INTRODUCTION:Amyotrophic lateral sclerosis (ALS) is a progressive and heterogeneous neurodegenerative disease that manifests itself in different phenotypes depending on the anatomical region affected. PATIENTS AND METHODS:Retrospective observational study of patients diagnosed with ALS in our healthcare area, classified by phenotypes to assess their relationship with functional respiratory variables, gas exchange, and sleep-related breathing disorders. The search period was from 2014 to 2024. RESULTS:Data from 201 patients were analyzed. The overall mean incidence of ALS was 3.8 cases (95%CI: 3.3-4.3)/100,000 inhabitants/year, while the prevalence was 9.4 cases (95%CI: 7.8-11.1)/100,000 inhabitants. The results indicated that the spinal phenotype is the most common (49.3%), while the bulbar phenotype presented greater respiratory involvement, with a lower forced vital capacity (FVC) (86% [IQR: 66.5-98]) and greater nocturnal desaturation CT90 8% (IQR: 2.3-32.5%). Likewise, a prevalence of respiratory disorders during sleep was observed, with approximately 50% mild obstructive sleep apnea (OSA), 30% moderate, and 15% severe. Severe OSA was recorded in 8% of patients with spinal ALS, 14% of patients with bulbar ALS, and 17% with other forms of ALS. CONCLUSIONS:The disease significantly affects respiratory function, especially in the bulbar phenotype, and respiratory disturbances during sleep are common. The heterogeneity of ALS highlights the importance of a personalized approach to patient management.
BACKGROUND:Intrapleural enzyme therapy (IET) is widely used for pleural infections, including complicated parapneumonic effusions and empyema; however, the role of saline lavage alone or in combination with IET remains uncertain. METHODS:The SCOPE trial was a two-centre, prospective, randomised superiority study designed to determine whether saline lavage alone or combined with IET was superior to IET alone in adults with pleural infection. Patients were randomised in a 1:1:1 ratio to saline lavage alone, saline lavage plus IET (urokinase and DNase) or IET alone. Patients and outcome assessors were blinded to the treatment allocation. The primary end-point was the duration of pleural drainage. Secondary end-points included radiographic resolution, need for additional interventions, length of hospital stay, mortality and adverse events. RESULTS:89 patients were analysed in three groups (saline lavage, n=30; saline+IET, n=30; IET, n=29). Baseline characteristics were broadly similar across the groups. The median (interquartile range) drainage duration was longer with saline lavage alone (4.0 (3.0-6.75) days) than with IET alone (3.0 (2.0-4.0) days; p=0.01) or saline lavage plus IET (3.0 (3.0-3.75) days; p=0.01). No significant difference was observed between the two IET-containing regimens (p=0.24). Secondary outcomes showed no clear advantage for saline lavage alone or in combination with IET. CONCLUSIONS:In pleural infection, saline lavage alone results in a longer drainage duration than IET. In this small superiority trial, adding saline lavage to IET did not demonstrate a clinically meaningful advantage over IET alone.
Previous studies have linked radon exposure with mortality in patients with chronic obstructive pulmonary disease (COPD), but there is little data assessing the role of indoor radon exposure with the risk of COPD or its severity in never smokers. Objective: To explore whether indoor radon exposure is associated with the risk or severity of COPD in never smokers. Multicentric, hospital-based, case–control study, located in Northwest Spain. The study included 300 never-smokers with COPD (cases) and 462 never smoking controls. COPD cases and their severity were included following Global Initiative for Obstructive Lung Disease 2019 (GOLD) criteria for COPD diagnosis. Radon concentrations were measured in each participant’s home using long-term (at least 3 months) alpha-track devices. Results were analyzed for cases and controls, and also for different lengths of residence in the last dwelling employing logistic regression. For cases, hospital admissions in the last 3 years due to COPD exacerbations and COPD severity were related to radon concentrations. (1) Indoor radon exposure was similar in cases and controls, but radon exposure showed a statistically significant association with COPD when length of residence in the same dwelling was > 40 years. There was an odds ratio (OR) of 2.79 (1.06–7.40) for those with highest (> 300 Bq/m3) vs. lowest radon exposure (< 100 Bq/m3); (2) in cases, the severity of airflow obstruction increased with higher indoor radon exposure, and the proportion of COPD cases with radon exposure above 300 Bq/m3 was higher in GOLD 4 (50
Introducción La esclerosis lateral amiotrófica (ELA) es una enfermedad neurodegenerativa progresiva y heterogénea que se manifiesta en diferentes fenotipos según la región anatómica afectada. Pacientes y métodos Estudio observacional retrospectivo de los pacientes diagnosticados en nuestra área sanitaria de ELA, clasificados por fenotipos para evaluar su relación con variables respiratorias funcionales, el intercambio gaseoso y los trastornos respiratorios durante el sueño. El periodo de búsqueda fue de 2014 a 2024. Resultados Se analizaron los datos de 201 pacientes. La incidencia media global de la ELA fue de 3,8casos (IC95%: 3,3-4,3)/100.000 habitantes/año, mientras que la prevalencia fue de 9,4casos (IC95%: 7,8-11,1)/100.000 habitantes. Los resultados indicaron que el fenotipo espinal es el más frecuente (49,3%), mientras que el fenotipo bulbar presentó mayor afectación respiratoria, con una capacidad vital forzada (CVF) más baja (86% [RIQ: 66,5-98]) y una mayor desaturación nocturna CT90 8% (RIQ: 2,3-32,5%). Asimismo, se observó una prevalencia de alteraciones respiratorias durante el sueño, con aproximadamente un 50% de apnea obstructiva del sueño (AOS) leve, un 30% moderada y un 15% grave. La AOS grave se registró en el 8% de los pacientes con ELA espinal, el 14%, de los pacientes con ELA bulbar y el 17% con otras formas de ELA. Conclusiones Este estudio destaca la heterogeneidad de la ELA según sus fenotipos y se subraya la importancia de implementar un enfoque personalizado en el manejo clínico de los pacientes. La enfermedad afecta significativamente a la función respiratoria, sobre todo en el fenotipo bulbar, y las alteraciones respiratorias durante el sueño son comunes. La heterogeneidad de la ELA resalta la importancia de un enfoque personalizado en el manejo de los pacientes.
Background and Objective:The use of humidification devices has been recommended during non-invasive mechanical ventilation (NIMV). However, existing recommendations on this topic remain limited and inconsistent. The objective of this narrative review is to summarize the current state of knowledge regarding the need for and timing of humidification during NIMV. Methods:A bibliographic review of the literature published over the last 30 years (time frame chosen arbitrarily) was conducted using the PubMed database to identify relevant studies addressing the role of humidification in NIMV, with a particular focus on clinical guidelines and expert consensus statements. Key Content and Findings:NIMV may induce undesirable physiological and histological effects, leading to patient discomfort and reduced tolerance, which can ultimately contribute to NIMV failure. For these reasons, most guidelines and experts recommend the use of humidification during NIMV. However, there is no consensus regarding the optimal timing for initiating humidification in the acute setting. The decision to apply humidification may depend on several factors, including the expected duration of NIMV, the underlying clinical diagnosis, and patient-reported symptoms. Overall, based primarily on physiological evidence, the literature suggests that heated humidifiers (HHs) may be superior to heat and moisture exchangers (HMEs). Nevertheless, this potential advantage of HH over HME is not consistently supported when clinical outcomes are considered. Conclusions:Current guidelines and expert recommendations generally advocate the use of additional humidification during NIMV, given the low humidity of inspired air in its absence and the possible adverse effects on upper airway function, patient comfort, and tolerance. However, further studies are needed to clarify the necessity of humidification during the acute phase of NIMV. While physiological data favor HH over HME, this superiority is less evident when clinical endpoints are evaluated. More real-world studies are warranted to determine the actual comparative benefits of each humidification method.
Background Chronic obstructive pulmonary disease (COPD) is frequently associated with cardiovascular diseases (CVD), which may adversely affect the prognosis of these patients. Objective To confirm that individuals with COPD and CVD utilize more healthcare resources, require more frequent hospitalizations, and have higher mortality rates compared to those without these comorbidities. Methods Population-based study including 7,391 COPD patients, classified into four groups according to the presence of heart failure (HF) and ischemic heart disease (IHD). Adjusted logistic regression and proportional hazards models were performed. Results Of the patients, 86.3% (6,378) had COPD alone; 8.1% (601) had COPD + HF; 3.9% (290) had COPD + IHD; and 1.7% (122) had all three conditions. Groups with CVD exhibited older age, higher cardiovascular risk, and increased mortality. Hospitalization was associated with older age, male sex, fewer spirometry tests performed in primary care, and the use of home oxygen therapy and non-invasive ventilation, and was linked to higher mortality compared to non-hospitalized patients.Mortality was higher among patients with COPD and CVD. It was associated with more emergency department visits, more hospital admissions, increased use of chest X-rays and CT scans, and a higher prevalence of depression. Conversely, it was also related to lower rates of vaccination, fewer spirometry tests in primary care, reduced dispensing of inhaled medications, and overall lower pharmaceutical expenditure. Conclusion The coexistence of COPD and CVD identifies subgroups of patients with poorer prognosis and increased healthcare resource utilization. Multidisciplinary management and prevention strategies are essential to improve outcomes and healthcare efficiency.
Background:Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that rarely affects the pleura, with pleural effusion (PE) reported in only 4% of cases. The characteristics of PE in patients with IgG4-RD are unknown. The objectives of this systematic review were to document the histological and biochemical characteristics of PF and pleural tissue, assess its clinical course, and determine the most effective treatments for the management of PE. Methods:A PRISMA literature search was conducted for published articles describing the characteristics of PE in IgG4-RD and discussing the approach to PE in this setting. Results:A total of 46 articles [55 patients and 57 pleural fluid (PF) samples] were included. Median age was 66 years (range, 28-86 years), with a male/female ratio of 2.9:1. PE was predominantly right-sided or bilateral (81.3%) and usually occupied <2/3 of the hemithorax (78.9%). PF generally had a serous appearance (84.2%) and was an exudate in 94.6% of cases with predominance (≥50%) of mononucleated cells in 96% (24/25). A predominance of eosinophils (≥10%) was observed in 40% (4/10 cases). As many as 66.7% of patients presented values of adenosine deaminase (ADA) ≥35 U/L (18/27), pH values remained within normal range 7.35-7.45 (15.4%) in only two patients. Three patients had glucose values <60 mg/dL (12.5%) and the PF of two patients was a chylothorax. Pleural biopsy was consistent with diagnosis in 95.6% of cases (43/45). In total, 92.7% of patients (51/55) received treatment with corticosteroids, and 9 (17.6%) received immunosuppressants. Of the four cases left untreated, 2 underwent chest drainage. Clinical course was benign in 97.7% of patients (43/44; a patient died of unknown causes). Conclusions:Patients with IgG4-RD and PE are usually men over 50 years of age with small/moderate, right-sided or bilateral PE. PF is most commonly a lymphocytic exudate with elevated ADA values. Pleural biopsy can help establish diagnosis. Treatment with corticosteroids, combined or not with immunosuppressants, was usually effective.
Cryptococcus neoformans infection occurs more frequently in immunocompromised individuals, with pulmonary and cerebral forms being the most common. Among pulmonary manifestations, pleural effusion in cryptococcosis is an exceptional presentation. We present a series of four cases of cryptococcosis with empyema caused by Cryptococcus neoformans diagnosed at a single centre and we conducted a systematic review of the literature on cases of cryptococcal pleural effusion. 32 were selected for data extraction and analysis. The median patient age was 59 years, with 72
Pulmonary ossification (PO) is a rare disease, especially in patients without a previous history of lung disease. PO has an indolent course that may progressively cause lung function impairment. It is characterized by the presence of ectopic metaplastic bone in the lungs. Although it is generally idiopathic, it may be associated with other diseases, such as interstitial lung diseases. High resolution computed tomography (HRCT) of the chest is the technology of choice for diagnosis, as it allows differential diagnosis with other diseases and spares the use of more aggressive diagnostic techniques. Distinctive radiological findings include two ossification patterns: dendriform and nodular (the most frequent). Each pattern is associated with specific histological findings and can be related to different entities or otherwise be idiopathic forms. This review provides an updated overview of the pathogenesis, diagnostic and histopathological criteria and prognostic factors. As specific treatment is not currently available, the objective is to prevent fibrotic interstitial progression. For a correct management of the disease, it is necessary to consider the associated comorbidities and closely monitor cases. The purpose of this approach is to prevent progression, in case it is associated with diffuse interstitial lung disease. Further research and long-term follow-up are necessary for a better understanding of the pathogenesis and prognosis of PO.
ABSTRACT We present the case of a 42‐year‐old woman on oral contraceptives that presented to the emergency department with pain and swelling in the left lower limb. Diagnosis of extensive deep vein thrombosis was established. A few minutes later, she exhibited signs of shock and hemodynamic instability, thus raising suspicion of high‐risk acute pulmonary thromboembolism. Prior to the administration of fibrinolytic treatment, a bedside transthoracic echocardiography was performed that excluded right ventricular dilatation. Then, the study was complemented with a thoraco‐abdominal computed tomography scan that demonstrated a large retroperitoneal hematoma as the cause of the shock. In conclusion, a transthoracic echocardiography should be performed before initiating thrombolytic therapy in hemodynamically instable patients with strong suspicion of high‐risk pulmonary embolism.
The term "asbestos" is used to refer to a group of silicate minerals that often break down into fibres and whose inhalation over time can cause a number of diseases, especially pleuropulmonary diseases. While the most serious complications are malignant diseases, inhalation of these fibres can also cause benign pleural diseases such as round atelectasis, pleural plaques, diffuse pleural thickening and non-malignant asbestos pleural effusion. Although asbestos is banned in most developed countries (in the European Union, since 2002), it is still used in developing countries. Despite these restrictions, the prevalence of diseases due to inhalation remains high due to the long latency period between the onset of exposure and the onset of disease. In this paper we review benign pleural diseases induced by asbestos exposure, update the diagnostic criteria for these disorders, and describe the approaches suggested so far to differentiate them from malignant pleural diseases.
Background:Although transbronchial lung cryobiopsy (TBLC) is gaining popularity in the diagnosis of interstitial lung disease (ILD), its diagnostic performance and safety are still a matter of debate. The objective of this study was to assess the diagnostic performance and safety of TBLC by a multidisciplinary committee (MDC). Methods:A diagnostic cross-sectional study was performed of all patients who underwent a TBLC between 2017 and 2024 in a center without previous experience in this technique. Results:A total of 117 patients undergoing a TBLC were included. A specific pathological diagnosis was achieved in 73/117 cases (62.4%) and a multidisciplinary diagnosis was obtained in 79/117 cases (67.5%). A positive result did not correlate with any variable. In total, 65 patients (55.6%) experienced a complication. Twenty-one patients had a pneumothorax (17.8%), and 11 (52.4%) needed a chest drain. The occurrence of complications was associated with age (with an inverse relationship) [odds ratio (OR), 0.920; 95% confidence interval (CI): 0.868-0.976; P=0.006] and the number of samples obtained (OR, 2.094; 95% CI: 1.111-3.947; P=0.02). Bleeding was observed in 44 cases (37.6%). Moderate (15; 12.8%) and severe (3; 2.6%) bleeding was associated with age (OR, 1.108; 95% CI: 1.021-1.202; P=0.01) and a lower number of biopsies performed (OR, 0.349; 95% CI: 0.169-0.719; P=0.004). Only a patient required mechanical ventilation. No 30-day mortality was observed. Conclusions:TBLC provides a specific diagnosis and spares a SLB in at least 2/3 of cases requiring a lung biopsy, with an acceptable incidence of pneumothorax and moderate/severe bleeding. TBLC should be further standardized to prevent inconsistencies in diagnostic performance estimates.
OBJECTIVE:The therapeutic approach for metastatic malignant pleural effusion depends on the patient's life expectancy. Can survival be accurately estimated in these patients using a risk-prediction model? METHODS:A prospective, single-center study was conducted to examine the prognostic value of pre-established variables (multivariate Cox model). Subsequently, a prognostic score was developed and validated. The inclusion period was 11 years long. Follow-up was conducted until death or for a minimum of 12 months. RESULTS:The derivation and validation cohorts included 475 and 205 patients, respectively. The prognostic score GASENT (Galicia, Age, Sex, ECOG-PS, Neutrophil/lymphocyte ratio, and Tumor type) was derived from the multivariate analysis of survival. Categorization of patients in the derivation cohort into low-, moderate-, or high-risk yielded median survival times of 477 days (377-665; n=159), 108 days (83-156; n=158), and 35 days (27-47; n=158), respectively. Survival rates at 1, 3, and 6 months were 92%, 83%, and 72%, respectively, for the low-risk group; 80%, 55%, and 36%, respectively, for the moderate-risk group; and 55%, 23%, and 13%, respectively, for the high-risk group. The analysis of areas under the curve revealed that the GASENT model was superior to the LENT score as a survival predictive model at 1 (0.777 vs. 0.737; p=0.009), 3 (0.810 vs. 0.778; p=0.009), and 6 months (0.812 vs. 0.780; p=0.007). CONCLUSIONS:The GASENT predictive model estimates survival in patients with metastatic malignant effusions with significantly greater accuracy than the scores categorizing patients by risk groups.
Nocardia spp. are a pathogen that can cause both localized infection in different organs and disseminated infection. Although it is not always associated with immunosuppression situations, the increase in these conditions, along with the development of novel diagnostic methods, makes this diagnosis increasingly common. In this manuscript, we describe three cases of pleural infection caused by different species of Nocardia. The lesson learned is that when faced with pleural infection, Nocardia infection should be ruled out, and if confirmed, performing an imaging study of the central nervous system is mandatory to exclude a brain abscess. Close monitoring is required to control the clinical course and prevent drug-induced toxicity.
Pleural effusion induces an increase in pleural pressure, resulting in a cascade of changes in the physiological parameters of the thoracic cavity. These changes include increased volume of the rib cage, abnormal gas exchange, impaired respiratory mechanics, and abnormal diaphragmatic movements. Such alterations have a significant clinical impact, including the development of specific symptoms such as dyspnoea. Dyspnoea secondary to pleural effusion has a complex physiopathology. Notably, the severity of dyspnoea often shows a poor correlation with effusion size and may not improve after fluid drainage. The symptomatic relief experienced by patients after fluid drainage is largely attributed to improvements in the length-tension relationship of the respiratory muscles. Importantly, dyspnoea in these cases results from the abnormal shape and restricted motion of the ipsilateral hemidiaphragm, coupled with an increased compensatory respiratory drive aimed at preserving adequate ventilation. Fluid drainage reduces diaphragmatic distension, restoring diaphragmatic mobility, reducing inspiratory neural drive, and enhancing the neuromechanical coupling of the diaphragm, all of which contribute to dyspnoea relief. This review explores current evidence regarding the pathophysiological mechanisms underlying dyspnoea in pleural effusion and the therapeutic effects of thoracentesis.
Under normal conditions, there is a small volume of fluid in the pleural space as a result of the balance between its inflow from the pleural capillaries and its drainage through the lymphatics. Pleural effusion (PE) will occur when this balance is disturbed. The initial step in its study is to determine whether PE it is a transudate or an exudate. The first is caused by an alteration in the hydrostatic or oncotic pressure of both the pleural capillaries and the pleural space, without any structural damage to the pleura and with a simple differential diagnosis. In the second, there will be an alteration in fluid flow (increased inflow due to increased permeability of the pleural capillaries or decreased reabsorption due to blocked lymphatic drainage) with damage to the pleural surfaces. Diagnosis is more difficult and more complex biochemical determinations are often used to determine the etiology. A careful clinical history and physical examination together with a good knowledge of the movement of pleural fluid and the information provided by its analysis, obtained by thoracentesis, a simple and safe technique, would allow the family physicians to establish the presumptive diagnosis of the etiology of pleural effusion in about 95% of cases. In this review we provide guidelines as to which specific markers may be useful in the diagnosis of pleural effusion in the Primary Care setting.
Certain obstetric and gynaecological diseases are associated with pleural effusion, including benign peripartum pleural effusion, endometriosis, ovarian hyperstimulation syndrome and Meigs syndrome. This review provides a comprehensive and detailed overview of this group of rare diseases. A thorough understanding of their unique characteristics is required to ensure early identification, correct diagnosis and appropriate management.