Background: Interstitial lung disease (ILD) leads to progressive lung function decline and significant respiratory symptoms. Although antifibrotic agents preserve lung function and reduce mortality in ILD, their impact on health-related quality of life (HRQoL) remains unclear.
Objectives: We aimed to evaluate whether antifibrotic agents improve HRQoL and their effectiveness in treating HRQoL-related symptoms in patients with ILD.
Design: Systematic review and meta-analysis.
Data sources and methods: A literature search was conducted using MEDLINE, EMBASE, and the Cochrane Library from inception to August 25, 2025. The search included terms related to ILD, antifibrotic agents, and measures of HRQoL. HRQoL outcomes were assessed using the St. George's Respiratory Questionnaire (SGRQ), including total and domain scores. Data were pooled using a random-effects model, with outcomes reported as mean differences (MD) or relative risks (RR) and heterogeneity evaluated using the I² statistic.
Results: A total of 13 randomized controlled trials were included. Antifibrotic agents showed significant improvement in SGRQ scores, particularly in the symptom (MD: -2.59, 95% confidence interval [CI]: -4.56 to -0.61; I² = 32%) and activity (MD: -2.88, 95% CI: -4.82 to -0.94; I² = 34%) domains. Antifibrotics reduced the rate of cough (RR: 0.77, 95% CI: 0.64-0.94; I² = 0%) and dyspnea (RR: 0.71, 95% CI: 0.56 to 0.89; I² = 0%). However, fatigue was frequently observed in patients treated with antifibrotics (RR: 1.48, 95% CI: 1.20-1.83; I² = 0%) compared with the non-antifibrotic group. Most trials were judged to have low-to-moderate risk of bias, and the certainty of evidence was rated very low for total SGRQ scores but low to moderate for domain-specific outcomes and symptoms.
Conclusion: Antifibrotic agents may improve HRQoL and reduce dyspnea and cough in patients with ILD, but the certainty of evidence is low, and they may increase fatigue, requiring careful monitoring.Trial registration:The study protocol was registered in PROSPERO (CRD42023450917).
Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT), as a rare and highly malignant neoplasm associated with a high mortality risk, is easily confused with SMARCA4-deficient nonsmall-cell lung cancer (NSCLC). To date, no standard and effective protocol for thoracic SMARCA4-UT has been established. Immunotherapy has demonstrated efficacy in advanced NSCLC, achieving unprecedented survival benefits. However, immune-related adverse events (irAEs) remain a significant clinical challenge. Here, we reported the first case of thoracic SMARCA4-UT with immune-related cystitis and hypothyroidism, in which the patient benefited from first-line immune checkpoint inhibitor (ICI)-based combination therapy, achieving a remarkable overall survival of over 100 weeks. Furthermore, we performed a review and analysis of the diagnosis, differential diagnosis, immunotherapy, and prognosis of thoracic SMARCA4-UT, proposing that first-line therapy combining immunotherapy with platinum-based chemotherapy (induction and maintenance phases) with or without radiotherapy, may improve the prognosis of such patients. Additionally, we hypothesized a potential role of macrophages in the pathogenesis of immune-related cystitis for the first time and detailed the clinicopathological characteristics and evidence-based management of this irAE.
Background: Females with cystic fibrosis (fwCF) are at increased risk of urinary incontinence (UI), likely due to chronic coughing and elevated intra-abdominal pressure. Prevalence rates reported in the literature vary widely, and no large multicenter study has been carried out to date.
Objective: To estimate the prevalence and severity of UI in fwCF and to investigate clinical variables associated with UI.
Design: A multicenter, cross-sectional study conducted across 21 Italian CF centers.
Methods: UI prevalence and severity were assessed using two validated questionnaires. A multivariable fractional polynomial approach was used to select variables for inclusion in the final logistic regression model to identify relevant associations with UI.
Results: UI was present in 218/542 females (40.2%, 95% Confidence Interval (CI): 36.1-44.5). Among children and adolescents, the prevalence was 12/160 (7.5%, 95% CI: 4.1-13), whereas among adults it was 206/382 (53.9%, 95% CI: 48.8-59). FwCF with UI showed a BMI of 0.2 Z score higher (95% CI: 0.1-0.4) than fwCF without UI; however, the overall prevalence of UI in fwCF overweight was 41% (95% CI: 30.2-52.7) compared to 40.1% (95%CI: 35.6-44.7) in fwCF with normal weight. Age (interquartile range-odds ratio (IQR-OR) 4.19, 95% CI: 2.80-6.28), days of hospitalization (IQR-OR 1.72, 95% CI: 1.42-2.08), and physical activity (OR 0.66, 95% CI: 0.53-0.82) were the only factors statistically associated with UI.
Conclusion: UI affects mostly adult fwCF and is associated with older age and longer hospitalization. Physical activity of ⩾150 min per week was also associated with a reduced probability of UI.
Background: There is uncertainty regarding the optimal next imaging modality for identifying likely malignant pulmonary lesions in patients with abnormal chest radiography, with or without respiratory symptoms.
Objectives: We compared the diagnostic accuracy of chest contrast-enhanced computed tomography (CECT) and positron emission tomography or positron emission tomography-computed tomography (PET/PET-CT) for identifying malignant pulmonary lesions.
Design: Systematic review and meta-analysisData sources and methods:We searched the PubMed, Embase, Scopus, and Cochrane CENTRAL databases to identify head-to-head diagnostic accuracy studies comparing CECT and PET/PET-CT for their ability to differentiate between benign and malignant pulmonary lesions. The risk of bias of included studies was assessed using the Quality Assessment of Diagnostic Accuracy Studies - Comparative (QUADAS-C) tool. Meta-analysis was performed using the bivariate random effects model.
Results: We included eight studies, comprising a total of 873 subjects. The pooled sensitivity and specificity of CECT (850 lesions) were 0.93 (95% CI, 0.89-0.96; I2 = 24.99%) and 0.54 (95% CI, 0.32-0.75; I2 = 84.00%). The pooled sensitivity and specificity of PET/PET-CT (851 lesions) were 0.87 (95% CI, 0.78-0.93; I2 = 65.15%) and 0.83 (95% CI, 0.63-0.94; I2 = 73.23%). Compared to CECT, PET/PET-CT had a lower relative sensitivity (relative ratio [RR], 0.93; 95% CI, 0.89-0.97; p < 0.01) and a higher relative specificity (RR, 1.69; 95% CI, 1.18-2.41; p < 0.01). After excluding the study with the largest sample size, PET/PET-CT was not less sensitive than CECT (RR, 0.99; 95% CI, 0.94-1.04; p = 0.73). There was a high/unclear risk of bias and applicability concerns in the population domain in six out of eight studies.
Conclusions: Based on limited evidence with applicability concerns, CECT of the chest may have a higher sensitivity but lower specificity than PET/PET-CT for identifying malignant lesions among patients with suspected lung cancer.
Trial registration: The protocol for the systematic review was prospectively registered on PROSPERO (CRD42024590904).
This review focuses on the advancements in the treatment of pulmonary hypertension (PH), especially after the Food and Drug Administration (FDA) approval of sotatercept and the advances in treatment recommendations after seven World Symposia on PH. PH, a complex and progressive condition defined hemodynamically by a mean pulmonary artery pressure >20 mmHg, encompasses multiple PH groups, each with distinct pathophysiological characteristics and treatment implications. Diagnosing PH can be challenging because symptoms like shortness of breath, fatigue, and chest pain are nonspecific. Contemporary treatment of pulmonary arterial hypertension aims to improve outcomes, symptoms, and overall quality of life, with a primary focus on preventing and treating right ventricular failure. Comprehensive risk stratification remains crucial, aiding in personalized therapy adjustments that improve patients' outcomes. This review also touches upon the limited treatment options for other PH groups, like PH associated with left heart disease, parenchymal lung diseases, and chronic thromboembolic PH, underscoring the need for expanded therapeutic options. Despite advances, challenges remain: diagnostic delays, misdiagnosis, absence of head-to-head clinical trials, and the timing of introducing newer treatments such as sotatercept are discussed, emphasizing an integrated approach that transcends vasodilation to target underlying disease mechanisms. Future directions envision a comprehensive risk stratification incorporating right ventricular function and a mechanism-based treatment paradigm, encouraging a tailored therapeutic approach in PH management.
Over the past decade, major clinical advances have been made in the healthcare and therapeutic development for cystic fibrosis (CF), a lethal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein. CFTR modulators represent innovative treatments that directly target the primary defects in the mutated CFTR protein and have demonstrated significant clinical benefits for many people with CF (pwCF) who are eligible for these treatments. In particular, the triple combination therapy composed of elexacaftor, tezacaftor, and ivacaftor (ETI) has changed the CF therapeutic landscape by significantly improving lung function, quality of life, and predicted survival rates. Here, we provided a comprehensive summary of the impact of ETI on clinical outcomes and the need for further research on long-term efficacy, side effects, pregnancy, possible drug-drug interactions, and extra-pulmonary manifestations. Moreover, a significant number of pwCF are unresponsive to these drugs or cannot afford their high costs. We, therefore, discussed health inequity issues and alternative therapeutic strategies under development aiming to obtain effective therapies for all pwCF.
Background: During the COVID-19 pandemic, telemedicine became crucial for monitoring chronic conditions, including respiratory diseases.
Objectives: This study, part of a larger cohort of COVID-positive patients, focuses on individuals with chronic obstructive pulmonary disease (COPD) monitored through an active home surveillance system (COD19).
Design: Longitudinal telematic active surveillance study.
Methods: The study included COVID-19-positive patients in home isolation, quarantined workers, and those discharged from ASST hospitals or emergency departments. At discharge, patients received a letter with isolation guidelines, a COD19 kit (oxygen meter, thermometer, protective devices), and instructions for monitoring clinical parameters. Regular phone check-ins by physicians were conducted, starting within 12 h of activation. A secure platform COD19 enabled data collection and communication between patients, healthcare providers, and regional authorities.
Results: The study involved 1288 patients, including 226 (17.5%) with COPD, who were older (p < 0.001), had a higher BMI (p = 0.006), and were more frequently admitted from home isolation (p < 0.001). COPD patients also had higher mean body temperature (p = 0.011) and respiratory rate (p = 0.035), with a non-significant trend toward lower SpO2 values. Monitoring outcomes indicated that COPD patients were more likely to require higher levels of care (p < 0.001), and the only two deceased patients were from this group. The remote monitoring service received positive feedback, with a median answering ratio of 92%, reflecting strong patient participation and manageable monitoring processes.
Conclusion: The findings underscore telemedicine's effectiveness in COPD management, ensuring continuity of care and smooth home-to-hospital transitions. The system enhanced accessibility, enabling consistent monitoring and timely interventions. As healthcare evolves, telemedicine remains a key tool in improving patient care and accessibility.
Background: Asthma is one of childhood's most prevalent chronic conditions significantly impacting the quality of life. Current asthma management lacks real-time, objective, and longitudinal monitoring reflected by a high prevalence of uncontrolled asthma. Long-term home monitoring promises to establish new clinical endpoints for timely anticipation. In addition, integrating eHealth interventions holds promise for timely and appropriate medical anticipation for controlling symptoms and preventing asthma exacerbations.
Objectives: This study aims to provide a pragmatic study design for gaining insight into longitudinal monitoring, assessing, and comparing eHealth interventions' short- and long-term effects on improving pediatric asthma care.
Design: The CIRCUS study design is a cohort multiple randomized controlled trial (cmRCT) with a dynamic cohort of 300 pediatric asthma patients.
Methods: The study gathers observational and patient-reported measurements at set moments including patient characteristics, healthcare utilization, and asthma, clinical, and environmental outcomes. Participants are randomly appointed to the intervention or control group. The effects of the eHealth interventions are assessed and compared to the control group, deploying the CIRCUS outcomes. The participants continue in the CIRCUS cohort after completing the intervention and its follow-up.
Results: This study was ethically approved by the Medical Research Ethics Committee (NL85668.100.23) on February 15th, 2024.
Discussion: The CIRCUS study can provide a rich and unique dataset that can improve insight into risk factors of asthma exacerbations and yield new clinical endpoints. Furthermore, the effects of eHealth interventions can be assessed and compared with each other both short- and long-term. In addition, patient groups within the patient population can be discerned to tailor eHealth interventions to personalized needs on improving asthma management.
Conclusion: In conclusion, CIRCUS can provide valuable clinical data to discern risk factors for asthma exacerbations, identify and compare effective scalable eHealth solutions, and improve pediatric asthma care.Trial registration: The protocol is registered at ClinicalTrials.gov (NCT06278662).
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