Disfunciones del metabolismo del surfactante pulmonar: caso clínico pediátrico / Pulmonary surfactant metabolism dysfunction: A pediatric clinical case report

Las enfermedades pulmonares intersticiales son patologías poco frecuentes en pediatría. Dentro de ellas, se incluyen las disfunciones del metabolismo del surfactante pulmonar, molécula anfipática cuya función es disminuir la tensión superficial y evitar el colapso alveolar. Se presenta el caso de un lactante de 6 meses, en seguimiento por bajo peso, que presentó dificultad respiratoria aguda y cianosis; la radiografía de tórax evidenció infiltrado intersticial, neumomediastino y neumotórax bilateral. Al interrogatorio, surgió antecedente materno de internación al año de vida, con requerimiento de oxigenoterapia prolongada y diagnóstico desconocido; presenta signos de hipoxia crónica. El paciente cursó internación con requerimiento de oxigenoterapia. Se realizaron estudios complementarios en búsqueda de etiología, sin resultados positivos. La tomografía de tórax evidenció opacidades en vidrio esmerilado, engrosamiento del intersticio septal y áreas de atrapamiento aéreo; con resultado de biopsia pulmonar y estudio genético se llegó al diagnóstico de disfunción del metabolismo del surfactante pulmonar.

Interstitial lung diseases are rare in pediatrics. They include dysfunctions in the metabolism of pulmonary surfactant, an amphipathic molecule that reduces surface tension and prevents alveolar collapse. Here we describe the case of a 6-month-old infant controlled for low weight, who presented with acute respiratory distress and cyanosis; his chest X-ray showed interstitial infiltrate, pneumomediastinum, and bilateral pneumothorax. During history-taking, it was noted that his mother had a history of hospitalization at 1 year old with unknown diagnosis, requiring prolonged oxygen therapy; she now shows signs of chronic hypoxia. The patient was hospitalized and required oxygen therapy. Ancillary tests were done to look for the etiology of the condition, with no positive results. A chest computed tomography showed groundglass opacities, thickening of the septal interstitium, and areas of air trapping; based on the results of a lung biopsy and a genetic study, pulmonary surfactant metabolism dysfunction was diagnosed.

Abordaje paliativo en enfermedades respiratorias avanzadas / Palliative Approach in Advanced Respiratory Diseases

Las enfermedades respiratorias crónicas avanzadas son prevalentes y producen deterioro de la calidad de vida, en particular la enfermedad pulmonar obstructiva crónica (EPOC), las enfermedades pulmonares intersticiales difusas (EPID) y las enfermedades neuromusculares progresivas con compromiso diafragmático (ENM). Quienes las padecen presentan síntomas persistentes que no son siempre adecuada-mente controlados por los tratamientos recomendados por las guías clínicas de mane-jo. El tratamiento paliativo de los síntomas persistentes es un punto relevante y suelen presentarse barreras para su implementación.Este artículo ofrece una revisión narrativa sobre una perspectiva latinoamericana acerca del rol de los cuidados paliativos en enfermedades respiratorias avanzadas.

Advanced chronic respiratory diseases are prevalent and cause deterioration in qual-ity of life, particularly chronic obstructive pulmonary disease (COPD), diffuse intersti-tial lung diseases (ILD) and progressive neuromuscular diseases with diaphragmatic involvement (NMD). Those who suffer from them usually present persistent symptoms that are not always adequately controlled by the treatments recommended by the clinical management guidelines. Palliative treatment of persistent symptoms is a relevant point, but the pal-liative approach usually presents barriers to its implementation.This article offers a narrative review over Latin American perspective on the role of pal-liative care in advanced respiratory diseases.

Estudio de sobrevida a largo plazo en pacientes adultos con enfermedades respiratorias crónicas atendidos por infección respiratoria aguda por coronavirus SARS-CoV-2 / Study of long-term survival in adult patients with chronic respiratory diseases treated for acute respiratory infection by coronavirus SARS-CoV-2

La enfermedad respiratoria aguda por coronavirus SARS-CoV-2 (COVID-19) se ha convertido en un grave problema de salud pública a nivel mundial. Objetivos: Examinar el uso de recursos sanitarios, riesgo de complicaciones y muerte en pacientes adultos con enfermedades respiratorias crónicas atendidos por COVID-19. Métodos: Estudio clínico descriptivo prospectivo realizado en pacientes adultos atendidos por COVID-19 en la Red de Salud UC Christus entre el 1 de abril y 31 de diciembre de 2020. Resultados: Se evaluaron 2.160 pacientes adultos, edad: 47 ± 17 años (rango: 18-100), 51,3% sexo masculino, 43,8% tenía comorbilidades, especialmente hipertensión (23,2%), diabetes (11,7%) y enfermedades respiratorias crónicas: asma (5%), EPOC (1,4%) y enfermedad pulmonar difusa (EPD: 0,8%). Los pacientes adultos con enfermedades respiratorias crónicas tuvieron mayor riesgo de hospitalización y uso de oxígeno suplementario; sin embargo, la evolución de los pacientes asmáticos y la sobrevida a los doce meses fue similar a los pacientes sin comorbilidades atendidos por COVID-19, mientras que en los pacientes con EPOC y EPD la admisión a la unidad de paciente crítico y riesgo de muerte fueron más elevados. En el análisis multivariado, los principales predictores clínicos asociados al riesgo de muerte en el seguimiento a doce meses en pacientes adultos con COVID-19 fueron la edad y admisión al hospital, mientras que el asma fue un factor protector. Conclusión: Los pacientes asmáticos tuvieron bajo riesgo de complicaciones y muerte asociados a COVID-19; mientras que los pacientes con EPOC y EPD tuvieron mayor riesgo de complicaciones y muerte en el seguimiento a largo plazo.

The acute respiratory disease associated to coronavirus SARS-CoV-2 (COVID-19) has become a serious public health problem worldwide. Objectives: To examine the use of healthcare resources, risk of complications and death in adult patients with chronic respiratory diseases treated for COVID-19. Methods: Prospective descriptive clinical study conducted in adult patients treated for COVID-19 in the UC Christus Healthcare Network between April 1 and December 31, 2020. Results: 2,160 adult patients were evaluated, age: 47 ± 17 years-old (range: 18-100), 51.3% male, 43.8% had comorbidities, especially hypertension (23.2%), diabetes (11.7%), and chronic respiratory diseases: asthma (5%), COPD (1,4%) and interstitial lung disease (ILD: 0.8%). Adult patients with chronic respiratory diseases were at higher risk for hospitalization and use of supplemental oxygen; however, the evolution of asthmatic patients and survival at twelve months was similar to that of adult patients without comorbidities treated for COVID-19, while in patients with COPD and ILD admission to the critical care unit and risk of death were higher. In the multivariate analysis, the main clinical predictors associated to 12-month mortality risk in adult patients with COVID-19 were age and hospital admission, while asthma was a protective factor. Conclusion: Asthmatic patients had minor risk of complications and mortality associated with COVID-19; while patients with COPD and ILD had a significant higher risk of complications and 12-month mortality.

Openreuma consensus on the role of nursing in the care of patients with rheumatoid arthritis and diffuse interstitial lung disease / Consenso de Openreuma sobre el papel de la enfermería en el cuidado de pacientes con artritis reumatoide y enfermedad pulmonar intersticial difusa / Consenso Openreuma sobre o papel da enfermagem no cuidado de pacientes com artrite reumatoide e doença pulmonar intersticial difusa

Objective.To develop practical recommendations, based on the best available evidence and experience, on the nursing management of patients with rheumatoid arthritis (RA) and interstitial lung disease (ILD). Methods. The usual consensus methodology was used, with a nominal group, systematic reviews (SRs), and Delphi survey. The expert panel, consisting of rheumatology nurses, rheumatologists, a psychologist, a physiotherapist, and a patient, defined the scope, the users, the topics on which to explore the evidence and on which to issue recommendations. Results.Three PICO questions evaluated the efficacy and safety of pulmonary rehabilitation and non-pharmacological measures for the treatment of chronic cough and gastroesophageal reflux by means of SR of the literature. With the results of the reviews, 15 recommendations were established for which the degree of agreement was obtained with a Delphi survey. Three recommendations were rejected in the second round. The 12 recommendations were in patient assessment (n=4); patient education (n=4); and risk management (n=4). Only one recommendation was based on available evidence, while the remaining were based on expert opinion. The degree of agreement ranged from 77% to 100%. Conclusion.This document presents a series of recommendations with the aim of improving the prognosis and quality of life of patients with RA-ILD. Nursing knowledge and implementation of these recommendations can improve the follow-up and prognosis of patients with RA who present with ILD.

Objetivo.Desarrollar recomendaciones prácticas, basadas en la mejor evidencia y experiencia disponible, sobre el manejo de enfermería de los pacientes con artritis reumatoide (AR) y enfermedad pulmonar intersticial (EPI). Métodos. Se utilizó la metodología de consenso en la que un panel de expertos (formado por enfermeras de reumatología, reumatólogos, una psicóloga, una fisioterapeuta y una paciente) definió el ámbito, los usuarios, los temas sobre los que explorar la evidencia y sobre los que emitir recomendaciones. Tres preguntas PICO evaluaron la eficacia y seguridad de la rehabilitación pulmonar y las medidas no farmacológicas para el tratamiento de la tos crónica y el reflujo gastroesofágico mediante la búsqueda de revisiones sistemáticas, excluyendo aquellas cuya calidad era baja, muy baja o críticamente baja, según la herramienta AMSTAR-2. Posteriormente, se hizo una reunión para la formulación de recomendaciones que se presentaron con un resumen de la evidencia a la encuesta Delphi. Resultados.Con los resultados de las revisiones se establecieron 15 recomendaciones cuyo grado de acuerdo osciló entre el 77% y el 100% en la una encuesta Delphi. Tres recomendaciones fueron rechazadas en la segunda ronda: una por la evidencia disponible y los dos restantes se basaron en la opinión de expertos. Las 12 recomendaciones restantes aprobadas se referían a la evaluación del paciente (n=4), a la educación del paciente (n=4) y a la gestión del riesgo (n=4). Conclusión. El conocimiento del consenso Openreumapor parte de enfermería y la aplicación sus 12 recomendaciones basadas en la mejor evidencia y experiencia puede mejorar el seguimiento y el pronóstico de los pacientes con AR que presentan EPI.

Objetivo.Desenvolver recomendações práticas, baseadas na melhor evidência e experiência disponíveis, sobre o manejo de enfermagem de pacientes com artrite reumatoide (AR) e doença pulmonar intersticial (DPI). Métodos.Foi utilizada a metodologia de consenso, com grupo nominal, revisões sistemáticas e levantamento Delphi. O painel de especialistas, formado por enfermeiros reumatologistas, reumatologistas, psicólogo, fisioterapeuta e paciente, definiu o escopo, os usuários, os tópicos sobre os quais explorar as evidências e sobre os quais emitir recomendações. Três questões do PICO avaliaram a eficácia e segurança da reabilitação pulmonar e medidas não farmacológicas para o tratamento da tosse crônica e refluxo gastroesofágico por meio de RS. Aqueles cuja qualidade era baixa, muito baixa ou criticamente baixa, de acordo com a ferramenta AMSTAR-2, foram excluídos. Posteriormente, realizou-se uma reunião para formular recomendações que foram apresentadas com um resumo das evidências ao inquérito Delphi. Resultados. Com os resultados das revisões, foram estabelecidas 15 recomendações cujo grau de concordância entre 77% e 100% foi obtido com uma pesquisa Delphi. Três recomendações foram rejeitadas na segunda rodada. As 12 recomendações referiam-se à avaliação do paciente (n=4); à educação do paciente (n=4); e ao gerenciamento de risco (n=4). Apenas uma recomendação foi baseada nas evidências disponíveis, enquanto as demais foram baseadas na opinião de especialistas. Conclusão. Este documento apresenta uma série de recomendações com o objetivo de melhorar o prognóstico e a qualidade de vida dos pacientes com AR-ILD. O conhecimento da enfermagem e a aplicação dessas recomendações podem melhorar o acompanhamento e o prognóstico de pacientes com AR com DPI.

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.

Inspiratory muscle training in interstitial lung disease: a systematic scoping review / Treinamento muscular inspiratório na doença pulmonar intersticial: revisão sistemática de escopo

ABSTRACT Inspiratory muscle training (IMT) has been described as one of the components of the treatment of chronic lung conditions such as obstructive and restrictive lung diseases. Although the number of studies showing results of IMT in patients with interstitial lung disease (ILD) is scarce when compared with studies in patients with COPD, evidence points to benefits of IMT in this population. This scoping review aimed to explore the role and the rationale of IMT in patients with ILD and to gather recent evidence on the effects of IMT in this population. The studies included in this review showed improvements in respiratory muscle function, quality of life, exercise capacity and dyspnea after ILD patients participated in programs that included stand-alone IMT or combined with pulmonary rehabilitation. There is still a gap in the literature to allow a clear conclusion on the indications of IMT as part of ILD treatment because of poor research design and small numbers of participants. Therefore, although IMT seems to have a positive effect in patients with ILD, current evidence prevents us from drawing a definite conclusion. Further studies need to be conducted using better research methodology to demonstrate and confirm the positive effects of IMT.

RESUMO O treinamento muscular inspiratório (TMI) já foi descrito como um dos componentes do tratamento de doenças pulmonares crônicas obstrutivas e restritivas. Embora os estudos que mostram os resultados do TMI em pacientes com doença pulmonar intersticial (DPI) sejam escassos em comparação com os estudos em pacientes com DPOC, as evidências indicam que o TMI traz benefícios para essa população. O objetivo desta revisão de escopo foi explorar o papel e a lógica do TMI em pacientes com DPI e reunir evidências recentes sobre os efeitos do TMI nessa população. Os estudos incluídos nesta revisão mostraram melhora na função muscular respiratória, qualidade de vida, capacidade de exercício e dispneia depois que pacientes com DPI participaram de programas com TMI apenas ou em conjunto com reabilitação pulmonar. Ainda há uma lacuna na literatura que impede que se chegue a uma conclusão clara a respeito da indicação do TMI como parte do tratamento de DPI em virtude da baixa qualidade do desenho dos estudos e do pequeno número de participantes. Portanto, embora os efeitos do TMI em pacientes com DPI sejam aparentemente positivos, as evidências atuais não permitem que se chegue a uma conclusão definitiva. São necessários mais estudos com metodologia de pesquisa melhor para demonstrar e confirmar os efeitos positivos do TMI.

Detection of Mycoplasma capricolum subsp. capripneumoniae and Mannheimia haemolytica as causative agents of pleuropneumonia in goats / Detección de Mycoplasma capricolum subsp. capripneumoniae y Mannheimia haemolytica como agentes causales de pleuro-neumonia en cabras

Abstract Objective: The aim of the study was detection of two major causative agents of pleuropneumonia, Mycoplasma capricolum subsp. capripneumoniae (Mccp) and Mannheimia haemolytica, in goats. To the best of our knowledge, this study is the first investigation of Mccp in Iran. Methods: 50 grossly suspected lungs to pleuropneumonia and 10 healthy samples were collected from Shiraz abattoir. Results: Histopathological evaluation of tissue samples showed various diagnosed pneumonias including 40% bronchointerstitial pneumonia (20 samples), 34% interstitial pneumonia (17 samples), 10% fibrinopurulent bronchopneumonia (5 samples), 12% purulent bronchopneumonia (6 samples) and 4% chronic pneumonia (2 samples). In molecular study, all 50 suspected samples and 10 healthy ones by PCR showed no Mccp positive sample, but the detection rate of M. haemolytica in suspected samples was 14% and in healthy lungs was zero. Conclusions: It may be concluded that goats referred to Shiraz abattoir is free of Mccp. Further sampling and molecular testing at the level of suspected herds to CCPP can be useful.

Resumen Objetivo: El objetivo del estudio fue la detección de dos agentes causantes principales de pleuroneumonía, Mycoplasma capricolum subsp. Capripneumoniae (Mccp) y Mannheimia haemolytica, en cabras. Hasta donde sabemos, este estudio es la primera investigación de Mccp en Irán. Métodos: 50 pulmones muy sospechosos de pleuroneumonía y 10 muestras sanas se obtuvieron del matadero de Shiraz. Resultados: La evaluación histopatológica de muestras de tejido mostró varias neumonías diagnosticadas, incluyendo 40% de neumonía broncointersticial (20 muestras), 34% de neumonía intersticial (17 muestras), 10% de bronconeumonía fibrinopurulenta (5 muestras), 12% de bronconeumonía purulenta (6 muestras) y 4% neumonía crónica (2 muestras). En un estudio molecular, las 50 muestras sospechosas y 10 sanas por PCR no mostraron una muestra positiva de Mccp, pero la tasa de detección de M. haemolytica en muestras sospechosas fue del 14% y en pulmones sanos fue cero. Conclusiones: se puede concluir que las cabras referidas al matadero Shiraz están libres de Mccp. La realización de muestreo adicional y pruebas moleculares a nivel de rebaños sospechosos para CCPP puede ser útil.

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.

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.

Experiencia de rehabilitación respiratoria en EPID en un Centro Latinoamericano / Experience in Pulmonary Rehabilitation in DILD (Diffuse Interstitial Lung Disease) in a Latin American Center

La rehabilitación respiratoria (RR) ha demostrado claramente ser una herramienta terapéutica beneficiosa en pacientes con EPOC por lo cual es recomendada en forma sistemática en todas las guías y consensos sobre el manejo de la enfermedad. Mientras tanto la evidencia sigue siendo más limitada en pacientes con enfermedades pulmonares intersticiales difusas (EPID).

Translation and cultural adaptation of the King's Brief Interstitial Lung Disease health status questionnaire for use in Brazil / Tradução e adaptação cultural do King's Brief Interstitial Lung Disease health status questionnaire

ABSTRACT Objective: To translate the King's Brief Interstitial Lung Disease (K-BILD) questionnaire to Portuguese and culturally adapt it for use in Brazil. The K-BILD quantifies the health status of patients with ILD. Methods: The process involved the following steps: authorization from the author of the original (English-language) questionnaire; translation of the questionnaire to Portuguese by three translators, working independently; merging of the translations by a committee of specialists; back-translation of the questionnaire to English; revision and readjustment of the back-translation by the committee of specialists; evaluation by the original author; revision of the back-translation; cognitive debriefing (verification of the clarity and acceptability of the Portuguese-language version in the target population-i.e., patients with ILD); and finalization of the Portuguese-language version. Results: In the cognitive debriefing step, 20 patients with ILD were interviewed. After the interviews, the clarity and acceptability index of each question was ≥ 0.8, which is considered acceptable. Conclusions: The Portuguese-language version of K-BILD appears to be easily administered to and understood by patients with ILD in Brazil. To our knowledge, this is the only instrument in Brazilian Portuguese that is designed to evaluate the impact that ILD has on the various aspects of the lives of those it affects.

RESUMO Objetivo: Traduzir e adaptar culturalmente o King's Brief Interstitial Lung Disease (K-BILD) health status questionnaire, utilizado para mensurar o estado de saúde em pacientes com doença pulmonar intersticial (DPI), para a língua portuguesa falada no Brasil. Métodos: As seguintes etapas foram realizadas: autorização do autor do instrumento original; preparação; tradução do K-BILD da língua inglesa para a língua portuguesa falada no Brasil por três tradutores independentes; unificação das traduções por um comitê de especialistas; retradução do português para o inglês; revisão e readequação da retradução pelo comitê de especialistas; avaliação do autor do original; revisão da versão em inglês; desdobramento cognitivo (observação da clareza e aceitabilidade do questionário traduzido na população-alvo); e elaboração da versão final em português. Resultados: Na etapa de desdobramento cognitivo foram entrevistados 20 pacientes com DPI. Após todas as entrevistas, o índice de clareza e aceitabilidade das questões foi igual ou maior que 0,8, indicando um índice de clareza aceitável para todas as questões. Conclusões: A versão do K-BILD mostrou ser compreensível e de fácil aplicação. Até onde sabemos, esse é atualmente o único instrumento disponível em português falado no Brasil dirigido à avaliação do impacto da DPI em vários aspectos da vida desses pacientes.

Síndrome de Sjögren primario y enfermedad quística pulmonar: reporte de un caso / Primary Sjögren syndrome and pulmonary cysts disease: case report

El síndrome de Sjögren (SS) es una enfermedad inflamatoria, autoinmunitaria crónica de etiología desconocida, caracterizada por infiltración del tejido glandular y extraglandular por linfocitos y células plasmáticas. Presenta manifestaciones glandulares (xeroftalmia y xerostomía) y extraglandulares (50%); v.gr.: pulmonares (11%) ­las más comunes son la enfermedad intersticial linfocítica y la enfermedad quística pulmonar (25%)­; las manifestaciones extraglandulares preceden, muchas veces, a las manifestaciones glandulares; por este motivo su diagnóstico se confunde o se retrasa. Caso clínico: paciente de sexo femenino, 71 años de edad, con antecedentes de hipertensión arterial e hipotiroidismo. Hospitalizada por astenia, tos seca y disnea de medianos esfuerzos. Revisión de aparatos y sistemas: xeroftalmia y xerostomía. Exámenes complementarios: Rx tórax: opacidades difusas redondeadas en ambos campos pulmonares; tomografía simple de tórax: múltiples lesiones quísticas predominantes en los lóbulos inferiores y calcificaciones nodulares. Se amplían los estudios para determinar la etiología: exámenes inmunoquímicos: anti SSA/Ro 200 U/ml (< 20 UE/ ml), anti SSB/La 84,4U/ml (< 20 UE/ml), anticuerpos antinucleares 1/160 (≤ 1/40), factor reumatoideo 49,9UI/ml (< 20 UI/ml). Se sospecha síndrome de Sjogren y realiza sialografía de parótidas que evidencia obstrucción y procesos inflamatorios de los conductos parotídeos. Biopsia de labio inferior: mucosa revestida por epitelio escamoso, edema intercelular y exocitosis linfocitaria. El estroma contiene infiltrado linfomononuclear disperso característico del SS. Una vez confirmado el diagnóstico, se trata con corticoides y tratamiento específico del ojo y boca seca.

Context: Sjögren's syndrome (SS) is an inflammatory, chronic autoimmune disease of unknown etiology, which is characterized by infiltration of glandular and extraglandular tissue by lymphocytes and plasma cells. It presents glandu-lar manifestations (xerophthalmia and xerostomia), as extraglandular (50%). Within these, there are pulmonary manifestations (11%), the most common manifestation being lymphocytic interstitial disease followed by cystic lung dis-ease (25%). Its manifestations often precede the glandular manifestations so that the diagnosis of it is confused or delayed. Case presentation: A 71-year-old female patient with a history of high blood pressure and hypothyroidism. Hospitalized for presenting asthenia, dry cough and dyspnea to the medium efforts. In the review of all systems, she referred xerophthalmia and xerostomia. The following exams were carried out: Rx thor-ax: rounded diffuse opacities in both pulmonary fields, is complemented with simple chest Tomography: Multiple pre-dominant cystic lesions in lower lobes and nodular calcifications. It was decided to expand the studies to determine etiology and see if it is related to its xerophthalmia and xerostomia characteris-tic of SS. Immunochemical tests were performed. The results: anti-SSA / Ro 200 U / mL (< 20 EU / mL); anti SSB / 84.4U / mL (< 20 EU / mL); antinuclear antibodies with fine granular pattern 1/160 (≤ 1/40) Rheumatoid factor 49.9UI / mL (< 20 IU / mL). With the suspicion of Sjogren's syndrome, parotid sialog-raphy is performed, obstruction is evidenced and chronic inflammatory pro-cesses of parotid ducts. Lower lip biopsy: Mucosa lined by squamous epitheli-um with intercellular edema and exocytosis of small lymphocytes. The stroma contains mild scattered lymphomononuclear infiltrate characteristic of SS. Con-firmed diagnosis of SS managed with corticosteroids and specific treatment of eye and dry mouth.

Patrón tomográfico de neumonía intersticial usual en paciente con exposición laboral / Tomographic pattern of usual interstitial pneumonia in a patient with occupational exposure

La neumonitis por hipersensibilidad (NH) puede ser causada por múltiples agentes presentes en el lugar de trabajo y en el hogar, como microbios, proteínas animales o vegetales y químicos orgánicos o inorgánicos. El diagnóstico basa en la evaluación de una serie de características clínicas incluyendo antecedentes de exposición significativa a un potencial antígeno, examen físico, tomografía computada de tórax, linfocitosis en el lavado bronquioalveolar, y, en casos seleccionados, histología. La NH puede ser aguda o crónica. Esta última puede ser fibrosante o no. La NH fibrosante tiene una amplia lista de diagnósticos diferenciales y tiene peor pronóstico. Describimos el caso de un varón, tabaquista, trabajador en una fábrica de quesos, con diagnóstico de NH "muy probable", y mala evolución.

Características clínicas, funcionais e sobrevida dos pacientes com pneumonia de hipersensibilidade do Ambulatório de Doenças Pulmonares Intersticiais do Hospital das Clinicas da Universidade Federal de Minas Gerais (HC-UFMG) / Clinical, functional and survival in patients with hypersensitivity pneumonia Center of Pulmonary Interstitial Disease of Hospital das Clínicas da Universidade Federal de Minas Gerais (HC- UFMG)

A pneumonia de hipersensibilidade (PH) constitui síndrome inflamatória causada pela resposta imune exagerada a partículas antigênicas inaladas. Objetivo: descrever características clínicas, radiológicas, funcionais esobrevida de pacientes com PH. Metodologia: estudo retrospectivo, envolvendo pacientes em acompanhamento no ambulatório de doenças pulmonares intersticiais do HC-UFMG no período de 2011 a 2015. Analisados dados clínicos, radiológicos, funcionais e morfológicos coletados através de um protocolo padronizado. Resultados: Dentre 139 pacientes com doenças intersticiais pulmonares, 34 (24%) tinham PH, com idade de 60,7 ± 18,1 anos. Exposição a mofo foi a etiologia mais frequente (19;55%). A forma crônica foi predominante (100% doas casos), assim como a presença de padrão reticular na tomografia de tórax (100%), com ou sem faveolamento. Observou-se distúrbio restritivo leve (CVF=69,00 ± 17,62L) associado a redução moderada da difusão de monóxido de carbono (59,20 ± 16,99%). O lavado broncoalveolar não auxiliou no diagnóstico e a biópsia transbrônquica foi diagnóstica em 5 (55%) casos e a biópsia cirúrgica foi conclusiva em 6 (85,7%). A sobrevida mediana foi de 75 meses. Conclusão: Nesta amostra de um ambulatório de referência, a PH respondeu por cerca de um quarto dos pacientes com doença intersticial pulmonar. A exposição a mofo foi a principal etiologia e a mortalidade significativa, o que reforça a importância da adoção de medidas preventivas através do reconhecimento precoce da exposição aos fatores de risco. (AU)

Hypersensitivity pneumonitis (HP) inflammatory is a syndrome caused by an excessive immune response to inhaled antigen particles. Objective:To describe clinical, radiological and functional characteristics and survival in patients with HP. Methodology: Retrospective study involving patients followed up at the pulmonary interstitial diseases clinic of the HC-UFMG from 2011 to 2015. Clinical, radiological, functional and morphological data were analysed from a standardized protocol. Results: Among 139 patients with interstitial lung disease, 34 (24%) had HP, aged 60.7 ± 18.1 years. Mold exposure was the most frequent cause (19; 55%). The chronic form predominated (100% donate cases), as well as the presence of reticular pattern on the chest tomography (100%), with or without honeycombing. There was mild restrictive lung disease (FVC = 69.00 ± 17,62L) associated with moderate reduction in carbon monoxide diffusion (59.20 ± 16.99%). Bronchoalveolar lavage did not help in the diagnosis and transbronchial biopsy was diagnostic in 5 (55%) cases; surgical biopsy was conclusive in 6 (85.7%). Median survival was 60 months. Conclusion: In this sample of a reference clinic, HP accounted for about a quarter of the patients with interstitial lung disease. The mold exposure was the main etiology and the mortality rate was significant, what reinforces the importance of adopting preventive measures through early recognition of exposure to risk factors. (AU)

Enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Revisión narrativa de la literatura / Intersticial pulmonary disease in patients with systemic sclerosis. Narrative review of the literatura / Doença pulmonar intersticial em pacientes com esclerose sistémica. Revisão narrativa da literatura

RESUMEN Introducción: la esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por la tríada de vasculopatía de pequeños vasos, activación del sistema inmune y aumento de los depósitos de matriz extracelular tanto en la piel como en los órganos internos. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, pasando a ser la primera causa de muerte, dada la disminución de la mortalidad por crisis renales con el advenimiento de los inhibidores de la enzima convertidora de angiotensina. La afección pulmonar puede ocurrir como hipertensión o enfermedad intersticial difusa; esta última es más común en pacientes con la variedad generalizada, en los primeros tres años de la enfermedad. Tiene mal pronóstico si no se inicia el tratamiento, cuya meta es detener el deterioro de la función pulmonar. Entre los tratamientos disponibles, la ciclofosfamida cuenta con la mejor evidencia, pero se están estudiando, con resultados preliminares satisfactorios, otros tratamientos como micofenolato mofetil, rituximab, trasplante autólogo de células hematopoyéticas y trasplante de pulmón. Objetivo: describir, de acuerdo con lo reportado en la literatura, la epidemiología, la fisiopatología, los métodos diagnósticos y el tratamiento de la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Métodos: revisión estructurada, no sistemática de la literatura, enfocada en los aspectos de interés, mencionados y que incluyó 52 artículos.

SUMMARY Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by the triad of small vessel vasculopathy, immune system activation and increased deposits of extracellular matrix in the skin and internal organs. In the last few years, pulmonary involvement has gained relevance since the introduction of angiotensin enzyme converter inhibitors with the subsequent decline in scleroderma renal crisis mortality, transforming scleroderma lung disease in the leading cause of mortality. Pulmonary involvement can manifest as hypertension or interstitial lung disease, which usually occurs in patients with generalized scleroderma in the first three years of the disease. Its prognosis is poor without treatment which is aimed at stopping pulmonary function deterioration. Among treatment options, cyclophosphamide has the best evidence, and mycophenolate mofetil, rituximab, and stem cell and lung transplantation are currently under investigation with positive preliminary results. Objective: To describe, according to reports in the literature, epidemiology, pathophysiology, diagnostic methods, and treatment of interstitial lung disease in systemic sclerosis. Methods: Structured, non-systematic literature review, focused on the aforementioned aspects of interest. It included 52 articles.

RESUMO Introdução: a esclerose sistémica é uma doença autoimune crônica, caracterizada pela tríada de vasculopatia de pequenos vasos, ativação do sistema imune e aumento dos depósitos de matriz extracelular tanto na pele como nos órgãos internos. Nos últimos anos, o compromisso pulmonar há cobrado grande importância, passando a ser a primeira causa de morte, dada a diminuição da mortalidade por crises renais com o advento dos inibidores da enzima convertedora de angiotensina. A afecção pulmonar pode ocorrer como hipertensão ou doença intersticial difusa; esta última é mais comum em pacientes com a variedade generalizada, nos primeiros três anos da doença. Tem mal prognóstico se não se inicia o tratamento, cuja meta é deter o deterioro da função pulmonar. Entre os tratamentos disponíveis, a ciclofosfamida conta com a melhor evidência, mas se está estudando, com resultados preliminares satisfatórios, outros tratamentos como micofenolato mofetil, rituximab, transplante autólogo de células hematopoiéticas e transplante de pulmão. Objetivo: Descrever, de acordo com o reportado na literatura, a epidemiologia, a fisiopatologia, os métodos diagnósticos e o tratamento da doença pulmonar intersticial em pacientes com esclerose sistémica. Métodos: revisão estruturada, não sistemática da literatura, enfocada nos aspectos de interesse, mencionados e que incluiu 52 artigos.

Caracterización clínica, serológica y patrón radiológico de una cohorte unicéntrica de pacientes con enfermedad pulmonar difusa / Clinical, serological and radiological pattern characterization of a monocentric cohort of patients with diffuse lung disease

Diffuse Lung Disease (DLD) is an important cause of morbidity and mortality, however in Chile we are lacking of epidemiological data on this condition. Our aim is communicate the first report of a prospective registry of DLD patients treated at bronchopulmonary unit of DIPRECA hospital. Methods: Cross-sectional study in patients referred to our bronchopulmonary unit under suspicion of DLD. Diagnosis was confirmed by chest computed tomography and informed consent was approved by patients. Data regarding clinical, serological, pulmonary function tests and echocardiography were collected from 2014 up to date. Results: 30 patients were analysed, their median of age was 76.5 years-old (Interquartile Range 68-80), 56.7% were women, median duration of disease: 4 years (IQR 1-10.6) and 43% has smoking history Most frequent signs and symptoms were crackles (97%), dyspnoea (90%) and cough (57%). Comorbidities: 3% had asthma, 3% chronic obstructive pulmonary disease and 6.7% connective tissue diseases. Radiological findings: 20% had DLD with usual interstitial pneumonia pattern (UIP), 23% DLD possible UIP, 30% DLD inconsistent with UIP, 14% chronic hypersensitivity pneumonitis and 13% nonspecific interstitial pneumonia. Serology: 18% had positive rheumatoid factor of which only one case had rheumatoid arthritis, 67% had positive antinuclear antibodies (ANA), 17% ANCA positive of which only one case of clinical vasculitis. Spirometry was mainly normal (52%) or restrictive (45%). Echocardiography showed pulmonary hypertension mainly mild in 52% of patients. No significant association was found between titles of ANA ≥ 1/320 and gender, smoking or radiological pattern. Conclusions: Our demographic and radiological findings are similar to those reported in literature; however, the highlights in our cohort are the increased frequency of female gender and positive ANA without history or clinical manifestation of connective tissue diseases.

La enfermedad pulmonar difusa (EPD) es causa importante de morbimortalidad; a pesar de esto no tenemos datos epidemiológicos en Chile. Nuestro objetivo es comunicar el primer reporte del registro prospectivo de pacientes con EPD atendidos en la unidad de broncopulmonar del hospital DIPRECA. Métodos: Estudio de corte transversal en pacientes derivados alpoliclínico broncopulmonar del hospital DIPRECA por sospecha de EPD. En caso de confirmación diagnóstica por tomografía computada de tórax y consentimiento informado aprobado por los pacientes, se compilaron datos clínicos, serológicos, pruebas de función pulmonar y ecocardiografía, desde 2014 hasta la fecha. Resultados: Se analizaron 30 pacientes la mediana de su edad fue 76,5 años (rango intercuartílico 68-80), 56,7% eran mujeres, duración mediana de la enfermedad: 4 años (RIC 1-10,6)y 43% con antecedentes de tabaquismo. Los síntomas y signos más frecuente fueron crujidos (97%), disnea (90%) y tos (57%). Comorbilidades: 3% tenía asma, 3% enfermedad pulmonar obstructiva crónica y 6,7% enfermedades del tejido conectivo. Hallazgos radiológicos: 20% tenía EPD con patrón de neumonía intersticial usual (UIP), 23% EPD posible UIP, 30% EPD inconsistente con UIP, 14% neumonitis por hipersensibilidad crónica y 13% neumonía intersticial no específica. Serología: 18% tenía factor reumatoide positivo de ellos sólo uno de los casos tenía artritis reumatoide, el 67% tenía anticuerpos antinucleares (ANA) positivos, 17% ANCA positivo de ellos sólo un caso tenía historia de vasculitis clínica. La espirometría fue mayoritariamente normal (52%) o restrictiva (45%). Ecocardiografía detectó hipertensión pulmonar mayoritariamente leve en 52% de los pacientes. No se encontró asociación significativa entre los títulos de ANA ≥ 1/320 en relación a género, tabaquismo o patrón radiológico. Conclusiones: Nuestros hallazgos demográficos y radiológicos son similares a los de la literatura; sin embargo, destaca en nuestra cohorte la mayor frecuencia de género femenino y ANA positivos sin historia o manifestación clínica de enfermedades del tejido conectivo.

Hard metal lung disease: a case series / Doença pulmonar por metal duro: uma série de casos

ABSTRACT Objective: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. Methods: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Results: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Conclusions: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis.

RESUMO Objetivo: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. Métodos: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013. Resultados: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3. Conclusões: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.

Combined acute interstitial pneumonitis and pancytopenia induced by low-dose methotrexate in a hemodialysis patient treated for bullous pemphigoid

Methotrexate has been widely used for many years in the treatment of a variety of diseases. Acute pneumonitis and bone marrow suppression are very serious side effects in methotrexate treatment. A 48-year-old man with end-stage renal disease undergoing chronic hemodialysis developed combined acute pneumonitis and pancytopenia after a cumulative dose of 20 mg methotrexate for bullous pemphigoid. Continuous renal replacement therapy (CRRT) can effi ciently decrease serum methotrexate concentration. A rapid improvement of clinical symptoms and resolution of pulmonary opacifi cation were found after CRRT. Blood cell counts returned to normal after component blood transfusion and cytokine supportive therapy. Patients with impaired renal function are at high risk of methotrexate toxicity, and low-dose methotrexate should be prescribed with great caution.

Hepatitis autoinmune y enfermedad pulmonar intersticial difusa: Una asociación poco frecuente: a propósito de un caso / A Patient with Autoimmune Hepatitis and Interstitial Lung Disease

Las enfermedades autoinmunes pueden comprometer el pulmón, como ocurre por ejemplo en las enfermedades del tejido conectivo, pero poco se ha publicado sobre la asociación entre la hepatitis autoinmune (HAI) y la enfermedad pulmonar intersticial (EPID). Se presenta una mujer de 57 años de edad, hipotiroidea, fumadora activa que consulta por tos seca y disnea. El examen físico reveló crepitantes aislados en bases pulmonares. La oximetría de pulso fue de 96%. El laboratorio mostró niveles elevados de las transaminasas, factor anti núcleo 1/80 y anticuerpo anti músculo liso 1/1280. La espirometría dejó en evidencia capacidad vital forzada del 85% y capacidad de difusión de monóxido de carbono del 53% del predicho. Ecografía hepática: hepatomegalia lobulada. Vías biliares intra y extrahepáticas conservadas, sin hipertensión portal. La biopsia hepática demostró infiltrado inflamatorio moderado y algunos plasmocitos con extensa hepatitis. A nivel acinar, se observaron focos necroinflamatorios aislados. Tricómico: bandas fibrosas gruesas que delimitan nódulos de regeneración. Tomografía de tórax: patrón reticular con vidrio esmerilado bilateral, algunas bronquiolectasias y sin panal de abeja. Se interpretó como HAI más EPID. La paciente rechazó la biopsia pulmonar. Hay muy pocos reportes en la literatura médica que describan la asociación entre la hepatopatía autoinmune y afectación intersticial pulmonar. Es importante determinar si la HAI comparte un mecanismo fisiopatológico común con la EPID, ya que tiene implicaciones diagnósticas y terapéuticas. Se deben tener en cuenta la HAI y la EPID, como enfermedades potencialmente relacionadas, con probables mecanismos fisiopatológicos en común.

Autoimmune diseases can compromise the lung, for example the connective tissue diseases, but little has been published about the association between autoimmune hepatitis (AH) and interstitial lung diseases (ILD). We present a 57 year old woman with hypothyroidism and current smoking habit who complains of dry cough and dyspnea. Physical examination detected isolated crackles in pulmonary bases. Pulse oximetry was 96%. Blood tests showed elevated aminotransferase levels, antinuclear antibody 1/80, anti-smooth muscle antibodies: 1/1280. Spirometry showed forced vital capacity of 85% of predicted value and diffusing capacity for carbon monoxide was 53% of predicted value. Hepatic ultrasonography showed diffuse, lobulated hepatomegaly. Intrahepatic and extrahepatic biliary tracts were preserved without portal hypertension. Liver biopsy demonstrated moderate inflammatory infiltrate and some plasmocytes with extensive hepatitis. At acinar level isolated necroinflammatory foci were observed. The Tricomic Technique showed thick fibrous bands that delimit regenerative nodules. The Chest Tomography showed diffuse interstitial opacities with bilateral ground glass, reticular pattern, few bronchiolectasis and no honeycombing. The case was interpreted as AH plus ILD. The patient refused lung biopsy. There are very few reports in the medical literature describing the association between autoimmune liver disease and pulmonary interstitial involvement. It is important to determine whether this type of autoimmune liver disease shares a common pathophysiological mechanism with ILD, because of the diagnostic and therapeutic implications (e.g. the decision to perform surgical lung biopsy) and possible prognostic significance. We should consider AH and ILD as related diseases with probable common pathophysiological mechanisms.