Prevalence and associated factors of experimentation with and current use of water pipes and electronic cigarettes among medical students: a multicentric study in Brazil / Prevalência de experimentação e uso atual de narguilé e cigarros eletrônicos e os fatores associados entre estudantes de medicina: estudo multicêntrico no Brasil

ABSTRACT Objective: To evaluate the prevalence of and factors associated with experimentation with and current use of water pipes and e-cigarettes among medical students. Methods: This was a cross-sectional multicentric study involving a convenience sample of students from medical schools in most Brazilian geographic regions. Information about experimentation with and current use of conventional cigarettes, water pipes, and e-cigarettes; beliefs and attitudes toward tobacco products; religiosity; and demographics were collected by means of an online structured questionnaire. We used descriptive statistics and logistic regression to analyze the association of those factors. Results: Our sample comprised 700 individuals from four Brazilian regions. Prevalence of experimentation with and current use of cigarettes, water pipes, and e-cigarettes were, respectively, 39.1% and 7.9%; 42.6% and 11.4%; and 13.1% and 2.3%. Water pipe experimentation was higher among those who had a sibling (adjusted OR = 2.64; 95% CI, 1.24-5.61) or friends (adjusted OR = 2.33; 95% CI, 1.63-3.31) who smoke. The same occurred regarding e-cigarette experimentation: siblings (adjusted OR = 2.76; 95% CI, 1.17-6.50) and friends (adjusted OR = 2.47; 95% CI, 1:45-4.22). Curiosity and scent/taste were the major reasons for water pipe use and e-cigarette experimentation. Although 93% of the responders learned about health damages of smoking during medical school classes, 51.4% reported having experimented with at least one of these tobacco products. Most responders who reported feeling the presence of God/the Holy Spirit in their lives were never experimenters of water pipes (59.2%) or e-cigarettes (55.3%). Conclusions: There is a high prevalence of experimentation with tobacco products among medical students whose siblings or friends smoke, despite their knowledge about smoking harms.

RESUMO Objetivo: Avaliar a prevalência de experimentação e uso atual de narguilé e cigarros eletrônicos e os fatores associados entre estudantes de medicina. Métodos: Estudo transversal multicêntrico com uma amostra de conveniência de estudantes de faculdades de medicina da maioria das regiões geográficas brasileiras. Informações sobre experimentação e uso atual de cigarros convencionais, narguilé e cigarros eletrônicos; crenças e atitudes em relação aos produtos do tabaco; religiosidade; e dados demográficos foram coletados por meio de um questionário on-line estruturado. Utilizou-se estatística descritiva e regressão logística para analisar a associação desses fatores. Resultados: Nossa amostra foi composta por 700 indivíduos de quatro regiões brasileiras. As prevalências de experimentação e uso atual de cigarros, narguilé e cigarros eletrônicos foram, respectivamente, de 39,1% e 7,9%; 42,6% e 11,4%; e 13,1% e 2,3%. A experimentação de narguilé foi maior entre aqueles que tinham irmãos (OR ajustada = 2,64; IC95%: 1,24-5,61) ou amigos (OR ajustada = 2,33; IC95%: 1,63-3,31) fumantes. O mesmo ocorreu em relação à experimentação de cigarros eletrônicos: irmãos (OR ajustada = 2,76; IC95%: 1,17-6,50) e amigos (OR ajustada = 2,47; IC95%: 1,45-4,22). Curiosidade e aroma/sabor foram os principais motivos para o uso de narguilé e a experimentação de cigarros eletrônicos. Embora 93% dos respondentes tenham aprendido sobre os danos do tabagismo à saúde nas aulas da faculdade de medicina, 51,4% relataram já ter experimentado pelo menos um desses produtos do tabaco. A maioria dos respondentes que relataram sentir a presença de Deus/Espírito Santo em suas vidas nunca experimentou narguilé (59,2%) ou cigarros eletrônicos (55,3%). Conclusões: Há uma alta prevalência de experimentação de produtos do tabaco entre estudantes de medicina cujos irmãos ou amigos fumam, apesar de terem conhecimento sobre os malefícios do tabagismo.

The role of Clinical-Functional Vulnerability Index-20 to detect quality of life in older adults assisted in primary care

SUMMARY OBJECTIVE: To determine the cutoff point of the Clinical-Functional Vulnerability Index-20 to detect poor quality of life in the elderly in Primary Health Care. METHODS: This cross-sectional study was carried out in Primary Health Care units of Ceilândia (DF, Brasil) between September 2019 and January 2020. Four hundred and fifty-eight individuals were included in the study and answered the Clinical-Functional Vulnerability Index-20 (IVCF-20) and the World Health Organization Quality of Life - WHOQOL-BREF (validated to access quality of life) instruments. Based on the WHOQOL-BREF answers, three subgroups were created: poor, good and undetermined quality of life. The receiver operating characteristic curve (using Youden index) showed the IVCF-20 cutoff point to detect poor quality of life (in individuals with good quality of life as the Control Group), and therefore diagnostic tests were performed. RESULTS: The IVCF-20 cutoff point to detect poor quality of life was ≥11. The area under the receiver operating characteristic curve was 0.97 (95%CI 0.95-0.98). The IVCF-20 diagnostic tests showed good sensitivity (88.1%) and specificity (84.9%). CONCLUSIONS: The IVCF-20 ≥11 detected poor quality of life adequately in the elderly in Primary Health Care. These data suggest that elderly individuals with IVCF ≥11 should attend appointments more often in Primary Health Care or geriatrics collaborative care, considering the impact that quality of life can have on the elderly population's mortality.

Association of health vulnerability with adverse outcomes in older people with COVID-19: a prospective cohort study

OBJECTIVES: Health vulnerability is associated with a higher risk of mortality and functional decline in older people in the community. However, few studies have evaluated the role of the Vulnerable Elders Survey (VES-13) in predicting clinical outcomes of hospitalized patients. In the present study, we tested the ability of the VES-13 to predict mortality and the need for invasive mechanical ventilation in older people hospitalized with coronavirus disease 2019 (COVID-19). METHODS: This prospective cohort included 91 participants aged ≥60 years who were confirmed to have COVID-19. VES-13 was applied, and the demographic, clinical, and laboratory variables were collected within 72h of hospitalization. A Poisson generalized linear regression model with robust variance was used to estimate the relative risk of death and invasive mechanical ventilation. RESULTS: Of the total number of patients, 19 (21%) died and 15 (16%) required invasive mechanical ventilation. Regarding health vulnerability, 54 (59.4%) participants were classified as non-vulnerable, 30 (33%) as vulnerable, and 7 (7.6%) as extremely vulnerable. Patients classified as extremely vulnerable and male sex were strongly and independently associated with a higher relative risk of in-hospital mortality (p<0.05) and need for invasive mechanical ventilation (p<0.05). CONCLUSION: Elderly patients classified as extremely vulnerable had more unfavorable outcomes after hospitalization for COVID-19. These data highlight the importance of identifying health vulnerabilities in this population.

The role of VES-13 to identify limited life expectancy in older adults in primary healthcare settings / O potencial do VES-13 na identificação da expectativa de vida limitada em idosos na atenção primária à saúde / Potencial del VES-13 para identificar la esperanza de vida limitada de adultos mayores en centros de atención primaria

ABSTRACT Objective To investigate the potential role of the Vulnerable Elders Survey to identify older adults with limited life expectancy in primary healthcare settings. Method This cross-sectional study was performed in all (nine) healthcare units in Jatai, Goiás (Brazil) from July to December 2018. A sample size of 407 older adults was obtained considering an older population (≥ 60 years old). Participants answered a questionnaire about sociodemographic and clinical characteristics, including the Vulnerable Elders Survey and the Suemoto index. We tested the association between limited life expectancy and the Vulnerable Elders Survey using multiple logistic regression analysis. Results The mean age was 68.9 ± 6.6 yo, and 58.0% were women. The mean score of the Vulnerable Elders Survey was 2.0 ± 2.2, the mean score of Suemoto index was 31.5 ± 21.1%, and 17.2% had limited life expectancy. The Vulnerable Elders Survey was associated with limited life expectancy (OR = 1.57; p = < 0.0001). Conclusion The Vulnerable Elders Survey was able to identify older adults with limited life expectancy in primary healthcare settings and can play a role in detecting older adults who would not benefit from screening and strict control of chronic diseases.

RESUMO Objetivo Investigar o potencial do instrumento Vulnerable Elders Survey para identificar idosos com expectativa de vida limitada, em ambientes de atenção primária à saúde. Método Estudo transversal realizado em todas as (nove) unidades de saúde de Jataí, Goiás (Brasil), no período de julho a dezembro de 2018. Obteve-se uma amostra de 407 idosos, considerando uma população ≥ 60 anos. Os participantes responderam a um questionário sobre características sociodemográficas e clínicas, incluindo o Vulnerable Elders Survey e o índice de Suemoto. Testamos a associação entre a expectativa de vida limitada e o Vulnerable Elders Survey usando análise de regressão logística múltipla. Resultados A idade média foi de 68,9 ± 6,6 anos, e 58,0% dos participantes eram mulheres. A pontuação média do Vulnerable Elders Survey foi de 2,0 ± 2,2, a pontuação média do índice de Suemoto foi de 31,5 ± 21,1%, e 17,2% dos participantes tinham expectativa de vida limitada. O Vulnerable Elders Survey foi associado a uma expectativa de vida limitada (OR = 1,57; p = < 0,0001). Conclusão O instrumento Vulnerable Elders Survey foi capaz de identificar idosos com expectativa de vida limitada em ambientes de atenção primária à saúde, além de poder auxiliar na detecção de idosos que não se beneficiariam com a triagem e o controle estrito de doenças crônicas.

RESUMEN Objetivo Investigar el potencial del instrumento Vulnerable Elders Survey para identificar adultos mayores con esperanza de vida limitada en centros de atención primaria. Método Se trata de un estudio transversal realizado en todas las (nueve) unidades sanitarias de Jataí, Goiás (Brasil) de julio a diciembre de 2018. Se consideró una población de ≥ 60 años, de la cual se obtuvo una muestra de 407 adultos mayores. Los participantes respondieron un cuestionario sobre características sociodemográficas y clínicas, incluyendo el Vulnerable Elders Survey y el índice de Suemoto. Se comprobó la asociación entre la esperanza de vida limitada y el Vulnerable Elders Survey, mediante el análisis de regresión logística múltiple. Resultados La edad promedio era de 68,9 ± 6,6 años y el 58,0% de los participantes pertenecía al sexo femenino. La puntuación media del Vulnerable Elders Survey resultó en 2,0 ± 2,2; la puntuación media del índice de Suemoto, 31,5 ± 21,1% y el 17,2% de los participantes tenía una esperanza de vida limitada. El Vulnerable Elders Survey estaba asociado a una esperanza de vida limitada (OR = 1,57; p = < 0,0001). Conclusión El instrumento Vulnerable Elders Survey ha sido capaz de identificar a los adultos mayores con una esperanza de vida limitada en los centros de atención primaria, además de ayudar en la detección de aquellos adultos mayores que no se beneficiarían con el triaje y el control estricto de las enfermedades crónicas.

VES-13 and WHOQOL-bref cutoff points to detect quality of life in older adults in primary health care

ABSTRACT OBJECTIVE: To determine Vulnerable Elders Survey (VES-13) and WHOQOL-bref cutoff points to detect poor quality of life (QoL) in older individuals. METHODS: This is a cross-sectional study, performed in all primary health care units in Samambaia, DF, Brazil. The data were collected from August 2016 to May 2017. The sample size of 466 older individuals treated in primary health care was obtained considering a 5% margin of error, 95% confidence level, 50% prevalence, and 20% possible losses, in a population of 13,259 older individuals. The subjects answered the VES-13 and WHOQOL-bref questionnaires. They were divided into 3 subgroups: poorQoL (older individuals with self-reported very poor or poor QoL AND very dissatisfied or dissatisfied with their health), goodQoL (very good or good QoL AND very satisfied or satisfied with Health) and indeterminateQoL (NOT belonging to poorQoL or goodQoL subgroups). A receiver-operating characteristic (ROC) curve was performed with poorQoL (case) versus goodQoL (control) to determine the cutoff score in VES-13 and WHOQOL-bref. A diagnostic test using these cutoffs was carried out in all older individuals (n = 466). RESULTS: The VES-13 and WHOQOL-bref cutoff points to detect poorQoL were ≥ 2 and < 60, respectively. The area under ROC curve of VES-13 and WHOQOL-bref was 0.741 (CI95% 0.659-0.823; p < 0.001) and 0.934 (CI95% 0.881-0.987; p < 0.001), respectively. In diagnostic tests, VES-13 showed 84% sensitivity and 98.2% negative predictive value, and WHOQOL-bref, 88% sensitivity and 99% negative predictive value. CONCLUSIONS: VES-13 score ≥ 2 and WHOQOL-bref score < 60 adequately detected poorQoL in patients treated in primary health care. Our data suggest that older individuals with these scores require special treatment such as geriatrics collaborative care to improve this scenario, considering QoL impact on mortality.

Functional health literacy in hypertensive elders at primary health care / Alfabetismo funcional en salud en personas hipertensas en la atención primaria / Alfabetismo funcional em saúde em pessoas idosas hipertensas na atenção primária

ABSTRACT Objective: to assess the relationship between inadequate functional health literacy and inadequate blood pressure control in older people with hypertension in Primary Health Care. Method: a cross-sectional study with sample calculated at 392. SAHLPA-18 tool was used for functional health literacy; blood pressure was measured; sociodemographic and clinical data were collected. Hierarchical logistic regression was used. Results: (high) inadequate blood pressure and (low) functional inadequate health literacy were present in 41.6% and 54.6% of the people, respectively. Factors associated with inadequate blood pressure were: inadequate functional health literacy, black-brown skin color, overweight-obesity, hypertension diagnosis time, non-adherence to exercise/diet, drug treatment. Schooling had no association with inadequate blood pressure Conclusion: hypertensive elderly people with inadequate health literacy were more likely to have inadequate blood pressure. Thus, health professionals need to value functional health literacy as a possible component to control blood pressure.

RESUMEN Objetivo: evaluar la relación entre alfabetismo funcional en salud inadecuada y control inadecuado de la presión arterial en personas ancianas hipertensas em la Atención Primaria de Salud. Método: estudio transversal com muestra calculada en 392. Se utilizó instrumento SAHLPA-18 para alfabetismo funcional en salud; a la presión arterial; recogidos datos sociodemográficos y clínicos. Se utilizo La regresión logística jerárquica. Resultados: La presión arterial inadecuada (alta) y el alfabetismo funcional en salud inadecuada (bajo) estaban presentes en el 41,6% y el 54,6% de las personas, respectivamente. Los factores asociados com la presión arterial inadecuada fueron: alfabetismo funcional en salud inadecuada, color parda-negra, sobrepeso-obesidad, tiempo de diagnóstico de la hipertensión, no adhesión a ejercicio/dieta, no adhesión al tratamiento medicamentoso. El nivel de escolaridad no tuvo asociación con la presión arterial inadecuada. Conclusión: las personas mayores hipertensas con alfabetismo funcional em salud inadecuada presentaron más posibilidades de tener presión arterial inadecuada. Así, los profesionales de La salud necesitan valorizar el alfabetismo funcional en salud como posible componente para controlar la presión arterial.

RESUMO Objetivo: avaliar a relação entre alfabetismo funcional em saúde inadequado e controle inadequado da pressão arterial em pessoas idosas hipertensas na Atenção Primária. Método: estudo transversal com amostra calculada em 392. Foi usado instrumento SAHLPA-18 para alfabetismo funcional em saúde; aferida a pressão arterial; coletados dados sociodemográficos e clínicos. Utilizou-se regressão logística hierárquica. Resultados: pressão arterial inadequada(alta) e alfabetismo funcional em saúde inadequado(baixo) estavam presentes em 41,6% e 54,6% das pessoas, respectivamente. Fatores associados com pressão arterial inadequada foram: alfabetismo funcional em saúde inadequado, cor parda-negra, sobrepeso-obesidade, tempo de diagnóstico da hipertensão, não adesão a exercício/dieta, não adesão a tratamento medicamentoso. O nível de escolaridade não teve associação com pressão arterial inadequada. Conclusão: pessoas idosas hipertensas com alfabetismo funcional em saúde inadequado apresentaram mais chance de ter pressão arterial inadequada. Assim, profissionais de saúde precisam valorizar o alfabetismo funcional em saúde como possível componente para controlar a pressão arterial.

Atualização do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos / Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update

As vasculites antineutrophil cytoplasmic antibody (ANCA, anticorpo anticitoplasma de neutrófilos) associadas (VAAs) são caracterizadas por uma inflamação sistêmica das artérias de pequeno e médio calibre (especialmente no trato respiratório superior e inferior, e nos rins). As VAAs compreendem a granulomatose de Wegener (agora chamada de granulomatose com poliangeíte), poliangeíte microscópica, VAA limitada ao rim e a síndrome de Churg-Strauss. Neste artigo, discutiremos as fases de tratamento dessas vasculites, como fase de indução (com ciclofosfamida ou rituximab) e fase de manutenção (com azatioprina, metotrexato ou rituximab). Além disso, discutiremos como manusear os casos refratários à ciclofosfamida.

In its various forms, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by a systemic inflammation of the small and medium-sized arteries (especially in the upper and lower respiratory tracts, as well as in the kidneys). The forms of AAV comprise Wegener's granulomatosis (now called granulomatosis with polyangiitis), microscopic polyangiitis, renal AAV, and Churg-Strauss syndrome. In this paper, we discuss the phases of AAV treatment, including the induction phase (with cyclophosphamide or rituximab) and the maintenance phase (with azathioprine, methotrexate, or rituximab). We also discuss how to handle patients who are refractory to cyclophosphamide.

Trombose em artérias pulmonares pequenas e médias em granulomatose de Wegener: um estudo com microscopia confocal por varredura a laser / Thrombosis in small and medium-sized pulmonary arteries in Wegener's granulomatosis: a confocal laser scanning microscopy study

OBJETIVO: A granulomatose de Wegener (GW) pode causar dano nas células endoteliais e fenômenos tromboembólicos. Entretanto, poucos estudos analisaram a microcirculação pulmonar - artérias pulmonares de pequeno/médio calibre (APPMC) - em pacientes com GW. O objetivo deste estudo foi quantificar trombos de fibrina em amostras de APPMC de pacientes com GW. MÉTODOS: Analisamos 24 APPMC de seis pacientes com GW e 16 APPMC de quatro pacientes controles sem WG. Utilizamos CD34 para a marcação do endotélio em todas as amostras e microscopia confocal a laser para detectar trombos de fibrina intravasculares. Calculamos a área total do vaso, a área livre do lúmen e a área trombótica. RESULTADOS: A média da área total do vaso foi similar no grupo GW e no grupo controle (32.604 µm² vs. 32.970 µm², p = 0,8793). Trombos foram identificados em 22 das 24 APPMC (91,67 por cento) no grupo GW, e em nenhuma do grupo controle (p < 0,0001; OR = 297 (IC95 por cento: 13,34-6.612). A média da área trombótica foi maior no grupo GW do que no grupo controle (10.068 µm² vs. 0.000 µm², p < 0,0001). Em contraste, a média da área livre do lúmen foi menor no grupo GW que no grupo controle (6.116 µm² vs. 24.707 µm², p < 0,0001). CONCLUSÕES: A microscopia confocal a laser mostrou uma associação significante entre trombose microvascular pulmonar e GW. Isso sugere um possível papel da trombose microvascular na fisiopatologia da GW pulmonar, evocando o potencial benefício da anticoagulação na GW pulmonar. Entretanto, novos estudos são necessários para confirmar nossos achados, assim como um ensaio clínico randomizado a fim de testar o papel da anticoagulação no tratamento de pacientes com GW pulmonar.

OBJECTIVE: Wegener's granulomatosis (WG) can cause endothelial cell damage and thromboembolic events. Nevertheless, there have been few studies on the pulmonary microcirculation-small and medium-sized pulmonary arteries (SMSPA)-in patients with WG. The objective of this study was to quantify fibrin thrombi in the SMSPA of patients with WG. METHODS: We analyzed 24 SMSPA samples collected from six patients with WG and 16 SMSPA samples collected from four patients without WG. In all samples, we used the endothelial cell marker CD34 and confocal laser scanning microscopy in order to detect intravascular fibrin thrombi. We calculated the total vessel area, the free lumen area, and the thrombotic area. RESULTS: The mean total vessel area was similar in the WG and control groups (32,604 µm² vs. 32,970 µm², p = 0.8793). Thrombi were present in 22 (91.67 percent) of the 24 WG group samples and in none of the control group samples (p < 0.0001; OR = 297; 95 percent CI: 13.34-6,612). The mean thrombotic area was greater in the WG group samples than in the control group samples (10,068 µm² vs. 0.000 µm²; p < 0.0001). In contrast, the mean free lumen area was smaller in the WG group samples than in the control group samples (6,116 µm² vs. 24,707 µm²; p < 0.0001). CONCLUSIONS: Confocal laser scanning microscopy revealed a significant association between pulmonary microvascular thrombosis and WG. This suggests a possible role of microvascular thrombosis in the pathophysiology of pulmonary WG, evoking the potential benefits of anticoagulation therapy in pulmonary WG. However, further studies are needed in order to confirm our findings, and randomized clinical trials should be conducted in order to test the role of anticoagulation therapy in the treatment of patients with pulmonary WG.

Acometimento pulmonar na doença de Behçet: uma boa experiência com o uso de imunossupressores / Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy

OBJETIVO: A doença de Behçet (DB) representa uma patologia sistêmica, cuja sobrevida se relaciona com a presença de acometimento pulmonar. Entretanto, sugere-se que pacientes com diferentes tratamentos podem apresentar diferentes prognósticos. O objetivo deste estudo foi avaliar a evolução clínica e tomográfica, bem como a sobrevida deste pacientes com acometimento pulmonar relacionado à DB acompanhados em nosso serviço. MÉTODOS: Uma análise retrospectiva de nossa experiência com pacientes com acometimento pulmonar relaionado a DB acompanhados de 1 de Janeiro de 1988 a 30 de Abril de 2006. Os dados clínicos, radiológicos, terapêuticos e de sobrevida foram obtidos dos prontuários médicos. RESULTADOS: Foram identificados 9 pacientes, com idade média de 34 ± 11,5 anos, sendo 7 deles do sexo masculino. Os achados radiológicos foram aneurisma de artéria pulmonar (AAP) em 8 pacientes, embolia pulmonar em 3 (resultando em uma incidencia de 5,11 casos/100 paciente-anos), hemorragia alveolar em 1 e hipertensão pulmonar em 1 de 9 doentes. O tratamento consistiu-se de prednisona mais clorambucil (ou ciclofosfamida ou micofenolato de mofetil) em todos os 9 pacientes, com resolução total ou parcial dos AAP. O paciente com AAP e hipertensão pulmonar também recebeu sildenafil e warfarina, com boa resposta clínica e tomográfica. A sobrevida de nossos pacientes foi de 88,8 por cento em 3 e 5 anos, com acompanhamento médio de 6,52 anos. CONCLUSÕES: Pacientes com acometimento pulmonar relacionado à DB podem apresentar boa sobrevida com tratamento imunossupressor, e a DB deve ser lembrada como uma possível causa de hipertensão pulmonar e hemorragia alveolar.

OBJECTIVE: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. METHODS: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. RESULTS: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8 percent, as was the five-year survival rate. CONCLUSIONS: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Uma rara causa de dispnéia com apresentação singular na tomografia computadorizada de tórax: síndrome de ativação macrofágica / A very rare cause of dyspnea with a unique presentation on a computed tomography scan of the chest: macrophage activation syndrome

A síndrome de ativação macrofágica é uma doença rara e potencialmente fatal. Ela ocorre devido a uma alteração no sistema imunológico, com excessiva proliferação de macrófagos, geralmente causando hepatoesplenomegalia, pancitopenia e disfunção hepática. Neste artigo, relatamos uma raríssima apresentação da síndrome de ativação macrofágica como insuficiência respiratória, bem como a primeira descrição de nódulo escavado, vidro fosco e consolidação na tomografia computadorizada de alta resolução de pulmão (simulando uma pneumonia ou hemorragia alveolar) e o sucesso terapêutico com o uso de imunoglobulina humana. Assim, sugerimos que a síndrome de ativação macrofágica seja colocada no diagnóstico diferencial de causas de insuficiência respiratória e que o rápido diagnóstico e tratamento seja imperativo para a boa evolução do paciente.

Macrophage activation syndrome is a rare and potentially life-threatening disease. It occurs due to immune dysregulation manifested as excessive macrophage proliferation, typically causing hepatosplenomegaly, pancytopenia and hepatic dysfunction. Here, we report an unusual case of macrophage activation syndrome presenting as dyspnea, as well as (reported here for the first time) high resolution computed tomography findings of an excavated nodule, diffuse ground glass opacities and consolidations (mimicking severe pneumonia or alveolar hemorrhage). The patient was successfully treated with human immunoglobulin. We recommend that macrophage activation syndrome be considered in the differential diagnosis of respiratory failure. Rapid diagnosis and treatment are essential to achieving favorable outcomes in patients with this syndrome.