disorders ranging from snoring to complete upper airway collapse and cessation of respiration leading to oxygen desaturation and sleep fragmen... PURPOSE OF REVIEW: Sleep disordered breathing (SDB) encompasses a spectrum of disorders ranging from snoring to complete upper airway collapse and cessation of respiration leading to oxygen desaturation and sleep fragmentation. SDB is highly prevalent in the pregnant population, particularly predisposed to SDB because of physiological changes such as hormonal changes, changes to body habitus, and respiratory mechanics due to the gravid uterus. RECENT FINDINGS: The disorder has been associated with adverse maternal outcomes in pregnancy such as gestational hypertension, preeclampsia, impaired glucose metabolism, severe maternal cardiovascular morbidity, and long-term morbidity, as well as maternal mental health conditions with potential societal consequences. There is also mounting evidence on the impact of SDB with regards to the fetal and neonatal outcomes, including preterm birth, altered growth trajectories, and congenital anomalies, as well as evidence of epigenetic changes in the offspring exposed to disordered breathing during sleep. SUMMARY: This review focuses on SDB in pregnancy and recent data demonstrating its impact on maternal morbidity and fetal and neonatal outcomes, and recent guidelines and data on treatment modalities and their impact on pregnancy health.

Evolving cystic fibrosis care models in the modulator era.

Martin I, Ratjen F, Flume P

Curr Opin Pulm Med · 2025 Nov · PMID 40916950 · Publisher ↗

PURPOSE OF REVIEW: The advent of CFTR modulators and the adoption of telemedicine during the COVID-19 pandemic have prompted reconsideration of cystic fibrosis (CF) care models. This review explores how care delivery may... PURPOSE OF REVIEW: The advent of CFTR modulators and the adoption of telemedicine during the COVID-19 pandemic have prompted reconsideration of cystic fibrosis (CF) care models. This review explores how care delivery may evolve in response to these changes. RECENT FINDINGS: Emerging evidence highlights the heterogeneity in response to CFTR modulators, with some patients continuing to experience disease progression. Preliminary trial data have explored therapy de-escalation, but long-term safety remains uncertain. Challenges in microbiological surveillance, particularly due to reduced sputum production, complicate monitoring. Early efforts to define "stability" have led to position statements advocating risk-stratified, hybrid care models. SUMMARY: CF care models should shift toward individualized, flexible approaches that prioritize equity and safety. Clinical trials and registry analyses will be essential to validate such models. Until then, conservative implementation with continued multidisciplinary support and objective monitoring are advised.

Glucagon-like peptide-1 receptor agonists for the treatment of obstructive sleep apnea.

D'Annibale DA, Mimoto M, McCowen KC … +1 more , Malhotra A

Curr Opin Pulm Med · 2025 Nov · PMID 40855981 · Full text

PURPOSE OF REVIEW: This review highlights the emerging data on the use of incretin therapies, including glucagon-like peptide-1 receptor agonists (GLP-1RA) and dual GLP-1RA and glucose-dependent insulinotropic peptide (G... PURPOSE OF REVIEW: This review highlights the emerging data on the use of incretin therapies, including glucagon-like peptide-1 receptor agonists (GLP-1RA) and dual GLP-1RA and glucose-dependent insulinotropic peptide (GIP) receptor agonists, on the treatment of obstructive sleep apnea (OSA). Given known cardiometabolic and neurocognitive consequences of OSA, optimizing treatment is essential. In the setting of widespread research efforts and clinical implementation of dual agonists in managing OSA, obesity and other cardiometabolic diseases, this review is timely. RECENT FINDINGS: Several randomized controlled trials and meta-analyses have shown GLP-1 and GIP receptor agonists to reduce apnea-hypopnea index (AHI) and body weight in patients with OSA. This impact has been demonstrated with the use of pharmacotherapy alone and in combination with traditional positive airway pressure (PAP) therapy. GLP-1RA may positively affect OSA through reducing systemic inflammation and decreasing adiposity, including via hormone changes, delayed gastric emptying, and central mechanisms impacting appetite regulation and sleep-wakefulness. SUMMARY: Novel pharmacological advances in individuals with OSA and obesity have shown promise in cardiometabolic disease control. Longitudinal follow-up to monitor the efficacy and adverse effects of incretin therapies, and further comparison studies with PAP therapy, are warranted.

Transforming sleep medicine: the evolving role of artificial intelligence.

Banjade P, Sharma M, Surani S

Curr Opin Pulm Med · 2025 Nov · PMID 40855966 · Publisher ↗

PURPOSE OF REVIEW: Artificial intelligence (AI) is in the era of rapid evolution. Like other healthcare fields, AI has significantly impacted sleep medicine. We aim to explain the evolving role of AI in sleep medicine an... PURPOSE OF REVIEW: Artificial intelligence (AI) is in the era of rapid evolution. Like other healthcare fields, AI has significantly impacted sleep medicine. We aim to explain the evolving role of AI in sleep medicine and provide clinicians with key information related to its benefits and limitations. RECENT FINDINGS: AI technologies, like machine learning and deep learning, improve the detection of sleep disorders, such as obstructive sleep apnea, insomnia, and narcolepsy, through advanced data analysis from tools like polysomnography and consumer sleep devices. AI also enables targeted therapies by endotyping sleep disorders, optimizing patient care, and reducing unnecessary treatments. On the other hand, there are many challenges that need to be addressed before using AI in clinical settings. Ethical issues regarding patient privacy, biases, and transparency regarding data use are some of the key challenges. SUMMARY: AI could transform sleep medicine by enhancing diagnostic accuracy and personalizing treatment plans. Effective collaboration between clinicians and AI experts is necessary to use AI optimally in clinical settings.

An update on sleep disordered breathing in spinal cord injury.

Berlowitz DJ, Graco M

Curr Opin Pulm Med · 2025 Nov · PMID 40832774 · Full text

PURPOSE OF REVIEW: Sleep disordered breathing (SDB) is a direct consequence of tetraplegic spinal cord injury (SCI), is highly prevalent in both tetraplegia and paraplegia, and is associated with worse daytime functionin... PURPOSE OF REVIEW: Sleep disordered breathing (SDB) is a direct consequence of tetraplegic spinal cord injury (SCI), is highly prevalent in both tetraplegia and paraplegia, and is associated with worse daytime functioning and reduced quality of life. Despite this, most people with SCI are undiagnosed and untreated for the disorder. This narrative review summarises research from the last 5 years on the epidemiology, pathophysiology, and consequences of SDB in SCI, as well as the current approaches to screening, diagnosis, and treatment of SDB in this population. RECENT FINDINGS: Previous research predominantly focussed on SDB in tetraplegia, however recent studies have established that people with paraplegia also experience substantially higher prevalence than the general population. SDB risk screening questionnaires are not helpful because SDB in SCI is so prevalent, and questionnaires alone cannot exclude true negative cases. Alternative treatments, such as mandibular advancement devices, are feasible and likely effective, and alternative care models may improve rates of diagnosis and access to treatments. SUMMARY: Recent research into SDB in SCI has identified novel, emergent themes, however researchers must collaborate more to achieve sample sizes that can deliver impact in this relatively rare population.

Does glucose metabolism and its consequences depend on the phenotype of obstructive sleep apnea?

Suwannakin A, Reutrakul S, Chirakalwasan N

Curr Opin Pulm Med · 2025 Nov · PMID 40802566 · Publisher ↗

PURPOSE OF REVIEW: Obstructive sleep apnea (OSA) is a common form of sleep-disordered breathing, with rising prevalence and increasingly recognized for its association with multisystem involvement, particularly abnormali... PURPOSE OF REVIEW: Obstructive sleep apnea (OSA) is a common form of sleep-disordered breathing, with rising prevalence and increasingly recognized for its association with multisystem involvement, particularly abnormalities in glucose metabolism. This review examined the relationship between OSA and glucose metabolism and associated cardiovascular outcomes. RECENT FINDINGS: OSA is a significant risk factor for the development of abnormal glucose metabolism and is strongly associated with incident cardiovascular disease, partly mediated by impaired glucose regulation. Emerging evidence highlights a bidirectional relationship between OSA and glucose dysregulation, including insulin resistance and type 2 diabetes. Specific OSA phenotypes such as rapid eye movement (REM)-related OSA and marked nocturnal desaturation have been associated with worsened glycemic control. However, current data show inconsistent improvements in glucose homeostasis following continuous positive airway pressure (CPAP) therapy, indicating the need for more targeted approaches. Meanwhile, weight loss by lifestyle modification, bariatric surgery or medications have been shown to improve OSA as well as glycemic control in people with diabetes. SUMMARY: Personalized strategies targeting specific OSA phenotypes may improve metabolic outcomes in patients with coexisting glucose dysregulation. Integrating metabolic biomarkers into clinical practice could enable earlier detection of maladaptive changes and support precision-guided interventions to improve long-term outcomes.

Evolving management of sarcoidosis in the real world.

Culver DA, Ribeiro Neto ML

Curr Opin Pulm Med · 2025 Sep · PMID 40767091 · Publisher ↗

PURPOSE OF REVIEW: The management of sarcoidosis has relied mainly on glucocorticoids for over 80 years. Innumerable review articles, as well as all current guidelines, position systemic steroids as first line therapy fo... PURPOSE OF REVIEW: The management of sarcoidosis has relied mainly on glucocorticoids for over 80 years. Innumerable review articles, as well as all current guidelines, position systemic steroids as first line therapy for moderate and severe sarcoidosis. Despite accumulating evidence of short term (mostly overt) and long term (often unrecognized) toxicities of steroids, and the example of specialists treating diseases like rheumatoid arthritis and inflammatory bowel disease, the treatment paradigm for sarcoidosis has remained stubbornly affixed to steroids. RECENT FINDINGS: In 2025, there is now compelling evidence to relegate steroids to a lesser role in the management of sarcoidosis. Nonsteroid agents, especially methotrexate, can be used as first line therapy for many patients. SUMMARY: As steroid use diminishes, the development and implementation of additional steroid-sparing agents will assume increased importance.

The fluid challenge for identification of pulmonary hypertension associated with left heart disease.

D'Alto M, Vergara A, Orlando A … +1 more , Naeije R

Curr Opin Pulm Med · 2025 Sep · PMID 40767090 · Publisher ↗

PURPOSE OF REVIEW: Left heart disease (LHD) is the commonest cause of pulmonary hypertension (PH). The differential diagnosis between PH associated with LHD (PH-LHD) and pulmonary arterial hypertension (PAH) may be diffi... PURPOSE OF REVIEW: Left heart disease (LHD) is the commonest cause of pulmonary hypertension (PH). The differential diagnosis between PH associated with LHD (PH-LHD) and pulmonary arterial hypertension (PAH) may be difficult. PH associated with LHD is causally related to chronically increased pulmonary artery wedge pressure (PAWP). However, PAWP may be "falsely" normal or high-normal at the time of diagnostic right heart catheterization. RECENT FINDINGS: Updated guidelines for step-by-step diagnosis of PAH and LHD leave nevertheless a proportion of patients with PH and diagnostic uncertainty. In these patients, several studies have shown that a PAWP >18 mmHg after a rapid infusion of 500 ml saline is associated with a high likelihood of LHD. Evidence has been accumulated that patients with PH, cardiovascular risk factors and a high-normal PAWP should have LHD excluded by a fluid challenge. Preliminary studies suggest that the test may be performed noninvasively by combining Doppler echocardiography and lung ultrasound showing respectively a ratio of trans-mitral flow E wave to mitral annulus tissue velocity e' (E/e') ≥12 and ≥5 B-lines. SUMMARY: A fluid challenge has a place in the step-by-step diagnostic work-up of patients referred for PH for the differential diagnosis between PH-LHD and PAH.

Sex differences in pulmonary (arterial) hypertension: does it matter?

Ventetuolo CE, Sherman-Roe AE

Curr Opin Pulm Med · 2025 Sep · PMID 40767089 · Full text

PURPOSE OF REVIEW: This review synthesizes the current prevailing theories behind the 'sex paradox' or 'sex puzzle' in pulmonary arterial hypertension (PAH), a disease marked by sexual dimorphism. To a lesser extent, we... PURPOSE OF REVIEW: This review synthesizes the current prevailing theories behind the 'sex paradox' or 'sex puzzle' in pulmonary arterial hypertension (PAH), a disease marked by sexual dimorphism. To a lesser extent, we also review sex differences in other forms of pulmonary hypertension. RECENT FINDINGS: Although more females than males develop PAH worldwide, female sex is associated with improved right ventricular (RV) function and survival. We review the role of sex chromosomes and sex hormones and their relationships to genomic and epigenetic regulation, immune function, and RV function, sex-based differences in therapeutic response and social determinants of health and intersectionality with gender in PAH pathobiology, prevalence and outcomes. We include experimental studies and observational human data that have led to the study of sex hormone modulation as a treatment strategy in PAH, with recently completed clinical trials. In addition, we explore potential future directions to help understand the mechanisms that underpin sex biases in pulmonary vascular disease, as well as those that may inform potential therapeutic targets. SUMMARY: Survival in PAH depends on RV function, and females have improved survival despite increased prevalence for reasons that remain unclear. While knowledge gaps remain, recent advancements offer promise and many future directions.
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