Introduction: 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. Methods: 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 analyses were performed to highlight an association between preprocedural features and clinical success. Results: A total of 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). Overall, 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. Conclusion: 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.

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.

Plain Language SummaryMachine learning is a powerful tool that helps predict when asthma symptoms suddenly become much worse. However, because asthma can affect people differently, we need a special kind of machine learning model that works best for asthma. We aimed to create a computer model using machine learning that can find out what things make asthma attacks severe in a certain type of asthma, which could help doctors catch and treat these attacks early, possibly saving money on medical care. We analyzed medical records of patients with type 2 inflammatory asthma exacerbations from May 2017 to September 2022, assessing various clinical variables and using four machine learning models. The computer model called LightGBM worked the best. It could tell which patients were more likely to have a bad asthma attack. It was good at telling them with very high accuracy. The LightGBM model is a strong tool for predicting when asthma attacks become severe in a certain type of asthma, giving doctors a way to prepare and treat patients early, which could help save money on medical bills.

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. Also, acute exacerbation of 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 was 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). Eighteen 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. The mean forced vital capacity (FVC) was 1.89 ± 0.73 L with HFNT and 2.43 ± 0.87 L without HFNT (p = 0.09). The mean FVC decline per year was 190 mL with HFNT versus 200 mL with standard of care. The mean DLCO % of predicted was 28.86 ± 14.51% with HFNT and 36.03 ± 19.18% with COT (p = 0.276). 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). The score for cough significantly improved after treatment with a mean score in the HFNT group being 46.67 ± 10.85 versus 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.

Introduction: 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. Case Presentation: 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. Conclusion: This case highlights a unique combination of heterozygous CFTR variants in a person with CF respiratory disease, which may be amendable to CFTR modulation therapy.

Introduction: Rapid On-Site Evaluation (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 (EBUS)-guided transbronchial needle aspiration. Methods: To re-evaluate the impact of ROSE, we completed a retrospective cohort analysis of prospectively maintained Greater Manchester EBUS database encompassing all linear EBUS procedures between 1 January 2017 and 31 December 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 and then stratified according to EBUS indication. Results: In total, 1,650 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%), NPV 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, and 17% for diagnosis of nodal metastases from extra-thoracic malignancy. Conclusion: 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.

Introduction: 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. Methods: This was a retrospective review of patients who underwent CTA and bronchoscopy for mild to massive and unexplained recurrent hemoptysis in two centers during a 6-year period. Results: Thirty-six patients were diagnosed with BDD by bronchial CTA and bronchoscopy. Abnormal vessels were observed by CTA in all 36 patients; 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 10 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 1 patient. Conclusion: 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.

Background: Pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is critical for symptom burden and prognosis. The prevalence of PH varies according to ILD 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 noninvasive diagnostic methods. Summary: Treatment options for PH-ILDs are limited and target mainly the underlying parenchymal lung disease. For decades, clinical trials of PH-ILD 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-min 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. Key Message: 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 patients with both PH and ILDs.