Accuracy of echocardiography and chest tomography for pulmonary hypertension screening in patients awaiting lung transplantation.

OBJECTIVE: To examine the accuracy of a pulmonary hypertension screening strategy based on a combination of echocardiographic data and tomographic measurements (pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio) in patients with chronic lung disease referred for lung transplantation. METHODS: A retrospective observational study with patients with pulmonary emphysema or fibrosis referred for transplantation between 2012 and 2016. Pulmonary hypertension was defined as mean pulmonary artery pressure ≥25mmHg, or between 21 and 24mmHg, with pulmonary vascular resistance >3 Wood units on right heart catheterization. Tomographic measurements were made by two independent radiologists. RESULTS: This sample comprised 13 patients with emphysema and 19 patients with pulmonary fibrosis. Of these, 18 had pulmonary hypertension. The level of agreement in tomographic measurements made by radiologists was high (intraclass correlation coefficients 0.936 and 0.940, for pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio, respectively). Areas under the ROC curves constructed for pulmonary artery diameter, pulmonary artery diameter to ascending aorta diameter ratio, and pulmonary artery systolic pressure as predictors of pulmonary hypertension were 0.540, 0.629 and 0.783, respectively. The sensitivity, specificity and negative predictive value of pulmonary artery systolic pressure ≥40mmHg were 67%, 79% and 65%, respectively. The combined criterion (pulmonary artery diameter to ascending aorta diameter ratio >1 and/or pulmonary artery systolic pressure ≥40mmHg) achieved sensitivity of 72%, specificity of 79%, and a negative predictive value of 69%. CONCLUSION: Measurements of pulmonary artery and ascending aorta diameter were highly reproducible. The association of pulmonary artery and aortic diameter >1 and/or pulmonary artery systolic pressure ≥40mmHg improved the sensitivity and the negative predictive value for pulmonary hypertension screening. This strategy demands prospective validation to assess safety and cost-effectiveness.

Relação entre exercício físico e sistema imunológico / Relationship between exercise and the immune system

O corpo humano tende sempre a procurar um estado de homeostase, buscando o equilíbrio entre todos os sistemas. O exercício físico está presente na rotina diária de indivíduos, mesmo com objetivos diferentes, porém a influência no sistema imunológico não é muitas vezes abordada como fator relevante. O sistema imune é responsável por proteger o organismo contra infecções e doenças, podendo ser modulado perante a resposta de exercícios físicos regulares. Tendo em vista que, atualmente, existe uma preocupação maior em tornar e manter a imunidade eficiente, a prática regular e moderada do exercício pode contribuir para uma maior eficácia desse sistema, dessa forma, podendo ser considerada uma proteção ao corpo humano. O objetivo dessa revisão foi sintetizar os dados de estudos presentes na literatura que demonstram a influência do exercício físico na resposta do sistema imunológico, tornando possível compreender as alterações moleculares, fisiológicas, metabólicas e celulares que levam a um tipo específico de resposta do organismo humano.

The human body always tends to seek a homeostasis state, trying to balance all systems. Physical exercise is present in the routine of individuals even with different goals, but the influence in the immune system isnt a relevant factor. The immune system is responsible for protecting the human body against some infections and diseases, and could be modulated in response by some regular physical exercise. At the moment there is a greater concern to keep efficient immunity, a practice of regular and moderate exercise can contribute to a better effectiveness of this system, thus, it can be considered a form of protection to the human body. The objective of this review was to synthesize some data from any studies presented in the literature that demonstrate the influence of physical exercise on the immune system response. Making it possible to understand the molecular mechanisms, physiological, metabolic and cellular changes that turn to a specific type of response in the human body.

Accuracy of echocardiography and chest tomography for pulmonary hypertension screening in patients awaiting lung transplantation / Acurácia do ecocardiograma e da tomografia de tórax para rastreamento de hipertensão pulmonar em pacientes aguardando transplante de pulmão

ABSTRACT Objective To examine the accuracy of a pulmonary hypertension screening strategy based on a combination of echocardiographic data and tomographic measurements (pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio) in patients with chronic lung disease referred for lung transplantation. Methods A retrospective observational study with patients with pulmonary emphysema or fibrosis referred for transplantation between 2012 and 2016. Pulmonary hypertension was defined as mean pulmonary artery pressure ≥25mmHg, or between 21 and 24mmHg, with pulmonary vascular resistance >3 Wood units on right heart catheterization. Tomographic measurements were made by two independent radiologists. Results This sample comprised 13 patients with emphysema and 19 patients with pulmonary fibrosis. Of these, 18 had pulmonary hypertension. The level of agreement in tomographic measurements made by radiologists was high (intraclass correlation coefficients 0.936 and 0.940, for pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio, respectively). Areas under the ROC curves constructed for pulmonary artery diameter, pulmonary artery diameter to ascending aorta diameter ratio, and pulmonary artery systolic pressure as predictors of pulmonary hypertension were 0.540, 0.629 and 0.783, respectively. The sensitivity, specificity and negative predictive value of pulmonary artery systolic pressure ≥40mmHg were 67%, 79% and 65%, respectively. The combined criterion (pulmonary artery diameter to ascending aorta diameter ratio >1 and/or pulmonary artery systolic pressure ≥40mmHg) achieved sensitivity of 72%, specificity of 79%, and a negative predictive value of 69%. Conclusion Measurements of pulmonary artery and ascending aorta diameter were highly reproducible. The association of pulmonary artery and aortic diameter >1 and/or pulmonary artery systolic pressure ≥40mmHg improved the sensitivity and the negative predictive value for pulmonary hypertension screening. This strategy demands prospective validation to assess safety and cost-effectiveness.

RESUMO Objetivo Avaliar a acurácia de uma estratégia de rastreamento de hipertensão pulmonar baseada na combinação de dados de ecocardiograma com as medidas derivadas da tomografia computadorizada (diâmetro da artéria pulmonar e razão entre diâmetro da artéria pulmonar e diâmetro da aorta ascendente) em pacientes pneumopatas crônicos encaminhados para transplante pulmonar. Métodos Estudo observacional retrospectivo realizado com pacientes com enfisema e fibrose pulmonar avaliados para transplante entre 2012 e 2016. Definiu-se hipertensão pulmonar como pressão arterial pulmonar média ≥25mmHg, ou entre 21 a 24mmHg, com resistência vascular pulmonar >3 unidades Wood no cateterismo direito. As medidas tomográficas foram realizadas por dois radiologistas independentes. Resultados Foram incluídos 13 pacientes com enfisema e 19 com fibrose pulmonar, sendo 18 com hipertensão pulmonar. Houve alta concordância entre os radiologistas em relação às medidas tomográficas (coeficientes de correlação intraclasse para diâmetro da artéria pulmonar de 0,936 e diâmetro da artéria pulmonar/diâmetro da aorta ascendente de 0,940). As áreas abaixo da curva ROC de diâmetro da artéria pulmonar, diâmetro da artéria pulmonar/diâmetro da aorta ascendente, e pressão sistólica da artéria pulmonar como preditores de hipertensão pulmonar foram 0,540, 0,629 e 0,783, respectivamente. A sensibilidade, especificidade e valor preditivo negativo da pressão de sistólica de artéria pulmonar ≥40mmHg foram 67%, 79% e 65%, respectivamente. O critério combinado de diâmetro da artéria pulmonar/diâmetro da aorta ascendente >1 e/ou pressão sistólica da artéria pulmonar ≥40mmHg mostrou sensibilidade de 72%, especificidade de 79%, e valor preditivo negativo de 69%. Conclusão Os diâmetros da artéria pulmonar e da aorta ascendente foram altamente reprodutíveis. A associação entre diâmetro da artéria pulmonar e diâmetro da aorta ascendente >1 e/ou pressão sistólica da artéria pulmonar ≥40mmHg melhorou a sensibilidade e o valor preditivo negativo para rastreamento de hipertensão pulmonar. Essa estratégia requer validação prospectiva para se avaliarem segurança e custo-efetividade.

Hypersensitivity to dipyrone in aspirin-exacerbated respiratory disease patients is associated with urticaria.

OBJECTIVE: To phenotype patients with aspirin-exacerbated respiratory disease (AERD) according to the presence of atopy, urticaria and level of peripheral eosinophils. METHODS: This study included adult asthmatic patients with AERD followed up at a tertiary hospital. They were classified according to atopy and/or urticaria, assessing clinical and laboratorial differences among the groups in order to identify possible aggravating factors of the disease. RESULTS: We included 73 patients, 78.1% being female with a mean age of 54.0 years. Severe asthma was observed in 68.5% and respiratory exacerbation with dipyrone in 67.1% of these patients. They had median total serum IgE of 191.6 IU/mL, mean peripheral eosinophils of 718.5 cells/mm3, and 50.7% were atopic. Urticaria was observed in 32.9% of them, and exacerbations were more often triggered by dipyrone (p = .016). Atopic patients were younger than nonatopic patients (p = .023), and had, on average, higher total serum IgE levels (p = .022). We observed a good correlation between asthma severity and peripheral eosinophils count (r2 = 026; p = .021). CONCLUSIONS: In this study, severe asthma was highly prevalent in AERD patients. Likewise, urticaria was quite prevalent and its presence was associated with dipyrone induced hypersensitivity reaction. Atopy was found in half of the patients, with no association with asthma severity. Patients with higher levels of peripheral eosinophils had more severe asthma. Dypirone hypersensitivity may be a marker for concomitant respiratory and cutaneous hypersensitivity reactions.

Diagnóstico e tratamento da DREA: realidades da prática clínica / Diagnosis and treatment of AERD: clinical practice reality

Introdução: A doença respiratória exacerbada por aspirina (DREA), caracterizada por asma, rinossinusite, polipose nasal e hipersensibilidade à aspirina, pode ser sugerida pela história, porém, o teste de provocação oral com a aspirina é o padrão ouro para o diagnóstico, e a dessensibilização com aspirina, uma boa opção terapêutica. O objetivo do trabalho foi avaliar as características clínicas e os resultados dos procedimentos de provocação e/ou de dessensibilização com aspirina nos pacientes com suspeita de DREA, bem como observar se houve correlação com a literatura. Métodos: Neste estudo retrospectivo, foram avaliados prontuários de pacientes adultos com suspeita de DREA, em acompanhamento em um hospital terciário e que foram submetidos à provocação e/ ou dessensibilização com aspirina. Dois protocolos foram utilizados para o teste de provocação: (a) cetorolaco nasal/aspirina oral, e (b) apenas aspirina oral. Foram avaliados: características clínicas, a positividade do teste e da dessensibilização e a comparação deste resultado com a história prévia. Resultados: Participaram do estudo 24 pacientes, com média de idade de 50,8 anos, sendo 54,2% do sexo feminino. Treze pacientes (54,2%) tinham asma grave, e seis (25%), asma alérgica. Média do volume expiratório forçado no primeiro segundo (VEF1) foi de 81,5% do valor predito. Dezenove pacientes (79,2%) referiam broncoespasmo e/ou urticária com anti-inflamatórios não esteroidais. Cinco pacientes não faziam associação com essas medicações. Independente do protocolo usado, onze pacientes (45,8%) apresentaram teste positivo, confirmando a DREA, sendo que seis pacientes (25%) foram submetidos à dessensibilização com aspirina. Oito pacientes (33,3%) apresentaram provocação negativa, e cinco (20,8%) não conseguiram completar a investigação devido à presença de urticária. Conclusões: Pacientes com suspeita de DREA deveriam ser submetidos à provocação com aspirina para confirmar o diagnóstico. Um quarto dos pacientes foi submetido à dessensibilização, entretanto, para a maioria dos pacientes não foi possível confirmar o diagnóstico ou o teste foi negativo.

Introduction: Aspirin-exacerbated respiratory disease (AERD) is characterized by asthma, rhinosinusitis, nasal polyps, and aspirin hypersensitivity. The condition may be suggested by the patient's medical history; however, oral provocation test with aspirin is the gold standard for diagnosis, and desensitization with aspirin, a good therapeutic option. The aim of this study was to evaluate the clinical characteristics and results obtained with aspirin provocation tests and/or desensitization in patients with suspected AERD, as well as to correlate these data with the literature available. Methods: In this retrospective study, the medical records of adult patients with suspected AERD followed at a tertiary hospital who underwent aspirin challenge and/or desensitization were evaluated. Two protocols were used for the challenge test: (a) nasal ketorolac/ oral aspirin; and (b) oral aspirin alone. Clinical characteristics and both test and desensitization positivity were evaluated, and the results were compared with data from the patient's history. Results: Twenty-four patients participated in the study, with a mean age of 50.8 years; 54.2% were female. Thirteen patients (54.2%) had severe asthma, and six (25%) had allergic asthma. Mean forced expiratory volume in 1 second (FEV1) was 81.5% of the predicted value. Nineteen patients (79.2%) reported bronchospasm and/or urticaria with nonsteroidal anti-inflammatory drugs. Five patients had no association with these medications. Regardless of the protocol used, eleven patients (45.8%) presented positive tests, confirming the diagnosis of AERD, and six patients (25%) underwent aspirin desensitization. Eight patients (33.3%) had negative results in the provocation test, and five (20.8%) failed to complete the investigation due to the presence of urticaria. Conclusions: Patients with suspected AERD should undergo aspirin challenge to confirm the diagnosis. One-fourth of our patients underwent desensitization, but for most patients, either it was not possible to confirm the diagnosis or the test resulted negative.

Endomiocardite de Loeffler ­ manifestação cardíaca da síndrome hipereosinofílica: relato de caso / Loeffler's endomyocarditis ­ cardiac manifestation of hypereosinophilic syndrome: case report'

Introdução: A síndrome hipereosinofílica é caracterizada por uma produção aumentada e contínua de eosinófilos e pode levar a lesões teciduais em múltiplos órgãos, como consequência da infiltração eosinofílica. Os pacientes apresentam eosinofilia absoluta no sangue periférico (> 1.500 eosinófilos/mm3) sem uma causa primária de eosinofilia. A manifestação cardíaca desta síndrome geralmente se apresenta como endomiocardite de Loeffler, que constitui uma miocardiopatia restritiva primária resultante da infiltração de eosinófilos no tecido cardíaco. Descrição do caso: Relatamos o caso raro de uma paciente de 64 anos com eosinofilia a esclarecer e comprometimento cardíaco, que teve o diagnóstico estabelecido a partir de exames de imagem. Comentários: Enfatizamos os aspectos clínicos e evolutivos, ressaltando as dificuldades diagnósticas e a importância da investigação de eosinofilias persistentes sem causa aparente, uma vez que o diagnóstico e tratamento precoce podem proporcionar melhores taxas de sobrevida e prognóstico nestes pacientes.

Introduction: The hypereosinophilic syndrome is characterized by an increased, continuous production of eosinophils, and it may lead to tissue damage in multiple organs as a consequence of eosinophilic infiltration. Patients with this syndrome present absolute eosinophil count > 1,500 eosinophils/mm3 in the peripheral blood without a primary cause for eosinophilia. The cardiac manifestation of this syndrome usually presents as Loeffler's endomyocarditis, a primary restrictive cardiomyopathy resulting from the infiltration of eosinophils into cardiac tissue. Case description: We report the rare case of a 64-year-old woman with eosinophilia and cardiac involvement, who had the diagnosis established based on imaging tests. Comments: We emphasize the clinical and evolutionary aspects of the condition, highlighting the diagnostic difficulties and the importance of investigating persistent eosinophilia without an apparent cause, as early diagnosis and treatment can provide better survival rates and improved prognosis in these patients.

Características de pacientes asmáticos com bronquiectasias / Characteristics of asthmatic patients with bronchiectasis

Introdução: A bronquiectasia é uma enfermidade caracterizada por dilatações anormais e irreversíveis de um ou mais brônquios. Pode estar associada a diversas outras comorbidades respiratórias, entre elas, a asma. Objetivo: Caracterizar os pacientes asmáticos com bronquiectasias acompanhados em um serviço terciário de alergia. Métodos: Análise retrospectiva de prontuários de pacientes asmáticos que apresentavam bronquiectasias nos exames de tomografia computadorizada de tórax. Os portadores de aspergilose broncopulmonar alérgica (ABPA) foram excluídos. Foram analisados os dados demográficos e exames complementares (espirometria, eosinófilos periféricos, IgE total e IgE específica). A caracterização das bronquiectasias foi avaliada conforme sua localização nos campos pulmonares. Resultados: Quarenta e nove pacientes foram selecionados, sendo 88% do sexo feminino. A média de idade do grupo era de 63 anos, e de tempo de doença de 43 anos. O tratamento prévio de tuberculose (TB), em algum momento da vida, foi relatado por 31% dos pacientes. Doença do refluxo gastroesofágico (DRGE) estava presente em 76% dos pacientes, e a história de tabagismo ativo ou passivo, em 65% dos pacientes. Mais de 80% dos pacientes estavam no step 4 de tratamento da asma, e apresentavam VEF1 reduzido. A média de eosinófilos periféricos foi de 253,4 cel/mm3, de IgE total foi de 458,8 UI/mL, e 69% dos pacientes eram sensibilizados para algum aeroalérgeno. Em relação à localização das bronquiectasias, o lobo superior direito foi o mais acometido, seguido pelo lobo superior esquerdo e lobo inferior direito, respectivamente. Conclusão: Neste estudo, 88% dos pacientes asmáticos com bronquiectasias apresentavam asma grave e função pulmonar alterada, apesar do tratamento otimizado. A bronquiectasia pode estar associada à dificuldade no controle da asma e, embora a história de TB prévia estivesse presente em 31% dos pacientes, a frequência elevada de comprometimento dos lobos superiores (49%) sugere uma frequência maior de doença pulmonar associada à TB.

Introduction: Bronchiectasis is a disease characterized by abnormal and irreversible dilatation of one or more bronchi. It may be associated with several other respiratory comorbidities including asthma. Objective: To characterize asthmatic patients with bronchiectasis seen at a tertiary allergy service. Methods: Retrospective analysis of medical records of asthmatic patients showing bronchiectasis on chest computed tomography scans was conducted. Patients with allergic bronchopulmonary aspergillosis (ABPA) were excluded. Demographic data and complementary tests (spirometry, peripheral eosinophils, total IgE and specific IgE) were analyzed. Bronchiectasis characterization was evaluated according to its location in the lung fields. Results: Forty-nine patients were selected, and 88% were female. Mean age in the group was 63 years, and disease time was 43 years. Previous treatment of tuberculosis (TB) at some point in life was reported by 31% of the patients. Gastroesophageal reflux disease (GERD) was present in 76% of the patients, and history of active or passive smoking in 65%. More than 80% of the patients were on step 4 of asthma treatment and showed reduced FEV1. Mean peripheral eosinophils was 253.4 cell/mm3, total IgE was 458.8 IU/mL, and 69% of the patients were sensitized to some aeroallergen. Regarding the location of bronchiectasis, the right upper lobe was most commonly affected, followed by the left upper lobe and the right lower lobe, respectively. Conclusion: In this study, 88% of asthmatic patients with bronchiectasis had severe asthma and altered pulmonary function despite optimized treatment. Bronchiectasis may be associated with difficulty in controlling asthma. Although previous history of TB was present in 31% of the patients, the high frequency of involvement of the upper lobes (49%) suggests a higher frequency of TB-associated lung disease.

Uma nova classe de doenças: doenças autoinflamatórias / A new class of diseases: autoinflammatory diseases

As doenças autoinflamatórias sistêmicas são um grupo de doenças raras recentemente descritas, mas que vêm ganhando espaço no cenário clínico. Caracterizam-se por alterações da imunidade inata, portanto sem a presença de linfócito T autorreator ou autoanticorpo, e que respondem ao bloqueio de uma única citocina. Esta revisão tem como objetivo analisar a base imunofisiológica destas doenças e descrever brevemente cada uma delas com suas características clínicas mais importantes.

Systemic autoinflammatory diseases are a group of rare diseases only recently described but rapidly growing in importance in the clinical setting. They are characterized by innate immunity impairment, i.e., absence of autoreactive T lymphocytes or autoantibodies, and respond to individual cytokine blockade. The objective of this review was to analyze the immunophysiological basis of these diseases and to briefly describe each of them along with their most relevant clinical characteristics.

Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.