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.

Recomendaciones para la rehabilitación pulmonar en pacientes con COVID-19 / Recommendations for pulmonary rehabilitation in patients with COVID-19

Introducción: La COVID-19 es una enfermedad que desencadena infecciones respiratorias graves como el síndrome respiratorio agudo severo, que puede requerir atención prolongada intra y extrahospitalaria. Objetivo: Brindar recomendaciones y pautas para una correcta evaluación y tratamiento del paciente con COVID-19 mediante la rehabilitación pulmonar. Materiales y métodos: Revisión de la literatura en las bases de datos MEDLINE, PEDRO, Scielo y en Google Scholar, a partir de las palabras clave: COVID-19, Síndrome de Dificultad Respiratoria del Adulto, Enfermedades pulmonares, Rehabilitación, pruebas de función respiratoria, Unidad de cuidado intensivo; seleccionando, aquellos artículos que presentaran información sobre rehabilitación en pacientes con COVID-19 y otras alteraciones con compromiso similar como el Síndrome Respiratorio Agudo y las Enfermedades Pulmonares Intersticiales Difusas. Resultados: Para realizar una intervención en paciente con COVID-19 u otra alteración respiratoria como Síndrome de Dificultad Respiratoria del Adulto y Enfermedad Pulmonar Intersticial Difusa, es necesario realizar un adecuado diagnóstico de la enfermedad, una minuciosa evaluación integral y una intervención basada en las necesidades de cada individuo. Conclusiones: El paciente intrahospitalario puede recibir intervención enfocada a evitar el deterioro funcional y una vez son dados de alta la rehabilitación pulmonar extrahospitalaria podría ser una alternativa eficaz en el tratamiento para los pacientes.

Introduction: COVID-19 is a disease that triggers serious respiratory infections such as severe acute respiratory syndrome, which requires treatment on an inpatient and outpatient basis. Objective: To provide recommendations and guidelines for the appropriate assessment and treatment of COVID-19 patients through pulmonary rehabilitation. Materials and methods: A literature review using MEDLINE, PEDRO, Scielo and Google Scholar databases and the keywords: COVID-19, Adult Respiratory Distress Syndrome, Pulmonary diseases, Rehabilitation, Respiratory function tests, Intensive Care Unit. Articles that described information regarding rehabilitation in COVID-19 patients and other similar dysfunctions such as Acute Respiratory Syndrome and Diffuse Interstitial Lung Diseases. Results: It is necessary to carry out an appropriate disease diagnosis, a detailed integral assessment, and an intervention based on the needs of each patient in order to intervene with either COVID-19 patients or cases with other respiratory dysfunctions such as Adult Respiratory Distress Syndrome and Diffuse Interstitial Lung Disease. Conclusions: Inpatients can be subjected to an intervention focused on avoiding functional failure. Once they are discharged, outpatient pulmonary rehabilitation could be an effective treatment alternative for these patients.

Carboxyhemoglobinemia as a laboratory finding in an outpatient

Resumen El monóxido de carbono (CO) es un gas producido principalmente por combustión incompleta de hidrocarburos. La intoxicación por exposición ambiental puede presentarse con síntomas inespecíficos y constituye la causa más importante de aumento de carboxihemoglobina (COHb). Su nivel en sangre depende de la duración de la exposición, la ventilación minuto y las concentraciones de CO y oxígeno en el ambiente. La elevada toxicidad radica en la hipoxia tisular que se genera. Se presenta el caso de un paciente masculino, 73 años, en seguimiento en el hospital por neumonía intersticial no específica como patología de base. En un control de laboratorio se encontró 11,9% de COHb, sin exposición a tabaco. No utilizaba calefacción a gas sino un panel cerámico eléctrico, recientemente pintado con esmalte sintético. La suspensión del uso del panel normalizó la COHb. El CO, producto de descomposición térmica del esmalte sintético, explica la causa de la intoxicación.

Abstract Carbon monoxide (CO) is a gas produced mainly by incomplete combustion of hydrocarbons. Poisoning from environmental exposure can present with nonspecific symptoms and is the most important cause of increased carboxyhemoglobin (COHb). Its blood level depends on the duration of exposure, minute ventilation, and the concentrations of CO and oxygen in the environment. The high toxicity lies in the tissue hypoxia that is generated. The case of a male patient, 73 years old, under follow-up in the hospital for non-specific interstitial pneumonia as the underlying pathology was presented. In a laboratory control, COHb 11,9% was found. There was no exposure to tobacco and there was no use of gas heating but of an electric ceramic panel, recently painted with synthetic enamel type paint. The suspension of the use of the panel normalised the COHb. The CO product of thermal decomposition of synthetic enamel explains the cause of poisoning.

Resumo O monóxido de carbono (CO) é um gás produzido principalmente pela combustão incompleta de hidrocarbonetos. A intoxicação por exposição ambiental pode se apresentar com sintomas inespecíficos e é a causa mais importante de aumento da carboxihemoglobina (COHb). Seu nível em sangue depende do tempo de exposição, da ventilação minuto e das concentrações de CO e oxigênio no ambiente. A alta toxicidade está na hipóxia tecidual gerada. Apresentamos o caso de um paciente do sexo masculino, 73 anos, em acompanhamento hospitalar por pneumonia intersticial inespecífica como patologia de bas. Em um controle laboratorial, achou-se 11,9% de COHb, sem exposição ao tabaco. Não utilizava aquecimento a gás e sim um painel elétrico cerâmico, recentemente pintado com esmalte sintético. A suspensão do uso do painel normalizou o COHb. O CO produto da decomposição térmica do esmalte sintético explica a causa da intoxicação.

Impacto del ejercicio aeróbico en enfermedades pulmonares intersticiales / Impact of aerobic exercise on patients with interstitial lung diseases

Resumen Introducción: El tratamiento integral para pacientes con enfermedades pulmonares intersticiales incluye entrenamiento físico, dados los efectos fisiológicos que se generan. El objetivo de este estudio es analizar el impacto del ejercicio aeróbico en el tratamiento para los pacientes con enfermedades pulmonares intersticiales. Métodos: Se realizaron búsquedas de ensayos clínicos (enero 2015- junio 2020) en bases de datos indexadas tales como: Ebsco, Scopus, Medline, Medscape, PubMed Central, EMBASE, Redalyc, DOAJ y OVID. Se utilizó la estrategia PICO, Cochrane y escala de PEDro en cada estudio seleccionado. Resultados: Se incluyeron 11 ensayos clínicos para esta revisión y la calidad de los estudios fue media-alta. Todos los estudios incluyeron el componente de entrenamiento con ejercicio aeróbico combinado con otras estrategias como el desarrollo de fuerza, resistencia y flexibilidad. Se demostraron cambios significativos en más del 50% de los estudios para el componente de la capacidad aeróbica, consumo máximo de oxígeno, función cardiovascular y calidad de vida. Sin embargo, otros estudios, no demostraron diferencias significativas en pruebas de función pulmonar, cambios en variables ventilatorias, de intercambio gaseoso o cardiovasculares tal como señalan algunos autores en los estudios realizados. Conclusiones: El ejercicio aeróbico demostró ser un componente que mejoró resultados en la capacidad aeróbica, evidenciados en la distancia y metros recorridos post caminata de 6 minutos, así como en la calidad de vida, consumo máximo de oxígeno, fuerza y resistencia, mas no en variables ventilatorias e intercambio gaseoso.

Abstract Introduction: Comprehensive treatment for patients with interstitial lung diseases includes physical training, given the physiological effects that are generated. The objective of this study is to analyze the impact of aerobic exercise in the treatment for patients with interstitial lung diseases. Methods: Clinical trials (January 2015- June 2020) were searched in indexed databases such as Ebsco, Scopus, Medline, Medscape, PubMed Central, EMBASE, Redalyc, DOAJ and OVID. PICO, Cochrane and PEDro scale were used in each selected study. Results: Eleven clinical trials were included for this review and the quality of the studies was medium to high. All studies included the training component of aerobic exercise combined with other strategies such as strength development, endurance, flexibility. Significant changes were demonstrated in over 50% of the studies for the aerobic capacity component, maximum oxygen consumption, cardiovascular function and quality of life. Other studies, however, did not demonstrate significant differences in lung function tests, changes in ventilatory, gas exchange or cardiovascular variables as indicated by some authors in the studies conducted. Conclusions: Aerobic exercise proved to be a component that improved results in aerobic capacity evidenced in distance and meters traveled after a 6-minute walk, as well as in quality of life, maximum oxygen consumption, strength and resistance, but not in ventilatory variables and exchange gaseous.

Síndrome antisintetasa: reporte de un caso / Antisynthetase syndrome: a case repor

El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.

Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.

Manifestaciones pulmonares al diagnóstico de 170 pacientes con vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos: cohorte retrospectiva de un centro universitario / Pulmonary manifestations at the diagnosis of 170 patients with vasculitis associated with anticytoplasmic neutrophil antibodies: retrospective cohort of a university center

INTRODUCCIÓN: Las Vasculitis Asociadas a Anticuerpos Anticitoplasma de Neutrófilos (VAA) son infrecuentes, pero de amplio espectro de presentación. Si bien el consenso de clasificación de Chapel Hill del año 2012, sigue vigente, la tendencia actual es clasificarlas de acuerdo al marcador inmunológico: anti-Proteinasa 3 (PR3) o anti-mieloperoxidasa (MPO). Las manifestaciones pulmonares clásicas son la hemorragia alveolar y los nódulos pulmonares. En los últimos 10 años se ha descrito la enfermedad pulmonar difusa (EPD). Los estudios epidemiológicos son escasos, y suelen representar en su mayoría poblaciones norteamericanas o europeas. El objetivo es describir las características del compromiso pulmonar al debut en VAA en un centro universitario. PACIENTES Y MÉTODO: De un total de 190 pacientes con diagnóstico de VAA se incluyeron 170 en seguimiento en nuestro centro. Se revisaron aspectos clínicos, demográficos, laboratorio e imagenológicos de los pacientes incluidos. RESULTADOS: De los 170 pacientes, 112 (65,88%) presentaron compromiso pulmonar. 106 (94,64%) de los pacientes fueron anticuerpos anti citoplasma de neutrófilos (ANCA) positivos; de estos, 56 (53,27%) MPO (+) y 39 (36,45%) PR-3 (+). Un tercio de los pacientes de ambos grupos presentó hemorragia alveolar. En los pacientes MPO (+) predomina la EPD (53,5%) y en PR-3 (+) los nódulos pulmonares (69,23%). Destaca la baja frecuencia de patología obstructiva asociada. CONCLUSIONES: El compromiso pulmonar en las VAA es prevalente y heterogéneo. En nuestra serie, destaca la frecuencia de EPD en VAA MPO (+), lo que releva la importancia del estudio con ANCA en paciente con diagnóstico y seguimiento por EPD.

INTRODUCTION: Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis (AAV) are uncommon, but of broad spectrum of presentation. Although the 2012 Chapel Hill classification consensus remains valid, the current trend is to classify them according to the immunological marker: anti-Proteinase 3 antibody (PR-3) or anti-Myeloperoxidase antibody (MPO). The classic pulmonary manifestations are alveolar hemorrhage and pulmonary nodules. Interstitial lung disease (ILD) has been described in the last 10 years. Epidemiological studies are scarce, and they usually represent mostly North American or European populations. The objective is to describe the characteristics of lung involvement upon debut in AAV in a university center. PATIENTS AND METHODS: Of a total of 190 patients diagnosed with AAV, 170 were included in follow-up at our center. Clinical, demographic, laboratory and imaging aspects of the included patients were reviewed. RESULTS: Of the 170 patients, 112 (65.88%) had lung involvement. 106 (94.64%) of the patients were ANCA (+); of these, 56 (53.27%) MPO (+) and 39 (36.45%) PR-3 (+). One third of the patients in both groups had alveolar hemorrhage. In MPO (+) patients, ILD predominates (53.5%) and in PR-3 (+) pulmonary nodules (69.23%). The low frequency of associated obstructive pathology stands out. CONCLUSIONS: Pulmonary manifestations in AAVs are frequent and heterogeneous. Locally, the association of ILD and AAV MPO (+) stands out, which highlights the importance of ANCA study in patients with diagnosis and follow-up by ILD.

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.

Taquipnea persistente de la infancia: hiperplasia de células neuroendocrinas, una revisión / Persistent tachypnea of infancy: neuroendocrine cell hyperplasia, a review

La hiperplasia de células neuroendocrinas de la infancia (HCNEI) constituye una de las enfermedades intersticiales más frecuentes en pediatría. Tanto su etiología como los mecanismos fisiopatológicos involucrados son inciertos. Suele presentarse en pacientes por lo demás sanos, durante los primeros meses de vida con taquipnea, retracciones costales, rales e hipoxemia. En la tomografía axial computada de tórax de alta resolución (TACAR) presenta imágenes características en vidrio esmerilado de distribución central y zonas de atrapamiento aéreo. Para el diagnóstico, además de la clínica y la TACAR, podemos recurrir a la biopsia en casos atípicos. Los hallazgos histológicos reflejan una arquitectura pulmonar normal y un aumento en el número de células neuroendocrinas. El manejo global es con medidas de sostén, ya que no se cuenta con un tratamiento específico. La sintomatología suele mejorar con la edad y el pronóstico es favorable.

Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases of childhood. The etiology and pathophysiological mechanisms involved are uncertain. It usually presents in otherwise healthy patients during the first months of life with tachypnea, rib retractions, crackles, and hypoxemia. High-resolution chest computed tomography (HRCT) shows ground-glass opacities of central distribution and areas of air trapping. For diagnosis purposes, in addition to clinical and HRCT features, a lung biopsy is indicated for atypical cases. Histological findings reflect normal architecture and an increased number of neuroendocrine cells. The management consists of supportive and preventive care, since there is no specific treatment. Symptoms usually improve with age and the prognosis is favorable.

Prevalence of latent tuberculosis infection among patients with interstitial lung disease requiring immunosuppression / Prevalência de infecção latente por tuberculose em pacientes com doença pulmonar intersticial com necessidade de imunossupressão

ABSTRACT Objective To characterize the prevalence of latent tuberculosis infection (LTBI) in patients with interstitial lung diseases (ILDs) requiring immunosuppression. Only 5 to 10% of individuals infected with Mycobacterium tuberculosis develop tuberculosis, and certain groups of patients have an increased risk of illness, such as the immunocompromised. Patients with ILDs are frequently treated with immunosuppressants and, therefore, might have a higher risk of developing the disease. Methods Prospective study conducted at the ILD reference center of the Federal University of Paraná from January 2019 to December 2020. The screening of LTBI was performed with the use of the tuberculin skin test (TST). Results The sample consisted of 88 patients, of whom 64.8% were women, with a mean age of 61.4 years. The most frequent diagnoses were autoimmune rheumatic disease ILD (38.6%) and hypersensitivity pneumonitis (35.2%). The most common immunosuppressant in use at the time of the TST was prednisone, either in combination with mycophenolate (19.3%) or alone (17.1%). The majority of participants had fibrotic lung disease, characterized by a reticular interstitial pattern on chest computed tomography (79.5%) and moderate to severe functional impairment (mean FVC 69.2%). A prevalence of LTBI of 9.1% (CI 95%, 2.1%-15.1%) was found, with a TST median of 13. Conclusion Patients with ILD who are treated with immunosuppressants are not commonly screened for LTBI, despite being under a greater risk of progression to active disease. This study suggests the need for a more cautious approach to these patients.

RESUMO Objetivo Caracterizar a prevalência de Infecção Latente por Tuberculose (ILTB) em pacientes com Doenças Pulmonares Intersticiais (DPIs) que necessitam de imunossupressão. Apenas 5 a 10% dos indivíduos infectados pelo Mycobacterium tuberculosis desenvolvem tuberculose, sendo que certos grupos de pacientes apresentam maior risco de doença, tais como os imunocomprometidos. Pacientes com DPIs são frequentemente tratados com imunossupressores, portanto, podem apresentar maior risco de desenvolver a doença. Métodos Estudo prospectivo conduzido no Centro de Referência para DPI da Universidade Federal do Paraná (UFPR), entre Janeiro de 2019 e Dezembro de 2020. O rastreio de ILTB foi realizado por meio da Prova Tuberculínica (PT). Resultados A amostra foi composta por 88 pacientes, dos quais 64,8% eram mulheres, com, em média, 61,4 anos de idade. Os diagnósticos mais frequentes foram DPI associada a doença reumática autoimune (DRAI) (38,6%) e pneumonite de hipersensibilidade (35,2%). Prednisona foi o imunossupressor mais comumente utilizado à época da PT, em combinação com micofenolato (19,3%) ou isoladamente (17,1%). A maioria dos participantes tinha doença pulmonar fibrótica, caracterizada por infiltrado reticular em tomografia computadorizada de tórax (79,5%), bem como comprometimento funcional moderado a grave (Capacidade Vital Forçada (CVF) média de 69,2%). Observou-se uma prevalência de ILTB de 9,1% (Intervalo de Confiança (IC) 95%, 2,1%-15,1%), com mediana da PT de 13. Conclusão Não é comum que pacientes com DPI tratados com imunossupressores sejam avaliados quanto à presença de ILTB, apesar de estarem sob um maior risco de progressão para doença ativa. Este estudo sugeriu a necessidade de uma abordagem mais cuidadosa em relação a esses pacientes.

Acometimento da função ventricular direita em pacientes com esclerose sistêmica e doença pulmonar intersticial: avaliação pelo doppler tecidual / Impairment of right ventricular function in patients with systemic sclerosis and interstitial lung disease: tissue doppler evaluation

Introdução: A esclerose sistêmica (ES) é uma doença autoimune do tecido conjuntivo que cursa com fibrose e disfunção microvascular. O envolvimento dos órgãos viscerais, incluindo os pulmões e o coração, é a principal causa de óbito na ES. Nesse contexto, analisamos a relação entre os parâmetros ventriculares direitos (VD) pela ecocardiografia com Doppler tecidual e o acometimento pulmonar em pacientes com ES. Métodos: Os pacientes que preencheram os Critérios de Classificação da ES de 2013 foram submetidos à ecocardiografia com Doppler tecidual para avaliação da função sistólica (fração de ejeção) ventricular esquerda (VE), enquanto a função sistólica do VD foi avaliada por meio da fração de variação de área do VD (fractional area change ­ FAC), velocidade (sistólica) do Doppler tecidual, índice de desempenho miocárdico (IDM) e excursão sistólica do plano anular tricúspide (TAPSE). A pressão sistólica pulmonar foi estimada por insuficiência tricúspide. A tomografia computadorizada de alta resolução (TCAR) de tórax avaliou a presença de fibrose pulmonar. De acordo com os resultados da TCAR, os pacientes foram divididos em 2 subgrupos: Grupo I, incluindo pacientes com fibrose pulmonar (n=26), e Grupo II sem fibrose (n=17). Resultados: Entre os 43 pacientes com ES, a maioria era do sexo feminino (86%) com idade de 51±12 anos. Todos os pacientes apresentavam função ventricular sistólica normal, avaliada pela FEVE>55% e FAC VD>35%. Não houve diferença significativa em termos de idade ou duração da doença para os grupos. Exceto pela diminuição das velocidades do Doppler tecidual em pacientes com fibrose pulmonar, todos os índices de desempenho do VD foram semelhantes. Conclusão: Em pacientes com ES e fibrose pulmonar, o Doppler tecidual identifica acometimento miocárdico longitudinal precoce do VD, apesar do desempenho sistólico radial preservado do VD.(AU)

Introduction: Systemic sclerosis (SSc) is an autoimmune tissue connective disease that courses with fibrosis and microvascular dysfunction. Involvement of the visceral organs, including the lungs and heart, is the main cause of death among patients with SSc. In this context, here we analyzed the relationship between right ventricle (RV) parameters assessed by tissue Doppler echocardiography and lung involvement in patients with SSc. Methods: Patients fulfilling the 2013 SSc Classification Criteria underwent tissue Doppler echocardiography for the assessment of left ventricular (LV) systolic function (ejection fraction) and RV fractional area change (FAC), tissue Doppler s' (systolic) velocity, myocardial performance index, and tricuspid annular plane systolic excursion for the assessment of RV systolic function. Pulmonary systolic pressure was estimated using tricuspid regurgitation. Chest high-resolution computed tomography was used to evaluate the presence of pulmonary fibrosis. The patients were divided into two subgroups accordingly: Group I, patients with pulmonary fibrosis (n=26); and Group II, those without fibrosis (n=17). Results: Among the 43 patients with SSc, most were female (86%), and the mean age was 51 ± 12 years. All patients had normal systolic ventricular function as evidenced by an LV ejection fraction > 55% and an RV FAC > 35%. No significant intergroup difference was noted in age or disease duration. Except for a decreased tissue Doppler s' velocity in patients with lung fibrosis, all indexes of RV performance were similar. Conclusion: In patients with SSc and pulmonary fibrosis, tissue Doppler identified early RV longitudinal myocardial involvement despite preserved RV radial systolic performance.(AU)

Oxigenoterapia domiciliaria y capacidad aeróbica en pacientes con enfermedad pulmonar intersticial difusa. Estudio transversal / Home Oxygen Therapy And Aerobic Capacity In Patients With Diffuse Interstitial Lung Disease. Cross-Sectional Study

Introducción: La enfermedad pulmonar intersticial difusa es un grupo de enfermedades que causan un trastorno de la capacidad aeróbica y calidad de vida, además, ocasionan una gran tasa de morbimortalidad para esta población. El uso de oxigenoterapia domiciliaria mayor a 15 horas diarias tiene beneficios en pacientes hipoxémicos crónicos, sin embargo, poco se ha comparado su uso con pacientes que no lo reciben. Objetivo: Describir las características clínicas, capacidad aeróbica funcional y calidad de vida relacionada con la salud de dos grupos de pacientes con enfermedad pulmonar intersticial difusa, uno con indicación de oxigenoterapia domiciliaria y otro grupo sin indicación. Métodos: Estudio descriptivo transversal, que incluyó 41 pacientes con enfermedad pulmonar intersticial difusa que firmaron consentimiento informado. En ambos grupos, características demográficas y clínicas, ansiedad/depresión, calidad de vida relacionada con la salud con el cuestionario Saint George y la capacidad aeróbica funcional con la prueba de marcha de seis minutos fueron medidas. Se compararon los grupos con la prueba t de student para muestras independientes. Resultados: El grupo enfermedad pulmonar intersticial difusa con oxigenoterapia domiciliaria presentó mayor porcentaje de antecedente de tabaquismo (p = 0,041), menor distancia caminada en la prueba de marcha de seis minutos (304,1 ± 108,7 vs. 390,3 ± 95,6 p = 0,01), y menor porcentaje de la distancia predicha (58,37 ± 20,45 vs. 73,34 ± 22,90, p = 0,034) frente al grupo enfermedad pulmonar intersticial difusa sin oxigenoterapia domiciliaria. Conclusiones: Los pacientes con enfermedad pulmonar intersticial difusa con indicación de oxigenoterapia domiciliaria presentan menor capacidad aeróbica funcional comparada con pacientes sin indicación(AU)

Introduction: Diffuse interstitial lung disease is a group of diseases that cause a disorder of aerobic capacity and quality of life; in addition, they cause a high rate of morbidity and mortality for this population. The use of home oxygen therapy greater than 15 hours a day has benefits in chronic hypoxemic patients, however, little has been compared to patients who do not receive it. Objective: Describe the clinical characteristics, functional aerobic capacity and health-related quality of life of two groups of patients with diffuse interstitial lung disease, one with indication for home oxygen therapy and another group without indication. Methods: A cross-sectional descriptive study included 41 patients with diffuse interstitial lung disease who signed informed consent. In both groups, demographic and clinical characteristics, anxiety/depression, health quality of life related with the Saint George questionnaire, and functional aerobic capacity with the six-minute gait test were measured. The groups were compared with the student's t-test for independent samples. Results: The diffuse interstitial lung disease group with home oxygen therapy presented a higher percentage of smoking history (p = 0.041), a shorter distance walked in the six-minute gait test (304.1 ± 108.7 vs. 390.3 ± 95.6 p = 0.01), and a lower percentage of the predicted distance (58.37 ± 20.45 vs. 73.34 ± 22.90, p = 0.034) compared to the diffuse interstitial lung disease group without home oxygen therapy. Conclusions: Patients with diffuse interstitial lung disease with indication of home oxygen therapy have lower functional aerobic capacity compared to patients without indication(AU)

Expert consensus on the diagnosis and treatment of anticancer drug-induced interstitial lung disease / 中华肿瘤杂志

Drug-induced interstitial lung disease (DILD) is the most common pulmonary adverse events caused by anti-cancer treatment. In recent years, with the development of clinical oncology, a large amount of novel anti-cancer drugs have been approved and widely used in clinical practice, and the incidence of anti-cancer drug related DILD is gradually increasing. DILD lacks specific clinical manifestations or diagnostic criteria. If not treated properly, it may leads to interruption or discontinuation of anti-cancer treatment, or even become life threat in severe cases. Therefore, the Anti-cancer Drug-induced Interstitial Lung Disease Management Group have reached a consensus on the diagnosis and management of anti-cancer DILD after several rounds of discussion. This consensus aims to improve clinicians' awareness of anti-cancer drug related-DILD and proposes an algorithm for the diagnosis and treatment of this disease, and to improve patients' prognosis and quality of life.

Interstitial lung disease due to anti-TNF use in the treatment of psoriasis

Abstract Psoriasis is a chronic inflammatory disease that affects the skin variably, according to genetic and environmental factors. Some patients may benefit from systemic treatment with immunobiological agents, drugs that can be accompanied by several adverse effects. A case of a 58-year-old patient undergoing treatment for psoriasis with adalimumab for five years is reported. Alterations compatible with interstitial pneumonia were detected with important regression after adalimumab discontinuation. This case is relevant due to the scarcity of reports on late pulmonary adverse effect of anti-TNF treatment of psoriasis.

Herpetic whitlow in a child with AIDS: the importance of Tzanck test in the diagnosis

Abstract Herpetic whitlow is a viral infection of the fingers caused by the herpes simplex virus. The disease has a bimodal age distribution, affecting children under 10 years of age and young adults between 20 and 30 years old. It can be easily mistaken for panaritium or bacterial cellulitis. In patients with AIDS, atypical, chronic and recurrent ulcerated lesions occur. The Tzanck test allows a quick and low-cost diagnosis of herpes simplex virus infection. The authors report the case of a child with AIDS with painful finger ulcers in which the diagnosis was confirmed by the Tzanck test.

Disfunción del transportador del surfactante pulmonar ABCA3: reporte de un caso peruano / Surfactant ABCA3 transporter dysfunction: a case report from Peru

Resumen Introducción: Los trastornos genéticos que afectan la homeostasis del surfactante pulmonar son una causa importante del síndrome de dificultad respiratoria en el recién nacido a término y de enfermedad pulmonar intersticial difusa en niños. El transportador ABCA3 (ATP binding cassette A3) interviene en la producción normal del surfactante que recubre el interior de las paredes alveolares y funciona como agente tensioactivo. Caso clínico: Recién nacido a término que presentó dificultad respiratoria a los 3 días de vida y requirió ventilación mecánica. Los estudios para determinar otras causas de enfermedad pulmonar fueron negativos. Se realizó una biopsia de pulmón para realizar estudios de microscopía óptica y microscopía electrónica. Esta última mostró pequeños cuerpos lamelares anómalos, además de condensaciones electrodensas periféricas, características de las mutaciones del transportador ABCA3. Se inició tratamiento con pulsos de metilprednisolona, hidroxicloroquina, azitromicina y corticoides inhalados a dosis altas, y la respuesta clínica y radiológica fue favorable durante el seguimiento. Conclusiones: La correlación de las características clínicas y de las imágenes (tomografía y microscopía electrónica) puede ser útil para el diagnóstico de la disfunción del surfactante pulmonar, especialmente en los países de bajos y medianos recursos que no disponen de estudios genéticos para determinar las diferentes mutaciones del transportador ABCA3. Este es uno de los primeros casos reportados en Perú con respuesta adecuada al tratamiento y evolución favorable durante el seguimiento.

Abstract Background: Genetic disorders affecting pulmonary surfactant homeostasis are a major cause of respiratory distress syndrome in full-term newborn and childhood interstitial lung disease. The ABCA3 transporter (ATP binding cassette A3) intervenes in the normal production of surfactant that covers the interior of alveolar walls and plays a fundamental role as a surfactant. Case report: Male term newborn who presented respiratory distress 3 days after birth and required mechanical ventilation. Studies to determine other causes of lung disease were negative. Lung biopsy was performed for the study with light microscopy and electron microscopy. Electron microscopy showed small abnormal lamellar bodies in addition to peripheral electrodense condensations characteristic of ABCA3 transporter mutation. Treatment was started with pulses of methylprednisolone, hydroxychloroquine, azithromycin, and high-dose inhaled corticosteroids, finding a favorable clinical and radiological response to follow-up. Conclusions: Correlation of clinical characteristics and images (tomography and electron microscopy) can be useful for the diagnosis of lung surfactant dysfunction, especially in low and medium-income countries where genetic studies to determine the different ABCA3 transporter mutations are not available. This is one of the first cases reported in Peru with an adequate response to treatment and favorable evolution to follow-up.

Neumonía Intersticial con características autoinmunes (IPAF): Reporte de casos / Interstitial pneumonia with autoimmune features (IPAF): Case reports

IPAF agrupa individuos con EPID y otras características clínicas, serológicas o pulmonares que derivan de una condición autoinmune sistémica subyacente, pero no cumplen con los criterios reumatológicos actuales para una ETC. La EPID, manifestación clínica frecuente de las ETC, puede aparecer en el contexto de una ETC conocida pero no es infrecuente que sea la primera y única manifestación de un ETC oculta. Identificar una ETC subyacente en pacientes que presentan con compromiso intersticial inicial puede ser un desafío; tales evaluaciones pueden optimizarse mediante un enfoque multidisciplinario. Presentamos el caso de tres pacientes, de diferente presentación, evolución y tratamiento, todos caracterizados hasta la fecha como IPAF.

IPAF groups individuals with ILD and other clinical, serologic, or pulmonary manifestations with an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a CTD. ILD is a frequent clinical manifestation of CTDs; may appear in the context of a well known CTD but is often the first and only manifestation of an unknown CTD. Identifying an underlying CTD in patients presenting with initial interstitial involvement can be challenging; such evaluations can be optimized using a multidisciplinary approach. We present the case of three patients, of different presentation, evolution and treatment, all characterized to date as IPAF.

Uniportal surgical biopsy, without orotraqueal intubation, without thoracic drainage in intersticial pulmonary disease: initial results / Biópsia cirúrgica uniportal, sem intubação orotraqueal, sem drenagem torácica na doença pulmonar intersticial: resultados iniciais

ABSTRACT Objective: interstitial lung disease comprises a group of lung diseases with wide pathophysiological varieties. This paper aims to report the video thoracoscopic surgical biopsy in patients with interstitial lung disease through a single minimal chest incision, without orotracheal intubation, without chest drainage, and without the use of neuromuscular blockers. Methods: this study is a series of 14 cases evaluated retrospectively, descriptively, where patients underwent a pulmonary surgical biopsy from January 2019 to January 2020. The patients included in the study had diffuse interstitial lung disease without a defined etiological diagnosis. Results: none of the patients had transoperative complications, there was no need for chest drainage in the postoperative period, and the patients pain, assessed using the verbal scale, had a mode of 2 (minimum value of 1 and maximum of 4) in the post immediate surgery and 1 (minimum value of 1 and maximum of 3) at the time of hospital discharge. The length of hospital stay was up to 24 hours, with 12 patients being discharged on the same day of hospitalization. Conclusion: therefore, it is concluded in this series of cases that the performance of uniportal video-assisted thoracoscopic surgery procedures to perform lung biopsies, without orotracheal intubation, without chest drainage, and without the use of neuromuscular blockers, bring benefits to the patient without compromising his safety. Further larger studies are necessary to confirm the safety and efficiency of this method.

RESUMO Objetivo: a doença pulmonar intersticial compreende um grupo de doenças pulmonares com grandes variedades fisiopatológicas. Este trabalho objetiva relatar a biópsia cirúrgica videotoracoscópica em pacientes com doença pulmonar intersticial por meio de incisão torácica mínima única, sem intubação orotraqueal, sem drenagem torácica e sem uso de bloqueadores neuromusculares. Métodos: este estudo é uma série de 14 casos avaliados de forma retrospectiva, descritiva, onde no qual os pacientes foram submetidos a biópsia cirúrgica pulmonar no período de janeiro de 2019 a janeiro de 2020. Os pacientes incluídos na pesquisa, apresentavam doença pulmonar intersticial difusa sem diagnóstico etiológico definido. Resultados: nenhum dos pacientes apresentou complicações transoperatórias, não houve necessidade de drenagem torácica no período pós-operatório e a dor dos pacientes, avaliada por meio da escala verbal, teve moda de 2 (valores mínimos de 1 e máximo de 4) no período de pós-operatório imediato e 1 (valores mínimos de 1 e máximos de 3) no momento da alta hospitalar. O tempo de permanência hospitalar foi de até 24 horas, sendo que 12 pacientes receberam alta no mesmo dia da internação. Conclusão: conclui-se, assim, que nesta série de casos, a realização de procedimentos de cirurgia toracoscópica videoassistida uniportais para realização de biópsias pulmonares, sem intubação orotraqueal, sem drenagem torácica e sem uso de bloqueadores neuromusculares trazem benefícios para o paciente sem comprometer sua segurança. Estudos maiores são necessários para comprovar tanto a segurança quanto à eficácia deste método.

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.

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.