Respiration最新文献

{"title":"Predictive factors of clinical success of therapeutic bronchoscopy in malignant central airway obstruction: results from the EpiGETIF registry.","authors":"Pascalin Roy, Lyria Amari, Sophie Laroumagne, Julien Legodec, Clément Fournier, Laurent Cellerin, Christine Lorut, François Gonin, Jean-Michel Vergnon, Thomas Egenod, Nicolas Favrolt, Pascal Schlossmacher, Valérian Bourinet, Loic Perrot, Samy Lachkar, Juliette Camuset, Amandine Briault, Tristan Degot, Christophe Gut-Gobert, Gilles Mangiapan, Jean-Yves Jasnot, Eric Briens, Adrian Crutu, Armelle Marceau, Bénédicte Toublanc, Maxime Dewolf, Julien Dutilh, Nathalie Germain, Julie Tronchetti, Philippe Astoul, Nicolas Guibert, Hervé Dutau","doi":"10.1159/000545568","DOIUrl":"https://doi.org/10.1159/000545568","url":null,"abstract":"<p><strong>Background and objective: </strong>Therapeutic bronchoscopy (TB) is considered a safe and effective treatment for patients with malignant central airway obstruction (MCAO). While many factors have been associated with technical success, it does not always translate in clinical success. Few factors to predict clinical response have been described. The objective of this study was to determine predictive factors of clinical success for patients with MCAO undergoing TB.</p><p><strong>Methods: </strong>We used the multicenter prospective registry EpiGETIF to collect data from patients with MCAO undergoing TB from January 2019 to June 2021. The criterion for clinical success was dyspnea measured on the Borg scale. Patients were classified as super responders if they had an improvement of 4 points after the procedure. Uni- and multivariate analysis were performed to highlight an association between preprocedural features and clinical success.</p><p><strong>Results: </strong>496 patients from 24 centers met inclusion criteria. The mean preprocedural Borg score was 6.5 ± 2.0 versus 2.2 ± 1.7 postprocedural (mean difference 4.3 ± 2.3). 302 patients (60.9%) were considered super responders. The only factor associated with super responders in multivariate analysis was a higher baseline Borg score. The only factor associated with non-super responders was a poor performance status and mechanical ventilation.</p><p><strong>Conclusion: </strong>Patients show good clinical results following TB for MCAO, influenced positively by a worse pre-procedure dyspnea and negatively by a worse performance status. No other data could help predict the effectiveness of TB, confirming the complexity of the process and heterogeneity of the target population.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-22"},"PeriodicalIF":3.5,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796204","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":"Endobronchial seeding of tuberculous granulomas after EBUS-TBNA of mediastinal lymph nodes.","authors":"Ales Rozman, Izidor Kern","doi":"10.1159/000545506","DOIUrl":"https://doi.org/10.1159/000545506","url":null,"abstract":"<p><p>A 36-year-old non-smoking immunocompetent female patient was admitted due to cough, weight loss, and general malaise. A CT scan revealed a tumor in the left upper lobe with pathological mediastinal lymph nodes. Bronchoscopic biopsy of the tumor and EBUS lymph nodes 11L, 7, and 4R were performed. Histological examination revealed granulomatous inflammation with necrosis and rare tuberculouse bacilli (Figure 1). Cultures remained negative, but Xpert MTB/RIF assay was positive for tuberculosis and negative for antibiotic resistance. The patient received standard six-month tuberculosis therapy, but the lymph nodes and the lesion itself slightly increased on the follow-up CT after six months, and new endobronchial lesions appeared, corresponding to the puncture sites. Upon repeat bronchoscopy, tumor-like growth were found at all three sites previously sampled with EBUS-TBNA, which were completely excised (Figure 2). Histological examination showed granulomas with necrosis but without the presence of bacteria, fungi, or TB bacilli. Xpert MTB/RIF was still marginally positive (Figure 3). The patient, who clinically improved, was not reintroduced to therapy but was kept under careful observation for one year. During this time, the changes on CT regressed, and sputum cultures remained negative. In this presented case, we describe iatrogenic fistulas that developed at the sites of puncture tracts created by previous EBUS-TBNA, through which tuberculosis spread into the airway lumen. Endobronchial seeding after EBUS-TBNA has been likely underreported in the literature (1-2). Similar fistulas could also form in the esophagus in the case of EUS-B, although they have not been reported so far. However, we believe it is important to highlight and recognize the tendency of tuberculosis to form fistulas when considering invasive diagnostics of mediastinal lymph nodes in suspected tuberculosis cases.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-7"},"PeriodicalIF":3.5,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143773155","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":"Machine learning-based model for predicting severe exacerbations in adult-onset type 2 inflammatory asthma.","authors":"JunJie Dai, Huaxiang Ling, Yaqin Liu, Rongchang Chen, Fei Shi","doi":"10.1159/000545039","DOIUrl":"https://doi.org/10.1159/000545039","url":null,"abstract":"<p><strong>Introduction: </strong>Currently, scholars have applied machine learning to the clinical prediction of acute asthma exacerbations. However, given the heterogeneity of inflammatory phenotypes in asthma, it is imperative to develop machine learning models tailored to specific asthma inflammatory phenotypes.</p><p><strong>Objective: </strong>To develop predictive models to identify risk factors for the severe exacerbations in adult-onset type 2 inflammatory asthma, which could help facilitate early diagnosis and intervention, potentially reducing healthcare costs.</p><p><strong>Methods: </strong>Retrospective analysis of patients with acute exacerbations of type 2 inflammatory asthma at Shenzhen People's Hospital from May 2017 to September 2022. Patients were categorized into mild-to-moderate exacerbation (n=300) and severe exacerbation groups (n=209). We collected clinical data from all participants, including demographic characteristics, laboratory results, pulmonary function test results, comorbidities, and asthma medication use. We tested four models: decision trees, logistic regression, random forests, and LightGBM. For each model, 80% of the dataset was used for training and 20% was used to validate the models. The area under (AUC) the receiver operator characteristic (ROC) curve was calculated for each model.</p><p><strong>Results: </strong>Multivariate Logistic regression revealed that low ACT scores, low FEV1/FVC ratio, a history of diabetes, high absolute neutrophil count, and a family history of asthma were independent risk factors for severe exacerbations of type 2 inflammatory asthma. LightGBM outperformed all other models, achieving the highest AUC of 0.9344, with sensitivity = 0.8293, specificity =0.9180, PPV = 0.8718, and NPV = 0.8889. The accuracy stood at 0.8824, with an F1 score of 0.8500. The top ten clinical variables impacting the prediction outcome in the LightGBM model were ACT score, FEV1/FVC ratio, age, lactate dehydrogenase, FEV1 % predicted, fasting blood glucose, angiotensin-converting enzyme, duration of disease, Neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio. Finally, through DCA, the clinical decision-support value of the LightGBM model was confirmed, demonstrating its maximum net benefit for type 2 asthma patients across a threshold probability range of 20% to 80%.</p><p><strong>Conclusions: </strong>We have developed and established a prediction model for severe exacerbations of adult-onset type 2 inflammatory asthma using the LightGBM machine learning approach, which exhibits good predictive performance. This model can aid in the early prediction and prevention of severe exacerbations of adult-onset type 2 inflammatory asthma.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-30"},"PeriodicalIF":3.5,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754248","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":"Lung function and symptoms in idiopathic pulmonary fibrosis treated with high flow nasal therapy for one year.","authors":"Francesca Simioli, Anna Annunziata, Maurizia Lanza, Maria Cardone, Antonietta Coppola, Antonella Marotta, Cecilia Calabrese, Giuseppe Fiorentino","doi":"10.1159/000545165","DOIUrl":"https://doi.org/10.1159/000545165","url":null,"abstract":"<p><p>Introduction Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease that subverts the normal structure of the lungs and finally causes respiratory failure. High flow nasal therapy (HFNT) is currently used in the acute setting for IPF with acute respiratory failure (ARF). Also, acute exacerbation of IPF (AE-IPF) and end-stage disease are common indications. Chronic cough is often an unmet need in IPF because it is partially responsive to common pharmacological treatment. Moreover, opioids have known adverse events. The aim of this paper is to investigate the effects and safety of chronic HFNT on lung function and symptoms of IPF. Methods This is a single center case-control study including patients affected by IPF. We included 35 adult patients with a consistent radiological diagnosis of IPF, clinical history of lung function decline and high prevalence of symptoms. All patients received the standard of treatment, particularly including antifibrotic drugs and conventional oxygen therapy (COT). 18 subjects were assigned to additional treatment with HFNT for 12 months. Results No significant differences were observed after the follow up with HFNT in terms of lung function. Results are showed in Figure 1. The mean FVC was 1.89 ± 0.73 L with HFNT and 2.43 ± 0.87 without HFNT (p=0.09). The mean FVC % of predicted is shown in Fig.1A; the mean FVC decline per year was 190 with HFNT vs 200 ml with standard of care. The mean DLCO % of predicted was 28.86 ± 14.51 % of predicted with HFNT and 36.03 ± 19.18 with COT (p=0.276), as shown in Fig.1B. No significant impact was observed on dyspnea, the mean borg scale value was 6.72 ± 2.22 after HFNT and 7.14 with COT (p=0.56) (Fig.1C). The score for cough significantly improved after treatment with a mean score in the HFNT group being 46.67 ± 10.85 vs 73.8 ± 18.43 (p<0.0001) with standard of care. Conclusions Long-term HFNT significantly reduces chronic cough in patients affected by IPF compared to COT. Lung function including FVC and DLCO is not significatively influenced.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-10"},"PeriodicalIF":3.5,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754334","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":"A unique combination of heterozygous CFTR gene variants in a person with cystic fibrosis and M. abscessus infection.","authors":"Arthur Lemson, Cedric Bosteels, Jakko van Ingen, Monique Reijers, Dineke Westra, Wouter Hoefsloot","doi":"10.1159/000545552","DOIUrl":"https://doi.org/10.1159/000545552","url":null,"abstract":"<p><strong>Introduction: </strong>Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene. A minority of people with CF carry two heterozygous CFTR mutations other than the common Phe508del, complicating diagnosis and treatment.</p><p><strong>Case presentation: </strong>We report the case of a 25-year-old South American male diagnosed with CF respiratory disease, characterized by a history of recurrent infections, pulmonary Mycobacterium abscessus infection, airway disease on high-resolution CT, and an elevated sweat chloride level (74 mmol/L). Exome sequencing identified a unique combination of CFTR mutations: a pathogenic frameshift variant (c.2052dup) and a variant of unknown clinical significance (c.710A>C). Notably, there were no signs of pancreatic insufficiency. Rectal mucosal organoid cultures demonstrated residual CFTR function with responsiveness to ivacaftor and the combination of elexacaftor, tezacaftor, and ivacaftor.</p><p><strong>Conclusion: </strong>This case highlights a unique combination of heterozygous CFTR variants in a person with late-onset CF respiratory disease, which may be amenable to CFTR modulation therapy.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-10"},"PeriodicalIF":3.5,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754331","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":"Impact of an interdisciplinary approach to the management of refractory dyspnoea on patient quality of life by a non-palliative respiratory care team: a pilot study.","authors":"Gregory Clark, Noemie Vuilleumier, Leo Vanini, Marc Marechal, Jean-Marc Fellrath","doi":"10.1159/000545313","DOIUrl":"https://doi.org/10.1159/000545313","url":null,"abstract":"<p><strong>Introduction: </strong>Refractory dyspnoea is a common symptom of chronic respiratory disease, often poorly recognized by healthcare professionals. Pharmacological treatments are of limited effectiveness. A multidisciplinary intervention carried out by a specialized palliative care team has demonstrated its effectiveness in helping patients improve their quality of life.</p><p><strong>Objective: </strong>The aim of this pilot study was to verify the effects of a multidisciplinary intervention carried out by a team not specialized in palliative care (physician, psychologist, physiotherapist, social worker, dietician).</p><p><strong>Methods: </strong>A longitudinal, single-arm interventional study was conducted between February 2019 and May 2022. All consecutive patients with refractory dyspnoea - referred by a pulmonologist - had a non-pharmacological paramedical intervention (ventilatory education, mindfulness meditation, respiratory rehabilitation, psychological and dietary support, etc.) tailored to their situation after an initial interview. The primary endpoint was the evolution of the Chronic Respiratory Questionnaire (CRQ) before and after the intervention.</p><p><strong>Results: </strong>Fifty-eight patients were referred; 56 patients underwent the intervention. Patients were comparable to previous studies on the basis of LCADL and ESAS scores. The CRQ increased from 4.7 ± 1.5 to 6.2 ± 1.5 pre- and post-intervention overall (P<0.001), with a significant change in all sub-items (fatigue, dyspnoea, emotional function, control).</p><p><strong>Conclusion: </strong>A non-specialized multidisciplinary palliative care team can help patients with refractory dyspnoea improve their quality of life.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-19"},"PeriodicalIF":3.5,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143731478","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":"Diagnosis of peripheral pulmonary lesions using forceps and 1.1 or 1.7 mm cryoprobes. A randomised trial.","authors":"Carolin Steinack, Jan Hendrik Rüschoff, Maurice Roeder, Silvan Manuel Vesenbeckh, Silvia Ulrich, Martina Haberecker, Miriam Nowack, Malcolm Kohler, Daniel P Franzen, Thomas Gaisl","doi":"10.1159/000545338","DOIUrl":"https://doi.org/10.1159/000545338","url":null,"abstract":"<p><strong>Introduction: </strong>Lung cancer screening has increased the detection of peripheral pulmonary lesions (PPLs). Accurate diagnosis for therapy and prognosis is crucial but challenging. Our study compares the safety and efficacy of transbronchial cryobiopsy with two probe diameters and forceps biopsy.</p><p><strong>Methods: </strong>This single-centre, investigator-initiated, open-label, randomised trial included patients with PPLs who required flexible bronchoscopy with radial endobronchial ultrasound-guided biopsy for histopathological diagnosis. Patients received a forceps biopsy and were randomly assigned to a cryobiopsy with a 1.1 mm (freezing time 7-10 sec) or a 1.7mm cryoprobe (freezing time 4-6 sec), respectively. The primary outcome was the diagnostic yield; secondary outcomes included total biopsy size, the proportion of malignant tissue, artefact-free alveolar space percentage, molecular pathology of the specimen and safety.</p><p><strong>Results: </strong>Fifty-four patients (66.52±9.81 years; 48.1% male) with a median nodule size of 24mm [IQR 19 to 30] were included. The overall diagnostic yield was similar in the 1.1 mm and 1.7 mm groups (75.9% vs. 88.0%, p=0.261), and the specimens obtained with the two different cryoprobes were equal in size, quality, and information about molecular pathology. There was no difference in procedural-related bleeding between the groups (p=0.847). Compared to forceps biopsies, cryobiopsies had a superior overall diagnostic yield (75.9% vs. 48.1%, p=0.001) and were better suited for further molecular analysis (p=0.001).</p><p><strong>Conclusion: </strong>The 1.1 mm and 1.7 mm cryoprobes displayed comparable diagnostic yield, ability to provide molecular pathology information, and safety. Forceps biopsy was inferior to cryobiopsy in all aspects except safety.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-20"},"PeriodicalIF":3.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711022","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":"Comparing diagnostic performance across two high-volume EBUS services in Greater Manchester with and without Rapid On-Site Evaluation (ROSE).","authors":"Haider Al-Najjar, Marwah Almadhi, Azita Rajai, Nadira Narine, Durgesh Rana, Haval Balata, Matthew Evison","doi":"10.1159/000545164","DOIUrl":"https://doi.org/10.1159/000545164","url":null,"abstract":"<p><strong>Introduction: </strong>ROSE entails immediate pathological evaluation of diagnostic specimens in the procedure room, facilitating real-time feedback to operator on specimen adequacy and diagnosis. There is ongoing debate about its role in the field of Endobronchial Ultrasound Transbronchial Needle Aspiration (EBUS-TBNA).</p><p><strong>Methods: </strong>Retrospective cohort analysis of prospectively maintained Greater Manchester EBUS database encompassing all linear EBUS procedures between 01/01/2017 and 31/12/2018 at the ROSE-centre versus all linear EBUS procedures in the same period at a non-ROSE centre. Diagnostic performance plus procedural factors such as lymph node (LN) stations sampled and sedation doses were examined then stratified according to EBUS indication.</p><p><strong>Results: </strong>In total 1650 consecutive EBUS procedures were examined across two centres. Using ROSE resulted in statistically significant reduction in number of nodes sampled, time to pathology and sedation doses for most indications. In 697 staging EBUS, sensitivity at the ROSE-centre was 95% (95% CI 91-97%), negative predictive value (NPV) 93% (95% CI 88-96%) with prevalence of mediastinal nodal metastases of 27% (103/376) versus non-ROSE sensitivity 85% (95% CI 79-90%), negative predictive value 86% (95% CI 80-90%) with prevalence of mediastinal nodal metastases of 32% (103/321). In 329 diagnostic EBUS, using ROSE resulted in a statistically significant reduction in number of LN stations sampled per procedure (median 1 station [1-1] with ROSE vs 2 [1-2], p <0.001). Diagnostic performance was higher at the ROSE centre including increases in sensitivity of 7% for diagnostic EBUS in advanced lung cancer, 20% for Isolated Mediastinal Hilar Lymphadenopathy (IMHL) and 17% for diagnosis of nodal metastases from extra-thoracic malignancy.</p><p><strong>Conclusion: </strong>This study suggests ROSE may provide additional value in diagnostic performance in EBUS and warrants further discussion in an evolving lung cancer and bronchoscopic landscape.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-19"},"PeriodicalIF":3.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693003","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":"Diagnosis and Treatment of Bronchial Dieulofy's Disease: A Case Series.","authors":"Huizhen Yang, Yuyan Liu, Jianghua Lou, Xianliang Chen, Quncheng Zhang, Xiaoju Zhang, Felix J F Herth","doi":"10.1159/000545261","DOIUrl":"https://doi.org/10.1159/000545261","url":null,"abstract":"<p><strong>Background: </strong>Bronchial Dieulafoy's disease (BDD) comprises rare vascular malformations. This study analyzes a series of BDD patients diagnosed through combined bronchial computed tomography arteriography (CTA) and bronchoscopy, addressing critical gaps in diagnostic standardization and therapeutic decision-making.</p><p><strong>Methods: </strong>This was a retrospective review of patients who underwent computed tomography arteriography and bronchoscopy for mild to massive and unexplained recurrent hemoptysis in two centers during a 6-year period.</p><p><strong>Results: </strong>Thirty-six patients were diagnosed with BDD by bronchial computed tomography arteriography (CTA) and bronchoscopy. Abnormal vessels were observed by CTA in all 36 patients; and twisted vessels protruding into the bronchial lumen were found in 14 patients. Mucosal eminence lesions in the ipsilateral lobar/segmental bronchus were detected by white light bronchoscopy in all patients. Bronchial artery embolization (BAE) was performed in ten patients and endobronchial intervention was attempted in 18 patients as the initial treatment. Eight patients received application of antibiotics and hemostatic drugs, only. Recurrent hemoptysis occurred in one patient.</p><p><strong>Conclusions: </strong>Vascular disease was the main cause of large to massive hemoptysis. CTA is a noninvasive method that could be used for first-line screening for bronchial vascular malformations. Bronchoscopic procedures could confirm the diagnosis of BDD. BAE is often the first choice, clinically, owing to the invasiveness of thoracic surgery and the patient's status. Bronchoscopic interventional therapy is an effective complement to BAE.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":3.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693010","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 hypertension associated with interstitial lung disease - a review on diagnosis and treatment with a focus on emerging therapies.","authors":"Léon Genecand, Julie Wacker, Ivan Guerreiro, Benoit Lechartier, Maurice Beghetti, Susanne Pohle, Andrei M Darie, Mona Lichtblau, Silvia Ulrich, Sabina A Guler, Frédéric Lador","doi":"10.1159/000544800","DOIUrl":"https://doi.org/10.1159/000544800","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is critical for symptom burden and prognosis. The prevalence of PH varies according to ILDs subgroups and severities. Establishing the diagnosis of PH associated with ILDs (PH-ILDs) is complex due to overlapping symptoms, late clinical signs and poor diagnostic performance of routine non-invasive diagnostic methods. Treatment options for PH-ILDs are limited and target mainly the underlying parenchymal lung disease. For decades, clinical trials of PH-ILDs treatment using pulmonary vasodilators have failed showing no benefits or even harm. Thus, most PH specific therapies are contra-indicated in PH-ILDs. Recently, the landmark INCREASE trial showed that inhaled treprostinil improved exercise capacity (6-minute walking distance) and NT-proBNP, stabilized forced vital capacity and reduced clinical worsening. Inhaled treprostinil was approved by the FDA in 2021, while approval in Europe and Switzerland is pending. After its approval in Europe and Switzerland, the optimal use of inhaled treprostinil will require a careful patient selection with comprehensive assessments (including right heart catheterization) by highly specialized expert centers treating both patients with PH and ILDs.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-23"},"PeriodicalIF":3.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693116","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}