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.

Fisiología respiratoria: fisiología de las enfermedades pulmonares restrictivas / Pathophysiology of pulmonary restrictive diseases

Las enfermedades restrictivas comprenden un grupo heterogéneo de trastornos que se caracterizan por una alteración en la distensibilidad pulmonar, generada por enfermedades del parénquima o intersticio pulmonar o por problemas extrapulmonares (alteraciones de la caja torácica o enfermedades neuromusculares). Presentan un patrón característico en las pruebas de función pulmonar, relación VEF1/ FVC normal o aumentada con CVF disminuida, VEF1 disminuido leve o normal y capacidad pulmonar total disminuida (CPT). Su manejo es complejo debido a la dificultad para establecer el diagnóstico diferencial, por lo que se recomienda una derivación precoz a un especialista en enfermedades respiratorias y el enfrentamiento por un equipo multidisciplinario.

Restrictive diseases comprise a heterogeneous group of disorders characterized by an alteration in lung compliance, generated by diseases of the lung parenchyma or interstitium, as well as by extrapulmonary problems (abnormalities of the rib cage or neuromuscular diseases). They present a characteristic pattern in pulmonary function tests, with decreased FVC (forced vital capacity), slightly decreased or normal FEV1 (forced expiratory volume in 1 second), normal or increased FEV1/FVC ratio, and decreased total lung capacity. Its management is complex due to the difficulty in establishing the differential diagnosis, so early referral to a specialist in respiratory diseases and confrontation by a multidisciplinary team is recommended.

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.

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.

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.

Hiperplasia de células neuroendocrinas: reporte de caso / Neuroendocrine cell hyperplasia: case report

A clinical case of Neuroendocrine Cell Hyperplasia is presented with a bibliographic review. An infant patient with respiratory distress syndrome, characterized by nasal flaring, retractions, and tachypnea with temporary resolution with the use of bronchodilators. However, the patient requires oxygen. With complementary examinations (negative viral panel twice) and epidemiology it is classified as a viral Bronchiolitis. Without improvement, extrapulmonar pathologies were suspected, discarding hearth disease, epilepsy, pathological gastroesophageal reflux. New tests were performed to rule out other pathologies, including immunological disorders. Those results were normal, so a high-resolution chest tomography was done which allowed the diagnosis of Neuroendocrine Cell Hyperplasia. During the follow up the child had improved and required oxygen until he was two years old. Neuroendocrine Cell Hyperplasia belongs to a huge group of less common interstitial disorders, which diagnosis is clinical and radiological. It can easily be confused with common respiratory disorders. For this reason, it is important to know about this disease to make an early diagnosis. Most of the cases had a gradual (months to years) improvement.

Se presenta un caso clínico de Hiperplasia de Células Neuroendocrinas y la revisión de la literatura. Paciente lactante menor con cuadro de dificultad respiratoria, caracterizado por aleteo nasal, retracciones y taquipnea persistente acompañada de desaturación. Sin adecuada respuesta al uso de broncodilatadores. Por exámenes complementarios, panel viral negativo en dos ocasiones y epidemiología, se le diagnostica una bronquiolitis viral. Por no presentar mejoría se completan estudios, descartándose neumonía atípica, cardiopatía, epilepsia, reflujo gastroesofágico patológico y compromiso inmunológico. El diagnóstico fue determinado en base a la clínica, junto con imágenes en vidrio esmerilado característicos en lóbulo medio y língula. En su seguimiento mejora paulatinamente, requiriendo soporte de oxígeno hasta los dos años. La Hiperplasia de Células Neuroendocrinas es una patología intersticial pulmonar poco frecuente, cuyo diagnóstico es clínico y radiológico. Puede ser fácilmente confundida con desórdenes respiratorios comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en meses o en los primeros años de vida.

El intersticio pulmonar y sus enfermedades en la infancia. Deficiencia de ABCA3. Seguimiento durante doce años. Perspectivas ¿Un nuevo paradigma genético? / The pulmonary interstitium and its diseases in childhood. ABCA3 deficiency. Follow-up for 12 years. Perspectives. A new genetic paradigm?

Introducción. Sobre la base de un caso clínico, se presenta la descripción del cuadro intersticial por deficiencia de ABCA3, de una paciente de catorce años de edad, en seguimiento durante doce años. Método. Evaluación clínica con extensos estudios para descartar otras patologías semejantes. El diagnóstico definitivo fue determinado por el estudio genético para deficiencias de ABCA3 y otros defectos genéticos realizados por el Dr. Larry Nogee, Hospital Johns Hopkins, EE. UU. Objetivos. Describir detalladamente la evolución de la paciente durante doce años, con énfasis en los estudios anteriormente mencionados. Sugerir la presencia de un cambio de paradigma pronóstico en lo que se conocía sobre la evolución de esta enfermedad intersticial pulmonar grave, tratar de mejorar la calidad de vida y posiblemente el pronóstico. Presentar los hallazgos de genética, anatomía patológica y radiología en consultas y evaluaciones por centros de referencia. Resultados. Realizado su diagnóstico de deficiencia genética de ABCA3, presentamos su seguimiento actualizado hasta el año 2020. Esta debe ser sospechada en niños pequeños desde el nacimiento y durante los primeros años ante la persistencia de cuadros pulmonares crónicos con desaturación de oxígeno e imágenes tomográficas que sugieren cuadro intersticial. Se decidió tratar el cuadro en los años 2019-2020, durante seis meses, según bibliografía y consultas con centros de referencia en los Estados Unidos, con la finalidad de determinar la posible mejoría de su patología y decidir la continuación o suspensión de la medicación. Se usaron pulsos con metilprednisolona- hidroxicloroquina y azitromicina. Se logró mantener estable su función pulmonar y mejorar notablemente su calidad de vida. (AU)

Introduction. A clinical case diagnosed with ABCA3 deficiency is described. Patient is now fourteen years old. She´s being followed up since she was two years old. Methodology. clinical follow ­ up with extensive studies to rule out other similar pathologies. Final diagnosis was done through genetic studies done at Johns Hopkins Hospital by Nogee LM. Objective. To present a detailed evolution description of twelve years' follow-up with the support of the aforementioned studies, to suggest a change in diagnostic ­ prognostic paradigm on what was known of mortality in this severe pulmonary interstitial pathology to improve life quality and possibly prognosis. Present the findings of genetics, pathological anatomy and radiology in consultations and evaluations by reference centers. Results. Having made her diagnosis of genetic ABCA3 deficiency, we present her up dated follow-up until 2020. This should be suspected in young children from birth and during the first years due to the persistence of chronic pulmonary symptoms with oxygen desaturation and tomographic images that suggest interstitial symptoms. It was decided to treat the condition in the years 2019-2020, for six months, according to the bibliography and consultations with reference centers in the United States, in order to determine the possible improvement of her pathology and decide to continue or suspend the medication. Pulses with methylprednisolone hydroxychloroquine and azithromycin were used. Her lung function was stable and her quality of life significantly improved. (AU)

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.

Cánula nasal de alto flujo en lactantes: experiencia en una unidad de paciente crítico / High flow nasal cannula in infants: Experience in a critical patient unit

Introducción: La cánula nasal de alto flujo (CNAF) es un método de soporte respiratorio cada vez más utilizado en pediatría por sus resultados y seguridad. Objetivo: Determinar la efectividad de la CNAF, evaluar factores asociados a fracaso y complicaciones relacionadas con su uso en lactantes. Pacientes y método: Se analizaron los datos demográficos, clínicos, gasométricos, radiológicos y complicaciones de los pacientes conectados a CNAF en una unidad crítica entre junio de 2012 y septiembre de 2014. Se compararon los pacientes que fracasaron con los respondedores a CNAF, considerándose fracaso la necesidad de un mayor soporte respiratorio durante las primeras 48 h de conexión. Se utilizó test de Kolmogorov Smirnov, U de Mann-Whitney, Chi cuadrado, test exacto de Fisher, correlaciones y Modelo de regresión logística binaria para p ≤ 0,05. Resultados: Un total de 109 pacientes. Mediana de edad y peso: 1 mes (0,2-20 meses) y 3,7 kg (2-10 kg); percentil 95: 3,7 meses y 5,7 kg respectivamente. El diagnóstico y patrón radiológico más frecuente fue bronquiolitis (53,2%) e infiltrado intersticial (56%). Un 70,6% respondió. Hubo diferencia significativa entre fracaso y respuesta en el diagnóstico (p = 0,013), radiografía (p = 0,018), contexto de conexión (p < 0,0001), pCO2 (mediana 40,7 mm Hg [15,4-67 mm Hg] versus 47,3 mm Hg [28,6-71,3 mm Hg], p = 0,004) y horas de CNAF (mediana 60,75 h [5-621,5 h] versus 10,5 h [1-29 h], p < 0,0001). El OR de PCO2 ≥ 55 mm Hg para fracaso fue 2,97 (IC 95%: 1,08-8,17; p = 0,035). Ningún paciente falleció ni registró complicaciones. Conclusión: El porcentaje de éxito observado fue similar a lo publicado. En esta muestra el fracaso de CNAF solo se asoció a una pCO2 inicial ≥ 55 mm Hg. Su uso se consideró seguro al no reportarse complicaciones relacionadas a su utilización. Se requiere de un estudio multicéntrico, aleatorizado y controlado para contrastar estos resultados.

Introduction: The high flow nasal cannula (HFNC) is a method of respiratory support that is increasingly being used in paediatrics due to its results and safety. Objective: To determine the efficacy of HFNC, as well as to evaluate the factors related to its failure and complications associated with its use in infants. Patients and method: An analysis was performed on the demographic, clinical, blood gas, and radiological data, as well as the complications of patients connected to a HFNC in a critical care unit between June 2012 and September 2014. A comparison was made between the patients who failed and those who responded to HFNC. A failure was considered as the need for further respiratory support during the first 48 hours of connection. The Kolmogorov Smirnov, Mann-Whitney U, chi squared and the Exact Fisher test were used, as well as correlations and a binary logistic regression model for P ≤ .05. Results: The study included 109 patients, with a median age and weight: 1 month (0.2-20 months) and 3.7 kg (2-10 kg); 95 percentile: 3.7 months and 5.7 kg, respectively. The most frequent diagnosis and radiological pattern was bronchiolitis (53.2%) and interstitial infiltration (56%). Around 70.6% responded. There was a significant difference between failure and response in the diagnosis (P = .013), radiography (P = 018), connection context (P < .0001), pCO2 (median 40.7 mmHg [15.4-67 mmHg] versus 47.3 mmHg [28.6-71.3 mmHg], P = .004) and hours on HFNC (median 60.75 hrs [5-621.5 hrs] versus 10.5 hrs [1-29 hrs], P < .0001). The OR of the PCO2 ≥ 55 mmHg for failure was 2.97 (95% CI; 1.08-8.17; P = .035). No patient died and no complications were recorded. Conclusion: The percentage success observed was similar to that published. In this sample, the failure of HFNC was only associated with an initial pCO2 ≥ 55 mmHg. On there being no complications reported as regards it use, it is considered safe, although a randomised, controlled, multicentre study is required to compare and contrast these results.

Enfermedad pulmonar difusa en esclerodermia: diagnóstico y tratamiento / Intetstitial lung disease in scleroderma: diagnosis and treatment

Scleroderma is among the connective tissue diseases (CTD) with more pulmonary involvement. More than half of scleroderma patients have some kind of interstitial lung disease (ILD), and this is currently the leading cause of death due to the disease itself. The main risk factor for ILD is the autoantibodie profile, and the period of greatest risk of developing ILD is among the first 3-5 years of disease. The most common histopathological form is NSIP, but the histopathological subtype has no influence on prognosis or response to treatment. Given the fact that some ILD patients will remain stable and because of the lack of a really effective and risk free treatment, immunosuppressive therapy is generally reserved for patients with extensive and / or progressive disease. The main risk factors for progressive disease are pulmonary extent and functional impairment. With currently available therapies the more realistic goal is stabilization of lung disease. The most widely used immunosuppressive induction therapy is Cyclophosphamide (CYC), but there is enough evidence to support the use of Mycophenolate Mofetil (MMF). As maintenance therapy, options are MMF and Azathioprine. Periodic clinical and functional reassessment is of vital importance, to monitor functional progression and/or the response to immunosuppressive therapy...

La esclerodermia está entre las enfermedades del tejido conectivo (ETC) que con mayor frecuencia presentan enfermedad pulmonar difusa (EPD), y se encuentra en más de la mitad de los pacientes. Actualmente la EPD representa la principal causa de muerte atribuible a la propia enfermedad. El principal factor de riesgo es el perfil de autoanticuerpos, y el período de mayor riesgo de desarrollar EPD son los primeros tres a cinco años de enfermedad. La forma histopatológica más frecuente es neumonía intersticial no específica (NINE), pero el subtipo histopatológico no tiene mayor influencia en el pronóstico ni en la respuesta al tratamiento. Dada la existencia de pacientes con EPD intrínsecamente estable y a la ausencia de una terapia realmente efectiva y exenta de riesgos, la inmunosupresión se reserva en general para pacientes con enfermedad extensa y/o progresiva. Lo parámetros que mejor se han correlacionado con el riesgo de progresión son la extensión radiológica y el compromiso de la función pulmonar. Con las terapias actualmente disponibles, el objetivo más realista es la estabilización del compromiso pulmonar. El Inmunosupresor más utilizado para la terapia de inducción es ciclofosfamida (CYC), pero existe evidencia suficiente que avala el uso de micofenolato mofetil (MMF). Como terapia de mantención las principales opciones son azatioprina y MMF. La reevaluación clínica y funcional periódica es fundamental, para monitorizar la progresión y/o la respuesta al tratamiento inmunosupresor...

Doenças intersticiais pulmonares no CTI: Quando pensar? Como conduzir? / Interstitial lung disease in ICU: When to think? How to manage?

As doenças intersticiais pulmonares podem ser secundárias a um processo agudo ou resultar de uma inflamação e cicatrização progressiva do parênquima pulmonar. Elas podem ser desafiadoras na terapia intensiva, tanto para o diagnóstico quanto para o tratamento. A exclusão de causas reversíveis de insuficiência respiratória e medidas de suporte são os pontos principais da terapia. É fundamental a revisão das imagens radiológicas prévias e a realização de anamnese detalhada. A coleta de lavado broncoalveolar por broncoscopia e/ou biopsia cirúrgica podem ser necessárias. Nas exacerbações agudas é recomendado corticosteróide e antibióticos. Frequentemente é necessária a ventilação mecânica para melhorar a troca gasosa e a baixa complacência pulmonar. A insuficiência respiratória é um final comum das doenças intersticiais crônicas e apresentam um mau prognóstico. Devido a isto, a ventilação mecânica deve ser oferecida nos casos com processos patológicos reversíveis ou nos candidatos à transplante pulmonar...

Interstitial lung disease (ILD) can be an acute process or the result of progressive inflammation and scarring of the pulmonary parenchyma. They can be challenging in the ICU both to diagnose and treatment. Exclusion of reversible causes of respiratory failure while administering supportive care are the mainstay treatment of patients with ILD with respiratory failure. It is fundamental to review previous imaging, detailed medication and occupational history. Bronchoscopy with bronchoalveolar lavage and/or surgical lung biopsy may be recommended. If an exacerbation syndrome is identified, corticosteroids and antibiotics are advised. Often mechanical ventilation is needed to control poor compliance and gas exchange. Respiratory failure is a common end of chronic ILD and carries a poor outcome. Mechanical ventilation should only be offered in select cases, most notably patients with reversible processes or those considered candidates for lung transplantation...

Enfermedad pulmonar intersticial en la niñez. Ocho años de experiencia / Childhood interstitial lung disease. Eight years experience

Se presenta la casuística de 8 años (2005-2013) de 27 enfermos con cuadros intersticiales en seguimiento efectivo. No fueron incluidas las displasias bronco-pulmonares, el daño pulmonar crónico por virus, la fibrosis quística y la aspiración recurrente. Método. Revisión de Historias Clínicas; tomografías computadas y biopsias pulmonares en actividad multidisciplinaria coordinada. Se modificaron las técnicas quirúrgicas de biopsia, radiología (tomografía computada) y se consultó a centros extranjeros con mayor experiencia en 14 casos. Resultados. Se presentan resultados de tomografías y de la clínica. La patología diagnosticada más frecuente fue la hemorragia pulmonar con 4 hemosiderosis pulmonares idiopáticas y 2 de otro origen (sangrado por celiaquía y hemorragia pulmonar del lactante). La segunda frecuencia, 4 casos, correspondió a posibles defectos del surfactante con genética incluida. Está representado casi todo el espectro de la patología intersticial. Los cuadros intersticiales pulmonares en la infancia seguramente son más frecuentes que lo supuesto hasta la fecha. Deben ser diagnosticados y tratados por equipos multidisciplinarios con énfasis especial en la técnica de biopsia, la tomografía computada y el futuro desarrollo de estudios genéticos avanzados...

Twenty-seven cases of interstitial lung disease in children with complete follow-up are presented. Bronchopulmonary dysplasia, viral chronic lung damage, cystic fibrosis and recurrent aspiration were not included. Methods. Detailed revision of 27 clinical records during the period 2005-2013. Results. computerized tomography, clinical symptoms, lung biopsies and diagnosis are shown. Coordinated multidisciplinary organization is underlined as necessary. Foreign Centers with larger experience were consulted in 14 cases; biopsy technique as well as computed tomography were accordingly modified. Most frequent finding was pulmonary hemorrhage of different origin: 4 idiopathic pulmonary hemosiderosis cases; one bleeding due to celiac disease and one acute pulmonary hemorrhage of infancy. Four cases of possible surfactant deficiencies were suspected (genetic studies included). Almost the whole spectrum of interstitial lung disease was found and surely it shall be found to be more frequent as long as a coordinated multidisciplinary effort is undertaken...

Enfermedad pulmonar intersticial del lactante / Interstitial lung disease in infants

Interstitial lung disease is an heterogeneous group of illnesses. Some of them only affect children under two years old and new classifications have been proposed for this age group. Proper clinical awareness, X-ray studies like thorax high-resolution computed tomography, and lung biopsy are required for diagnosis. Clinically, there is tachipnea, intercostal retractions, rales, interstitial images at X-ray, hypoxemia and restrictive lung disease. Steroids are the most used treatment; in severe cases lung transplantation might be the sole option. Some of them presents a good prognosis; for others it is often fatal when it appears in the neonatal period.

La enfermedad pulmonar intersticial (EPI) constituye un grupo heterogéneo de patologías. Algunas son exclusivas de niños menores de 2 años, para los cuales se ha propuesto un nuevo esquema de clasificación. El diagnóstico requiere de sospecha clínica, estudio radiológico, y en general biopsia pulmonar. Hay presencia de taquipnea, retracciones, crepitaciones, hipoxemia, infiltrados intersticiales y enfermedad restrictiva. El tratamiento de elección son los corticoides, en casos severos el trasplante. Algunas presentan buen pronóstico, otras iniciadas en período neonatal tienen a menudo una evolución fatal.

Destaques das diretrizes de doenças pulmonares intersticiais da Sociedade Brasileira de Pneumologia e Tisiologia / Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases

As doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

Neumonía intersticial linfoidea asociada a inmunodeficiencia común variable / Interstitial lymphoid pneumonia associated with common variable immunodeficiency

La neumonía intersticial linfoidea (NIL) es una rara entidad, descripta como hiperplasia no neoplásica del tejido linfoide pulmonar y clasificada dentro de las enfermedades intersticiales idiopáticas. Se la ha descripto asociada a infección por HIV, fenómenos autoinmunes, hipergamaglobulinemia policlonal o menos frecuentemente a hipogammaglobulinemia. Presentamos una paciente de 66 años de edad con diabetes mellitus, síndrome de Sjögren e hipertensión arterial, derivada a nuestro centro por tos seca y disnea clase funcional II-III. En el examen físico presentaba rales tipo “velcro” bibasales y esplenomegalia. Los estudios de laboratorio evidenciaron plaquetopenia (50 000/mm³) e hipogammaglobulinemia; la tomografía axial computarizada (TAC) de tórax mostró compromiso intersticial bibasal, con nódulos peribroncovasculares menores a un centímetro. Se realizó una videotoracoscopia con biopsia pulmonar, la anatomía patológica mostró hallazgos compatibles con neumonía intersticial linfoidea. Se inició tratamiento con metilprednisona 40 mg/día vía oral e infusión endovenosa de gammaglobulina 500 mg/kg, mensual, evolucionando con mejoría clínico-radiológica. Por persistencia de plaquetopenia, asociada a hiperesplenismo, se realizó esplenectomía con buena respuesta. En la anatomía patológica no se observo clonalidad linfoide.

The interstitial lymphoid pneumonia (LIP) is an uncommon disorder, described as non-neoplastic lung lymphoid tissue hyperplasia and classified as an interstitial lung disease. It has been described in association with HIV infection, autoimmune disorders, policlonal hypergammaglobulinemia and less frequently, with hypogammaglobulinemia. We report the case of a 66 year old female patient with a history of diabetes, Sjögren syndrome and hypertension. She was referred to our hospital due to a dry cough and dyspnea (FC II-III). The physical examination showed bilateral dry crackles and splenomegaly. Laboratory studies showed thrombocytopenia (50 000/ mm³) and hypogammaglobulinemia. A computed tomography thoracic scan showed interstitial bilateral basal lung infiltrates with small peribroncovascular nodules. A lung biopsy was made by thoracoscopy and reported LIP. Initial treatment consisted of oral methilprednisone, 40 mg daily, and once a month intravenous gammaglobulin 500 mg/kg, with good clinical and radiological response. A splenectomy was done due to persistent thrombocytopenia associated with hypersplenism, with good response of the platelets counts. No lymphoid clonal or other associated disease was detected.

Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase / Myositis-related interstitial lung disease and antisynthetase syndrome

Em pacientes com miosite, é comum o comprometimento pulmonar, e a presença de anticorpos anti-aminoacil-RNAt sintetase (anti-ARS) é preditora da presença ou do desenvolvimento de doença pulmonar intersticial (DPI). Uma entidade clínica distinta - a síndrome antissintetase - é caracterizada pela presença de anticorpos anti-ARS, miosite, DPI, artrite, fenômeno de Raynaud e "mãos de mecânico". O mais comum anticorpo anti-ARS é o anti-Jo-1. Anticorpos anti-ARS mais recentemente descritos podem conferir um fenótipo que é distinto daquele de pacientes com positividade para anti-Jo-1, sendo caracterizado por uma menor incidência de miosite e uma maior incidência de DPI. Nos pacientes com DPI relacionada à síndrome antissintetase, a resposta a medicações imunossupressoras é em geral favorável.

In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

Pulmonary interstitial emphysema complicating pneumonia in an unventilated term infant.

A case of pulmonary interstitial emphysema with pneumothorax and pneumomediastinum complicating pneumonia in a 6-week-old infant is reported. The patient had no history of resuscitation, bag and mask ventilation, nasal continuous positive airway pressure or mechanical ventilation.

Doenças pulmonares intersticiais crônicas na criança: [revisão] / Chronic interstitial lung diseases in children: [review]

As doenças pulmonares intersticiais (DPIs) da criança constituem um grupo heterogêneo de doenças raras que têm sido definidas e classificadas de acordo com as experiências e as pesquisas em adultos. Entretanto, os pneumologistas pediátricos vêm observando que o espectro clínico é mais amplo nas crianças, e que muitas destas doenças evoluem e respondem ao tratamento de forma diferente. Além disso, provavelmente devido a estágios diferentes de desenvolvimento e maturação pulmonares, novas formas clínicas têm sido descritas, principalmente em lactentes, ampliando a classificação nessa faixa etária. A compreensão de que nem a definição nem as classificações estabelecidas se aplicam inteiramente ao grupo pediátrico tem motivado a realização de estudos multicêntricos com o objetivo de estudá-las melhor, unificando as estratégias diagnósticas e terapêuticas. Fizemos a revisão atualizando a conceituação das DPIs no grupo pediátrico, considerando as particularidades desse grupo na utilização do esquema de classificação dessas doenças para adultos e revendo o histórico dos esforços para uma melhor compreensão do problema com os estudos multicêntricos. Foram ressaltadas as diferenças na apresentação clínica, procurando realçar os novos conhecimentos sobre as doenças recém descritas nas crianças pequenas. Alertamos também para a necessidade de ser seguida uma rotina padronizada de investigação laboratorial, radiológica e de processamento das biópsias à luz desses conhecimentos. É importante lembrar que, do grupo das novas doenças descritas, as alterações genéticas do surfactante devem constar também do diagnóstico diferencial das DPIs dos adultos, podendo se apresentar nesse grupo como uma das doenças classificadas como idiopáticas.

Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.