RESUMEN
INTRODUCCIÓN: La Fibrosis Pulmonar Idiopática (FPI) es una de las enfermedades respiratorias crónicas del adulto de mayor impacto y letalidad, diversos estudios epidemiológicos muestran tendencias progresivas al aumento de las tasas de mortalidad por FPI. En Chile no existen reportes sobre las tendencias de las tasas de mortalidad por FPI. El objetivo del presente estudio es determinar las tendencias de la mortalidad por FPI en Chile entre los años 2002 y 2015. MÉTODO: Estudio descriptivo de diseño ecológico, a partir de la información de bases de datos secundarias de libre disposición de las estadísticas vitales del Departamento de Estadísticas e Información de Salud (DEIS) y del Instituto Nacional de Estadísticas (INE) de Ministerio de Salud de Chile entre los años 2002 y 2015 se obtuvieron las tasas crudas de mortalidad por fibrosis pulmonar idiopática en población de 45 años y más en ambos sexos y las tasas ajustadas por sexo y edad por regiones, se calculó también la frecuencia mensual de las muertes por FPI y se compararon las tasas medias de mortalidad por regiones. RESULTADOS: Se observó un incremento progresivo de la tasa nacional cruda de mortalidad por FPI entre los años 2002 a 2015, la que fue de 18,5 fallecidos por 100.000 habitantes en el año 2002 hasta 24,6 fallecidos por 100.000 habitantes en el año 2015 con una pendiente de ascenso por año de +0,27 por 100.000 habitantes (p = 0,013). En las mujeres las tasas fueron más altas que en los hombres, pero las pendientes de ascenso no presentaron diferencias entre sexos. En la gran mayoría de las regiones las tasas ajustadas presentaron tendencias significativas al ascenso y las tasas medias más altas se presentaron en las regiones del norte de Chile. Se observó un comportamiento estacional de las muertes siendo las frecuencias más altas en los meses de invierno. CONCLUSIONES: Las tasas de mortalidad por FPI en Chile presentan una tendencia progresiva al aumento, con marcadas diferencias regionales lo que lleva a considerar, entre otros factores, influencia ambiental y contaminación del aire y de suelos que se debieran investigar para poder realizar intervenciones de salud pública que permitan reducir la mortalidad de esta enfermedad en nuestro país.
BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is one of the chronic respiratory diseases in adults with the greatest impact and high case fatality rate. Various epidemiological studies show progressive trends towards increasing IPF mortality rates. In Chile there are no national reports on country and regional trends in IPF mortality rates. The objective of this study is to determine trends in mortality due to IPF in Chile from year 2002 to 2015. METHOD: Epidemiological study of ecological design based on information from public databases of vital statistics of the Department of Health Statistics and Information (DEIS) and the National Institute of Statistics (INE) of the Ministry of Health of Chile. Crude mortality rates due to IPF in the population aged 45 years and over in both sexes were obtained from years 2002 to 2015. Besides the adjusted mortality rates for sex and age by region, the monthly frequency of IPF deaths during the same period and the average mortality rates by Chilean regions were calculated. RESULTS: A progressive increasing trend in the crude national IPF mortality rate was observed between years 2002 to 2015, which went from 18.5 deaths per 100,000 inhabitants in 2002 to 24.6 deaths per 100,000 inhabitants in 2015 with a slope of ascent per year of +0.27 per 100,000 inhabitants (p = 0.013); female rates were higher than men rates, but without differences in the slopes between sexes; in the vast majority of the regions the rates showed significant upward trends with the higher ones in the northern regions of Chile. A seasonal behavior of the death's frequency was observed being the highest in the winter term. CONCLUSIONS: Mortality rates due to IPF in Chile show a progressive upward trend, with marked regional differences which leads to consider, among other factors, environmental influence and air and soil contamination that should be investigated to carry out public health interventions that allow reducing the mortality of this disease in our country.
Asunto(s)
Masculino , Femenino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Fibrosis Pulmonar Idiopática/mortalidad , Estaciones del Año , Chile/epidemiología , Epidemiología Descriptiva , Estadísticas Vitales , Mortalidad/tendencias , Distribución por Sexo , Estudios EcológicosRESUMEN
Prediction models for survival at baseline evaluation have been proposed in idiopathic pulmonary fibrosis (IPF) but include diffusion capacity of the lung for carbon monoxide, a test not available in many places. The aim of the present study was to develop a simple new mortality risk scoring system for patients with IPF at initial evaluation without diffusion capacity of the lung for carbon monoxide measurement.A total of 173 patients, 72% males, mean age 70 years, 64% smokers/ex-smokers, were included in a retrospective study. The diagnosis was made by surgical lung biopsy in 40 (23%); in the remaining patients, a usual interstitial pneumonia pattern was present in high-resolution computed tomography. Patients with forced expiratory volume in 1 second/forced vital capacity ratio (FEV1/FVC) <0.70 were excluded. Dyspnea was evaluated by magnitude of task on the Mahler scale (Chest 1984). Peripheral oxygen saturation was measured by oximetry at rest and at the end of a 4âminutes step test or a 6-minute walk test.At the end of the follow-up period, 154 (89%) of the patients had died. Based on the univariate Cox proportional-hazards model, survival (P ≤ .10) was related directly to the dyspnea score, presence of cough, lower values of FVC% and FEV1%, lower rest and oxygen desaturation during exercise, and greater FEV1/FVC. By Cox multivariate analysis, the results remained correlated to the survival dyspnea score, FVC%, and exercise peripheral oxygen saturation. A score, using these variables, was developed and was able to discriminate among 3 groups, with high, low, and intermediate survival curves.A prognostic score, taking into account dyspnea, FVC%, and oxygen desaturation during exercise, can estimate survival in IPF.
Asunto(s)
Monóxido de Carbono/metabolismo , Fibrosis Pulmonar Idiopática/mortalidad , Pulmón/metabolismo , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Fibrosis Pulmonar Idiopática/metabolismo , Fibrosis Pulmonar Idiopática/fisiopatología , Pulmón/fisiopatología , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Pruebas de Función Respiratoria , Estudios Retrospectivos , Medición de RiesgoRESUMEN
INTRODUCTION: Chronic hypersensitivity pneumonitis (CHP) is an interstitial lung disease with limited treatment response and bad prognosis. Sometimes it is indistinguishable from idiopathic pulmonary fibrosis (IPF) becoming one of the main differential diagnosis. The aim of our study is to compare survival and functional decline between these two entities. METHODS: Survival and functional decline more than 10% in FVC were compared using Kaplan Meier (KM) method between patients with CHP and IPF. Cox proportional hazard analysis was used to identify independent predictors of survival and functional decline. RESULTS: 146 patients were included, 54 with CHP and 92 with IPF. KM rate for 2 years survival was 0.71 (CI 95% 0,6-0,8) for CHP group and 0,83 (CI 95% 0,66 - 0,92) for IPF (p=0,027). Nevertheless this difference disappeared using Cox proportional hazard analysis, the adjusted HR for survival among CHP patients was 0,53 (CI 95% 0,25-1,15) (p=0,11). There was no difference in functional evolution between the two groups. KM rate for a decline more than or equal to 10% was 0,64 for CHP (CI 95% 0,43-0,79) and 0,78 for IPF (IC 95% 0,6-0,88) (p=0,22). This observation did not change after using Cox proportional hazard analysis. CONCLUSIONS: Our study shows that both IPF and CHP are fibrosing interstitial diseases with a similar evolution and survival. It might be possible that therapeutic approach in patients with CHP should change in the light of these observations.
Asunto(s)
Alveolitis Alérgica Extrínseca/mortalidad , Fibrosis Pulmonar Idiopática/mortalidad , Anciano , Alveolitis Alérgica Extrínseca/diagnóstico , Argentina/epidemiología , Enfermedad Crónica , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Masculino , Persona de Mediana Edad , Análisis de Supervivencia , Tasa de Supervivencia/tendencias , Tomografía Computarizada por Rayos X/métodosRESUMEN
This study was conducted to evaluate whether a pulmonary rehabilitation program (PRP) is independently associated with survival in patients with idiopathic pulmonary fibrosis (IPF) undergoing lung transplant (LTx). This quasi-experimental study included 89 patients who underwent LTx due to IPF. Thirty-two completed all 36 sessions in a PRP while on the waiting list for LTx (PRP group), and 53 completed fewer than 36 sessions (controls). Survival after LTx was the main outcome; invasive mechanical ventilation (IMV), length of stay (LOS) in intensive care unit (ICU) and in hospital were secondary outcomes. Kaplan-Meier curves and Cox regression models were used in survival analyses. Cox regression models showed that the PRP group had a reduced 54.0% (hazard ratio = 0.464, 95% confidence interval 0.222-0.970, p = 0.041) risk of death. A lower number of patients in the PRP group required IMV for more than 24 hours after LTx (9.0% vs. 41.6% p = 0.001). This group also spent a mean of 5 days less in the ICU (p = 0.004) and 5 days less in hospital (p = 0.046). In conclusion, PRP PRP completion halved the risk of cumulative mortality in patients with IPF undergoing unilateral LTx.
Asunto(s)
Fibrosis Pulmonar Idiopática/rehabilitación , Fibrosis Pulmonar Idiopática/cirugía , Trasplante de Pulmón , Adulto , Anciano , Femenino , Humanos , Fibrosis Pulmonar Idiopática/mortalidad , Estimación de Kaplan-Meier , Trasplante de Pulmón/métodos , Masculino , Persona de Mediana Edad , Atención Perioperativa , Pronóstico , Recuperación de la Función , Rehabilitación/métodos , Respiración Artificial , Pruebas de Función Respiratoria , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To determine the cut-off point for the six-minute walk distance (6MWD) that indicates lower survival time in patients with idiopathic pulmonary fibrosis (IPF) in Brazil. METHODS: This was retrospective study carried out in two referral centers for IPF. The 6MWT was performed twice, considering the highest value of the 6MWD. Various cut-off points were estimated, in absolute values and in percentage of predicted values, using ROC curves, the Kaplan-Meier method, and data from other studies. RESULTS: The sample comprised 70 patients with IPF. The mean age was 71.9 ± 6.4 years, and 50 patients (71.4%) were male. The mean FVC was 76.6 ± 18.2% of predicted value. The mean SpO2 at rest before and after 6MWT were 93.8 ± 2.5% and 85.3 ± 6.5%, respectively. The median survival time was 44 months (95% CI: 37-51 months). The mean 6MWD was 381 ± 115 m (79.2 ± 24.0% of predicted). After the analyses, the best cut-off points for estimating survival were 6MWD < 330 m and < 70% of predicted. The median survival time of patients with a 6MWD < 330 m was 24 months (95% CI: 3-45 months), whereas that of those with a 6MWD ≥ 330 m was 59 months (95% CI: 41-77 months; p = 0.009). Similarly, the median survival times of those with a 6MWD < 70% and ≥ 70% of predicted, respectively, were 24 months (95% CI: 13-35 months) and 59 months (95% CI: 38-80 months; p = 0.013). Cox multivariate regression models including age, sex, smoking status, SpO2 at the end of the 6MWT, and FVC% showed that 6MWD remained significantly associated with survival (p = 0.003). CONCLUSIONS: Values of 6MWD < 330 m and < 70% of predicted value were associated with lower survival time in IPF patients in Brazil.
Asunto(s)
Prueba de Esfuerzo/métodos , Fibrosis Pulmonar Idiopática/mortalidad , Prueba de Paso , Brasil , Femenino , Humanos , Fibrosis Pulmonar Idiopática/prevención & control , Masculino , Modalidades de Fisioterapia , Curva ROC , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
ABSTRACT Objective: To determine the cut-off point for the six-minute walk distance (6MWD) that indicates lower survival time in patients with idiopathic pulmonary fibrosis (IPF) in Brazil. Methods: This was retrospective study carried out in two referral centers for IPF. The 6MWT was performed twice, considering the highest value of the 6MWD. Various cut-off points were estimated, in absolute values and in percentage of predicted values, using ROC curves, the Kaplan-Meier method, and data from other studies. Results: The sample comprised 70 patients with IPF. The mean age was 71.9 ± 6.4 years, and 50 patients (71.4%) were male. The mean FVC was 76.6 ± 18.2% of predicted value. The mean SpO2 at rest before and after 6MWT were 93.8 ± 2.5% and 85.3 ± 6.5%, respectively. The median survival time was 44 months (95% CI: 37-51 months). The mean 6MWD was 381 ± 115 m (79.2 ± 24.0% of predicted). After the analyses, the best cut-off points for estimating survival were 6MWD < 330 m and < 70% of predicted. The median survival time of patients with a 6MWD < 330 m was 24 months (95% CI: 3-45 months), whereas that of those with a 6MWD ≥ 330 m was 59 months (95% CI: 41-77 months; p = 0.009). Similarly, the median survival times of those with a 6MWD < 70% and ≥ 70% of predicted, respectively, were 24 months (95% CI: 13-35 months) and 59 months (95% CI: 38-80 months; p = 0.013). Cox multivariate regression models including age, sex, smoking status, SpO2 at the end of the 6MWT, and FVC% showed that 6MWD remained significantly associated with survival (p = 0.003). Conclusions: Values of 6MWD < 330 m and < 70% of predicted value were associated with lower survival time in IPF patients in Brazil.
RESUMO Objetivo: Determinar o ponto de corte da distância no teste de caminhada de seis minutos (DTC6) em relação à menor sobrevida em pacientes com fibrose pulmonar idiopática (FPI) no Brasil. Métodos: Estudo retrospectivo realizado em dois centros de referência para FPI. O TC6 foi realizado em duplicata, considerando-se o maior valor da DTC6. Vários pontos de corte foram estimados, em valores absolutos e em percentual do previsto, utilizando curvas ROC, método de Kaplan-Meier e dados de outros estudos. Resultados: A amostra envolveu 70 pacientes com FPI, com média de idade de 71,9 ± 6.4 anos, sendo 50 homens (71,4%). A média de CVF foi de 76,6 ± 18.2% do previsto. As médias de SpO2 em repouso antes e depois do TC6 foram de 93,8 ± 2,5% e 85,3 ± 6,5%, respectivamente. A mediana de sobrevida foi de 44 meses (IC95%: 37-51 meses). A média da DTC6 foi 381 ± 115 m (79,2 ± 24,0% do previsto). Após as análises, os melhores pontos de corte para estimar a sobrevida foram de DTC6 < 330 m e < 70% do previsto. A mediana de sobrevida foi de 24 meses (IC95%: 3-45 meses) para aqueles com DTC6 < 330 m comparada a 59 meses (IC95%: 41-77 meses) para aqueles com DTC6 ≥ 330 m (p = 0,009). Similarmente, a mediana de sobrevida foi de 24 meses (IC95%: 13-35 meses) para pacientes com DTC6 < 70% do previsto e de 59 meses (IC95%: 38-80 meses) para aqueles com DTC6 ≥ 70% do previsto (p = 0,013). Modelos de regressão multivariada de Cox incluindo idade, sexo, tabagismo, SpO2 ao final do TC6 e CVF% mostraram que a DTC6 permaneceu significativamente associada à sobrevida (p = 0,003). Conclusões: Valores de DTC6 < 330 m e < 70% do valor previsto associaram-se à menor sobrevida em pacientes com FPI no Brasil.
Asunto(s)
Humanos , Masculino , Femenino , Prueba de Esfuerzo/métodos , Fibrosis Pulmonar Idiopática/mortalidad , Prueba de Paso , Brasil , Tasa de Supervivencia , Estudios Retrospectivos , Curva ROC , Modalidades de Fisioterapia , Fibrosis Pulmonar Idiopática/prevención & controlRESUMEN
The predictive value of the decline in FVC by ≥10% on survival in patients with fibrotic hypersensitivity pneumonitis is unknown. Of 112 patients included, 66 (59%) had surgical lung biopsies. Patients with ≥10% decline in predicted FVC after 6-12 months had a significantly increased risk of all-cause mortality (median survival 53 months, 95% CI 37 to 69 vs 139 months, 95% CI 66 to 212 months, p=0.007). On multivariate analysis remained associated with increasing mortality: decline in FVC by ≥10% (HR 4.13, 95% CI 1.96 to 8.70, p=0.005), lower FVC% (HR 1.03, 95% CI 1.01 to 1.05, p=0.003) and with decreasing mortality improvement with antigen avoidance (HR 0.18, 95% CI 0.04 to 0.77, p=0.021).
Asunto(s)
Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/mortalidad , Tomografía Computarizada por Rayos X , Capacidad Vital , Adulto , Anciano , Lavado Broncoalveolar , Femenino , Estudios de Seguimiento , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodosRESUMEN
ABSTRACT Objective: To analyze mortality from idiopathic pulmonary fibrosis (IPF) in Brazil over the period 1979-2014. Methods: Microdata were extracted from the Brazilian National Ministry of Health Mortality Database. Only deaths for which the underlying cause was coded as International Classification of Diseases version 9 (ICD-9) 515 or 516.3 (until 1995) or as ICD version 10 (ICD-10) J84.1 (from 1996 onward) were included in our analysis. Standardized mortality rates were calculated for the 2010 Brazilian population. The annual trend in mortality rates was analyzed by joinpoint regression. We calculated risk ratios (RRs) by age group, time period of death, and gender, using a person-years denominator. Results: A total of 32,092 deaths were recorded in the study period. Standardized mortality rates trended upward, rising from 0.24/100,000 population in 1979 to 1.10/100,000 population in 2014. The annual upward trend in mortality rates had two inflection points, in 1992 and 2008, separating three distinct time segments with an annual growth of 2.2%, 6.8%, and 2.4%, respectively. The comparison of RRs for the age groups, using the 50- to 54-year age group as a reference, and for the study period, using 1979-1984 as a reference, were 16.14 (14.44-16.36) and 6.71 (6.34-7.12), respectively. Men compared with women had higher standardized mortality rates (per 100,000 person-years) in all age groups. Conclusion: Brazilian IPF mortality rates are lower than those of other countries, suggesting underdiagnosis or underreporting. The temporal trend is similar to those reported in the literature and is not explained solely by population aging.
RESUMO Objetivo: Analisar a mortalidade por fibrose pulmonar idiopática (FPI) no Brasil no período de 1979-2014. Métodos: Foram extraídos microdados do Sistema de Informações de Mortalidade do Ministério da Saúde cuja causa básica de óbito tenha sido codificada conforme a Classificação Internacional das Doenças, 9ª edição, códigos 515 ou 516.3 (até 1995), e 10ª versão, código J84.1 (a partir de 1996). Os coeficientes de mortalidade padronizados foram calculados para a população brasileira de 2010. A tendência anual da mortalidade foi analisada pelo método de regressão por pontos de inflexão. Foram calculadas as razões de risco (RR) por faixa etária, período analisado e gênero, utilizando-se como denominador pessoas-ano. Resultados: Foram registrados 32.092 óbitos no período estudado. O coeficiente de mortalidade padronizado mostrou-se ascendente, passando de 0,24/100.000 habitantes em 1979 para 1,10/100.000 em 2014. A tendência anual de crescimento identificou dois pontos de inflexão, em 1992 e 2008, gerando três retas com crescimento anual percentual de 2,2%, 6,8% e 2,4%, respectivamente. As RR (IC95%) por faixa etária, elegendo a faixa de 50-54 anos como referência, e por período estudado, elegendo o período de 1979-1984 como referência, foram de 16,14 (14,44-16,36) e de 6,71 (6,34-7,12), respectivamente. Homens, comparados a mulheres, apresentaram taxas de mortalidade padronizadas (por 100.000 pessoas-ano) superiores em todas as faixas etárias. Conclusões: Os coeficientes de mortalidade brasileiros por FPI são inferiores aos de outros países, podendo indicar subdiagnóstico ou subnotificação. A tendência temporal é semelhante à descrita na literatura e não é justificada apenas pelo envelhecimento populacional.
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Fibrosis Pulmonar Idiopática/mortalidad , Brasil/epidemiología , Factores Sexuales , Mortalidad/tendenciasRESUMEN
Idiopathic pulmonary fibrosis has poor prognosis and effective therapies are scarce. In the search for treatments that can modify the course of the disease, nintedanib (BIBF 1120), a tyrosine kinase inhibitor, has emerged as an alternative. However, its role is still unclear. To answer this question, we searched in Epistemonikos database, which is maintained by screening multiple sources of information. We identified seven systematic reviews including seven randomized trials overall. We extracted data, conducted a meta-analysis and generated a summary of findings table using the GRADE approach. We concluded nintedanib probably decreases the risk of acute exacerbations, and might reduce mortality in idiopathic pulmonary fibrosis. On the other hand, it is probably not associated with serious adverse events.
La fibrosis pulmonar idiopática es una enfermedad de mal pronóstico y cuyas terapias efectivas son escasas. En la búsqueda de tratamientos que puedan modificar el curso de la enfermedad ha surgido como una alternativa el nintedanib (BIBF 1120), un inhibidor de tirosina kinasa. Sin embargo, su rol aún no está claro. Para responder esta pregunta utilizamos la base de datos Epistemonikos, la cual es mantenida mediante búsquedas en múltiples fuentes de información. Identificamos siete revisiones sistemáticas que en conjunto incluyen siete estudios aleatorizados. Extrajimos los datos, realizamos un metanálisis y preparamos una tabla de resumen de los resultados utilizando el método GRADE. Concluimos que nintedanib probablemente disminuye el riesgo de exacerbaciones agudas, y podría reducir la mortalidad en la fibrosis pulmonar idiopática. Por otra parte, probablemente no se asocia a eventos adversos serios.
Asunto(s)
Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Indoles/uso terapéutico , Inhibidores de Proteínas Quinasas/uso terapéutico , Bases de Datos Factuales , Humanos , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/fisiopatología , Indoles/efectos adversos , Indoles/farmacología , Inhibidores de Proteínas Quinasas/efectos adversos , Inhibidores de Proteínas Quinasas/farmacología , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del TratamientoRESUMEN
Idiopathic pulmonary fibrosis has an ominous prognosis and there are virtually no effective therapies. It has been suggested that pirfenidone, an antifibrotic agent, could change its course. Searching in Epistemonikos database, which is maintained by screening multiple databases, we identified 13 systematic reviews comprising nine trials addressing the question of this article, seven of which are randomized and whose results were analyzed in this summary. We combined the evidence using meta-analysis and generated a summary of findings following the GRADE approach. We concluded pirfenidone decreases disease progression and mortality in idiopathic pulmonary fibrosis. Although it is associated with frequent gastrointestinal and cutaneous adverse effects, these are generally not severe.
Se ha planteado que la pirfenidona, un agente antifibrótico, podría cambiar el curso de la fibrosis pulmonar idiopática, una enfermedad de pronóstico ominoso y para la cual prácticamente no existen terapias efectivas. Utilizando la base de datos Epistemonikos, la cual es mantenida mediante búsquedas en múltiples bases de datos, identificamos trece revisiones sistemáticas que en conjunto incluyen nueve estudios primarios, siete de los cuales son aleatorizados y cuyos resultados se analizaron en este resumen. Extrajimos los datos, realizamos un metanálisis y preparamos tablas de resumen de los resultados utilizando el método GRADE. Concluimos que la pirfenidona disminuye la progresión de la enfermedad y la mortalidad en la fibrosis pulmonar idiopática. Si bien se asocia a efectos adversos gastrointestinales y cutáneos frecuentes, estos en general no son severos.
Asunto(s)
Antiinflamatorios no Esteroideos/uso terapéutico , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Piridonas/uso terapéutico , Antiinflamatorios no Esteroideos/efectos adversos , Antiinflamatorios no Esteroideos/farmacología , Progresión de la Enfermedad , Humanos , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/fisiopatología , Piridonas/efectos adversos , Piridonas/farmacología , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
OBJECTIVE: To analyze mortality from idiopathic pulmonary fibrosis (IPF) in Brazil over the period 1979-2014. METHODS: Microdata were extracted from the Brazilian National Ministry of Health Mortality Database. Only deaths for which the underlying cause was coded as International Classification of Diseases version 9 (ICD-9) 515 or 516.3 (until 1995) or as ICD version 10 (ICD-10) J84.1 (from 1996 onward) were included in our analysis. Standardized mortality rates were calculated for the 2010 Brazilian population. The annual trend in mortality rates was analyzed by joinpoint regression. We calculated risk ratios (RRs) by age group, time period of death, and gender, using a person-years denominator. RESULTS: A total of 32,092 deaths were recorded in the study period. Standardized mortality rates trended upward, rising from 0.24/100,000 population in 1979 to 1.10/100,000 population in 2014. The annual upward trend in mortality rates had two inflection points, in 1992 and 2008, separating three distinct time segments with an annual growth of 2.2%, 6.8%, and 2.4%, respectively. The comparison of RRs for the age groups, using the 50- to 54-year age group as a reference, and for the study period, using 1979-1984 as a reference, were 16.14 (14.44-16.36) and 6.71 (6.34-7.12), respectively. Men compared with women had higher standardized mortality rates (per 100,000 person-years) in all age groups. CONCLUSION: Brazilian IPF mortality rates are lower than those of other countries, suggesting underdiagnosis or underreporting. The temporal trend is similar to those reported in the literature and is not explained solely by population aging.
Asunto(s)
Fibrosis Pulmonar Idiopática/mortalidad , Anciano , Anciano de 80 o más Años , Brasil/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mortalidad/tendencias , Factores SexualesRESUMEN
OBJECTIVE: To evaluate the changes in lung function in the first year after single lung transplantation in patients with idiopathic pulmonary fibrosis (IPF). METHODS: We retrospectively evaluated patients with IPF who underwent single lung transplantation between January of 2006 and December of 2012, reviewing the changes in the lung function occurring during the first year after the procedure. RESULTS: Of the 218 patients undergoing lung transplantation during the study period, 79 (36.2%) had IPF. Of those 79 patients, 24 (30%) died, and 11 (14%) did not undergo spirometry at the end of the first year. Of the 44 patients included in the study, 29 (66%) were men. The mean age of the patients was 57 years. Before transplantation, mean FVC, FEV1, and FEV1/FVC ratio were 1.78 L (50% of predicted), 1.48 L (52% of predicted), and 83%, respectively. In the first month after transplantation, there was a mean increase of 12% in FVC (400 mL) and FEV1 (350 mL). In the third month after transplantation, there were additional increases, of 5% (170 mL) in FVC and 1% (50 mL) in FEV1. At the end of the first year, the functional improvement persisted, with a mean gain of 19% (620 mL) in FVC and 16% (430 mL) in FEV1. CONCLUSIONS: Single lung transplantation in IPF patients who survive for at least one year provides significant and progressive benefits in lung function during the first year. This procedure is an important therapeutic alternative in the management of IPF.
Asunto(s)
Fibrosis Pulmonar Idiopática/fisiopatología , Fibrosis Pulmonar Idiopática/cirugía , Trasplante de Pulmón , Adulto , Anciano , Femenino , Volumen Espiratorio Forzado/fisiología , Humanos , Fibrosis Pulmonar Idiopática/mortalidad , Trasplante de Pulmón/mortalidad , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Capacidad Vital/fisiologíaRESUMEN
AbstractObjective: To evaluate the changes in lung function in the first year after single lung transplantation in patients with idiopathic pulmonary fibrosis (IPF).Methods: We retrospectively evaluated patients with IPF who underwent single lung transplantation between January of 2006 and December of 2012, reviewing the changes in the lung function occurring during the first year after the procedure.Results: Of the 218 patients undergoing lung transplantation during the study period, 79 (36.2%) had IPF. Of those 79 patients, 24 (30%) died, and 11 (14%) did not undergo spirometry at the end of the first year. Of the 44 patients included in the study, 29 (66%) were men. The mean age of the patients was 57 years. Before transplantation, mean FVC, FEV1, and FEV1/FVC ratio were 1.78 L (50% of predicted), 1.48 L (52% of predicted), and 83%, respectively. In the first month after transplantation, there was a mean increase of 12% in FVC (400 mL) and FEV1 (350 mL). In the third month after transplantation, there were additional increases, of 5% (170 mL) in FVC and 1% (50 mL) in FEV1. At the end of the first year, the functional improvement persisted, with a mean gain of 19% (620 mL) in FVC and 16% (430 mL) in FEV1.Conclusions: Single lung transplantation in IPF patients who survive for at least one year provides significant and progressive benefits in lung function during the first year. This procedure is an important therapeutic alternative in the management of IPF.
ResumoObjetivo: Avaliar as alterações de função pulmonar no primeiro ano após transplante de pulmão unilateral em pacientes portadores de fibrose pulmonar idiopática (FPI).Métodos: Foi avaliada retrospectivamente a variação da função pulmonar de portadores de FPI submetidos a transplante pulmonar unilateral entre janeiro de 2006 e dezembro de 2012 no decorrer do primeiro ano após o procedimento.Resultados: Dos 218 pacientes submetidos a transplante pulmonar durante o período do estudo, 79 (36,2%) eram portadores de FPI. Desses 79 pacientes, 24 (30%) foram a óbito e 11 (14%) não realizaram espirometria ao final do primeiro ano. Dos 44 pacientes incluídos no estudo, 29 (66%) eram homens. A média de idade dos pacientes foi de 57 anos.Antes do transplante, as médias de CVF, VEF1 e relação VEF1/CVF foram de 1,78 l (50% do previsto), 1,48 l (52% do previsto) e 83%, respectivamente. No primeiro mês após o transplante, houve um aumento médio de 12% tanto na CVF (400 ml) como no VEF1 (350 ml). No terceiro mês após o transplante, houve um aumento adicional médio de 5% (170 ml) na CVF e de 1% (50 ml) no VEF1. Ao final do primeiro ano, a melhora funcional foi persistente, com um ganho médio de 19% (620 ml) na CVF e de 16% (430 ml) no VEF1.Conclusões: O transplante pulmonar unilateral em portadores de FPI que sobrevivam por pelo menos um ano proporciona importante e progressivo benefício na sua função pulmonar no decorrer do primeiro ano. Este procedimento é uma importante alternativa terapêutica no manejo da FPI.
Asunto(s)
Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fibrosis Pulmonar Idiopática/fisiopatología , Fibrosis Pulmonar Idiopática/cirugía , Trasplante de Pulmón , Volumen Espiratorio Forzado/fisiología , Fibrosis Pulmonar Idiopática/mortalidad , Trasplante de Pulmón/mortalidad , Pruebas de Función Respiratoria , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Capacidad Vital/fisiologíaRESUMEN
La FPI predomina en el sexo masculino, en edades avanzadas, con tos y/o disnea progresivas. Un 5% se presenta como una forma familiar. La tomografía axial computarizada de tórax, fundamental en el diagnóstico, en al menos un 50% hace innecesaria la biopsia. El diagnóstico es conjunto con clínicos, radiólogos y patólogos. La sobrevivencia media es de tres a cinco años desde el diagnóstico. La historia natural es un deterioro progresivo, pero hay formas rápidas y también pueden aparecer exacerbaciones que ensombrecen el pronóstico. Diversas comorbilidades se han descrito como la hipertensión pulmonar, la asociación con enfisema y el reflujo gastroesofágico. Sólo recientemente aparecen fármacos útiles, que son la Pirfenidona y el Nintedanib. El clásico esquema de prednisona, azatriopina y N-acetil cisteina, se ha demostrado ineficaz. Otros recursos que pueden utilizarse como complementos útiles en la enfermedad son el oxígeno, la rehabilitación, las terapias antirreflujo y el manejo sintomático de la tos.
PF appears mainly in aged males, with progressive cough and dyspnea. In 5% of the cases the disease presents as a familial form. CT scan is key in diagnosis of the disease. In no less than 50% biopsy is unnecessary but diagnosis must be made in conjunction with clinician, radiologist and pathologist. Median survival is 3 to 5 years from diagnosis. Natural history is a progressive deterioration but there are fast evolution cases and exacerbation of the disease that make worse the prognosis. Pulmonary hypertension, the association with emphysema and gastroesophageal reflux has been described as comorbidities of the disease. Last year has been published the positive results of therapeuticall trials with two new drugs, Pirfenidone and Nintedanib. The classical regime for IPF with Prednisone, Azathriopine and Acetylcysteine has been showed as useless. Oxygen, Pulmonary rehabilitation, gastroesophageal reflux and cough management are complementary treatment for the disease.
Asunto(s)
Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Biopsia , Biomarcadores , Tomografía Computarizada por Rayos X , Comorbilidad , Evolución Clínica , Tasa de Supervivencia , Factores de Riesgo , Fibrosis Pulmonar Idiopática/genética , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/tratamiento farmacológicoRESUMEN
BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is the most prevalent of all interstitial lung diseases. The usual underlying pathological picture is an interstitial pneumonia (UIP). AIM: To describe the evolution of a Chilean cohort of patients with IPF. MATERIAL AND METHODS: Patients with the disease were identified at the pathology registry of National Institute of Thoracic Diseases, Santiago, Chile. Patients were included if they had surgical biopsy of UIP and compatible clinical and radiological characteristics. The medical records of included patients were reviewed, recording clinical information and lung function test results. Survival was analyzed obtaining death records from the Chilean National Identification Service. RESULTS: Data from 142 patients with a mean age of 58 years (42% men), were analyzed. Mean initial lung function showed a forced vital capacity (FVC) of 73%, carbon monoxide diffusing capacity (DLCO) of 57% and a distance covered in 6-minute walk (6MWT) of 95% of expected normal values. The median survival was 80 months. Predictors of survival were a DLCO of less than 40% and an oxygen saturation at the end of the 6MWT of less than 89%. CONCLUSIONS: Survival in this group of patients was higher than the figures reported elsewhere. DLCO and the fall of oxygen saturation after walking were predictors of mortality, as previously described in other populations.
Asunto(s)
Fibrosis Pulmonar Idiopática/mortalidad , Biopsia , Estudios de Cohortes , Femenino , Humanos , Fibrosis Pulmonar Idiopática/patología , Pulmón/patología , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
BACKGROUND: The usual interstitial pneumonia (UIP) pattern of lung injury may occur in the setting of connective tissue disease (CTD), but it is most commonly found in the absence of a known cause, in the clinical context of idiopathic pulmonary fibrosis (IPF). Our objective was to observe and compare longitudinal changes in pulmonary function and survival between patients with biopsy-proven UIP found in the clinical context of either CTD or IPF. METHODS: We used longitudinal data analytic models to compare groups (IPF [n = 321] and CTD-UIP [n = 56]) on % predicted FVC (FVC %) or % predicted diffusing capacity of the lung for carbon monoxide (Dlco %), and we used both unadjusted and multivariable techniques to compare survival between these groups. RESULTS: There were no significant differences between groups in longitudinal changes in FVC % or Dlco % up to diagnosis, or from diagnosis to 10 years beyond (over which time, the mean decrease in FVC % per year [95% CI] was 4.1 [3.4, 4.9] for IPF and 3.5 [1.8, 5.1] for CTD-UIP, P = .49 for difference; and the mean decrease in Dlco % per year was 4.7 [4.0, 5.3] for IPF and 4.3 [3.0, 5.6] for CTD-UIP, P = .60 for difference). Despite the lack of differences in pulmonary function, subjects with IPF had worse survival in unadjusted (log-rank P = .003) and certain multivariable analyses. CONCLUSIONS: Despite no significant differences in changes in pulmonary function over time, patients with CTD-UIP (at least those with certain classifiable CTDs) live longer than patients with IPF--an observation that we suspect is due to an increased rate of mortal acute exacerbations in patients with IPF.
Asunto(s)
Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/fisiopatología , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/fisiopatología , Pulmón/fisiopatología , Anciano , Biopsia , Femenino , Humanos , Estimación de Kaplan-Meier , Estudios Longitudinales , Pulmón/patología , Masculino , Persona de Mediana Edad , Análisis Multivariante , Capacidad de Difusión Pulmonar/fisiología , Pruebas de Función Respiratoria , Tasa de Supervivencia , Capacidad Vital/fisiologíaRESUMEN
Background: Idiopathic Pulmonary Fibrosis (IPF) is the most prevalent of all interstitial lung diseases. The usual underlying pathological picture is an interstitial pneumonia (UIP). Aim: To describe the evolution of a Chilean cohort of patients with IPF. Material and Methods: Patients with the disease were identified at the pathology registry of National Institute of Thoracic Diseases, Santiago, Chile. Patients were included if they had surgical biopsy of UIP and compatible clinical and radiological characteristics. The medical records of included patients were reviewed, recording clinical information and lung function test results. Survival was analyzed obtaining death records from the Chilean National Identification Service. Results: Data from 142 patients with a mean age of 58 years (42% men), were analyzed. Mean initial lung function showed a forced vital capacity (FVC) of 73%, carbon monoxide diffusing capacity (DLCO) of 57% and a distance covered in 6-minute walk (6MWT) of 95% of expected normal values. The median survival was 80 months. Predictors of survival were a DLCO of less than 40% and an oxygen saturation at the end of the 6MWT of less than 89%. Conclusions: Survival in this group of patients was higher than the figures reported elsewhere. DLCO and the fall of oxygen saturation after walking were predictors of mortality, as previously described in other populations.
Asunto(s)
Femenino , Humanos , Masculino , Persona de Mediana Edad , Fibrosis Pulmonar Idiopática/mortalidad , Biopsia , Estudios de Cohortes , Fibrosis Pulmonar Idiopática/patología , Pulmón/patología , Pruebas de Función Respiratoria , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
OBJECTIVE: To study the expression of COX-1 and COX-2 in the remodeled lung in systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF) patients, correlating that expression with patient survival. METHODS: We examined open lung biopsy specimens from 24 SSc patients and 30 IPF patients, using normal lung tissue as a control. The histological patterns included fibrotic nonspecific interstitial pneumonia (NSIP) in SSc patients and usual interstitial pneumonia (UIP) in IPF patients. We used immunohistochemistry and histomorphometry to evaluate the expression of COX-1 and COX-2 in alveolar septa, vessels, and bronchioles. We then correlated that expression with pulmonary function test results and evaluated its impact on patient survival. RESULTS: The expression of COX-1 and COX-2 in alveolar septa was significantly higher in IPF-UIP and SSc-NSIP lung tissue than in the control tissue. No difference was found between IPF-UIP and SSc-NSIP tissue regarding COX-1 and COX-2 expression. Multivariate analysis based on the Cox regression model showed that the factors associated with a low risk of death were younger age, high DLCO/alveolar volume, IPF, and high COX-1 expression in alveolar septa, whereas those associated with a high risk of death were advanced age, low DLCO/alveolar volume, SSc (with NSIP), and low COX-1 expression in alveolar septa. CONCLUSIONS: Our findings suggest that strategies aimed at preventing low COX-1 synthesis will have a greater impact on SSc, whereas those aimed at preventing high COX-2 synthesis will have a greater impact on IPF. However, prospective randomized clinical trials are needed in order to confirm that. .
OBJETIVO: Estudar a expressão de COX-1 e COX-2 em áreas pulmonares remodeladas em pacientes com esclerose sistêmica (ES) ou fibrose pulmonar idiopática (FPI) e correlacioná-la com a sobrevida desses pacientes. MÉTODOS: Examinamos espécimes de biópsia pulmonar a céu aberto de 24 pacientes com ES e de 30 pacientes com FPI, utilizando-se tecido pulmonar normal como controle. Os padrões histológicos incluíram pneumonia intersticial não específica (PINE) fibrótica em pacientes com ES e pneumonia intersticial usual (PIU) nos pacientes com FPI. Imuno-histoquímica e histomorfometria foram usadas para avaliar a expressão celular de COX-1 e COX-2 em septos alveolares, vasos e bronquíolos, sua correlação com provas de função pulmonar e seu impacto na sobrevida. RESULTADOS: A expressão de COX-1 e COX-2 em septos alveolares foi significativamente maior em FPI-PIU e ES-PINE do que no tecido controle. Não houve diferença entre FPI-PIU e ES-PINE quanto à expressão de COX-1 e COX-2. A análise multivariada baseada no modelo de regressão de Cox mostrou que os fatores associados a baixo risco de morte foram ter idade menor, valores elevados de DLCO/volume alveolar, FPI, e alta expressão de COX-1 em septos alveolares, ao passo que os fatores associados a alto risco de morte foram ter idade maior, valores baixos de DLCO/volume alveolar, ES (com PINE) e baixa expressão de COX-1 em septos alveolares. CONCLUSÕES: Nossos resultados sugerem que estratégias de prevenção de baixa síntese de COX-1 terão maior impacto sobre a ES, ao passo que as de prevenção de alta síntese de COX-2 terão maior impacto sobre a FPI. Porém, são necessários ensaios clínicos randomizados prospectivos para confirmar essa hipótese. .
Asunto(s)
Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Remodelación de las Vías Aéreas (Respiratorias) , Ciclooxigenasa 1/análisis , /análisis , Fibrosis Pulmonar Idiopática/metabolismo , Esclerodermia Sistémica/metabolismo , Factores de Edad , Biopsia , Estudios de Seguimiento , Inmunohistoquímica , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/patología , Pulmón/patología , Análisis Multivariante , Alveolos Pulmonares/fisiopatología , Pruebas de Función Respiratoria , Tasa de Supervivencia , Esclerodermia Sistémica/mortalidad , Esclerodermia Sistémica/patologíaRESUMEN
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation. METHODS: We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania. RESULTS: Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome. CONCLUSIONS: Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation.
Asunto(s)
Ecocardiografía Doppler , Hemodinámica , Fibrosis Pulmonar Idiopática/mortalidad , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Trasplante de Pulmón , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Factores de Riesgo , Resistencia VascularRESUMEN
OBJECTIVE: To study the expression of COX-1 and COX-2 in the remodeled lung in systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF) patients, correlating that expression with patient survival. METHODS: We examined open lung biopsy specimens from 24 SSc patients and 30 IPF patients, using normal lung tissue as a control. The histological patterns included fibrotic nonspecific interstitial pneumonia (NSIP) in SSc patients and usual interstitial pneumonia (UIP) in IPF patients. We used immunohistochemistry and histomorphometry to evaluate the expression of COX-1 and COX-2 in alveolar septa, vessels, and bronchioles. We then correlated that expression with pulmonary function test results and evaluated its impact on patient survival. RESULTS: The expression of COX-1 and COX-2 in alveolar septa was significantly higher in IPF-UIP and SSc-NSIP lung tissue than in the control tissue. No difference was found between IPF-UIP and SSc-NSIP tissue regarding COX-1 and COX-2 expression. Multivariate analysis based on the Cox regression model showed that the factors associated with a low risk of death were younger age, high DLCO/alveolar volume, IPF, and high COX-1 expression in alveolar septa, whereas those associated with a high risk of death were advanced age, low DLCO/alveolar volume, SSc (with NSIP), and low COX-1 expression in alveolar septa. CONCLUSIONS: Our findings suggest that strategies aimed at preventing low COX-1 synthesis will have a greater impact on SSc, whereas those aimed at preventing high COX-2 synthesis will have a greater impact on IPF. However, prospective randomized clinical trials are needed in order to confirm that.