Predictors and changes of physical activity in idiopathic pulmonary fibrosis.

BACKGROUND: Different clinical predictors of physical activity (PA) have been described in idiopathic pulmonary fibrosis (IPF), but studies are lacking evaluating the potential role of muscle strength and anxiety and depression symptoms in PA limitation. Moreover, little is known about the impact of changes in PA in the course of the disease. The aim of the present study was to investigate the relationship between baseline PA and a wide range of variables in IPF, to assess its longitudinal changes at 12 months and its impact on progression free-survival. METHODS: PA was assessed by accelerometer and physiological, clinical, psychological factors and health-related quality of life were evaluated in subjects with IPF at baseline and at 12 month follow-up. Predictors of PA were determined at baseline, evolution of PA parameters was described and the prognostic role of PA evolution was also established. RESULTS: Forty participants with IPF were included and 22 completed the follow-up. At baseline, subjects performed 5765 (3442) daily steps and spent 64 (44) minutes/day in moderate to vigorous PA. Multivariate regression models showed that at baseline, a lower six-minute walked distance, lower quadriceps strength (QMVC), and a higher depression score in the Hospital Anxiety and Depression scale were associated to lower daily step number. In addition, being in (Gender-Age-Physiology) GAP III stage, having a BMI ≥ 25 kg/m2 and lower QMVC or maximum inspiratory pressure were factors associated with sedentary behaviour. Adjusted for age, gender and forced vital capacity (FVC) (%pred.) a lower progression-free survival was evidenced in those subjects that decreased PA compared to those that maintained, or even increased it, at 12 months [HR 12.1 (95% CI, 1.9-78.8); p = 0.009]. CONCLUSION: Among a wide range of variables, muscle strength and depression symptoms have a predominant role in PA in IPF patients. Daily PA behaviour and its evolution should be considered in IPF clinical assessment and as a potential complementary indicator of disease prognosis.

Prognostic factors of progressive fibrotic hypersensitivity pneumonitis: a large, retrospective, multicentre, observational cohort study.

Background: Fibrotic hypersensitivity pneumonitis (fHP) is an immune-mediated interstitial lung disease caused by sensitisation to chronic allergen inhalation. This study aimed to determine prognostic indicators of progression and mortality in fHP. Methods: This was a retrospective, multicentre, observational, cross-sectional cohort study of consecutive patients diagnosed with fHP from 1 January 2012 to 31 December 2021. Multivariate Cox regression analyses were used to calculate hazard ratios (HRs) with 95% confidence intervals for predictors of progression and survival. Results: A total of 403 patients were diagnosed with fHP: median (interquartile range) age 66.5 (14.0) years, 51.9% females and 55.1% never-smokers. The cause of fHP was mainly fungal (39.7%) or avian (41.4%). Lung biopsy was performed in 269 cases (66.7%). In the whole cohort the variables that were related to mortality or lung transplant were older age (HR 1.08; p<0.001), percentage predicted forced vital capacity (HR 0.96; p=0.001), lymphocytosis in bronchoalveolar lavage (BAL) (HR 0.93; p=0.001), presence of acute exacerbation during follow-up (HR 3.04; p=0.001) and GAP (gender, age and lung physiology) index (HR 1.96; p<0.01). In the group of biopsied patients, the presence of fibroblastic foci at biopsy (HR 8.39; p<0.001) stands out in multivariate Cox regression analyses as a highly significant predictor for increased mortality or lung transplant. GAP index (HR 1.26; p=0.009), lymphocytosis in BAL (HR 0.97; p=0.018) and age (HR 1.03; p=0.018) are also predictors of progression. Conclusions: The study identified several prognostic factors for progression and/or survival in fHP. The presence of fibroblastic foci at biopsy was a consistent predictor for increased mortality and the presence of lymphocytosis in BAL was inversely related to mortality.

Biological Biomarkers in Respiratory Diseases. / Biomarcadores biológicos en las enfermedades respiratorias.

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area.

Coagulation disorders and thromboembolic disease in COVID-19: review of current evidence in search of a better approach.

The new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been an unprecedented global health problem, causing more than 20 million infections and more than 900,000 deaths (September 2020). The SARS-CoV-2 infection, known as COVID-19, has various clinical presentations, from asymptomatic or mild catarrhal processes to severe pneumonia that rapidly progresses to acute respiratory distress syndrome (ARDS) and multiple organ failure. In the last few months, much scientific literature has been devoted to descriptions of different aspects of the coagulation disorders and arterial and venous thrombotic complications associated with COVID-19, particularly venous thromboembolism (VTE). These studies have revealed that SARS-CoV-2 could lead to a prothrombotic state reflecting the high cumulative incidence of associated thrombotic events, particularly in patients admitted to intensive care units (ICUs). As regards the coagulopathy observed in association with SARS-CoV-2 infection, the mechanisms that activate coagulation have been hypothesized as being linked to immune responses, through the release of pro-inflammatory mediators that interact with platelets, stimulate the expression of tissue factor, induce an upregulation of plasminogen activator inhibitor-1, suppress the fibrinolytic system and lead to endothelial dysfunction, triggering thrombogenesis. D-dimer elevation has been recognized as a useful biomarker of poor prognosis, although the best cut-off point for predicting VTE in COVID-19 patients has still not been clarified. This review will try to update all the available scientific information on this important topic with enormous clinical and therapeutic implications.

Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association.

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7-23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients' survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

Diagnostic delay of associated interstitial lung disease increases mortality in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. 106 patients were included. The multivariate analysis showed that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01-1.23, p = 0.035). Also, age (HR 1.33, 95% CI 1.09-1.62, p = 0.0045), DLCO (%) (HR 0.85, 95% CI 0.73-0.98, p = 0.0246), and final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39-0.99, p = 0.0465) were independent predictor variables of mortality, as well as GAP index (HR 4.65, 95% CI 1.59-13.54, p = 0.0051) and CPI index (HR 1.12, 95% CI 1.03-1.22, p = 0.0092). The withdrawal of MTX or LFN after ILD diagnosis was associated with disease progression in the COX analysis (HR 2.18, 95% CI 1.14-4.18, p = 0.019). This is the first study that highlights the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

[Case reports in Archivos de Bronconeumología: the journal's butterfly collection]. / La colección de mariposas de Archivos de Bronconeumología.

The aim of the case reports section of Archivos de Bronconeumología is to bring together unusual cases representing a substantial contribution to our understanding of the pathophysiology or other clinical and biological features of a disease. During 2007, 17 case descriptions were published in this section. They dealt with such diverse topics as lung cancer surgery, interventional radiology, and idiopathic interstitial diseases. The objective of this review is to call attention to the contributions of these cases by summarizing their main observations.

Biomarcadores biológicos en las enfermedades respiratorias / Biological Biomarkers in Respiratory Diseases

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

[Translated article] Biological Biomarkers in Respiratory Diseases / Biomarcadores biológicos en las enfermedades respiratorias

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

Respiratory diseases registries in the national registry of rare diseases.

This report describes the general characteristics, objectives and organizational aspects of the registries of rare respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans' cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis.

[The clinical view through the Archives: the clinical notes of 2009]. / La clínica vista a través de Archivos: las notas clínicas del 2009.

The Clinical Notes published in 2009 serve as a resource to reflect on clinical aspects relevant to different clinical entities. Through this review an attempt is likewise made to bring the reader closer to the clinical reality of our environment.

Triple terapia en fibrosis pulmonar idiopática / Triple Therapy in Idiopathic Pulmonary Fibrosis

No disponible

[Clinical perspective as seen in Archivos de Bronconeumología]. / La clínica vista a través de Archivos.

This article discusses the current main clinical problems of thoracic surgery and pneumology by means of the articles published in 2008 in the "clinical notes" section of the journal Archivos de Bronconeumología. The 18 cases published -which deal with topics ranging from pharmacologic toxicity to lung function- will enable us to discuss the main novelties in each one of the various fields and highlight future areas for research.

Registros de enfermedades respiratorias integrados en el Registro Nacional de Enfermedades Raras / Respiratory Diseases Registries in the National Registry of Rare Diseases

En el presente trabajo se describen las características generales, objetivos y aspectos organizativos de los registros de enfermedades respiratorias minoritarias integrados en el Registro Nacional de Enfermedades Raras del Instituto de Investigación de Enfermedades Raras (IIER), con el objetivo de dar a conocer su existencia y fomentar la participación de los profesionales. Se recoge información sobre registros de las siguientes enfermedades: déficit de alfa-1 antitripsina, estenosis traqueal idiopática, histiocitosis pulmonar de células de Langerhans del adulto, linfangioleiomiomatosis, proteinosis alveolar y sarcoidosis

This report describes the general characteristics, objectives, and organizational aspects of the registries of minority respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans’ cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis (AU)

La clínica vista a través de Archivos: las notas clínicas del 2009 / The Clinical View through the Archives: The Clinical Notes of 2009

Las notas clínicas publicadas en el año 2009 sirven como elemento de trabajo para reflexionar sobre aspectos clínicos relevantes de diferentes entidades clínicas. Mediante esta revisión se pretende asimismo acercar al lector a la realidad clínica de nuestro entorno(AU)

The Clinical Notes published in 2009 serve as a resource to reflect on clinical aspects relevant to different clinical entities. Through this review an attempt is likewise made to bring the reader closer to the clinical reality of our environment(AU)

La clínica vista a través de archivos / Clinical Perspective by Means of archivos

A través de los artículos publicados en el año 2008 en la sección “Notas clínicas” de la revista Archivos de Bronconeumología, el presente artículo discurre por los principales problemas clínicos de la cirugía torácica y la neumología actual. Los 18 casos publicados, que abarcan desde la toxicidad farmacológica a la función pulmonar, nos permitirán discutir las principales novedades en cada uno de los diversos campos y señalar las futuras áreas de investigación(AU)

This article discusses the current main clinical problems of thoracic surgery and pneumology by means of the articles published in 2008 in the “clinical notes” section of the journal Archivos de Bronconeumología. The 18 cases published —which deal with topics ranging from pharmacologic toxicity to lung function— will enable us to discuss the main novelties in each one of the various fields and highlight future areas for research(AU)

La colección de mariposas de ARCHIVOS DE BRONCONEUMOLOGÍA / Case reports in Archivos de Bronconeumología: the journal’s butterfly collection

'Notas clínicas' es una sección de ARCHIVOS DE BRONCONEUMOLOGÍAen la que se pretende reunir 'casos clínicos deexcepcional observación que supongan una aportación importanteen la clínica, fisiopatología o biología del proceso'. Durante el año 2007 se publicaron en dicha sección 17 casosque abarcaban aspectos tan diferentes como la cirugía delcáncer de pulmón, la radiología intervencionista o las enfermedadesintersticiales idiopáticas. El objetivo de este artículoes resaltar y resumir las principales observaciones derivadasde estos casos clínicos

The aim of the case reports section of Archivos de Bronconeumología is to bring together unusual cases representing a substantial contribution to our understandingof the pathophysiology or other clinical and biologicalfeatures of a disease. During 2007, 17 case descriptions werepublished in this section. They dealt with such diverse topicsas lung cancer surgery, interventional radiology, andidiopathic interstitial diseases. The objective of this review isto call attention to the contributions of these cases bysummarizing their main observations