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.

Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity / Diferentes caras de la fibrosis pulmonar idiopática con capacidad vital forzada preservada

Introduction Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. Methods Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. Results 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern. Conclusions Patients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Introducción La fibrosis pulmonar idiopática (FPI) es progresiva e irreversible. Existen algunas discrepancias sobre la estadificación de la FPI, especialmente en las fases leves. La capacidad vital forzada (FVC) superior al 80% se ha considerado una FPI temprana o leve incluso para el diseño de ensayos clínicos. Métodos Estudio español multicéntrico, observacional, retrospectivo de pacientes con FPI diagnosticados entre 2012-2016, en función de los criterios ATS/ERS, que presentaban FVC mayor o igual al 80% al diagnóstico. Se analizaron características clínicas y demográficas, función pulmonar, patrón radiológico, tratamiento y seguimiento. Resultados Se incluyeron 225 pacientes con FPI, el 72,9% eran varones. La edad media fue de 69,5 años. El patrón predominante en la tomografía computarizada de alta resolución (TCAR) fue compatible con neumonía intersticial usual (NIU) (51,6%). El 84,7% de los pacientes presentó síntomas respiratorios (disnea de esfuerzo o tos) y el 33,33% mostró desaturación con una saturación de oxígeno inferior al 90% en la prueba de la marcha de los 6min (PM6M). El tratamiento antifibrótico se inició en el momento del diagnóstico en el 55,11% de los pacientes. La mediana de la CVF fue del 89,6% (RIC 17) y el 58,7% de los pacientes presentó una disminución de la capacidad pulmonar de difusión del monóxido de carbono (DLCO) por debajo del 60% del valor teórico; la mayoría presentó progresión funcional (61,4%) y una mayor mortalidad a los 3 años (20,45%). Se observó una correlación estadísticamente significativa de la mortalidad a los 3 años entre una DLCO<60% y el patrón radiológico compatible con UIP. Conclusiones Los pacientes con FVC conservada pero que presentan patrón radiológico de NIU y disminución moderada-grave de la de DLCO en el momento del diagnóstico se asociaron a un mayor riesgo de progresión, fallecimiento o tener que recibir un trasplante de pulmón. Por lo tanto, en estos casos, una FVC preservada no sería representativa de una FPI temprana o leve

Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. METHODS: Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. RESULTS: 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern. CONCLUSIONS: Patients with preserved FVC but presenting UIP radiological pattern and moderate-severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Detection of Autoantibodies in Bronchoalveolar Lavage in Patients with Diffuse Interstitial Lung Disease. / Detección de autoanticuerpos en el lavado broncoalveolar en pacientes con enfermedad pulmonar intersticial difusa.

INTRODUCTION: Serum autoantibodies support the diagnosis of interstitial lung disease (ILD) related to systemic autoimmune diseases (SAD-ILD). Nevertheless, their presence in the bronchoalveolar lavage (BAL) has not been explored. OBJECTIVES: To demonstrate the presence of autoantibodies in the BAL of ILD patients at onset of clinical evaluation, its relation with serum autoantibodies and to analyze clinical features of patients with autoantibodies in BAL. METHODS: Autoantibodies against extractable nuclear antigens (ENAs) were analyzed by immunoblot in the BAL of 155 patient with suspected diagnosis of ILD and 10 controls. RESULTS: Seven ENAs were detected in the BAL of 19 patients (Anti-Ro52, Anti-Ro60, CENP-B, Anti-La, Jo-1, Sm/RNP and Anti-SL70). The most frequent ENA was anti-Ro52 (13 patients; 68,4% of positives ones). Seven patients presented more than one ENAs. Fourteen were diagnosed of SAD-ILD, 3 of interstitial pneumonia with autoimmune features, one of non-specific idiopathic pneumonia and other of silicosis. In 10 cases (52%) IgA autoantibodies were also detected. The autoantibodies observed in BAL were also detected in the serum of 17 patients (90%). There were no significant clinical differences with the patients with SAD-ILD or interstitial pneumonia with autoimmune features with patients with negative BAL. CONCLUSION: The study of ENAs in BAL is feasible and can be a useful tool in the ILD initial algorithm, specifically sustaining the suspected diagnosis of SAD-ILD.

Mapping IPF helps identify geographic regions at higher risk for disease development and potential triggers.

BACKGROUND AND OBJECTIVE: The relationship between IPF development and environmental factors has not been completely elucidated. Analysing geographic regions of idiopathic pulmonary fibrosis (IPF) cases could help identify those areas with higher aggregation and investigate potential triggers. We hypothesize that cross-analysing location of IPF cases and areas of consistently high air pollution concentration could lead to recognition of environmental risk factors for IPF development. METHODS: This retrospective study analysed epidemiological and clinical data from 503 patients registered in the Observatory IPF.cat from January 2017 to June 2019. Incident and prevalent IPF cases from the Catalan region of Spain were graphed based on their postal address. We generated maps of the most relevant air pollutant PM2.5 from the last 10 years using data from the CALIOPE air quality forecast system and observational data. RESULTS: In 2018, the prevalence of IPF differed across provinces; from 8.1 cases per 100 000 habitants in Barcelona to 2.0 cases per 100 000 in Girona. The ratio of IPF was higher in some areas. Mapping PM2.5 levels illustrated that certain areas with more industry, traffic and shipping maintained markedly higher PM2.5 concentrations. Most of these locations correlated with higher aggregation of IPF cases. Compared with other risk factors, PM2.5 exposure was the most frequent. CONCLUSION: In this retrospective study, prevalence of IPF is higher in areas of elevated PM2.5 concentration. Prospective studies with targeted pollution mapping need to be done in specific geographies to compile a broader profile of environmental factors involved in the development of pulmonary fibrosis.

Síndrome hepatopulmonar y enfermedad pulmonar intersticial difusa: una asociación poco conocida / Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination

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Cáncer de pulmón en pacientes con combinación de fibrosis pulmonar y enfisema y fibrosis pulmonar idiopática. Estudio descriptivo en una serie española / Lung Cancer in Patients With Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis. A Descriptive Study in a Spanish Series

Introducción: La información sobre la asociación del cáncer de pulmón (CP) y combinación de fibrosis pulmonar y enfisema (CFPE) es limitada y procedente casi exclusivamente de series asiáticas. El objetivo principal del estudio fue valorar el impacto del CP en la supervivencia en la CFPE y en pacientes diagnosticados de fibrosis pulmonar idiopática (FPI). Métodos: Se realizó un estudio retrospectivo con los datos de pacientes con CFPE y FPI diagnosticados en nuestro centro en un periodo de 5 años. Resultados: Se incluyó a 66 pacientes, 29 en el grupo de CFPE y 37 pacientes con FPI. Nueve tenían un diagnóstico de CP (6 con CFPE y 3 con FPI); 6 pacientes (67%) recibieron tratamiento paliativo a pesar de que 3 de ellos presentaban estadios I y II. No hubo diferencias significativas en la mortalidad global de los 2 grupos; sin embargo, en los pacientes con CP la supervivencia fue significativamente menor con respecto a los que no tenían CP (p = 0,044). Las causas más frecuentes de muerte fue la insuficiencia respiratoria secundaria a la exacerbación de la fibrosis pulmonar (44%). En el análisis multivariante, la odds ratio de morir en los pacientes con CP respecto a los pacientes sin CP fue de 6,20 (p = 0,037, intervalo de confianza [IC] del 95%: 1,11 a 34,48). Conclusión: El CP empeora la supervivencia de estas 2 entidades. El manejo diagnóstico y terapéutico del CP se ve dificultado por el mayor riesgo de complicaciones posteriores al tratamiento elegido, incluso tras el tratamiento paliativo (AU)

Introduction: Information on the association of lung cancer (LC) and combined pulmonary fibrosis and emphysema (CPFE) is limited and derived almost exclusively from series in Asian populations. The main objective of the study was to assess the impact of LC on survival in CPFE patients and in patients with idiopathic pulmonary fibrosis (IPF). Methods: A retrospective study was performed with data from patients with CFPE and IPF diagnosed in our hospital over a period of 5 years. Results: Sixty-six patients were included, 29 with CPFE and 37 with IPF. Nine had a diagnosis of LC (6 with CPFE and 3 with IPF). Six patients (67%) received palliative treatment even though 3 of them were diagnosed atstage I-II. Overall mortality did not differ significantly between groups; however, in patients with LC, survival was significantly lower compared to those without LC (P =.044). The most frequent cause of death was respiratory failure secondary to pulmonary fibrosis exacerbation (44%). In a multivariate analysis, the odds ratio of death among patients with LC compared to patients without LC was 6.20 (P =.037, 95% confidence interval: 1.11 to 34.48). Conclusions: Lung cancer reduces survival in both entities. The diagnostic and therapeutic management of LC is hampered by the increased risk of complications after any treatment modality, even after palliative treatment (AU)

Lung Cancer in Patients With Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis. A Descriptive Study in a Spanish Series. / Cáncer de pulmón en pacientes con combinación de fibrosis pulmonar y enfisema y fibrosis pulmonar idiopática. Estudio descriptivo en una serie española.

INTRODUCTION: Information on the association of lung cancer (LC) and combined pulmonary fibrosis and emphysema (CPFE) is limited and derived almost exclusively from series in Asian populations. The main objective of the study was to assess the impact of LC on survival in CPFE patients and in patients with idiopathic pulmonary fibrosis (IPF). METHODS: A retrospective study was performed with data from patients with CFPE and IPF diagnosed in our hospital over a period of 5 years. RESULTS: Sixty-six patients were included, 29 with CPFE and 37 with IPF. Nine had a diagnosis of LC (6 with CPFE and 3 with IPF). Six patients (67%) received palliative treatment even though 3 of them were diagnosed atstage i-ii. Overall mortality did not differ significantly between groups; however, in patients with LC, survival was significantly lower compared to those without LC (P=.044). The most frequent cause of death was respiratory failure secondary to pulmonary fibrosis exacerbation (44%). In a multivariate analysis, the odds ratio of death among patients with LC compared to patients without LC was 6.20 (P=.037, 95% confidence interval: 1.11 to 34.48). CONCLUSIONS: Lung cancer reduces survival in both entities. The diagnostic and therapeutic management of LC is hampered by the increased risk of complications after any treatment modality, even after palliative treatment.

Fibroelastosis pleuropulmonar: ¿es también una entidad idiopática? / Pleuroparenchymal Fibroelastosis: Is it Also an Idiopathic Entity?

La fibroelastosis pleuropulmonar (FEPP) es una entidad rara que ha sido recientemente incluida en el último consenso internacional multidisciplinar sobre neumonías intersticiales idiopáticas. Comparte rasgos clínicos con otras neumonías intersticiales crónicas (disnea de esfuerzo, tos seca, entre otros), y radiológicamente se caracteriza por afectación pleural y pulmonar de predominio en lóbulos superiores. Sus principales hallazgos histológicos incluyen fibrosis de la pleura visceral con prominente fibroelastosis subpleural y parenquimatosa. El conocimiento de sus características se basa en el creciente número de casos descritos en la literatura, por lo cual se desconocen varios aspectos sobre su etiología, patogénesis e historia natural. Aunque algunos casos han sido identificados como idiopáticos, la FEPP ha sido asociada como complicación tras el trasplante de médula ósea, trasplante pulmonar y tratamiento con quimioterapia, especialmente con agentes alquilantes. El neumotórax espontáneo o iatrogénico tras las pruebas invasivas para su diagnóstico es una complicación frecuentemente comunicada. La evolución descrita de la FEPP es variable, desde lentamente progresiva hasta casos con rápido deterioro clínico. No hay ningún tratamiento con evidencia de eficacia, y el trasplante pulmonar se erige como la única opción en aquellos pacientes que cumplan los criterios. El reconocimiento y difusión de las características de esta entidad son fundamentales para elevar el grado de sospecha clínica y permitir un adecuado manejo diagnóstico por parte del equipo multidisciplinar

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE are essential to raise the level of clinical suspicion for this entity, and to implement appropiate diagnostic and management by the multidisciplinary team

Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias.

Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.

Pulmonary hemodynamic profile in chronic obstructive pulmonary disease.

INTRODUCTION: Few data are available in regards to the prevalence of pulmonary hypertension (PH) in the broad spectrum of COPD. This study was aimed at assessing the prevalence of PH in a cohort of COPD patients across the severity of airflow limitation, and reporting the hemodynamic characteristics at rest and during exercise. METHODS: We performed a retrospective analysis on COPD patients who underwent right-heart catheterization in our center with measurements obtained at rest (n=139) and during exercise (n=85). PH was defined as mean pulmonary artery pressure (mPAP) ≥25 mmHg and pulmonary capillary wedge pressure <15 mmHg. Exercise-induced PH (EIPH) was defined by a ratio of ΔmPAP/Δcardiac output >3. RESULTS: PH was present in 25 patients (18%). According to the Global Initiative for Chronic Obstructive Lung Disease (GOLD) classification, PH prevalence in GOLD 2 was 7% (3 patients); 25% (14 patients) in GOLD 3; and 22% (8 patients) in GOLD 4. Severe PH (mPAP ≥35 mmHg) was identified in four patients (2.8%). Arterial partial oxygen pressure was the outcome most strongly associated with PH (r=-0.29, P<0.001). EIPH was observed in 60 patients (71%) and had a similar prevalence in both GOLD 2 and 3, and was present in all GOLD 4 patients. Patients with PH had lower cardiac index during exercise than patients without PH (5.0±1.2 versus 6.7±1.4 L/min/m(2), respectively; P=0.001). CONCLUSION: PH has a similar prevalence in COPD patients with severe and very-severe airflow limitation, being associated with the presence of arterial hypoxemia. In contrast, EIPH is highly prevalent, even in moderate COPD, and might contribute to limiting exercise tolerance.

Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management.

Enfermedad pulmonar obstructiva crónica y ventrículo izquierdo / Chronic Obstructive Pulmonary Disease and Left Ventricle

Numerosos estudios han puesto de manifiesto que la interacción entre la enfermedad pulmonar obstructiva crónica (EPOC) y la comorbilidad cardiovascular es compleja y bidireccional, puesto que cada una de estas entidades complica el pronóstico de la otra. El avance en las técnicas de imagen ha dado paso a una mejor caracterización de las cavidades cardíacas, hecho que ha permitido el estudio de la relación que existen entre ciertos parámetros de función cardíaca con variables clínicas y funcionales en la EPOC. A pesar de que las alteraciones cardíacas en la EPOC han sido adscritas fundamentalmente al ventrículo derecho, diversos estudios han descrito que el ventrículo izquierdo también se puede afectar en esta enfermedad. Una mejor compresión de los mecanismos involucrados y de sus implicaciones clínicas permitirá establecer estrategias de abordaje diagnóstico y terapéutico en los pacientes donde coexistan estas 2 entidades

Several studies have shown that the interaction between chronic obstructive pulmonary disease (COPD) and cardiovascular comorbidity is complex and bidirectional, since each of these diseases complicates the prognosis of the other. Recent advances in imaging technology have led to better characterization of cardiac chambers and allowed the relationship between certain cardiac function parameters and COPD clinical and functional variables to be explored. Although cardiac abnormalities in COPD have been mainly associated with the right ventricle, several studies have reported that the left ventricle may also be affected in this disease. A better understanding of the mechanisms involved and their clinical implications will establish diagnostic and therapeutic strategies for patients with both these conditions

Chronic obstructive pulmonary disease and left ventricle.

Several studies have shown that the interaction between chronic obstructive pulmonary disease (COPD) and cardiovascular comorbidity is complex and bidirectional, since each of these diseases complicates the prognosis of the other. Recent advances in imaging technology have led to better characterization of cardiac chambers and allowed the relationship between certain cardiac function parameters and COPD clinical and functional variables to be explored. Although cardiac abnormalities in COPD have been mainly associated with the right ventricle, several studies have reported that the left ventricle may also be affected in this disease. A better understanding of the mechanisms involved and their clinical implications will establish diagnostic and therapeutic strategies for patients with both these conditions.

Histiocitosis pulmonar de células de Langerhans en adultos: aproximación a la realidad española / Adult pulmonary Langerhans' cell histiocytosis: Approach to the reality of the Spanish population

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[Adult pulmonary Langerhans' cell histiocytosis: approach to the reality of the Spanish population]. / Histiocitosis pulmonar de células de Langerhans en adultos: aproximación a la realidad española.

BACKGROUND AND OBJECTIVE: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare respiratory disease closely associated with smoking. The aim of the study is to know the reality of PLCH in the Catalonian population. MATERIAL AND METHODS: We included all patients diagnosed of PLCH in 8 different Catalonian hospitals from April 2011 to December 2012. Epidemiologic, clinical, radiological, functional, diagnostic and therapeutic variables were collected, both at the beginning and at the last visit of the process. RESULTS: Thirty two patients were included. Overall, patients were middle-aged (38 years), with no gender predominance. Most of them were smokers (71.9%). The main pulmonary function values (forced vital capacity 75.9%, forced expiratory volume in one second 68.9%, diffusing capacity of the lung for carbon monoxide 59.6%) and radiological findings (cysts 89.7%, nodules 60.3%) showed no significant changes during evolution. However, the disease progressed with a worsening lung function in 34.4% of cases. The analysis of the functional outcome (forced vital capacity, forced expiratory volume in one second, and diffusing capacity of the lung for carbon monoxide) did not reveal significant differences according to the persistence or not of smoking (P<0.05). CONCLUSION: Our patients with PLCH had similar clinical and radiological characteristics to those described in other populations. The results observed regarding the relationship with smoking suggest that there may be other factors beyond smoking, which influence on the evolution of the disease. It therefore seems advisable to design prospective studies to investigate this aspect.

Echocardiographic abnormalities in patients with COPD at their first hospital admission.

Cardiovascular disease accounts for significant morbidity and mortality in chronic obstructive pulmonary disease (COPD). Its prevalence and mechanisms of association have not been elucidated. The study aimed to assess the prevalence of echocardiographic abnormalities and potential risk factors in patients with COPD at their first exacerbation requiring hospital admission. Transthoracic echocardiography was prospectively performed in 342 patients (forced expiratory volume in 1 s 52 ± 16% predicted) 3 months after discharge. Significant cardiac alterations were present in 64% of patients; 27% left- and 48% right-heart disorders. The most common were right ventricle enlargement (30%) and pulmonary hypertension (19%). Left ventricle enlargement was present in 6%, left ventricle systolic dysfunction in 13%, left ventricle diastolic impairment in 12% and left atrial dilatation in 29%. Echocardiographic abnormalities were unrelated to COPD severity and were more frequent in patients with self-reported cardiac disease. They were also observed in 63% of patients with no known cardiac disease or cardiovascular risk factors other than smoking. We conclude that cardiac abnormalities are highly prevalent in COPD patients at the time of their first severe exacerbation, even in the absence of established cardiac disease or cardiovascular risk factors. Considering the prognostic and therapeutic implications of cardiac comorbidity, echocardiography should be considered in the assessment of patients with clinically significant COPD.

Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease.

Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DL(co)) and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.

Anemia is a mortality predictor in hospitalized patients for COPD exacerbation.

BACKGROUND: Anemia is a recognized prognostic factor in many chronic illnesses, but there is limited information about its impact on outcomes in patients hospitalized for acute COPD exacerbation (AECOPD). AIM: To investigate whether anemia exerts an effect on mortality in patients admitted for AECOPD after one year of follow-up. Methods. From November 2007 to November 2009 we recruited 117 patients who required hospitalization due to an AECOPD. Clinical, functional and laboratory parameters on admission were prospectively assessed. Patients were followed up during one year. Mortality and days-to-death were collected. RESULTS: Mean age 72 (SD ± 9); FEV1 37.4 (SD ± 12); mortality after 1 year was 22.2%. Mean survival: 339 days. Comparing patients who died to those who survived we found significant differences (p < 0,000) in hemoglobin (Hb) (12.4 vs 13.8 mg/dl) and hematocrit (Ht) (38 vs 41%). Anemia (Hb < 13 g.dl⁻¹) prevalence was 33%. Those who died had experienced 3.5 exacerbations in previous year vs 1.5 exacerbations in the case of the survivors (p = 0.000). Lung function and nutritional status were similar, except for percentage of muscle mass (%) (35 vs 39%; p = 0.015) and albumin (33 vs 37 mg/dl; p = 0.039). These variables were included in a Multivariate Cox Proportional Hazards Model; anemia and previous exacerbations resulted as independent factors for mortality. Mortality risk for patients with anemia was 5.9(CI: 1.9-19); for patients with > 1 exacerbation in the previous year was 5.9(CI: 1.3-26.5). CONCLUSION: Anemia and previous exacerbations were independent predictors of mortality after one year in patients hospitalized for AECOPD.