Superposición Asma-EPOC: los hallazgos en el estudio EPOC AR / Asthma-COPD Overlap: Findings of the EPOC AR Study

Introducción: Asma y EPOC son enfermedades heterogéneas, algunos pacientes comparten características clínicas de ambas. Existen incertidumbres en los criterios para definir superposición asma-EPOC (ACO) y la prevalencia es entre el 15% y el 25% de la población adulta con obstrucción crónica del flujo aéreo. Motiva este estudio determinar la prevalencia de ACO en Argentina, que es desconocida. Objetivos: Primario: Determinar prevalencia de ACO en el estudio EPOC-AR. Se cundarios: Evaluar y analizar las características clínicas de los pacientes con ACO, la gravedad de los síntomas, la frecuencia y gravedad de exacerbaciones. Describir y comparar el tratamiento entre ACO vs. EPOC puros. Base de datos del estudio EPOC.AR: Espirometrías, asma, atopía o rinitis, síntomas respiratorios: CAT (prueba de evaluación de EPOC) y mMRC (Medical Research Council modificado), frecuencia de exacerbaciones/año previo, comorbilidades y tratamientos. Guías GOLD 2017 para determinar grados de obstrucción espirométrica y Grupos A, B, C y D. Criterios diagnósticos de ACO (comité expertos USA, Europa del Este y Asia-Denver 2015): CRITERIOS MAYORES: 1. Obstrucción persistente (FEV1/FVC pos-BD <70% o LIN) en ≥ 40 años. 2. TBQ ≥ 10 paquetes/año, contaminación ambiental o biomasa. 3. Historia documentada de asma antes de los 40 años o respuesta pos-BD ≥ 400 mL en FEV1. CRITERIOS MENORES: 1. Historia documentada de atopía o rinitis alérgica. 2. Res puesta pos-BD en FEV1 > 200 mL. 3. Recuento de eosinófilos en sangre periférica ≥ 300 células-Ul-1 (no realizado en EPOC.AR). Prueba de Chi-cuadrado, Chi-cuadrado de Pearson, razón de verosimilitud, asociación lineal por lineal. Resultados: EPOC (n498), n95 con criterios de ACO, masculino (53,4%) y edad pro medio 63,6 años. El 1%, sin asma y respuesta BD ≥ 400 mL; el 32,7%, asmáticos (3,6% respuesta BD ≥ 400 mL y el 14,5%, entre 200-400 mL); n23 respuesta BD ≥ 400 mL (4,6%). Prevalen cia ACO: 19,08% (IC 15,6-22,5) y del 2,6% del total de la población de EPOC.AR. En población ACO vs. EPOC, se detectó: menor promedio de edad y de FEV1 pre BD (p < 0,01), mayor respuesta BD (p < 0,05), mayor frecuencia de sibilancias (p < 0,01; IC 2,75-7,64), mayor frecuencia de diagnóstico previo de asma (p < 0,01; IC 3,79-10,05) y el 26,08% tenían antecedentes familiares de asma. Mayor uso de ATB (p < 0,05) e ICS/LABA (p < 0,05; IC 1,1-5,3). Mayor frecuencia de exacerbaciones (12,47%; IC 9,56-15,39) que motivaron indicación de medicación en un 90,48% y 2,49 veces más de alteraciones en actividades diarias y ausentismo laboral. No se registraron diferencias significativas entre pacientes con ACO frente a EPOC puros en frecuencia de grupos A, B, C y D. Conclusiones: La prevalencia de ACO fue del 19,08% en pacientes EPOC del es tudio EPOC.AR; tenían significativamente menor edad, mayor grado de obstrucción, frecuencia de sibilancias, uso de antibióticos/año previo y CI (LABA/CI). Destacamos la importancia de identificar este fenotipo para un tratamiento adecuado por sus impli cancias clínicas, y deterioro en calidad de vida.

Background: Asthma and COPD are heterogeneous diseases, and some patients share clinical features of both conditions. There are uncertainties about the criteria to define asthma-COPD overlap (ACO), and its prevalence is 15-25% in the adult population with chronic airflow obstruction. The purpose of this study was to determine the prevalence of ACO in Argentina, which is unknown. Objectives: Primary: to determine the prevalence of ACO in the EPOC.AR study. Secondary: to evaluate and analyze the clinical features of patients with ACO, the severity of the symptoms, and the frequency and severity of exacerbations. to describe and compare the treatment of ACO with that of pure COPD. Database of the EPOC.AR study: spirometries, asthma, atopy or rhinitis, respiratory symptoms: CAT (COPD Assesment Test) and mMRC (Modified Medical Research Council) scale, frequency of exacerbations/previous year, comorbidities and treatments. 2017 GOLD Guides (Global Initiative for Chronic Obstructive Lung Disease) to determine airflow obstruction degrees and Groups A, B, C, and D. ACO diagnostic criteria (expert committee from USA, East Europe and Asia that took place in Denver, 2015): MAJOR CRITERIA: 1. Persistent obstruction (post-BD [bronchodilator] FEV1/FVC (forced expiratory volume in the first second/forced vital capacity) < 70% or LLN [lower limit of normal] ) in ≥ 40 years. 2. SM (smoking) ≥ 10 packs/year, air pollution or biomass. 3. Documented history of asthma before 40 years or post-BD response ≥ 400 ml in FEV1. MINOR CRITERIA: 1. Documented history of atopy or allergic rhinitis. 2. Post-BD response in FEV1 > 200 ml. 3. Peripheral blood eosinophil count ≥ 300 cells-Ul-1 (not performed in EPOC.AR). Chi-Square Test, Pearson's Chi Square Test, likelihood ratio, linear-by-linear association. Results: COPD (n 498), n 95 with ACO criteria, males (53.4%), mean age 63.6 years. 1% without asthma and BD response ≥ 400 ml; 32.7% asthmatics (3.6% with BD response ≥ 400 ml and 14.5% between 200-400 ml); n 23 with BD response ≥ 400 ml (4.6%). ACO prevalence: 19.08% (CI [Confidence Interval] 15.6-22.5) and 2.6% of the total population of EPOC.AR. In the comparison between the ACO and COPD populations, we detected the following: lower mean age and pre-BD FEV1 (p < 0.01), higher frequency of BD response (p < 0.05), higher frequency of sibilance (p < 0.01; CI 2.75-7.64), higher frequency of previous asthma diagnosis (p < 0.01; CI 3.79-10.05); and 26.08% had family history of asthma. Greater use of ATBs (antibiotics) (p < 0.05) and ICS (inhaled corticosteroids)/ LABA (long-acting beta- adrenergic agonists) (p < 0.05; CI 1.1-5.3). Higher frequency of exacerbations (12.47%; CI 9.56-15.39) that motivated the indication of medication in 90.48% and 2.49 times more alterations in daily activities and absence from work. There weren't any significant differences between patients with ACO and pure COPD regarding frequency of groups A, B, C and D. Conclusions: the prevalence of ACO was 19.08% in the COPD patients of the EPOC. AR study; they were significantly younger, with higher degree of obstruction, frequency of sibilance, use of antibiotics/previous year and inhaled corticosteroids (LABA/IC). We emphasize the importance of identifying this phenotype in order to use a suitable treat ment, given its clinical implications and deterioration in quality of life.

Asthma-COPD Overlap: Findings of the EPOC.AR Study / Superposición Asma-EPOC: los hallazgos en el estudio EPOC AR

ABSTRACT Background: Asthma and COPD are heterogeneous diseases, and some patients share clinical features of both conditions. There are uncertainties about the criteria to define asthma-COPD overlap (ACO), and its prevalence is 15-25% in the adult population with chronic airflow obstruction. The purpose of this study was to determine the prevalence of ACO in Argentina, which is unknown. Objectives: Primary: to determine the prevalence of ACO in the EPOC.AR study. Secondary: to evaluate and analyze the clinical features of patients with ACO, the severity of the symptoms, and the frequency and severity of exacerbations. to describe and compare the treatment of ACO with that of pure COPD. Database of the EPOC.AR study: spirometries, asthma, atopy or rhinitis, respiratory symptoms: CAT (COPD Assesment Test) and mMRC (Modified Medical Research Council) scale, frequency of exacerbations/previous year, comorbidities and treatments. 2017 GOLD Guides (Global Initiative for Chronic Obstructive Lung Disease) to determine airflow obstruction degrees and Groups A, B, C, and D. ACO diagnostic criteria (expert committee from USA, East Europe and Asia that took place in Denver, 2015): MAJOR CRITERIA: 1. Persistent obstruction (post-BD [bronchodilator] FEV1/FVC (forced expiratory volume in the first second/forced vital capacity) < 70% or LLN [lower limit of normal] ) in ≥ 40 years. 2. SM (smoking) ≥ 10 packs/year, air pollution or biomass. 3. Documented history of asthma before 40 years or post-BD response ≥ 400 ml in FEV1. MINOR CRITERIA: 1. Documented history of atopy or allergic rhinitis. 2. Post-BD response in FEV1 > 200 ml. 3. Peripheral blood eosinophil count ≥ 300 cells-Ul-1 (not performed in EPOC.AR). Chi-Square Test, Pearson's Chi Square Test, likelihood ratio, linear-by-linear association. Results: COPD (n 498), n 95 with ACO criteria, males (53.4%), mean age 63.6 years. 1% without asthma and BD response ≥ 400 ml; 32.7% asthmatics (3.6% with BD response ≥ 400 ml and 14.5% between 200-400 ml); n 23 with BD response ≥ 400 ml (4.6%). ACO prevalence: 19.08% (CI [Confidence Interval] 15.6-22.5) and 2.6% of the total population of EPOC.AR. In the comparison between the ACO and COPD populations, we detected the following: lower mean age and pre-BD FEV1 (p < 0.01), higher frequency of BD response (p < 0.05), higher frequency of sibilance (p < 0.01; CI 2.75-7.64), higher frequency of previous asthma diagnosis (p < 0.01; CI 3.79-10.05); and 26.08% had family history of asthma. Greater use of ATBs (antibiotics) (p < 0.05) and ICS (inhaled corticosteroids)/ LABA (long-acting beta- adrenergic agonists) (p < 0.05; CI 1.1-5.3). Higher frequency of exacerbations (12.47%; CI 9.56-15.39) that motivated the indication of medication in 90.48% and 2.49 times more alterations in daily activities and absence from work. There weren't any significant differences between patients with ACO and pure COPD regarding frequency of groups A, B, C and D. Conclusions: the prevalence of ACO was 19.08% in the COPD patients of the EPOC. AR study; they were significantly younger, with higher degree of obstruction, frequency of sibilance, use of antibiotics/previous year and inhaled corticosteroids (LABA/IC). We emphasize the importance of identifying this phenotype in order to use a suitable treat ment, given its clinical implications and deterioration in quality of life.

RESUMEN Introducción: Asma y EPOC son enfermedades heterogéneas, algunos pacientes comparten características clínicas de ambas. Existen incertidumbres en los criterios para definir superposición asma-EPOC (ACO) y la prevalencia es entre el 15% y el 25% de la población adulta con obstrucción crónica del flujo aéreo. Motiva este estudio determinar la prevalencia de ACO en Argentina, que es desconocida. Objetivos: Primario: Determinar prevalencia de ACO en el estudio EPOC-AR. Se cundarios: Evaluar y analizar las características clínicas de los pacientes con ACO, la gravedad de los síntomas, la frecuencia y gravedad de exacerbaciones. Describir y comparar el tratamiento entre ACO vs. EPOC puros. Base de datos del estudio EPOC.AR: Espirometrías, asma, atopía o rinitis, síntomas respiratorios: CAT (prueba de evaluación de EPOC) y mMRC (Medical Research Council modificado), frecuencia de exacerbaciones/año previo, comorbilidades y tratamientos. Guías GOLD 2017 para determinar grados de obstrucción espirométrica y Grupos A, B, C y D. Criterios diagnósticos de ACO (comité expertos USA, Europa del Este y Asia-Denver 2015): CRITERIOS MAYORES: 1. Obstrucción persistente (FEV1/FVC pos-BD <70% o LIN) en ≥ 40 años. 2. TBQ ≥ 10 paquetes/año, contaminación ambiental o biomasa. 3. Historia documentada de asma antes de los 40 años o respuesta pos-BD ≥ 400 mL en FEV1. CRITERIOS MENORES: 1. Historia documentada de atopía o rinitis alérgica. 2. Respu esta pos-BD en FEV1 > 200 mL. 3. Recuento de eosinófilos en sangre periférica ≥ 300 células-Ul-1 (no realizado en EPOC.AR). Prueba de Chi-cuadrado, Chi-cuadrado de Pearson, razón de verosimilitud, asociación lineal por lineal. Resultados: EPOC (n498), n95 con criterios de ACO, masculino (53,4%) y edad pro medio 63,6 años. El 1%, sin asma y respuesta BD ≥ 400 mL; el 32,7%, asmáticos (3,6% respuesta BD ≥ 400 mL y el 14,5%, entre 200-400 mL); n23 respuesta BD ≥ 400 mL (4,6%). Prevalen cia ACO: 19,08% (IC 15,6-22,5) y del 2,6% del total de la población de EPOC.AR. En población ACO vs. EPOC, se detectó: menor promedio de edad y de FEV1 pre BD (p < 0,01), mayor respuesta BD (p < 0,05), mayor frecuencia de sibilancias (p < 0,01; IC 2,75-7,64), mayor frecuencia de diagnóstico previo de asma (p < 0,01; IC 3,79-10,05) y el 26,08% tenían antecedentes familiares de asma. Mayor uso de ATB (p < 0,05) e ICS/LABA (p < 0,05; IC 1,1-5,3). Mayor frecuencia de exacerbaciones (12,47%; IC 9,56-15,39) que motivaron indicación de medicación en un 90,48% y 2,49 veces más de alteraciones en actividades diarias y ausentismo laboral. No se registraron diferencias significativas entre pacientes con ACO frente a EPOC puros en frecuencia de grupos A, B, C y D. Conclusiones: La prevalencia de ACO fue del 19,08% en pacientes EPOC del es tudio EPOC.AR; tenían significativamente menor edad, mayor grado de obstrucción, frecuencia de sibilancias, uso de antibióticos/año previo y CI (LABA/CI). Destacamos la importancia de identificar este fenotipo para un tratamiento adecuado por sus impli cancias clínicas, y deterioro en calidad de vida.

Key factors associated with uncontrolled asthma - the Asthma Control in Latin America Study.

Objective: This study aimed to estimate asthma control at specialist treatment centers in four Latin American countries and assess factors influencing poor asthma control.Methods: Patients aged ≥12 years with an asthma diagnosis and asthma medication prescription, followed at outpatient specialist centers in Argentina, Chile, Colombia, and Mexico, were included. The study received all applicable ethical approvals. The Asthma Control Test (ACT) was used to classify patients as having controlled (ACT 20-25) or uncontrolled (ACT ≤19) asthma. Frequency and statistical tests were used to assess the association between hospital admissions/exacerbations/emergency department (ED) visits and uncontrolled asthma; multivariate logistic regression was used to assess the association of uncontrolled asthma with clinical/demographic variables.Results: A total of 594 patients were included. Overall controlled-asthma prevalence was 43.4% (95% confidence interval [CI]: 39.0, 47.4). Patients with uncontrolled asthma were more likely to be women (adjusted odds ratio [aOR]: 1.85; p = 0.003), non-white (aOR: 2.14; p < 0.001), obese (aOR: 1.71; p = 0.036), to have a low monthly family income (aOR: 1.75; p = 0.004), to have severe asthma (aOR:1.59; p = 0.26), and, compared with patients with controlled asthma, to have a higher likelihood of asthma exacerbations (34.5% vs. 15.9%; p < 0.001), hospital admissions (6.9% vs. 3.1%; p = 0.042), and ED visits (34.5% vs. 15.9%; p < 0.001) due to asthma.Conclusions: Even in specialist ambulatory services, fewer than half of patients were classified as having controlled asthma. The proportion of uncontrolled patients varied according to clinical and demographic variables.

Demographic Characteristics and Clinical Outcomes in Patients from Latin America Versus the Rest of the World: A TIOSPIR® Post-Hoc Analysis.

INTRODUCTION: Geographical variations may impact outcomes in chronic obstructive pulmonary disease (COPD). We evaluated differences in baseline characteristics and outcomes between patients enrolled in Latin America compared with the rest of the world (RoW) in the TIOtropium Safety and Performance In Respimat® (TIOSPIR®) trial. METHODS: TIOSPIR®, a 2-3-year, randomized, double-blind trial (n=17116; treated set), compared safety and efficacy of once-daily tiotropium Respimat® 5 and 2.5µg with tiotropium HandiHaler® 18µg. This post-hoc analysis pooled data from all treatment arms to assess mortality, exacerbations, cardiac events, and serious adverse events (SAEs) between both regions. RESULTS: At baseline, patients enrolled in Latin America (n=1000) versus RoW (n=16116) were older, with higher pack-years of smoking history and more exacerbations, but less cardiac history. In this analysis, patients in Latin America versus RoW had an increased risk of death (hazard ratio [HR] [95% confidence interval (CI)]: 1.52 [1.24-1.86]; P<.0001) or moderate-to-severe exacerbation (HR [95% CI]: 1.29 [1.18-1.41]; P<.0001), but a lower risk of severe exacerbation (HR [95% CI]: 0.82 [0.68-0.98]; P=.0333). SAE rates in Latin America were lower versus RoW (incidence rate ratio [IRR] [95% CI]: 0.82 [0.72-0.92]), including cardiac disorders (IRR [95% CI]: 0.68 [0.48-0.97]). Risk of major adverse cardiovascular events were similar (HR [95% CI]: 0.99 [0.71-1.40]; P=.9677). CONCLUSIONS: TIOSPIR® patients in Latin America had a higher risk of death or moderate-to-severe exacerbation, but a lower risk of severe exacerbation than those in RoW. Geographical differences may impact outcomes in COPD trials.

Prevalence of COPD in 6 Urban Clusters in Argentina: The EPOC.AR Study. / Prevalencia de enfermedad pulmonar obstructiva crónica en 6 aglomerados urbanos de Argentina: el estudio EPOC.AR.

INTRODUCTION: The prevalence of chronic obstructive pulmonary disease (COPD) has not been studied in Argentina. OBJECTIVES: To determine the prevalence and relevant clinical characteristics of COPD in a representative sample. MATERIAL AND METHODS: We performed a cross-sectional study in a population of adults aged ≥ 40 years randomly selected by cluster sampling in 6 urban locations. Subjects answered a structured survey and performed pre- and post-bronchodilator spirometry (PBD). COPD was defined as FEV1/FVC ratio < 0.7 predicted value. The total prevalence was estimated for each cluster with its 95% confidence interval (CI). RESULTS: Of 4,599 surveys and 3,999 spirometries, 3,469 were considered of adequate quality (86.8%) for our study. The prevalence of COPD was 14.5% (CI: 13.4-15.7). The distribution of COPD cases according to FEV1 (GOLD 2017) was stage 1: 38% (CI: 34-43); stage 2: 52% (CI: 47-56); stage 3: 10% (CI: 7-13); and stage 4: 1% (CI: 0-2), and according to the refined ABCD (GOLD 2017) assessment: A: 52% (CI: 47-56); B: 43% (CI: 39-48); C: 1% (CI: 0-2); D: 4% (CI: 2-6). The rate of underdiagnosis was 77.4% (CI 73.7-81.1%) and diagnostic error 60.7% (CI 55.1-66.3%). A significant association was found between COPD and age (OR 3.77 in individuals 50-59 years of age and 19.23 in those > 80 years), male gender (OR 1.62; CI 1.31-2), smoking (OR 1.95; CI 1.49-2.54), low socioeconomic status (OR 1.33; CI 1.02-1.73), and previous tuberculosis (OR 3.3; CI 1.43-7.62). CONCLUSIONS: We estimate that more than 2.3 million Argentineans have COPD, with high rates of underdiagnosis and diagnostic error.

Recomendaciones de vacunación en adultos con enfermedades respiratorias: Documento de la Asociación Argentina de Medicina Respiratoria para los neumonólogos / Recommendations on Vaccination for Adults with Respiratory Diseases. Document of the Argentina Association of Respiratory Medicine for Pulmonologists

El neumonólogo de adultos acostumbra a prescribir vacunas. Este documento hecho por expertos en aspectos de la especialidad que involucran vacunar a pacientes con enfermedades respiratorias, perteneciente a la Asociación Argentina de Medicina Respiratoria, resumió la información disponible proponiendo una participación activa en la vacunación contra influenza (VAG), neumococo (VAN), pertusis y zoster. El Ministerio de Salud (MSAL) en Argentina, como el CDC y su comité de consulta sobre inmunización (ACIP) en Estados Unidos, elaboran calendarios y recomendaciones para vacunación. La ACIP recomienda la VAG a mayores de 6 meses sin contraindicaciones; el MSAL a mayores de 65 años y a quienes tengan comorbilidades (incluye enfermedades respiratorias y tabaquismo) o contacto con personas vulnerables. La clásica VAN polisacárida de 23 serotipos es recomendada para adultos con riesgo de enfermedad invasiva, incluyendo a mayores de 65 años, revacunando a los inmunosuprimidos y una única vez a los mayores de 65 que hubieran sido vacunados 5 años antes o más; la ACIP recomienda dar la VAN conjugada de 13 serotipos, más inmunogénica, secuencialmente con la polisacárida de 23, en adultos con factores de riesgo y en mayores de 65 años. Sugerimos usarla en menores de 65 con comorbilidad respiratoria. El neumonólogo debe recordar al menos 2 vacunas más: dar el refuerzo decenal contra difteria y tétanos (DT) en mayores de 18, una vez con vacuna triple acelular (difteria, pertusis y tétanos) protegiendo contra pertusis y reduciendo su transmisión. El herpes zoster produce un rash cutáneo vesicular doloroso. Uno cada 2 mayores de 85 sufrirán al menos un ataque de herpes zoster. La vacuna reduce más del 50% la incidencia y más del 60% la neuralgia post herpética; el ACIP la recomienda en mayores de 60 años. Un gran número de los pacientes con afecciones pulmonares crónicas tienen esa edad.

The pulmonologist uses to prescribe vaccines to adult patients. Experts of the Argentina Association of Respiratory Medicine who are specialists in areas involving vaccination of patients with respiratory diseases prepared this document which summarizes the available information and proposes an active prescription of the infuenza, pneumococcus, pertussis and herpes zoster vaccinations. The Ministry of Health in Argentina as the CDC and its Advisory Committee on Immunization Practices (ACIP) in the USA, made recommendations on vaccination indications and schedules. The ACIP recommends influenza vaccination to persons older than 6 months of age without any contraindication. The Ministry of Health recommends this vaccination to persons over 65 years of age, to those with morbidities (including respiratory diseases and smoking habit) and to persons in contact with high risk people. The classic 23-valent polysaccharide pneumococcal vaccine is recommended for adults at risk of invasive disease, including persons over 65 years of age. Revaccination is recommended to immunosuppressed patients and persons over 65 years of age at 5-year intervals. The ACIP recommends vaccination with the 13-valent serotypes polysaccharide pneumococcal vaccine, which is more immunogenic, sequentially with the 23-valent vaccine in adults with risk factors and over 65 years of age. We suggest this practice in patients under 65 years of age with respiratory morbidities. The pulmonologist must remember at least two other vaccines: a booster vaccination every 10 years of diphtheria and tetanus vaccine to persons over 18 years of age, and once the triple acellular vaccine (diphtheria, pertussis and tetanus) to protect against pertussis and reduce transmission. Herpes zoster (shingles) causes a painful vesicular rash; 50% of persons over 85 years suffer at least one bout of herpes zoster. The vaccine reduces more than 50% incidence and more than 60% postherpetic neuralgia. This vaccine is recommended by ACIP for persons over 60 years. In this age group there are many patients with chronic lung conditions.

Síndrome de Hughes-Stovin / Hughes-Stovin Syndrome

El síndrome de Hughes-Stovin (SHS) es una enfermedad rara, caracterizada por la presencia de tromboflebitis y aneurismas de las arterias pulmonares y/o bronquiales. Si bien la etiología y patogénesis del SHS se desconoce, se han identificado asociaciones con ciertas infecciones y angiodisplasia. Pertenece al grupo de las vasculitis generalizadas, con un mecanismo patológico muy parecido al de la enfermedad de Behçet (EB), incluso considerándose como una variante de esta última. Las manifestaciones clínicas incluyen tos, disnea, fiebre, toracodinia y hemoptisis. Es fundamental realizar un diagnóstico y tratamiento de manera temprana debido a que estos pacientes presentan un alto riesgo de ruptura de los aneurismas de arteria pulmonar. El tratamiento se basa en el uso de agentes inmunosupresores.

The Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. Early diagnosis and treatment are important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture. Treatment usually involves immunosuppressive agents.

Alpha-1 Antitrypsin Deficiency in COPD Patients: A Cross-Sectional Study.

INTRODUCTION: Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder associated with early onset chronic obstructive pulmonary disease (COPD) and liver disease. It is also a highly under-diagnosed condition. As early diagnosis could prompt specific interventions such as smoking cessation, testing of family members, genetic counselling and use of replacement therapy, screening programs are needed to identify affected patients. OBJECTIVE: To estimate the prevalence of severe AATD in COPD patients by routine dried blood spot testing and subsequent genotyping in patients with alpha-1 antitrypsin (AAT) levels below an established threshold. MATERIALS AND METHODS: Cross-sectional study of adult COPD patients attending the Hospital Dr. Antonio Cetrángolo (Buenos Aires, Argentina) between 2009 and 2012. The study consisted of capillary blood collection via finger stick to determine AAT levels, clinical evaluation and lung function tests. Genotype was determined in AAT-deficient patients. RESULTS: A total of 1,002 patients were evaluated, of whom 785 (78.34%) had normal AAT levels, while low AAT levels were found in 217 (21.66%). Subsequent genotyping of the latter sub-group found: 15 (1.5%, 95% CI 0.75-2.25) patients with a genotype associated with severe AATD, of whom 12 were ZZ (1.2%, 95% CI 0.52-1.87) and 3 SZ (0.3%, 95% CI 0-0.64). The remaining 202 patients were classified as: 29 Z heterozygotes (2.89%, 95% CI 1.86-3.93), 25 S heterozygotes (2.5%, 95% CI 1.53-3.46) and 4 SS (0.4%, 95% CI 0.01-0.79). A definitive diagnosis could not be reached in 144 patients (14.37%, 95% CI 12.2-16.54). CONCLUSION: The strategy using an initial serum AAT level obtained by dried blood spot testing and subsequent genotyping was a satisfactory initial approach to a screening program for severe AAT, as a definitive diagnosis was achieved in 87% of patients. However, results were not obtained for logistical reasons in the remaining 13%. This major obstacle may be overcome by the use of dried blood spot phenotyping techniques. We believe this approach for detecting AATD in COPD patients, in compliance with national and international guidelines, is supported by our results.

Estudio multicéntrico sobre prevalencia de tabaquismo en mujeres embarazadas en 15 centros de salud de argentina / Multicenter Study on Prevalence of the Tobacco Smoking Habit among Pregnant Women in 15 Health Centers of Argentina

Introducción: No se conoce con exactitud cuál es el número de mujeres que fuman durante el embarazo y es difícil su evaluación, ya que muchas de ellas niegan ser fumadoras en sus consultas médicas. Ante la falta de datos epidemiológicos en Argentina, se realizó esta encuesta a fin de estimar la prevalencia de consumo de tabaco en una población de mujeres embarazadas en distintos centros de salud de la Argentina. Material y métodos: Se realizó un estudio prospectivo, transversal, no terapéutico, descriptivo, a través de una encuesta anónima a mujeres gestantes en cualquier trimestre y sin límite de edad. El cuestionario utilizado fue diseñado a tal fin por un grupo de especialistas en neumonología de la Sección Neumonología Clínica de la Asociación Argentina de Medicina Respiratoria. Resultados y discusión: La prevalencia de fumadoras del total de encuestadas fue del 19.8%. La casuística analizada, al igual que la de otros autores, demostró que un alto porcentaje de embarazadas mantiene el consumo de cigarrillos a pesar de conocer los riesgos de la adicción sobre el feto y la madre.

Introduction: It is not known exactly the number of women who smoke during pregnancy and its estimate is difficult because many of them deny being smokers at the medical consultation. In the absence of epidemiological data in Argentina, this survey was conducted to estimate the prevalence of the tobacco smoking habit in a population of pregnant women in different health centers in Argentina. Material and methods: We performed a prospective, cross sectional, non-therapeutic, and descriptive, through an anonymous survey of pregnant women in any quarter and without age limit. The questionnaire was designed for this purpose by a group of specialists of the Pulmonary Clinic Pulmonary Section of the Argentina Association of Respiratory Medicine. Results and discussion: The prevalence of smoking habit of the total respondents was 19.8%. The data analysis, like in other publications, showed that a high percentage of pregnant keeps the smoking habit despite knowing the risks of addiction on the fetus and mother.

Diagnóstico de secuestro pulmonar mediante tomografía computada / Diagnosis of lung sequestration by computed tomography

Hombre de 45 años que consulta derivado por su médico de cabecera para evaluación de radiografía de tórax de catastro (Fig. 1) donde se pone en evidencia una opacidad redondeada en base izquierda que no hace signo de la silueta con el corazón. Se solicita tomografía computada (TC) de tórax helicoidal con contraste endovenso que pone de manifiesto una hiperdensidad de bordes lobulados en el segmento posterobasal del lóbulo inferior izquierdo, que presenta sutiles imágenes aéreas en su interior. Se identifica un nivel hidroaéreo en su porción cefálica. Se encuentra irrigada por una arteria que emerge de la pared izquierda del tercio inferior de la aorta torácica descendente y su drenaje venoso es a través de una rama de la vena pulmonar inferior izquierda. Se encuentra rodeada por un área de disminución de la densidad del parénquima pulmonar. Las imágenes descritas impresionan en relación a un secuestro pulmonar intraloba

Frecuencia de trastornos respiratorios del sueño en pacientes con enfermedad difusa del parénquima pulmonar / Frecuency of sleep related disorders in patients with diffuse lung diseases

Introducción: Los pacientes con enfermedad pulmonar difusa (EPD) a menudo presentan fragmentación del sueño con microdespertares frecuentes. En este grupo de pacientes, la desaturación nocturna de oxígeno es frecuente con una prevalencia de 50- 80%. Se agrava en el sueño REM y se asocia con una menor calidad de vida, pudiendo contribuir al daño vascular a largo plazo. La presencia de apneas obstructivas del sueño (AOS) se presenta en el 20% de la población adulta general. Objetivos: Describir las características de los parámetros respiratorios clínicos y fisiológicos y la frecuencia de los trastornos respiratorios del sueño (TRS), especialmente de AOS, en pacientes con EPD. Materiales y métodos: Se incluyeron prospectivamente pacientes con diagnóstico establecido de EPD que consultaron en un hospital especializado en enfermedades respiratorias entre julio de 2010 y enero de 2012. Se les realizó polisomnografía (PSG) y estudio de función pulmonar (CVF y DLCO). Resultados: Se estudiaron 41 pacientes con edad promedio de 61.5 ± 12.6 años. El 54% eran hombres (n = 22). Los diagnósticos de EPD fueron neumonía intersticial usual (NIU) en el 70.7%, neumonía intersticial no específica (NINE) en el 14.6%, neumonitis por hipersensibilidad (NHS) en el 9.8% y proteinosis alveolar pulmonar (PAP) en el 4.8%. El índice de masa corporal (IMC) medio fue 29.4 ± 4.4 kg/m². En el 80% fue = 25 y en el 41% = 30. La CVF media fue de 2.18 ± 0.7 l y 64.8 ± 16.8 del porcentaje del predictivo. La DLCO ajustada a la Hb media fue de 10.3±3.6 ml/mmHg/min y 46.9 ± 14 del porcentaje del valor predictivo. Con respecto a la PSG, la SpO2 basal media fue de 93.9 ±3.4%, la saturación media durante el sueño fue 89.8 ±7% y en el 39% de los casos la SpO2 media era < 90%. En el 90% de los casos la SpO>2 mínima durante el sueño fue menor de 90%. El 34% de los casos presentaba CT 90 (tiempo con SpO2 < 90%) > 50%. De los 13 pacientes con SpO2 basal despierto > 95%, 3 presentaron un CT90 = 20% yv de los 27 pacientes con SpO2 basal > 92%, 10 cursaban con un CT90 = 20%.v Del total de los pacientes, el 48.8% presentaba AOS. El índice de apneas-hipoapneasv (IAH) medio fue 11.4 y el 20% de los pacientes presentó IAH = 15. Con respecto a la escala de somnolencia de Epworth (ESE), el 45% de los pacientes conv AOS presentaba una ESE = 10, que resultó significativamente mayor en comparación conv el grupo sin AOS. Del grupo de pacientes con IAH = 15, el 62.5% presentaban ESE < 10. Conclusiones: Un número importante de pacientes sin hipoxemia en reposo presentan desaturación durante el sueño. En este estudio, la frecuencia de AOS fue del 48.8%. Además, el grupo de pacientes con EPD con AOS presentó mayor compromiso oximétrico medido a través de CT90. Globalmente, el grupo de pacientes con AOS presentaba mayores valores de FVC (71.25% vs 58.67 p = 0.019).

Background: Patients with interstitial lung disease (ILD) often have sleep fragmentation with frequent arousals. In this group of patients, nocturnal oxygen desaturation is common, with a prevalence of 50-80%. It is worse in Rapid Eye Movement (REM) sleep, is associated with reduced quality of life and can contribute to long-term vascular damage. The presence of obstructive sleep apnea (OSA) occurs in 20% of the general adult population. Aims: To describe the characteristics of clinical and physiological respiratory parameters and frequency of respiratory sleep disorders (RSD), especially OSA, in patients with ILD. Materials and Methods: We prospectively included ILD patients who attended a specialized hospital between July 2010 and January 2012. Polysomnography (PSG) and study of pulmonary function: Forced Vital Capacity (FVC) and Diffusing Lung Capacity for Carbon Monoxide (DLCO) were performed. Results: We studied 41 patients with a mean age of 61.5 ± 12.6 years. 54% were male (n = 22). ILD diagnoses were usual interstitial pneumonia (UIP) in 70.7%, nonspecific interstitial pneumonia (NSIP) in 14.6%, hypersensitivity pneumonitis (HP) in 9.8% and pulmonary alveolar proteinosis (PAP) in 4.8%. The body mass index (BMI) was 29.4 ± 4.4 kg/m²; it was = 25 in 80% of patients and = 30 in 41%. The mean FVC was 2.18 ± 0.7 l and 64.8 ± 16.8 in the percentage of predictive value. The DLCO adjusted to Hb mean was 10.3 ± 3.6 ml/mmHg/min and 46.9 ± 14 in the percentage of predictive value. Regarding the PSG, the mean baseline SpO2 was 93.9 ± 3.4%, the mean saturation during sleep was 89.8 ± 7%, and the mean SpO2 was < 90% in 39% of cases. In 90% of cases the minimum SpO2 during sleep was less than 90%; 34% of patients had CT 90 (time with SpO2 < 90%) > 50%. In 13 patients with baseline SpO2 > 95%, 3 had CT90 = 20% and 10 of the 27 patients with basal SpO2 > 92%, had CT90 = 20%. Of all patients, 48.8% had OSA. The mean apnea-hypopnea index ...

Manifestaciones clínicas, diagnóstico y tratamiento de la vasculitis de Wegener / Wegener vasculitis: clinical manifestations, diagnosis findings and treatment

La granulomatosis de Wegener es una vasculitis sistémica rara y, como consecuencia del compromiso de múltiples órganos, los pacientes afectados pueden consultar con diversos especialistas. En su patogenia se relaciona con un proceso inflamatorio que afecta la pared vascular. El cuadro clásico de la enfermedad muestra compromiso tanto respiratorio como renal, aunque también pueden estar comprometidos otros órganos. Esta revisión describe el valor diagnóstico de los ANCA y la importancia de las imágenes en el diagnóstico y seguimiento de la enfermedad. Se describen los criterios diagnósticos y el tratamiento, así como los efectos adversos de los fármacos utilizados. Además, se enfatiza en la necesidad de un diagnóstico rápido, para lo cual es necesaria la alta presunción diagnóstica de la enfermedad.

Presentación de un caso de vasculitis de Wegener / Presentation of a case of Wegener’s vasculitis

Se presenta una paciente con granulomatosis de Wegener en su forma localizada y se efectúa una actualización de las manifestaciones clínicas, el diagnóstico y el tratamiento.

Geographic differences in clinical characteristics and management of COPD: the EPOCA study.

AIMS: Data on differences in clinical characteristics and management of COPD in different countries and settings are limited. We aimed to characterize the profile of patients with COPD in a number of countries and their treatment in order to evaluate adherence to recommendations of international guidelines. METHOD: This was an observational, international, cross-sectional study on patients with physician-diagnosed COPD. Demographic and clinical characteristics, risk factors, and treatment were collected by their physician via an internet web-based questionnaire developed for the study. RESULTS: A total of 77 investigators from 17 countries provided data on 833 patients. The countries with the highest number of patients included were: Argentina (128), Ecuador (134), Spain (162), and Hong Kong (153). Overall, 79.3% were men and 81% former smokers, with a mean FEV1 = 42.7%, ranging from 34.3% in Hong Kong to 58.8% in Ecuador. Patients reported a mean of 1.6 exacerbations the previous year, with this frequency being significantly and negatively correlated with FEV1 (%) (r = -0.256; p < 0.0001). Treatment with short-acting bronchodilators and theophyllines was more frequent in Ecuador and Hong Kong compared with Spain and Argentina, and in patients belonging to lower socioeconomic levels (p < 0.0001 for all comparisons). Inadequacy of treatment with inhaled corticosteroids and theophyllines was high, with significant differences among countries. CONCLUSIONS: Differences in the clinical characteristics and management of COPD were significant across countries. Adherence to international guidelines appears to be low. Efforts should be made to disseminate and adapt guidelines to the socioeconomic reality of different settings.

[Non invasive positive pressure ventilation in patients with COPD exacerbation]. / Ventilacion no invasiva en pacientes con exacerbacion de epoc.

This is a prospective study on the implementation of the non-invasive positive pressure ventilation (NPPV) to treat respiratory failure resulting from exacerbation of chronic obstructive pulmonary disease (COPD) in patients hospitalized in a Pneumological Unit. From January 2000 to January 2003, 39 patients were included during 54 different exacerbation events after being evaluated under international standards. They were classified as severe and very severe patients on the basis of their FEV1 values of 26%. Twenty nine patients presented co-morbidities. As a consequence of the NPPV treatment, the pH values increased between the first and last register as well as the pCO2 dropped in the same period. The initial mean pH values were 7.25 reaching mean values of 7.33 at 2 hours and 7.39 at the discharge; the corresponding pCO2 mean values were 83.8 mmHg, 67.8 mmHg and 54.2 mmHg. Thirty five patients out of 39 were discharged after a mean hospitalization length of 13.6 days. Four patients died. Apropriate training of health care staff in general facilities could allow the implementation of NPPV in addition to usual medical care to treat exacerbation of COPD. High morbidity situations could arise during hospitalization, so invasive ventilation must be necessary.

Ventilación no invasiva en pacientes con exacerbacion de EPOC / Non invasive positive pressure ventilation in patients with COPD exacerbation

Se realizó un estudio prospectivo sobre la utilización de la ventilación mecánica no invasiva (VNI) en pacientes internados en Clínica Médica Neumonológica por exacerbación de la enfermedad pulmonar obstructiva crónica (EPOC), con el objetivo de evaluar la evolución, los cambios gasométricos, las comorbilidades y la mortalidad de los pacientes internados. Desde enero 2000 a enero 2003 ingresaron al estudio 39 pacientes, evaluados según normas internacionales en 54 internaciones, siendo clasificados como de grado grave y muy grave, dado que la medición del volumen espiratorio forzado en un segundo (VEF1) era del 26%. Veintinueve pacientes (74.4%) presentaron alguna comorbilidad. Como consecuencia de la aplicación de la VNI, el pH se incrementó entre la primera y tercera medición. El pH promedio inicial fue de 7.25 llegando a 7.33 a las 2 horas y a 7.39 al alta, en tanto que la pCO2 con promedio inicial de 83.8 mm Hg llegó a 67.8 mm Hg y 54.2 mm Hg en el mismo período. Treinta y cinco de los 39 pacientes fueron dados de alta con un período de internación promedio de 13.6 días. Cuatro pacientes (10.3%) fallecieron. Se concluye que con la aplicación de la VNI en pacientes con exacerbación de EPOC, el pH y la PaCO2 cambian significativamente en las muestras sucesivas, y que la adecuada capacitación del equipo de salud puede permitir el tratamiento de estos pacientes en áreas de menor complejidad. Deben ser tenidas en cuenta las posibles complicaciones que pueden sufrir los pacientes durante la internación, que pueden requerir la aplicación de ventilación invasiva.

This is a prospective study on the implementation of the non-invasive positive pressure ventilation (NPPV) to treat respiratory failure resulting from exacerbation of chronic obstructive pulmonary disease (COPD) in patients hospitalized in a Pneumological Unit. From January 2000 to January 2003, 39 patients were included during 54 different exacerbation events after being evaluated under international standards. They were classified as severe and very severe patients on the basis of their FEV1 values of 26%. Twenty nine patients presented co-morbidities. As a consequence of the NPPV treatment, the pH values increased between the first and last register as well as the pCO2 dropped in the same period. The initial mean pH values were 7.25 reaching mean values of 7.33 at 2 hours and 7.39 at the discharge; the corresponding pCO2 mean values were 83.8 mmHg, 67.8 mmHg and 54.2 mmHg. Thirty five patients out of 39 were discharged after a mean hospitalization length of 13.6 days. Four patients died. Apropriate training of health care staff in general facilities could allow the implementation of NPPV in addition to usual medical care to treat exacerbation of COPD. High morbidity situations could arise during hospitalization, so invasive ventilation must be necessary.

[Updated Argentine consensus on chronic obstructive pulmonary disease]. / Nuevo consenso Argentino de la enfermedad pulmonar obstructiva crónica.

Due to the increase in morbidity and mortality from Chronic Obstructive Pulmonary Disease (COPD), a group of chest physicians updates the basic knowledge on COPD since the last Consensus in 1994 in order to prepare guidelines for its diagnosis, prevention and treatment. The authors review the definition of COPD together with the most recent information on its pathophysiology. The clinical presentation is summarized together with functional evaluation and imaging. Early diagnosis by means of functional testing (i.e., spirometry) is stressed, emphasizing smoking cessation as the only measure that has been shown to alter the outcome of the disease. Smoking cessation strategies are described as well as pharmacological and non-pharmacological treatment. In the pharmacological section, the use of inhaled bronchodilators (anticholinergic, beta 2 adrenergic agonists or both) is considered the first option for treatment. Long acting bronchodilators improve patient compliance and treatment effectiveness. Inhaled corticosteroids are indicated for patients with severe airways obstruction and known response to corticosteroids. These agents can diminish the number of exacerbations. Annual influenza vaccination is recommended in all patients. In the non-pharmacological section, surgery for emphysema is suggested in particular cases. Rehabilitation, including exercise training, kinesitherapy and nutrition, is a useful tool for patients who are physically disabled. Long-term oxygen therapy at home improves survival in patients with severe chronic hypoxemia. Other therapeutic interventions such as non-invasive home ventilation have specific indications. Acute exacerbations must be aggressively treated with oxygen, corticosteroids, antibiotics, combination of bronchodilators, and eventually respiratory support. The role of public education of the patients and their families is essential in the prevention and treatment of the disease.

Nuevo consenso argentino de la enfermedad pulmonar obstructiva cronica / Update argentine consensus on chronic obstructive pulmonary disease

Debido ai aumento de Ia morbi mortalidad de Ia enfermedad pulmonar obstructiva crónica (EPOC), especialistas en medicina respiratoria actualizan los conocimientos básicos acerca de esta enfermedad desde el último Consenso Ilevado a cabo en 1994 para efectuar recomendaciones para su diagnóstico, prevención y tratamiento. Los autores revisan Ia definición de EPOC, conjuntamente con los últimos conocimìentos de Ia fisiopatología de Ia enfermedad. Se resume Ia presentación clínica, diagnóstico por imágenes y evaluación funcional. Se enfatiza ia importancia dei diagnóstico precoz mediante Ia exploración funcional y Ia prevención a través de Ia cesación dei tabaquismo, única medida capaz de alterar Ia evolución. Se describen Ias estrategias para dejar de fumar, así como el tratamiento farmacológico y no farmacológico. En el primero, Ia administración de broncodilatadores inhalados anticolinérgicos, beta 2 agonistas adrenérgicos o ambos son Ia primera opción. Los broncodilatadores de acción prolongada mejoran Ia adherencia ai tratamiento y su efectìvidad. Los corticoides inhalados se indican en pacientes con marcada obstrucción ai flujo aéreo y respuesta conocida a corticoides. Su uso disminuye Ia frecuencia de Ias exacerbaciones. La vacunación antigripal anual está recomendada en todos los pacientes. Con respecto a Ias intervenciones no farmacológicas, Ia cirugía deienfisema se recomienda en especiales circunstancias. La rehabilitación que incluya ejercicios, kinesioterapia y nutrición, es una herramienta útil en pacientes con limitación en Ia actividad física habitual. La oxigenoterapia domiciliaria prolongada mejora Ia sobrevida de pacientes con hipoxemia crónica severa. Otras intervenciones terapéuticas como Ia ventilación no invasiva domiciliaria tienen indicación muy específica. Las exacerbaciones agudas deben ser tratadas agresivamente con oxigeno, corticoides, antibióticos, asociación de broncodilatadores, y eventualmente asistencia respiratoria. El rol de Ia educación pública, dei paciente y su familia, es fundamen tal en Ia prevención y tratamiento de esta enfermedad.