Diffuse lung involvement in rheumatoid arthritis: a respiratory physician's perspective / 中华医学杂志(英文版)

The lungs are one of the most common extra-articular organs involved in rheumatoid arthritis (RA), which is reported to occur in up to 60% to 80% of RA patients. Respiratory complications are the second leading cause of death due to RA. Although there is a wide spectrum of RA-associated respiratory diseases, interstitial lung disease is the most common manifestation and it impacts the prognosis of RA. There has been progress in understanding the management and progression of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and RA-associated respiratory diseases recently, for example, opportunistic pulmonary infectious diseases and toxicity from RA therapies. From a chest physicians' perspective, we will update the diagnosis and treatment of RA-associated ILD, methotrexate-associated lung disease, and the complication of Pneumocystis jiroveci pneumonia in RA in this review.

Taquipnea persistente como manifestación inicial de hiperplasia de células neuroendocrinas: reporte de un caso clínico / Persistent tachypnea as initial manifestation of hyperplasia of neuroendocrine cells: case report

Se realiza revisión de la literatura y presentación de un caso clínico de Hiperplasia de Células Neuroendocrinas en paciente lactante masculino que inicia su padecimiento a los 3 meses de vida con dificultad respiratoria caracterizada por retracciones subcostales y taquipnea persistente, posterior-mente a los 8 meses de edad se agrega hipoxemia respirando aire ambiente que requiere uso de oxígeno suplementario continuo. Tiene antecedente de tres hospitalizaciones, con diagnóstico de Bronquiolitis y Neumonía atípica, realizándose panel viral respiratorio con reporte negativo. El paciente persiste con sintomatología respiratoria a pesar de tratamientos médicos, por lo que se deriva a neumología pediátrica, unidad de enfermedad pulmonar intersticial del lactante, iniciando protocolo de estudio, se realiza tomografía tórax de alta resolución, que evidencia imágenes en vidrio despulido en lóbulo medio y región lingular, además de atrapamiento aéreo. Se concluye el diagnóstico de Hiperplasia de Células neuroendocrinas con base a la clínica y hallazgos tomográficos. La Hiperplasia de Células Neuroendocrinas es una patología pulmonar intersticial poco frecuente, cuyo diagnóstico es clínico y radiológico, en la minoría de los casos se requiere biopsia pulmonar para confirmación. Puede ser fácilmente confundida con otras enfermedades respiratorias 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 los primeros años de vida.

A review of the literature and presentation of a clinical case of Neuroendocrine Cell Hyperplasia in a male infant patient who begins his condition at 3 months of age with respiratory distress characterized by subcostal retractions and persistent tachypnea is presented. After 8 months of age hypoxemia is added requiring continuous oxygen therapy. He has a history of three hospitalizations, with a diagnosis of bronchiolitis and atypical pneumonia, respiratory viral panel has a negative report. The patient persists with respiratory symptoms despite medical treatments, so it is referred to pediatric pulmonology, initiating study protocol for interstitial lung disease of the infant. A high resolution chest tomography is performed, which evidences images in polished glass in the middle lobe and lingular region, in addition to air entrapment. The diagnosis of neuroendocrine cell hyperplasia is concluded based on clinical and tomographic findings. Neuroendocrine Cell Hyperplasia is a rare interstitial pulmonary pathology, whose diagnosis is clinical and radiological. Lung biopsy is required only in the minority of cases for confirming diagnosis. It can be easily confused with other common respiratory diseases, so it is important to suspect it to make an early diagnosis. Most cases evolve with decline in symptoms, improving spontaneously in the first years of life.

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.

Comportamiento clínico radiológico de la enfermedad intersticial difusa sintomática en pacientes con afecciones reumatológicas / Clinical radiological behavior of symptomatic diffuse interstitial disease in patients with rheumatological conditions

Introducción: Las enfermedades pulmonares intersticiales difusas constituyen un grupo amplio de afecciones, con manifestaciones clínicas, expresión radiológica y alteraciones funcionales del intersticio pulmonar. Objetivo: Describir el comportamiento clínico y radiológico de la enfermedad intersticial difusa sintomática en pacientes diagnosticados con enfermedades reumáticas. Métodos: Se realizó un estudio observacional, descriptivo transversal, con una población compuesta por 33 pacientes con manifestaciones respiratorias secundarias a enfermedades reumáticas entre enero de 2020 a enero de 2021 en el Hospital Universitario Clínico Quirúrgico Arnaldo Milián Castro. La muestra estuvo conformada por la totalidad de los pacientes. El análisis estadístico se llevó a cabo por medio de estadística descriptiva de cada variable. Resultados: Existió un mayor número de mujeres afectadas (75,8 por ciento) por esclerosis sistémica (31 por ciento), artritis reumatoide (27 por ciento) y lupus eritematoso sistémico (21 por ciento). La afectación respiratoria predominante fue de 81,8 por ciento con compromiso intersticial asociada a un patrón radiológico de neumonitis intersticial usual con 5 casos (21,4 por ciento), todos ligados a artritis reumatoide. Para 25 casos (75,8 por ciento) hubo un retraso diagnóstico menor de 1 año; la enfermedad reumática antecedió a la manifestación respiratoria en 21 casos (63,6 por ciento). Conclusiones: El compromiso intersticial fue la manifestación respiratoria más frecuente; los patrones de neumonitis intersticial usual y la neumonía intersticial no específica predominaron como hallazgos imagenológicos. La secuencia diagnóstica apuntó hacia las enfermedades reumáticas como el debut del proceso(AU)

Introduction: Diffuse interstitial lung diseases constitute a wide group of conditions, with clinical manifestations, radiological expression and functional alterations of the pulmonary interstitium. Objective: To describe the clinical and radiological behavior of symptomatic diffuse interstitial disease in patients diagnosed with collagenosis. Methods: An observational, descriptive, cross-sectional study was carried out. The population consisted of 33 patients with respiratory manifestations secondary to rheumatic diseases. The sample consisted of all the patients. Statistical analysis was carried out by means of descriptive statistics for each variable. Results: There was a greater number of women affected (75.8 percent) by systemic sclerosis (31percent), rheumatoid arthritis (27 percent) and systemic lupus erythematosus (21 percent). The predominant respiratory involvement was 81.8 percent with interstitial involvement associated with a radiological pattern of usual interstitial pneumonitis with 5 cases (21.4 percent), all linked to rheumatoid arthritis. For 25 cases (75.8 percent) there was a diagnostic delay of less than 1-year, rheumatic diseases preceded the respiratory manifestation in 21 cases (63.6 percent). Conclusions: Interstitial involvement was the most frequent respiratory manifestation; the patterns of usual interstitial pneumonitis and nonspecific interstitial pneumonia as predominated imaging findings. The diagnostic sequence pointed towards rheumatic diseases were the debut of the process(AU)

Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease / 中国医学科学杂志(英文版)

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital. Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 μmol/L, significantly higher than that in the ILD group (123 μmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 μmol/L, Nemenyi = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenyi = 43.853, P= 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR= 1.044, 95%CI: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis. Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.

Neumonía intersticial con características autoinmunes: reporte de caso / Interstitial Pneumonia with Autoimmune Features: case report

La neumonía intersticial con características autoinmunes por sus siglas en inglés, es una entidad en la que existe un compromiso pulmonar intersticial y hallazgos clínicos y paraclínicos que sugieren una enfermedad del tejido conectivo, aunque no cumplen criterios diagnósticos para ninguna de estas. Con fines de investigación, en el 2015 se describieron criterios para esta entidad, en los que se incluyeron características de los dominios clínicos, serológicos y morfológicos, con diversos patrones de compromiso pulmonar. En la actualidad, hay un aumento en el interés de esta entidad, pues algunos autores sugieren que se pueda tratar de una enfermedad autoinmune per se, cuyo órgano blanco principal sería el pulmón. Dado su reciente reconocimiento, son pocos los casos descritos en la literatura. Con el propósito de contribuir a la mejor identificación de esa entidad, presentamos el caso de una paciente de 68 años con afectación pulmonar en quien después de descartar otras causas se llegó al diagnóstico de neumonía intersticial con características autoinmunes al cumplir los criterios de cada dominio requerido. Se inició tratamiento con micofenolato mofetilo a dosis de 2,5 mg/día. En su evolución clínica, la paciente presentó mejoría y fue dada de alta con tratamiento ambulatorio(AU)

Interstitial pneumonia with autoimmune features is a condition in which patients can have clinical and serological findings suggesting of a connective tissue disease associated with an interstitial lung disease, nonetheless no criteria for an specific connective tissue disease are meeting. In 2015 classification criteria where proposed, the diagnosis is made in the presence of a combination of features from clinical, serological and morphological domain with various patterns of pulmonary involvement. Currently there is an increase in the interest of this condition, as some authors suggest that it can be an autoimmune pathology per se, whose main target organ would be the lung. Given its recent recognition, there are few cases described in the literature and therefore in order to contribute to the better identification of that entity, we present the case of a 65 year old patient with lung involvement in whom after ruling out other etiological causes reached the diagnosis of I Interstitial pneumonia with autoimmune by meeting criteria of each required domain(AU)

Esophageal manometry in systemic sclerosis: findings and association with clinical manifestations

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.

RESUMO INTRODUÇÃO A esclerose sistêmica (ES) é uma doença autoimune que afeta vários órgãos de etiologia desconhecida, caracterizada por dano vascular e fibrose da pele e órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. OBJETIVOS Relacionar o perfil das alterações na eletromanometria (ME), o perfil de acometimento da pele, a pneumopatia intersticial (PI) e os sintomas esofágicos em pacientes com ES. MÉTODO Trata-se de um estudo observacional, transversal, realizado no ambulatório de SSC do Hospital das Clínicas da Universidade Federal de Uberlândia. Após aprovação pelo Comitê de Ética e assinatura dos termos de consentimento, 50 pacientes foram inicialmente convidados, de 04/12/2014 a 25/06/2015. Eles foram submetidos às investigações usuais de acordo com o quadro clínico. A análise estatística foi descritiva em porcentagem, média e desvio padrão. O teste Qui-quadrado foi utilizado para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos. RESULTADOS 91,9% dos pacientes apresentaram alterações manométricas. 37,8% tinham envolvimento do corpo esofágico e do esfíncter esofágico inferior. 37,8% tinham IP. 24,3% apresentaram a forma difusa da ES. Não há associação entre alterações manométricas e manifestações clínicas (sintomas cutâneos, pulmonares e gastrointestinais). CONCLUSÃO O presente estudo confirma que as alterações da motilidade esofágica detectadas pela EM são frequentes em pacientes com SSC, mas podem não estar relacionadas ao envolvimento cutâneo, à de DPI ou às queixas gastrointestinais dos pacientes.

Los reumatólogos en la pandemia COVID-19 / Rheumatologists in the COVID-19 pandemic

Desde fines del 2019 enfrentamos el brote de una nueva infección por coronavirus llamada COVID-19, que rápidamente se transformó en una pandemia y llegó a nuestro país a principios del 2020. Esto ha traído muchas preguntas y desafíos, específicamente en nuestros pacientes con enfermedades autoinmunes, que tienen tradicionalmente mayor riesgo de contraer infecciones y de complicarse por estas. Por otra parte, en el tratamiento actual del síndrome respiratorio agudo severo causado por el coronavirus SARS-CoV-2 se están usando e investigando varios medicamentos inmunosupresores e inmunomoduladores del arsenal reumatológico para controlar la respuesta inmune exagerada que se produce en el huésped en el COVID-19 grave. En esta revisión analizamos la literatura existente hasta el momento sobre pacientes reumatológicos y COVID-19, medicamentos reumatológicos en investigación y en uso para el manejo de la infección por SARS-CoV-2, y resumimos ciertas recomendaciones de manejo específicas para nuestros pacientes.

Since the end of 2019 we have been facing the outbreak of a new coronavirus infection called COVID-19, which quickly turned into a pandemic arriving in Chile in early 2020. This has brought with it many questions and challenges, specifically for our patients with autoimmune diseases, which have an increased risk of infections due to their disease and the use of immunosuppresant and corticosteroid drugs. On the other hand, in the current treatment of severe acute respiratory syndrome caused by the SARS-CoV-2 coronavirus, several immunosuppressive and immunomodulatory drugs in the rheumatologic arsenal are being used and investigated to control the exaggerated immune response that occurs in the host in serious COVID -19 cases. In this review we investigated the literature to date on rheumatology patients and COVID-19, rheumatology drugs under investigation and in use for the management of SARS-CoV-2 infection, and have summarized certain specific management recommendations for our patients

Disfagia orofaríngea y neumopatías en la infancia / Oropharyngeal dysphagia and childhood pneumopathies

OBJETIVOS: Presentar brevemente las principales patologías pulmonares y sus interferencias en la alimentación de niños. MÉTODOS: Se trata de una Actualización, basada en referencias bibliográficas actuales y referencias en pediatría. RESULTADOS: Se presentaron las posibles alteraciones de deglución en niños con: Bronquilitis Viral Aguda, Displasia Broncopulmonar, Enfermedades Intersticiales Pulmonares y Neumonias Aspirativas. CONCLUSIONES: Este material sirve para dirigir la atención del público de atención en salud en general, para trastornos de deglución niños neumópatas.

OBJECTIVES: Present briefly the main pulmonary pathologies and their interferences in the feeding of children. METHODS: This is an Update, based on current bibliographical references and references in pediatrics. RESULTS: Possible alterations of swallowing were presented in children with: Acute Viral Bronchitis, Bronchopulmonary Dysplasia, Pulmonary Interstitial Diseases and Aspirative Pneumonia. CONCLUSIONS: This material serves to direct the attention of the health care public in general, for children swallowing disorders pneumatics.

Correlation between nailfold capillaroscopic findings and presence of interstitial lung disease in systemic sclerosis patients / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients.@*METHODS@#We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters.@*RESULTS@#Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array.@*CONCLUSION@#Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc.

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review / Pneumomediastino espontâneo na dermatomiosite: uma série de casos e revisão de literatura

OBJECTIVES: To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS: This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

OBJETIVOS: Descrever série de casos de pneumomediastino espontâneo em portadores de dermatomiosite e revisar a literatura. MÉTODOS: Trata-se de série de casos, único centro, relatando 9 pacientes com pneumomediastino e dermatomiosite definida, acompanhados de 2005 a 2017. RESULTADOS: A mediana da idade dos pacientes foi de 33 anos. Sintomas constitucionais estavam presentes em 88,8% dos pacientes. Houve acometimento cutâneo e pulmonar em todos os casos, acometimento das articulações em 11,1%, trato gastrointestinal em 44,4% e musculatura em 77,7% dos pacientes. Enfisema subcutâneo foi observado em 55,5% e pneumotórax em 11,1%, respectivamente. A fraqueza muscular foi observada em 77,7% dos casos, com um nível médio de creatinofosfoquinase sérica de 124U/L. Com base nos resultados da revisão da literatura, a análise geral mostrou que: os fatores de risco associados ao pneumomediastino espontâneo foram: história de pneumopatia intersticial, níveis normais ou baixos de enzimas musculares, uso prévio de glicocorticoide sistêmico; >50% dos pacientes tiveram enfisema subcutâneo; houve alta mortalidade como consequência da gravidade da doença pulmonar intersticial. CONCLUSÕES: Nossa série de casos revelou que o pneumomediastino é uma complicação rara na dermatomiosite e que ocorre em pacientes com história de pneumopatia intersticial e pode ser acompanhada por enfisema subcutâneo e pneumotórax.

Neumonitis intersticial descamativa en el paciente pediátrico / Desquamative interstitial penumonitis in a pediatric patient

Introducción: la neumonitis intersticial descamativa es una entidad caracterizada en la clínica por mostrar tos, disnea, cianosis e hipercapnia, con un patrón restrictivo de las pruebas funcionales respiratorias, debido a la presencia de fibrosis pulmonar, cuya frecuencia es inusual en Pediatría. Presentación del caso: adolescente que fue remitida al Hospital Docente Pediátrico del Cerro por sospecha de dengue, al referir fiebre de 38 °C de 3 días de evolución, acompañada de dolores musculares en miembros inferiores, escalofríos y cefalea, por lo cual fue internada en la sala de misceláneas. Durante su evolución mostró dificultad respiratoria, tos seca, taquipnea, taquicardia y disminución del murmullo vesicular en la base del pulmón izquierdo. Se observó en la radiografía de tórax una opacidad en dicha zona y fue tratada con antibióticos. En etapa posterior se trasladó a la Unidad de Cuidados Intensivos por ocurrir un incremento de las lesiones pulmonares e insuficiencia respiratoria; por ello, se indicó ventilación mecánica, variedad presión controlada. Posteriormente se aisló en hemocultivo y secreciones bronquiales, Pseudomona Stutzeri, evento considerado como una sepsis asociada a cuidados sanitarios. Se planteó un distrés respiratorio del adulto en niños que no involucionó, y falleció en un cuadro de insuficiencia respiratoria a los 19 días de estadía. Conclusiones: esta paciente mostró síntomas y signos sugestivos de una infección pulmonar bacteriana de evolución tórpida. Los hallazgos necrópsicos describen la presencia de una bronconeumonía bacteriana como causa directa, y una neumonitis intersticial descamativa, como entidad básica del fallecimiento(AU)

Introduction: desquamative interstitial pneumonitis is a characterized condition in the clinical field since it shows cough, dysnea, cyanosis and hypercapnia, with a restrictive pattern of the functional respiratory tests due to the presence of pulmonary fibrosis that is unusual in pediatrics. Case presentation: a female adolescent was referred to the pediatric teaching hospital of Cerro on suspicion of dengue since she presented with 38 °C for three days, accompanied with muscle aches in lower limbs, chills and headache. She was admitted to a general ward. During her progression, she showed respiratory distress, unproductive cough, tachypnea, tachycardia and reduction of vesicular murmur in the left lung basis. The thoracic X ray showed opacity in the area and was treated with antibiotics. In a later phase, she was moced to the intensive care unit due to increase in pulmonary lesions and respiratory failure. She was also under mechanical ventilation with controlled pressure. Later, Pseudomona Stutzeri was isolated in blood culture and bronchial secretions, an event considered to be health care-associated sepsis. It was stated that this case was a respiratory distress of adult in a child that evolved and finally the adolescent died of respiratory failure 19 days after her hospitalization. Conclusions: this patient showed symptoms and signs suggestive of bacterial pulmonary infection of torpid progression. The necropsis finding describe the presence of bacterial bronchopneumonia as a direct cause and desquamative interstitial pneumonitis as the basic condition for death(AU)

Syndrome in question: antisynthetase syndrome (anti-PL-7)

Abstract: Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.

Pneumonia intersticial aguda (síndrome de Hamman-Rich): relato de caso e revisão de literatura / Acute intersticial pneumonia (Hamman-Rich syndrome): a case report and literature review

A Pneumonia Intersticial Aguda (PIA) é uma rara e fulminante forma de doença pulmonar difusa descrita por Hamman e Rich em 1935. Pneumonia Intersticial Aguda é classificada como Pneumonia Intersticial Idiopática, e é a mais aguda e agressiva em seu curso. Apresenta-se clinicamente de forma similar a Síndrome da Angústia Respiratória Aguda (SARA), e representa provavelmente uma parte dos casos de síndrome da Angústia Respiratória Aguda idiopática. O caso relatado é de uma paciente de 55 anos, portadora de Obesidade e Esquizofrenia, que apresentou Insuficiência Respiratória Aguda e síndrome da Angústia Respiratória Aguda grave, com piora clínica rápida e evolução para óbito com menos de 3 dias de internação hospitalar. O diagnóstico de Pneumonia Intersticial Aguda foi feito na necropsia. O objetivo deste trabalho é fazer uma breve revisão sobre a Pneumonia Intersticial Aguda e relatar um caso clínico desta doença, que é rara e com pouca descrição na literatura, devendo ser lembrada como diagnóstico diferencial nos casos de Síndrome da Angústia Respiratória Aguda

Acute Interstitial Pneumonia (AIP) is a rare and fulminant diffuse lung disease described by Hamman and Rich in 1935. Acute Interstitial Pneumonia is classified as idiopathic interstitial pneumonia, and is the most aggressive and acute in its course. It is presented as Adult Respiratory Distress Syndrome (ARDS), and it is one of the cases of idiopathic as Adult Respiratory Distress Syndrome. This is a case of a 55 years-old patient, with obesity and schizophrenia who presented with acute respiratory insufficiency and severe as Adult Respiratory Distress Syndrome, with rapid clinical deterioration and progression to death in less than three days of hospitalization. The diagnosis of Acute Interstitial Pneumonia was made at necropsy. The objective of this paper is to briefly review the Acute Interstitial Pneumonia and to report a case of this disease, which is rare with limited description in the literature. It should be included as a differential diagnosis in cases of Adult Respiratory Distress Syndrome

Enfermedad pulmonar difusa: causa de hipertensión pulmonar persistente antes del año de vida / Diffuse lung disease: cause of persistent pulmonary hypertension before one year of age

En pediatría, la enfermedad vascular pulmonar es multifactorial y heterogénea. Si bien comparte algunas características con la hipertensión pulmonar en los adultos, hay diferencias en las comorbilidades y condiciones asociadas, la coexistencia de enfermedades genéticas o del desarrollo. Las enfermedades pulmonares intersticiales pueden ser causantes de esta entidad. Una de ellas es la displasia alvéolo-capilar con mal alineamiento de las venas pulmonares, una patología infrecuente pero con 100% de mortalidad, caracterizada por la falla en la formación del tejido pulmonar que da por resultado final la alteración en la difusión de gases. Se describe un caso clínico de una paciente de 5 meses de edad estudiada a partir de sospechar una cardiopatía congénita con hipoxemia persistente, a cuyo diagnóstico se llega por la biopsia pulmonar.

Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.

Elevated Erythrocyte Sedimentation Rate Is Predictive of Interstitial Lung Disease and Mortality in Dermatomyositis: a Korean Retrospective Cohort Study

Interstitial lung disease (ILD) is a major cause of death in patients with dermatomyositis (DM). This study was aimed to examine the utility of the erythrocyte sedimentation rate (ESR) as a predictor of ILD and prognostic marker of mortality in patients with DM. One hundred-and-fourteen patients with DM were examined, including 28 with clinically amyopathic DM (CADM). A diagnosis of ILD was made based on high resolution computed tomography (HRCT) scans. The association between elevated ESR and pulmonary impairment and mortality was then examined. ILD was diagnosed in 53 (46.5%) of 114 DM patients. Cancer was diagnosed in 2 (3.8%) of 53 DM patients with ILD and in 24 (92.3%) of those without ILD (P or = 30 mm/hour had significantly higher mortality than those with ESR < 30 mm/hour (P = 0.002, log-rank test). Patients with a persistently high ESR despite immunosuppressive therapy was associated with higher mortality than those with a normalized ESR (P = 0.039, log-rank test). Elevated ESR is associated with increased mortality in patients with DM due to respiratory failure. Thus, monitoring ESR should be an integral part of the clinical care of DM patients.

A rare case of ulcerative colitis with diffuse parenchymal lung disease, spontaneous pneumomediastinum and subcutaneous emphysema.

A 50-year-old male, a tobacco smoker, who was known to have ulcerative colitis presented with dry cough, chest pain, dysponea and frequent passage of blood and mucous mixed stools. Physical examination revealed clubbing, subcutaneous emphysema of upper chest and auscultatory findings of crunching sound over pre-cordial area and basal crepitations. Spirometry was suggestive of restrictive pattern. High resolution computed tomography (HRCT) of thorax revealed pneumomediastinum, subcutaneous emphysema, bilateral diffuse centrilobular nodules and ground-glass haziness with mosaic pattern along with posterior basal fibrotic changes. The present case documents the uncommon pulmonary involvement of spontaneous pneumomediastinum and subcutaneous emphysema diffuse parenchymal lung disease, in a patient with ulcerative colitis.

Acute cor pulmonale due to lymphocytic interstitial pneumonia in a child with AIDS

BACKGROUND: Acute cor pulmonale is a clinical syndrome with signs of right-sided heart failure resulting from sudden increase of pulmonary vascular resistance. CASE PRESENTATION: A five-year-old male, infected by human immunodeficiency virus (HIV), was admitted at the division of infectious diseases of this hospital with cough, tachydyspnea, fever, and breathing difficulty. Computed tomography scan showed ground-glass opacities, cystic lesions, and bronchiectasis. The patient had nasal flaring, intercostal and subcostal retractions, and keeled chest. Abdomen was depressible; liver was 3 cm from the right-costal border, while spleen was 6 cm from the left-costal border. Echocardiogram examinations showed signs of acute cor pulmonale characterized by pulmonary hypertension and increased right-heart chamber dimensions. DIAGNOSTICS OUTCOME: Acquired immunodeficiency syndrome (AIDS)-B3, lymphocytic interstitial pneumonia (LIP), and acute cor pulmonale. Regressions of pulmonary hypertension and of right-heart chamber were observed after 30 days of highly active antiretroviral therapy (HAART) and chloroquine therapy. CONCLUSION: AIDS should be considered in children with recurrent pneumonia that is mostly associated with LIP rather than cystic fibrosis.