Eficacia y tolerabilidad Resultados del tratamiento para la fibrosis pulmonar idiopática. Experiencia de la vida real / Efficacy and Tolerability Outcomes of Treatment for Idiopathic Pulmonary Fibrosis. Real-life Experience

Introducción: la fibrosis pulmonar idiopática (FPI) es una enfermedad progresiva y cró-nica con muy mal pronóstico. Actualmente, existen dos fármacos para esta patología. El propósito de nuestro estudio es evaluar los efectos del tratamiento en los pacientes de una consulta en vida real.

Introduction: idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease with a very poor prognosis. Two drugs are currently available for this disease. The purpo-se of our study is to evaluate the effects of treatment in patients in a real-life practice.

Potential biomarkers for diagnosis and disease evaluation of idiopathic pulmonary fibrosis / 中华医学杂志(英文版)

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterized by progressive lung fibrogenesis and histological features of usual interstitial pneumonia. IPF has a poor prognosis and presents a spectrum of disease courses ranging from slow evolving disease to rapid deterioration; thus, a differential diagnosis remains challenging. Several biomarkers have been identified to achieve a differential diagnosis; however, comprehensive reviews are lacking. This review summarizes over 100 biomarkers which can be divided into six categories according to their functions: differentially expressed biomarkers in the IPF compared to healthy controls; biomarkers distinguishing IPF from other types of interstitial lung disease; biomarkers differentiating acute exacerbation of IPF from stable disease; biomarkers predicting disease progression; biomarkers related to disease severity; and biomarkers related to treatment. Specimen used for the diagnosis of IPF included serum, bronchoalveolar lavage fluid, lung tissue, and sputum. IPF-specific biomarkers are of great clinical value for the differential diagnosis of IPF. Currently, the physiological measurements used to evaluate the occurrence of acute exacerbation, disease progression, and disease severity have limitations. Combining physiological measurements with biomarkers may increase the accuracy and sensitivity of diagnosis and disease evaluation of IPF. Most biomarkers described in this review are not routinely used in clinical practice. Future large-scale multicenter studies are required to design and validate suitable biomarker panels that have diagnostic utility for IPF.

Seguimiento de 20 pacientes portadores de fibrosis pulmonar idiopática tratados con nintedanib / Follow-up of 20 patients with idiopathic pulmonary fibrosis treated with nintedanib

Se presenta un estudio observacional compasivo de seguimiento de 20 pacientes portadores de Fibrosis Pulmonar Idiopática tratados con Nintedanib, que muestra que Nintedanib es un medicamento en general bien tolerado, sin efectos adversos serios, que otorga una sobrevida más prolongada que la que cabría esperar en pacientes con esta enfermedad.

A compassionate observational follow-up study of 20 patients with Idiopathic Pulmonary Fibrosis treated with Nintedanib is presented, showing that Nintedanib is a generally well-tolerated drug, with no serious adverse effects, that grants a longer survival in real-life patients.

Prevalencia y letalidad de enfermedades pulmonares intersticiales en la Región de Los Ríos, Chile / Prevalence and lethality of interstitial pulmonary diseases in the Region of Los Ríos, Chile

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is a subtype of interstitial lung disease (ILD) that significantly affects people's quality of life, whose prevalence and mortality has increased. AIM: To determine the prevalence and lethality of ILD in the Los Ríos Region between the years 2018 and 2019. MATERIAL AND METHODS: Review of a database of all patients attended at an outpatient clinic of the regional hospital. Those patients with the diagnosis of ILD and seen between 2018 and 2019 were selected. Mortality and its causes were verified with death certificates. RESULTS: In the study period, 339 cases with ILD, aged 71 ± 10 years (64% women) were identified. The calculated ILD prevalence was 84 cases per 100,000 inhabitants, with a higher predominancy in Futrono and Paillaco communes. IPF and Connective Tissue Diseases were the predominant subtypes. Overall lethality was 18%, with more deaths among patients with IPF (n = 31). CONCLUSIONS: In the Los Ríos Region, ILDs have a relevant prevalence and lethality.

Protocolo Clínico e Diretrizes Terapêuticas de diagnóstico e tratamento da Fibrose Pulmonar Idiopática (FPI) no Estado de Goiás ­ versão 2022 / Clinical Protocol and Therapeutic Guidelines for the diagnosis and treatment of Idiopathic Pulmonary Fibrosis (IPF) in the State of Goiás ­ version 2022

A fibrose pulmonar idiopática (FPI) é uma forma específica de pneumonia intersticial idiopática crônica, fibrosante e de caráter progressivo. Ela ocorre primariamente em adultos idosos, predominantemente nas sexta e sétima décadas, além de ser restrita aos pulmões. O padrão histológico e/ ou radiológico associado à FPI é o de pneumonia intersticial usual (PIU). Uma vez confirmado o padrão histológico de PIU associado à FPI, se estabelece um prognóstico significativamente pior do que o observado em outras pneumonias intersticiais crônicas. Daí a necessidade do estabelecimento de diagnósticos acurados de FPI, o que, sem dúvida, é um processo desafiador (5).

Successful Rescue of Acute Exacerbation of Idiopathic Pulmonary Fibrosis after Surgery for Lung Cancer: Case Report / 中国肺癌杂志

Idiopathic Pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease with unknown cause, which is closely related to lung cancer. A serious complication called Acute exacerbation of IPF (AE-IPF) is prone to occur after lung resection. It progresses rapidly without effective treatment and has a poor prognosis. A typical case of AE-IPF after lung cancer surgery was reported, and its clinical characteristics, imaging features, diagnosis and treatment were summarized.
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Efficacy of Qingfei oral liquid for idiopathic pulmonary fibrosis in rats and related network pharmacology study / 浙江大学学报·医学版

To investigate the therapeutic effect and mechanism of Qingfei oral liquid in idiopathic pulmonary fibrosis. Seventy-two male SD rats were divided into control group, model group, pirofenidone group and Qingfei group with 18 animals in each group. The idiopathic pulmonary fibrosis was induced in last three groups by intratracheal injection of bleomycin; pirofenidone group was given oral administration of pirofenidone b.i.d for 21 d, and Qingfei group was given Qingfei oral liquid 3.6 mL/kg q.d for Lung tissues were obtained for HE staining, Masson staining and transforming growth factor (TGF)-β immunohistochemical staining. Superoxide dismutase (SOD), malondialdehyde (MDA) and glutathione (GSH) were detected in tissue homogenates. The BATMAN-TCM database was used to retrieve the chemical components and their corresponding targets of Qingfei oral solution by network pharmacology method, and then the component-target-disease network diagram was constructed. Finally, the pathway enrichment analysis was carried out to explore the molecular mechanism of Qingfei oral liquid against idiopathic fibrosis. Histopathology results showed that Qingfei oral liquid had a similar relieving effect on pulmonary fibrosis as the positive drug pirfenidone; TGF-β secretion had a significant reduction in lung tissues of Qingfei group; and Qingfei oral liquid had better regulatory effect on SOD, MDA and GSH than pirfenidone. The results of component-target-disease network and pathway enrichment analysis showed that the related molecular pathways were concentrated in inflammation, extracellular matrix and cytokines. Qingfei oral liquid has a good therapeutic effect on idiopathic pulmonary fibrosis in rats via regulation of inflammation, extracellular matrix and cytokines.

Tendencia de la mortalidad por fibrosis pulmonar idiopática en Chile / Trend in idiopathic pulmonary fibrosis in Chile

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.

Correlaciones entre el test de velocidad en 4 metros y el test de caminata en 6 min en enfermos respiratorios crónicos / Correlations between the 4 meter speed test and the 6-minute walking test in chronic respiratory patients

Resumen La prueba de velocidad de marcha en 4 metros (T4M) es considerada sustituto de la caminata en 6 min (TC6M) en EPOC. Sin embargo, no ha sido bien investigada en otras enfermedades respiratorias. Durante un año estudiamos pacientes que concurrieron a nuestra Unidad para realizar el TC6M midiendo la velocidad alcanzada en 4 metros, 2 h previo a realizar el TC6M. De 162 pacientes 99 eran mujeres. La edad media fue de 65 años, peso de 73 kg, talla de 158 cm, IMC 29,4 kg/m2. 36% tenían fibrosis pulmonar idiopática, 17% EPOC, GOLD IV,11% EPOC, GOLD III, 12% apnea de sueño y 12% otras enfermedades. No hubo diferencia para los distintos diagnósticos en ambos tests. La distancia media en TC6Mfue 368,5 m la velocidad: 1,01 m/s en T4M.Hubo una correlación positiva significativa entre ambos test: alto rendimiento en T4M es equivalente a un alto rendimiento en el TC6M. Hubo correlación negativa con la edad y positiva con la estatura. Al año de seguimiento 16 pacientes habían fallecido, siendo estos los que habían obtenido los más bajos rendimientos en ambos tests (T4M: 0,69 m/s y 248,1 m en TC6M) La posibilidad de sobrevida cayó a 20% en aquellos individuos que alcanzaron una velocidad inferior a 0,69 m/s. Es posible sustituir el TC6M por el T4M en pacientes con diferentes patologías respiratoria, podemos predecir la muerte por cualquier causa si un sujeto camina a una velocidad ≤ 0,69 m/s, T4M es barato y fácil de realizar en atención primaria, sirviendo como evaluación de riesgo para referir a un centro más complejo.

4-Meter Gait Speed Test (4MGST) a frailty test, is considered a surrogate for the 6-Minute Walk Test (6MWT) in COPD. However, it has not been investigated in other respiratory conditions. Over a year, we studied patients attending our Unit for evaluation with 6MWT, measuring the speed they achieved walking 4 m, 2 h before performing 6MWT. 162 patients (99 women) were studied; series' mean values were: age 65 years-old; body weight, 73 kg; height, 158 cm and BMI, 29.4 kg/m2. 36% of them had idiopathic pulmonary fibrosis, 17% GOLD IV COPD, 11% GOLD III COPD, 12% pulmonary arterial hypertension, 12% obstructive apnea-hypopnea syndrome, and 12% other conditions. ANOVA showed no difference between diagnostic categories for both test. Average score in 6MWT was 368.5 m and 1.01 m/s in 4MGST. Pearson correlation coefficient revealed significant positive correlation between results of both tests: high score in 4MGST is equivalent to high score in 6MWT. There was negative correlation with age and positive correlation with height. At one year follow-up, 16 patients had died. They obtained significantly lower scores in both tests (4MGST: 0.69 m/s and 6MWT: 248.1 m) Survival chance fell to 20% for patients walking slower than 0.69 m/s. It is possible to replace TC6M with T4M in patients with different respiratory pathologies, we can predict death for any cause if a subject walked at ≤ speed at 0.69 m/s. T4M is cheap and easy to perform in primary care, serving as a risk assessment to refer to a more complex center.

Importance of chest HRCT in the diagnostic evaluation of fibrosing interstitial lung diseases / Importância da TCAR de tórax na avaliação de pneumopatias intersticiais fibrosantes

ABSTRACT Many conditions result in chronic interstitial lung disease (ILD), being classified as fibrosing ILDs, including idiopathic pulmonary fibrosis, connective tissue diseases, sarcoidosis, and fibrotic hypersensitivity pneumonitis. HRCT plays an important role in the clinical evaluation of fibrosing ILDs. Current treatment perspectives are encouraging and reinforce the need for HRCT scans of adequate technical quality for early detection of fibrosing ILD. Despite efforts in this regard, the significance and management of imaging findings of early interstitial lung abnormalities have yet to be clarified. After identification of CT findings consistent with fibrosing ILD, radiologists must be able to identify characteristic morphological patterns and, in some cases, features of specific clinical entities. In cases in which HRCT features are not sufficiently specific for a definitive diagnosis, HRCT can aid in selecting the best site for surgical lung biopsy. CT follow-up is useful for identifying progressive fibrosing ILDs and detecting complications unrelated to the underlying disease, including infections, acute exacerbations, and neoplasms. Automated quantification tools have clinical applicability and are likely to be available for use in imaging analysis in the near future. In addition, incorporation of CT evaluation into scoring systems based on clinical and functional parameters for staging fibrosing disease is likely to become valuable in determining prognosis. Knowledge of the clinical applications of CT evaluation is essential for specialists managing patients with fibrosing ILD and can have a positive impact on the clinical course of the disease.

RESUMO Inúmeras doenças determinam dano intersticial crônico no parênquima pulmonar e são agrupadas com a denominação de pneumopatias intersticiais fibrosantes, incluindo fibrose pulmonar idiopática, doenças do colágeno, sarcoidose, pneumonite por hipersensibilidade fibrótica etc. Entre os métodos complementares à avaliação clínica, a TCAR tem um papel relevante. Perspectivas atuais de tratamento são encorajadoras e reforçam a necessidade de realização de estudos com técnica adequada, visando a detecção confiável de acometimento intersticial fibrosante o mais precocemente possível. Embora esforços tenham sido direcionados nesse sentido, o significado e manejo de anormalidades pulmonares intersticiais incipientes, detectadas nos estudos de imagem, ainda não são claros. Uma vez detectado o acometimento fibrosante, é importante que o radiologista conheça aspectos característicos de determinados padrões morfológicos e reconheça elementos que possam apontar para entidades clínicas específicas. Em casos nos quais a especificidade dos achados não é suficiente para a suspeição diagnóstica, as imagens de TC servem de guia para a escolha de sítios para biópsia cirúrgica. O seguimento evolutivo é útil para a determinação de pneumopatias fibrosantes progressivas e para a detecção de complicações não relacionadas à doença de base, como infecções, exacerbação aguda e neoplasias. Ferramentas automatizadas de quantificação têm aplicabilidade clínica e devem estar acessíveis para a análise imagética no futuro próximo. Além disso, a incorporação da avaliação tomográfica a escores com parâmetros clínicos e funcionais de estadiamento do acometimento fibrosante poderá se tornar valiosa na determinação prognóstica. O conhecimento das diversas aplicabilidades clínicas do método tomográfico é fundamental aos especialistas que acompanham esses pacientes, podendo impactar positivamente sua trajetória clínica.

Perfil farmacoeconômico do tratamento da fibrose pulmonar idiopática demandado por idosos no estado do Pará / Pharmacoeconomic profile of the treatment of idiopathic pulmonary fibrosis demanded by the elderly in the state of Pará

Objetivo: O objetivo deste estudo foi realizar o levantamento de custo dos medicamentos antifibróticos para o tratamento da fibrose pulmonar idiopática no estado do Pará. Métodos: Trata-se de uma pesquisa documental do tipo descritiva, retrospectiva e quantitativa, referente às tecnologias pirfenidona e nintedanibe, demandadas entre os meses de junho de 2016 a junho de 2019. Para a obtenção dos dados, acessaram-se os relatórios de dispensação por paciente, notas fiscais relacionadas à aquisição dos medicamentos, além de planilha Excel de cadastro e acompanhamento de processos dos anos correspondentes, disponibilizados pela Secretaria de Estado de Saúde. Resultados: Foram atendidos 81 processos destinados à aquisição dos medicamentos (2 em 2016, 13 em 2017, 31 em 2018 e 35 em 2019); dos quais 29 solicitando nintedanibe e 52, pirfenidona. Quanto aos gastos, identificou-se que, em 2016, se pagou R$ 38.673,32 para a aquisição nintedanibe. Em 2017, foi R$ 158.881,27 para a aquisição de pirfenidona e R$ 322.277,67 para o atendimento de nintedanibe. Para 2018, percebeu-se o aumento impactante de pirfenidona (R$ 627.959,33), se comparada aos anos anteriores, enquanto o nintedanibe totalizou R$ 670.337,55. Já para o primeiro semestre de 2019 foram investidos R$ 620.393,55 para a pirfenidona e R$ 464.079,84 para nintedanibe. Conclusões: Identificou-se que a demanda de tecnologias em saúde destinadas aos portadores de fibrose pulmonar idiopática no Pará segue em constante crescente, por ser uma tecnologia inovadora que ainda não está incorporada no Sistema Único de Saúde, fazendo-se necessários critérios que regulamentem a sustentabilidade do acesso ao tratamento dessa doença rara

Objective: The objective of this study was to perform the cost survey of antifibrotic drugs for the treatment of idiopathic pulmonary fibrosis in the state of Pará. Methods: This is a descriptive, retrospective and quantitative documentary research on Pirfenidone and Nintedanibe technologies, demanded from June 2016 to June 2019. To obtain the data were accessed dispensing reports per patient, Invoices related to the purchase of medicines, in addition to Excel spreadsheet for registration and monitoring of processes of the corresponding years, available from the Secretary of State for Health. Results: 81 cases were received for the purchase of medicines (2 in 2016, 13 in 2017, 31 in 2018 and 35 in 2019); of which 29 requesting nintedanibe and 52, pirfenidone. Regarding expenses, it was identified that in 2016, R$ 38,673.32 was paid for the acquisition of nintedanibe. In 2017, it was R$ 158,881.27 for the acquisition of pirfenidone and R$ 322,277.67 for the service of nintedanibe. For 2018, there was a significant increase in pirfenidone (R$ 627,959.33) compared to the previous year, while nintedanibe totaled R $ 670,337.55. For the first half of 2019, R$ 620,393.55 was invested for pirfenidone and R$ 464,079.84 for nintedanibe. Conclusions: It was identified that the demand for health technologies for patients with idiopathic pulmonary fibrosis in Pará continues to grow, as it is an innovative technology that is not yet incorporated into the Unified Health System. sustainability of access to treatment for this rare disease

Role of various imbalances centered on alveolar epithelial cell/fibroblast apoptosis imbalance in the pathogenesis of idiopathic pulmonary fibrosis / 中华医学杂志(英文版)

There have been recent extensive studies and rapid advancement on the pathogenesis underlying idiopathic pulmonary fibrosis (IPF), and intricate pathogenesis of IPF has been suggested. The purpose of this study was to clarify the logical relationship between these mechanisms. An extensive search was undertaken of the PubMed using the following keywords: "etiology," "pathogenesis," "alveolar epithelial cell (AEC)," "fibroblast," "lymphocyte," "macrophage," "epigenomics," "histone," acetylation," "methylation," "endoplasmic reticulum stress," "mitochondrial dysfunction," "telomerase," "proteases," "plasminogen," "epithelial-mesenchymal transition," "oxidative stress," "inflammation," "apoptosis," and "idiopathic pulmonary fibrosis." This search covered relevant research articles published up to April 30, 2020. Original articles, reviews, and other articles were searched and reviewed for content; 240 highly relevant studies were obtained after screening. IPF is likely the result of complex interactions between environmental, genetic, and epigenetic factors: environmental exposures affect epigenetic marks; epigenetic processes translate environmental exposures into the regulation of chromatin; epigenetic processes shape gene expression profiles; in turn, an individual's genetic background determines epigenetic marks; finally, these genetic and epigenetic factors act in concert to dysregulate gene expression in IPF lung tissue. The pathogenesis of IPF involves various imbalances including endoplasmic reticulum, telomere length homeostasis, mitochondrial dysfunction, oxidant/antioxidant imbalance, Th1/Th2 imbalance, M1-M2 polarization of macrophages, protease/antiprotease imbalance, and plasminogen activation/inhibition imbalance. These affect each other, promote each other, and ultimately promote AEC/fibroblast apoptosis imbalance directly or indirectly. Excessive AEC apoptosis and impaired apoptosis of fibroblasts contribute to fibrosis. IPF is likely the result of complex interactions between environmental, genetic, and epigenetic factors. The pathogenesis of IPF involves various imbalances centered on AEC/fibroblast apoptosis imbalance.

Clinical evaluation on idiopathic pulmonary fibrosis / 中南大学学报(医学版)

Idiopathic pulmonary fibrosis (IPF) is a chronic fatal pulmonary disease characterized by complex illness condition. There is no effective treatment at present except lung transplantation. The comprehensive evaluation is helpful for the management of patients with IPF in hierarchical stages. Therefore, it is very important to evaluate IPF by various independent factors. At present, the commonly used methods for clinical evaluation on IPF include assessment of health-related quality of life, assessment of physiological function, assessment of imaging, assessment of laboratory examination, and multi-dimensional assessment system. However, there are different advantages and disadvantages on diverse evaluation methods for the evaluation of IPF.

Resultados del primer registro nacional de fibrosis pulmonar idiopática en Chile / Results of the first national register of idiopathic pulmonary fibrosis in Chile

INTRODUCCIÓN: La FPI es la neumonía intersticial idiopática más común, con cifras de incidencia y prevalencia que varían en el mundo por la poco uniforme manera de recolectar casos en los diferentes estudios. No hay cifras publicadas sobre la epidemiología de la FPI en Chile ni Latinoamérica. Se hace relevante conocerlas por la carga sanitaria que representan los pacientes con FPI y por la aprobación reciente de drogas antifibróticas de alto costo. El objetivo de este estudio fue realizar un registro de pacientes con FPI atendidos por neumólogos chilenos de diversos regiones del país, con los medios diagnósticos que habitualmente utilizan en la vida real. PACIENTES Y MÉTODOS: Se utilizó una encuesta electrónica en línea diseñada por el grupo de enfermedades pulmonares difusas del Instituto Nacional del Tórax para registro de pacientes con diagnóstico de FPI según criterios ATS/ERS/JRS/ALAT desde junio de 2015 a junio de 2017. RESULTADOS: 40 de los 200 neumólogos invitados enviaron casos de FPI de las 13 regiones del país, completando un total de 700 pacientes. 2/3 eran casos antiguos, un número similar de hombres y mujeres, 73% tienen patrón definitivo de UIP (Usual Interstitial Pneumonia) en tomografía axial computarizada, la mayoría eran pacientes sobre 60 años y en solo 16% se solicitó biopsia para diagnóstico. CONCLUSIONES: Un registro de 700 casos representa un número muy importante de pacientes con FPI en Chile que nos permite acercarnos a la caracterización de la cohorte y a fortalecer una red de especialistas dedicados al cuidado de estos pacientes y sus familias.

INTRODUCTION: Idiopathic pulmonary fibrosis is the most common idiopathic interstitial pneumonia, with incidence and prevalence figures varying worldwide because of the inconsistent way of collecting cases in different studies. There are no published figures on the epidemiology of IPF in Chile or Latin America. It is relevant to know them because of the health burden of patients with IPF and the recent approval for treatment purposes of high cost antifibrotic drugs. The objective of this study was to develop a clinical registry of patients with IPF treated by Chilean pulmonologists from different regions of the country, using the diagnostic means they usually use in real life. PATIENTS AND METHODS: An online electronic survey was designed by the group of diffuse pulmonary diseases of the "Instituto Nacional del Tórax" to register patients with diagnosis of IPF from June 2015 to June 2017 according to ATS/ERS/JRS/ALAT criteria. RESULTS: 40 of the 200 invited pulmonologists sent IPF cases from the country's 13 regions, completing a total of 700 patients. 2/3 were old cases, a similar number of men and women, 73% had definitive UIP pattern in CT, the majority were patients over 60 years old and in only 16% biopsy was requested for diagnosis. CONCLUSIONS: A register of 700 cases represents a very important number of patients with IPF in Chile that allows us to approach the characterization of the cohort and to strengthen a network of specialists dedicated to the care of these patients and their families.

Transplante pulmonar em pacientes com fibrose pulmonar familiar / Lung transplant in patients with familial pulmonary fibrosis

RESUMO Objetivo A fibrose pulmonar familiar (FPF) é definida como uma doença pulmonar intersticial idiopática que afeta dois ou mais membros da mesma família. Nesses pacientes, os resultados têm sido insatisfatórios, apresentando alto risco de morte e disfunção crônica do enxerto pulmonar (CLAD) após o transplante de pulmão. O objetivo do presente estudo foi comparar o resultado de curto e longo prazo do transplante de pulmão em pacientes com FPF e pacientes transplantados por outras doenças pulmonares intersticiais. Métodos Foram coletados retrospectivamente dados clínicos pré e pós-transplante de 83 pacientes com fibrose pulmonar submetidos a transplante de pulmão. Os pacientes foram divididos em aqueles com fibrose pulmonar familiar (n = 9, grupo FPF) e aqueles com fibrose pulmonar não familiar (n = 74, grupo controle). Resultados O grupo FPF foi composto de quatro mulheres e cinco homens, sendo 44,5% ex-fumantes. A maioria apresentou tomografia computadorizada e evidência patológica de pneumonia intersticial usual. Os pacientes com FPF tiveram níveis significativamente menores de hemoglobina e hematócrito. Não foram observadas outras diferenças nas características pré e pós-transplante em relação ao grupo controle. A evolução clínica pós-operatória foi semelhante nos dois grupos. Não foi constatada diferença significativa na sobrevida de um ano livre de CLAD e na sobrevida em geral. Conclusão A evolução pós-transplante de pacientes com FPF foi semelhante à dos pacientes com fibrose pulmonar não familiar, embora mais pacientes com FPF tivessem anemia pré-transplante. O resultado a curto e longo prazo foi comparável em ambos os grupos. O transplante de pulmão provou ser uma opção válida para pacientes com FPF, assim como para pacientes com outros tipos de fibrose pulmonar.

ABSTRACT Objective Familial pulmonary fibrosis (FPF) is defined as an idiopathic interstitial lung disease affecting two or more members of the same family; poor outcome with high risk of death and chronic lung allograft dysfunction (CLAD) after lung transplant has been reported in these patients. The present study aimed to compare the short- and long-term outcome of lung transplants in patients with FPF and patients transplanted because of other interstitial lung diseases. Method Clinical pre- and post-transplant data from 83 consecutive patients with pulmonary fibrosis who underwent lung transplant at our centre were collected retrospectively. Patients were divided into those with familial (n=9 FPF group) and those with non-familial pulmonary fibrosis (n=74 controls). Results The FPF group was composed of 4 females and 5 males; 44.5% were ex-smokers. The majority presented their CT scan and pathology evidence of usual interstitial pneumonia. Patients with FPF had significantly lower pre-transplant levels of haemoglobin and haematocrit. No other differences in pre- and post-transplant characteristics were observed concerning controls. The clinical post-operative course was similar in the two groups. No significant difference in one-year CLAD-free survival and overall survival was observed. Conclusion The post-transplant course of patients with FPF was similar to patients with non-familial pulmonary fibrosis, although more patients with FPF had pre-transplant anaemia. Short- and long-term outcome was comparable in both groups. Lung transplant proved to be a valid option for patients with FPF as it was for patients with other types of pulmonary fibrosis.

Padrões de tomografia computadorizada de alta resolução na doença pulmonar intersticial (DPI): prevalência e prognóstico / High resolution computed tomography patterns in interstitial lung disease (ILD): prevalence and prognosis

RESUMO Objetivo Correlacionar a prevalência e o prognóstico de cada padrão de TCAR de pneumonia intersticial usual (PIU) típica, provável e indeterminada com o diagnóstico clínico multidisciplinar de doença pulmonar intersticial (DPI). Métodos Incluímos todos os pacientes com diagnóstico multidisciplinar de DPI com padrão de TCAR de PIU típica, PIU provável ou indeterminada para PIU. Dados clínicos e histopatológicos, teste de função pulmonar e status de sobrevida foram obtidos retrospectivamente. O diagnóstico final foi validado por uma equipe multidisciplinar. Resultados Foram incluídos no estudo 244 pacientes, com média de idade de 68 ± 13 anos sendo 52,5% do sexo masculino. Em um total de 106 pacientes com padrão típico de PIU, 62% tiveram o diagnóstico multidisciplinar de FPI, 20% de pneumonia por hipersensibilidade crônica (PHC) e 10% de DPI relacionada à doença do tecido conjuntivo (DPI-DTC). Dos 114 casos com provável PIU, DPI-DTC correspondeu a 39%, FPI a 31%, pneumonia intersticial descamativa a 11%, doença pulmonar relacionada a medicamentos a 9% e PHC a 8%. Nos 24 pacientes com TC indeterminada para PIU, o DPI-DTC foi o diagnóstico final em 33%, seguido por pneumonia intersticial descamativa (21%) e FPI (13%). Pacientes com PIU típica apresentaram maior probabilidade de morrer ou realizar transplante de pulmão no seguimento (17,9% e 11,3%, respectivamente). Conclusões FPI, PHC e DPI-DTC foram os principais diagnósticos diferenciais em pacientes com padrão de TCAR de PIU típica, provável e indeterminada. Pacientes com padrão de PIU típico na TCAR tiveram maior probabilidade de morrer ou realizar transplante de pulmão no seguimento.

ABSTRACT Objective To correlate the prevalence and prognosis of each HRCT pattern of typical, probable, and indeterminate usual interstitial pneumonia (UIP) with the clinical multidisciplinary diagnosis of interstitial lung disease (ILD). Methods We included all patients with a multidisciplinary diagnosis of ILD with an HRCT pattern of typical UIP, probable UIP, or indeterminate for UIP. Clinical and histopathological data, pulmonary function tests, and survival status were retrospectively obtained. The final diagnosis was validated by a multidisciplinary team. Results A total of 244 patients were included in the study, with a mean age of 68 ±13 years and being 52.5% males. In a total of 106 patients with typical UIP pattern, 62% had the multidisciplinary diagnosis of IPF, 20% had chronic hypersensitivity pneumonitis (CHP), and 10% had connective tissue disease-related ILD (CTD-ILD). Out of the 114 cases with probable UIP, CTD-ILD corresponded to 39%, IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. In the 24 patients with CT indeterminate for UIP, CTD-ILD was the final diagnosis in 33%, followed by desquamative interstitial pneumonia (21%), and IPF (13%). Patients with typical UIP were more likely to die or had lung transplantation in the follow-up (17.9% and 11.3%, respectively). Conclusion IPF, CHP, and CTD-ILD were the main differential diagnoses in patients with HRCT patterns of typical, probable and indeterminate UIP. Patients with HRCT typical UIP pattern were more likely to die or had lung transplantation in the follow-up.