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

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

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

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

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

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

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

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

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

Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

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