European Respiratory Review最新文献

{"title":"Anti-HLA antibodies in lung transplantation: diagnostic pitfalls and future directions.","authors":"Andrea Zajacova, Eva Revilla-Lopez, Miray Guney, Steffi De Pelsmaeker, Marie-Paule Emonds, Maarten Naesens, Berta Saez-Gimenez, Bart M Vanaudenaerde, Robin Vos","doi":"10.1183/16000617.0298-2025","DOIUrl":"10.1183/16000617.0298-2025","url":null,"abstract":"<p><p>Lung transplant recipients face high rejection rates, causing significant disease burden and limiting long-term outcomes. Among immunological factors affecting lung allografts, anti-human leukocyte antigen (HLA) antibodies, particularly donor-specific anti-HLA antibodies (DSAs), are key mediators of antibody-mediated rejection. Yet, the biology, detection and interpretation of DSAs remain incompletely defined across the pre-, peri- and post-transplant continuum. HLAs are highly polymorphic immune recognition molecules, and donor-recipient mismatches drive alloimmune responses. HLA typing is used to assess genetic disparity, but low-resolution approaches risk misclassifying mismatches and DSAs, whereas high-resolution typing improves diagnostic accuracy yet is not universally implemented. Standard HLA antibody monitoring assays report median fluorescence intensity. However, median fluorescence intensity does not convey information on the affinity, avidity or functional capacity of these antibodies, and may be affected by technical factors such as bead saturation, the Hook effect or binding to denatured HLAs. Subclass profiling adds further complexity: IgG1 and IgG3 are potent complement activators, whereas IgG2 and IgG4 have weaker or regulatory roles and differ in clearance by apheresis. Functional assays, including C1q- or C3d-binding HLA antibody detection assays and emerging endothelial or natural killer-cell-based platforms, offer additional insights into antibody-binding, complement activation and cytotoxic potential, but are not routinely applied in current clinical practice.Prospective mechanistic studies are required to define the clinical benefit, cost-effectiveness and optimal integration of these advanced immunological tools into routine practice pre-, peri- and post-lung transplant.DSAs drive lung allograft injury, yet their detection and interpretation remain inconsistent. Implementation of high-resolution HLA typing and functional assays may improve risk assessment and guide future clinical practice.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12976880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147431777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Old and emerging therapies for childhood interstitial lung disease (chILD): a systematic review.","authors":"Valentina Tonazzo, Raimondo Junior Castaldo, Stefania Zanconato, Silvia Carraro, Valentina Agnese Ferraro","doi":"10.1183/16000617.0062-2025","DOIUrl":"10.1183/16000617.0062-2025","url":null,"abstract":"<p><p>Childhood interstitial lung disease (chILD) is a heterogeneous group of rare lung diseases that comprises more than 200 entities. The diagnostic process for chILD might require multiple investigations, which often include a combination of clinical assessments, imaging studies, genetic testing and invasive procedures. Given the prolonged diagnostic timeline, supportive treatments are typically initiated followed by more targeted therapies generally postponed until the underlying cause of the disease is identified. This systematic review aims to describe the current knowledge regarding different treatment strategies for chILD and critically appraise, compare and qualitatively summarise the current evidence on old and emerging therapies for chILD. Of the 5775 publications returned from the initial search, 100 studies met the inclusion criteria, of which 50% were reviews or task force reports from several scientific societies, 41% case reports or case series, 5% randomised controlled trials and 4% preclinical studies on emerging therapies. In order to provide a clearer description of the data analysed, we describe available therapeutic options for chILD in general, organised by treatment type, and then we report treatments categorised by specific pathology.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12976882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147431909","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact of elexacaftor-tezacaftor-ivacaftor on cardiometabolic risk factors: a systematic review.","authors":"Heather Girouard, Farah Jaber, Josh Gharib, Valérie Boudreau, Laure Alexandre-Heymann, Adèle Coriati","doi":"10.1183/16000617.0197-2025","DOIUrl":"10.1183/16000617.0197-2025","url":null,"abstract":"<p><p>Elexacaftor-tezacaftor-ivacaftor (ETI) is a highly effective modulator therapy associated with significant improvements in respiratory function and quality of life among people living with cystic fibrosis (CF). However, ETI has also been shown to result in significant weight gain and there is emerging interest regarding traditional cardiometabolic risk factors. This systematic review investigated the effects of ETI on cardiometabolic risk factors (obesity/overweight, body composition, glucose metabolism, lipids, blood pressure and bone parameters) in people living with CF. This review adhered with Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines and was registered in PROSPERO (CRD42025630118). The following databases were searched in December 2024 and June 2025: PubMed, Scopus, Web of Science and Embase. A total of 54 publications met inclusion criteria (including ETI treatment duration >4 months, >10 total participants, empirical studies) and underwent data extraction. Risk of bias was assessed using National Institutes of Health tools. Results were prepared in tabular format and narrative summary. There was notable heterogeneity in study design and results. In published studies to date, ETI initiation was generally followed by a decrease in glycated haemoglobin, but minimal change in other glycaemic or insulinaemic parameters. Some studies reported increased blood pressure, hypertension and overweight/obesity classification, and cholesterol levels. The observational design and short duration of most studies limit the ability to conclude direct effects of ETI, particularly in the long term. However, we conclude there is likely increased risk of certain traditional cardiometabolic risk factors in ageing people with CF, thus highlighting the importance of clinical surveillance and prompt intervention if needed.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12933258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147304310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Physical inactivity in chronic airways disease: an important candidate in the treatable traits paradigm.","authors":"Benjamin Griffiths, Reem Alajmi, Ian J Clifton, Rebecca J Birch, Daniel Peckham, Oliver J Price","doi":"10.1183/16000617.0165-2025","DOIUrl":"10.1183/16000617.0165-2025","url":null,"abstract":"<p><strong>Background: </strong>Physical inactivity is a common and potentially modifiable trait in individuals with chronic airways disease, yet disease-specific physical activity profiles and clinical determinants remain poorly defined.</p><p><strong>Methods: </strong>We conducted a systematic review and meta-analysis in accordance with PRISMA guidelines to characterise physical activity profiles across the spectrum of chronic airways disease. Studies reporting objectively measured physical activity in adults with COPD, asthma, noncystic fibrosis bronchiectasis, cystic fibrosis or primary ciliary dyskinesia were included. Primary outcomes were daily step count and time spent in moderate-to-vigorous physical activity (MVPA). Univariate and multivariate regression analysis was used to explore disease-specific determinants and associations with established clinical outcome measures.</p><p><strong>Results: </strong>236 studies (353 cohorts, n=25 278 with chronic airways disease) met the eligibility criteria. The mean daily step count was 5494 (95% CI 5152-5636) and MVPA was 48.2 min·day^-1 (95% CI 33.8-62.6), with the lowest levels observed in COPD. Physical activity levels were consistently lower than matched healthy controls. Disease-specific determinants of physical activity remained elusive; body mass index and percent predicted forced expiratory volume in 1 s (FEV₁) were significant in COPD and asthma. Step count associated positively with FEV₁ % pred and 6-min walk distance, and negatively with modified Medical Research Council scores.</p><p><strong>Conclusion: </strong>Physical inactivity is highly prevalent across chronic airways diseases and is consistently associated with established clinical outcome measures. These findings highlight the clinical relevance of objective physical activity assessment and support its consideration within the treatable traits framework as part of routine disease evaluation and management.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12933259/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147304323","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment and management of post-COVID-19 pulmonary complications: a rapid review.","authors":"Wade Michaelchuk, Lesley J J Soril, Dacia Chiarieri-Hirsch, Emily Giroux, Julie Shatto, Andrea S Gershon, Samir Gupta, Michael K Stickland, Grace Y Lam","doi":"10.1183/16000617.0010-2025","DOIUrl":"10.1183/16000617.0010-2025","url":null,"abstract":"<p><p>The rising global prevalence of post-COVID-19 condition (PCC) underscores the substantial and ongoing burden faced by individuals following severe acute respiratory syndrome coronavirus 2 infection. The volume of emerging evidence regarding pulmonary-related PCC complications highlights the urgent need for current, evidence-informed guidelines to ensure timely assessment and effective treatment for those affected by PCC. Thus, the aim of this review was to synthesise existing research on the management and treatment of pulmonary complications in individuals with PCC. A rapid review of published and grey literature focused on pulmonary-related PCC complications was completed in November 2023 and updated in June 2025, in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. We identified 73 unique articles, including 12 guidance documents, 24 secondary studies (including 11 systematic reviews with meta-analyses, eight systematic reviews and three scoping reviews) and 37 primary research studies (13 randomised controlled trials) and narratively synthesised their findings. Guidance documents addressed workup and management for pulmonary-related PCC complications, recommending the use of pulmonary function testing with diffusing capacity and the importance of ruling out other conditions. Although evidence regarding the use of medical and pharmacological interventions for treatment of pulmonary-related PCC complications were limited and inconclusive, the current evidence base suggested potential effectiveness of a multidisciplinary rehabilitation approach for pulmonary-related PCC treatment, involving specialist consultations and tailored rehabilitation programmes. The heterogeneity in study quality and risk of bias warrants cautious interpretation of the findings. The current evidence and evolving healthcare landscape suggest the need for updated, evidence-informed clinical guidance.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12933261/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147304126","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bronchiectasis in children: a systematic review of cytokine profile, immune cell phenotypes and microbiota in bronchoalveolar lavage fluid.","authors":"Christian Magnus Kragh Thomsen, Simon Kromann-Thomsen, Signe Thim, Sune Rubak","doi":"10.1183/16000617.0071-2025","DOIUrl":"10.1183/16000617.0071-2025","url":null,"abstract":"<p><strong>Background: </strong>Paediatric noncystic fibrosis bronchiectasis (NCFB) is a chronic respiratory condition characterised by airway infection, chronic inflammation, mucociliary dysfunction and structural lung damage. Emerging evidence highlights the importance of the local immune response and microbial environment in driving disease progression.</p><p><strong>Objectives: </strong>This systematic review aimed to summarise contemporary evidence on bronchoalveolar lavage (BAL)-derived cytokine levels, immune cell phenotypes and microbiota in paediatric NCFB, with a focus on their role in disease pathogenesis, phenotype classification and potential to inform targeted interventions.</p><p><strong>Methods: </strong>The review followed PRISMA guidelines and was registered with PROSPERO (CRD42024520391). Six electronic databases were searched for studies investigating BAL cytokines, immune cells or microbiota in paediatric NCFB. Data extraction followed a pre-defined template and methodological quality was assessed using the RoB 2.0 and AXIS tools.</p><p><strong>Results: </strong>20 studies were included. Methodological and clinical heterogeneity precluded meta-analysis. Across studies, neutrophils consistently dominated the cellular profile, closely associated with pathogen detection, particularly <i>Haemophilus influenzae</i> and BAL-derived immunological biomarkers. Cytokine profiling demonstrated consistent elevations of pro-inflammatory mediators, notably interleukin-8, with variable associations to disease severity and pathogen-specific immune responses. Microbiota analyses, though limited, suggest that paediatric NCFB is not defined by a distinct microbial signature of the lower airways.</p><p><strong>Conclusion: </strong>Current evidence supports a model in which neutrophilic inflammation, driven by dysregulated cytokine responses and potentially sustained by microbial dysbiosis, is central to the pathophysiology of paediatric NCFB. Key gaps remain regarding dysbiosis, adaptive immunity and the longitudinal trajectories of immune mediators. Addressing these may support biomarker development and targeted therapies.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12933260/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147304104","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mechanistic basis for the antifibrotic actions of cAMP-based therapies.","authors":"Marc Peters-Golden, Sean M Fortier","doi":"10.1183/16000617.0265-2025","DOIUrl":"10.1183/16000617.0265-2025","url":null,"abstract":"<p><p>The human and economic impact of idiopathic pulmonary fibrosis and other interstitial lung diseases is enormous, and available therapies are of limited utility. A decade after the introduction of the first antifibrotic agents, two new agents are on the horizon. Nerandomilast is an inhibitor of phosphodiesterase 4B, while treprostinil is an analogue of prostacyclin. Both agents increase intracellular cAMP. Although the smooth muscle relaxant properties of agents that increase cAMP have long been leveraged for the treatment of airway and vascular diseases, potential antifibrotic actions of cAMP elevation are much less well appreciated by clinicians and researchers. The purpose of this review is to discuss the mechanistic underpinnings for a beneficial role of cAMP in fibrotic lung diseases. We briefly review the pathogenesis of fibrotic lung disease, the anatomy of the cAMP pathway, and the myriad ways in which this pathway is disrupted in fibrotic diseases. We then focus on the pleiotropic actions by which cAMP opposes the aberrant phenotypes of immune cells, fibroblasts, and epithelial cells that characterise fibrotic diseases. Finally, we highlight some unanswered questions about, and future opportunities for optimising, therapeutic interventions that leverage the cAMP pathway.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12914378/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146217918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary diffusing capacity for nitric oxide in disease: a scoping review.","authors":"Thomas Radtke, Hà Pham-Ngoc, Thong Hua-Huy, Connie C W Hsia, Holger Dressel, Anh Tuan Dinh-Xuan","doi":"10.1183/16000617.0145-2025","DOIUrl":"10.1183/16000617.0145-2025","url":null,"abstract":"<p><strong>Objective: </strong>This scoping review aims to map the available studies on single-breath pulmonary diffusing capacity for nitric oxide (<i>D</i> _LNO) in various clinical diseases and identify gaps for future research.</p><p><strong>Methods: </strong>We followed the JBI methodology for scoping reviews. A systematic literature search was conducted in Embase, MEDLINE (EBSCOhost), PubMed, Scopus, Web of Science and the Cochrane Library to identify studies on <i>D</i> _LNO from 1983 to 2025. Two reviewers screened abstracts using Rayyan software and extracted data using standardised templates implemented into REDCap (Research Electronic Data Capture).</p><p><strong>Results: </strong>We identified 1638 studies, of which 69 met the eligibility criteria. These studies represented respiratory (n=22), immunological/genetic/systemic (n=14), post-COVID-19 sequelae (n=13), cardiovascular (n=9), metabolic/endocrine/renal (n=6), environmental-related (n=3) and other (n=2) conditions. There was substantial heterogeneity in disease categories, sample sizes and reporting methods. Nearly half of the studies (49.3%) used convenience sampling, where participant selection processes are often poorly described; 62.3% included <50 participants. Only 18.8% reported a sample size calculation, and 10.1% registered or published a study protocol. Disparate findings within disease categories were often difficult to interpret; and small sample sizes limited generalisability. In some specific conditions, <i>D</i> _LNO showed sensitivity in detecting interstitial and fibrotic changes compared to conventional single-breath pulmonary diffusing capacity for carbon monoxide (<i>D</i> _LCO) while simultaneous <i>D</i> _LNO-<i>D</i> _LCO measurements permitted the detection of pulmonary vascular-haematological perturbations.</p><p><strong>Conclusion: </strong>Single-breath <i>D</i> _LNO-<i>D</i> _LCO may provide additional pathophysiological insight by assessing the relative contributions from membrane and blood resistance of diffusion. Larger studies are required to clarify its interpretation across disease states.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12914377/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146218661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Scoping out sarcoidosis: the evolving role of bronchoscopy.","authors":"Prince Ntiamoah, Felix Wireko, Ajay Wagh, Raul Mendoza-Ayala, Francisco Almeida, Joseph Cicenia, Manuel L Ribeiro Neto","doi":"10.1183/16000617.0122-2025","DOIUrl":"10.1183/16000617.0122-2025","url":null,"abstract":"<p><p>The bronchoscopic approach to diagnosing sarcoidosis has evolved significantly with the advent of advanced endoscopic and imaging-guided modalities. Bronchoscopy remains the cornerstone for both histological confirmation and therapeutic intervention, offering minimally invasive access to the mediastinum, lung parenchyma and airways. This review integrates traditional techniques, including bronchoalveolar lavage, endobronchial biopsy and transbronchial biopsy, with advanced modalities such as endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial lung cryobiopsy, endobronchial ultrasound-guided intranodal forceps biopsy and mediastinal cryobiopsy. Emerging tools, including elastography and confocal laser endomicroscopy, are also explored for their potential to enhance diagnostic precision. A stage-based diagnostic algorithm is proposed to guide procedural choices tailored to clinical presentation. The role of bronchoscopy in diagnosing stage 0 and extrapulmonary sarcoidosis is discussed. This comprehensive update synthesises recent evidence to provide a practical framework for clinicians navigating complex diagnostic scenarios in sarcoidosis.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12914379/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146218596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.","authors":"Annalisa Villa, Sébastien Sanges, Vincent Sobanski, Nicolas Lamblin, Edoardo Rosato, Laurent Godinas, Eric Hachulla, Marion Delcroix, David Montani, David Launay","doi":"10.1183/16000617.0137-2025","DOIUrl":"10.1183/16000617.0137-2025","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a severe disease characterised by a progressive thickening and obliteration of pulmonary vessels, resulting in increased vascular resistance, elevated pulmonary artery pressures, and right heart failure. Among the various conditions associated with PAH, systemic sclerosis (SSc) is the most common in Western countries. Compared to other forms of PAH, SSc-PAH presents with a more aggressive clinical course, poorer response to conventional therapies and a worse prognosis. However, despite these differences, the overall management of SSc-PAH remains close to idiopathic PAH; and therefore, there is a crucial need for treatment strategies dedicated to this disease. To help fill this gap, we assessed the level of evidence currently available on SSc-PAH management in a systematic literature review that compiled data regarding conventional therapies, immunosuppressants, nonconventional drugs and surgical/interventional procedures. For each study, we highlighted the results specific to the connective tissue disease or SSc subgroups, the haemodynamic characteristics of the patients, and their comorbidities. By doing so, we identified critical gaps in the field, consisting mostly of the lack of studies focusing on SSc-PAH, a substantial heterogeneity in haemodynamic severity (with notable scarcity of data for mild PAH) and the systematic exclusion of relevant comorbidities (such as interstitial lung disease). Building on these data and our cumulative experience, we provide pragmatic, experience-based suggestions tailored to the management of SSc-PAH, that tries to capture the full scope of clinical situations encountered in these patients and help clinicians manage difficult cases where robust data are lacking.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12914380/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146219278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}