RESUMEN
Background: Lymphangioleiomyomatosis (LAM) is a rare disease that can occur sporadically (S-LAM) or associated with the tuberous sclerosis complex (TSC-LAM). The natural history of LAM is not completely understood, including whether there is a difference between the clinical courses of the two forms. This study aimed to compare the clinical, functional and tomographic features between S-LAM and TSC-LAM, and evaluate the annual rates of change in lung function. Methods: This retrospective cohort study included patients with LAM followed up between 1994 and 2019. Clinical, functional and imaging variables were evaluated, and the lung cysts were automatically quantified. Quality of life and predictors of lung function impairment were accessed, and the annual rate of lung function decline was compared between S-LAM and TSC-LAM. Results: Of the 107 patients included, 77 had S-LAM and 30 had TSC-LAM. Although patients with TSC-LAM had a higher prevalence of renal angiomyolipomas and neurological and dermatological manifestations, pulmonary function tests were similar. Patients with S-LAM had a greater rate of forced expiratory volume in 1â s decline and a higher extent of cysts. Pneumothorax, desaturation in the 6-minute walking test and a higher extent of lung cysts were predictors of functional impairment. A greater impact on vitality and emotional health was observed in the TSC-LAM. Conclusion: Greater functional decline and a higher cystic extension were found in patients with S-LAM. Our study provides a broad clinical, functional and tomographic characterisation of patients with LAM, adding valuable information to the existing evidence to better understand the two forms of the disease.
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BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) determines reduced exercise capacity. The speculated mechanisms of exercise impairment in PLCH are ventilatory and cardiocirculatory limitations, including pulmonary hypertension (PH). RESEARCH QUESTION: What are the mechanisms of exercise limitation, the exercise capacity, and the prevalence of dynamic hyperinflation (DH) and PH in PLCH? STUDY DESIGN AND METHODS: In a cross-sectional study, patients with PLCH underwent an incremental treadmill cardiopulmonary exercise test with an evaluation of DH, pulmonary function tests, and transthoracic echocardiography. Those patients with lung diffusing capacity for carbon monoxide (Dlco) < 40% predicted and/or transthoracic echocardiogram with tricuspid regurgitation velocity > 2.5 m/s and/or with indirect PH signs underwent right heart catheterization. RESULTS: Thirty-five patients were included (68% women; mean age, 47 ± 11 years). Ventilatory and cardiocirculatory limitations, impairment suggestive of PH, and impaired gas exchange occurred in 88%, 67%, 29%, and 88% of patients, respectively. The limitation was multifactorial in 71%, exercise capacity was reduced in 71%, and DH occurred in 68% of patients. FEV1 and Dlco were 64 ± 22% predicted and 56 ± 21% predicted. Reduction in Dlco, an obstructive pattern, and air trapping occurred in 80%, 77%, and 37% of patients. FEV1 and Dlco were good predictors of exercise capacity. The prevalence of PH was 41%, predominantly with a precapillary pattern, and mean pulmonary artery pressure correlated best with FEV1 and tricuspid regurgitation velocity. INTERPRETATION: PH is frequent and exercise impairment is common and multifactorial in PLCH. The most prevalent mechanisms are ventilatory, cardiocirculatory, and suggestive of PH limitations. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02665546; URL: www.clinicaltrials.gov.
Asunto(s)
Prueba de Esfuerzo/métodos , Tolerancia al Ejercicio , Histiocitosis de Células de Langerhans , Rendimiento Físico Funcional , Pruebas de Función Respiratoria/métodos , Estudios Transversales , Evaluación de la Discapacidad , Ecocardiografía/métodos , Femenino , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/fisiopatología , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Masculino , Persona de Mediana Edad , Ventilación PulmonarRESUMEN
INTRODUCTION: Serum vascular endothelial growth factor-D (VEGF-D) is a lymphangiogenic growth factor that is considered a valuable tool in the diagnosis of lymphangioleiomyomatosis (LAM). Previous studies have reported a wide variability in VEGF-D serum levels in LAM patients and it seems to be associated with pulmonary impairment and lymphatic involvement. METHODS: We conducted a cross-sectional study from 2009 to 2017 that evaluated VEGF-D serum levels in a cohort of LAM patients who were never treated with mTOR inhibitors and compared them to healthy age-matched volunteers. Clinical and functional parameters were assessed and correlated with their respective serum VEGF-D levels. RESULTS: One hundred and four patients were included in the analysis. Serum VEGF-D levels were higher in LAM patients compared to healthy controls: 796 (404-1588) versus 162 (117-232) pg/mL, respectively (p < 0.001). Patients with tuberous sclerosis complex-LAM, TSC-LAM (20%), had higher levels of VEGF-D when compared to patients with sporadic LAM (80%) [1005 (641-2732) vs. 772 (370-1383), p = 0.05]. Serum VEGF-D levels were weakly correlated with DLCO (r = - 0.26, p = 0.001) and lymphatic involvement was more frequent in those with serum VEGF-D levels equal or above 800 pg/mL (35% vs. 13%, p = 0.02). CONCLUSIONS: In LAM, serum VEGF-D is weakly associated with lung function impairment and strongly associated with lymphatic involvement. VEGF-D is validated for use in Brazilian patients with LAM whose characteristics must be accounted for when evaluating their serum VEGF-D levels.
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Linfangioleiomiomatosis/sangre , Factor D de Crecimiento Endotelial Vascular/sangre , Adulto , Biomarcadores/sangre , Brasil , Estudios de Casos y Controles , Estudios Transversales , Progresión de la Enfermedad , Tolerancia al Ejercicio , Femenino , Volumen Espiratorio Forzado , Humanos , Pulmón/fisiopatología , Linfangioleiomiomatosis/diagnóstico , Linfangioleiomiomatosis/fisiopatología , Sistema Linfático/fisiopatología , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Capacidad de Difusión Pulmonar , Regulación hacia ArribaRESUMEN
ABSTRACT Objective: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. Methods: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Results: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Conclusions: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis.
RESUMO Objetivo: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. Métodos: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013. Resultados: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3. Conclusões: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.
Asunto(s)
Humanos , Masculino , Adulto , Persona de Mediana Edad , Aleaciones/efectos adversos , Aleaciones/toxicidad , Cobalto/efectos adversos , Cobalto/toxicidad , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Profesionales/diagnóstico , Exposición Profesional/efectos adversos , Tungsteno/efectos adversos , Tungsteno/toxicidad , Corticoesteroides/uso terapéutico , Biopsia , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/patología , Pulmón/diagnóstico por imagen , Pulmón/patología , Enfermedades Profesionales/tratamiento farmacológico , Enfermedades Profesionales/patología , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
A 27-year-old female patient was referred to our outpatient clinic with a 1-year history of shortness of breath when walking fast on level ground or when climbing stairs. Symptoms worsened after a second episode of spontaneous left pneumothorax, when a chest tube was placed in another hospital for complete lung expansion. During this hospitalization, an open lung biopsy was performed. There was no history of rhinorrhea, nasal congestion, cough, hemoptysis, wheezing, or expectoration.
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Fibrosis Quística/diagnóstico , Neoplasias Pulmonares/diagnóstico , Pulmón , Linfangioleiomiomatosis , Nódulos Pulmonares Múltiples/diagnóstico , Sirolimus/administración & dosificación , Esclerosis Tuberosa , Adulto , Antibióticos Antineoplásicos/administración & dosificación , Diagnóstico Diferencial , Manejo de la Enfermedad , Femenino , Humanos , Pulmón/diagnóstico por imagen , Pulmón/patología , Linfangioleiomiomatosis/diagnóstico , Linfangioleiomiomatosis/fisiopatología , Linfangioleiomiomatosis/terapia , Esclerosis Tuberosa/diagnóstico , Esclerosis Tuberosa/fisiopatología , Esclerosis Tuberosa/terapiaRESUMEN
OBJECTIVE:: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. METHODS:: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. RESULTS:: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. CONCLUSIONS:: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis. OBJETIVO:: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. MÉTODOS:: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013. RESULTADOS:: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3. CONCLUSÕES:: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.
Asunto(s)
Aleaciones/efectos adversos , Aleaciones/toxicidad , Cobalto/efectos adversos , Cobalto/toxicidad , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Profesionales/diagnóstico , Exposición Profesional/efectos adversos , Tungsteno/efectos adversos , Tungsteno/toxicidad , Corticoesteroides/uso terapéutico , Adulto , Biopsia , Humanos , Pulmón/diagnóstico por imagen , Pulmón/patología , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/patología , Masculino , Persona de Mediana Edad , Enfermedades Profesionales/tratamiento farmacológico , Enfermedades Profesionales/patología , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). METHODS: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. RESULTS: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. CONCLUSIONS: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.
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Inmunosupresores/uso terapéutico , Neoplasias Pulmonares/tratamiento farmacológico , Linfangioleiomiomatosis/tratamiento farmacológico , Sirolimus/uso terapéutico , Adulto , Femenino , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Linfangioleiomiomatosis/diagnóstico por imagen , Persona de Mediana Edad , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). METHODS: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. RESULTS: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. CONCLUSIONS: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols. .
OBJETIVO: A linfangioleiomiomatose (LAM) é uma doença rara que é atualmente considerada uma neoplasia de baixo grau com potencial metastático e evolução variável. Os inibidores de mammalian target of rapamycin (mTOR), como o sirolimo e o everolimo, recentemente se tornaram uma opção para o tratamento de pacientes com LAM, especialmente daqueles com manifestações extrapulmonares. O objetivo deste estudo foi descrever quatro casos de pacientes com LAM no Brasil que apresentaram melhora, especialmente das manifestações extrapulmonares, após tratamento com sirolimo (em doses de 1-4 mg/dia). MÉTODOS: Descrevemos quatro casos de pacientes com LAM e diferentes manifestações extrapulmonares tratados com sirolimo. RESULTADOS: Após o tratamento com sirolimo por 12 meses, um paciente apresentou resolução do quilotórax de difícil manejo, um paciente teve redução significativa do volume do angiomiolipoma renal, e uma paciente apresentou regressão importante de linfangioleiomiomas retroperitoneais e linfonodomegalias abdominais. Após tratamento com sirolimo por 6 meses, um paciente apresentou redução significativa de volumoso linfangioleiomioma retroperitoneal. CONCLUSÕES: Nossos achados confirmam que os inibidores de mTOR são benéficos para pacientes com LAM, especialmente para aqueles com manifestações extrapulmonares, tais como angiomiolipomas renais, linfangioleiomiomas e derrames de origem quilosa. Entretanto, alguns pontos, tais como a dose ideal, a duração do tratamento e os efeitos adversos em longo prazo, ainda precisam ser esclarecidos para que os inibidores de mTOR possam ser incorporados na abordagem da LAM. .
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Adulto , Femenino , Humanos , Persona de Mediana Edad , Inmunosupresores/uso terapéutico , Neoplasias Pulmonares/tratamiento farmacológico , Linfangioleiomiomatosis/tratamiento farmacológico , Sirolimus/uso terapéutico , Neoplasias Pulmonares , Linfangioleiomiomatosis , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that promotes pulmonary cystic destruction and impairs pulmonary function. We aim to describe features and clinical course of LAM patients from Brazil. METHODS: We described the clinical and functional features, performance in six minute walk test (6MWT), management details, survival and clinical course of 84 LAM patients followed in a Brazilian reference centre. RESULTS: All subjects were women, the average age at onset of symptoms was 38 years, and the average at diagnosis was 42 years. The major symptoms during the course of the disease were dyspnoea and pneumothorax. The patients experienced impaired quality of life, with worse scores in the physical and emotional domains. The most common abnormalities in pulmonary function tests were an obstructive pattern and reduced diffusion capacity, whereas a quarter of the patients had normal spirometric results. In the 6MWT, although patients had preserved exercise capacity, more than half of the patients had significant desaturation. Hormonal blockage and doxycycline were the most common treatment modalities employed in our patients. The survival probability from diagnosis was 90% at 5 years, whereas the mean annual rate of decline in FEV1 was 60 ± 78 mL. CONCLUSIONS: Clinical and functional features of the LAM patients from our centre are similar to those from other countries. Our sample showed preserved exercise capacity, with desaturation in the 6MWT, and impaired quality of life. Survival was similar, whereas the annual rate of decline of FEV1 was slightly lower than in recent studies.
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Prueba de Esfuerzo , Pulmón/fisiopatología , Linfangioleiomiomatosis/diagnóstico , Calidad de Vida , Pruebas de Función Respiratoria , Encuestas y Cuestionarios , Adulto , Brasil , Progresión de la Enfermedad , Doxiciclina/uso terapéutico , Emociones , Tolerancia al Ejercicio , Femenino , Volumen Espiratorio Forzado , Antagonistas de Hormonas/uso terapéutico , Humanos , Linfangioleiomiomatosis/tratamiento farmacológico , Linfangioleiomiomatosis/mortalidad , Linfangioleiomiomatosis/fisiopatología , Linfangioleiomiomatosis/psicología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Análisis de Supervivencia , Factores de Tiempo , Resultado del TratamientoRESUMEN
Relato de caso de paciente de 38 anos, feminina, com lúpus eritematoso sistêmico (LES) que apresentou evento tromboembólico arterial agudo em membro inferior direito. A investigação evidenciou a presença de anticorpos antifosfolípides e vegetação asséptica em válvula mitral, endocardite de Libman-Sacks (eLS). São discutidas as possíveis causas de eventos tromboembólicos arteriais no LES, com ênfase nas recomendações atuais para diagnóstico e tratamento da eLS.
Case report of a 38-year-old female patient with systemic lupus erythematosus (SLE) who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE). Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE.
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Adulto , Femenino , Humanos , Anticuerpos Antifosfolípidos/inmunología , Endocarditis/etiología , Endocarditis/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Trombosis/etiología , Trombosis/inmunologíaRESUMEN
Case report of a 38-year-old female patient with systemic lupus erythematosus (SLE) who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE). Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE.
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Anticuerpos Antifosfolípidos/inmunología , Endocarditis/etiología , Endocarditis/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Trombosis/etiología , Trombosis/inmunología , Adulto , Femenino , HumanosRESUMEN
A micobacteriosa atípica é uma doença causada por micobactérias não tuberculosas. Os microorganismos são encontrados na natureza e podem causar doença tanto em imunocomprometidos quanto em imunocompetentes. Alguns pacientes são mais susceptíveis, como os pneumopatas. O acometimento é principalmente pulmonar, mas também pode haver infecção do tecido linfático, pele, subcutâneo e ossos. Os sintomas são inespecíficos e difíceis de serem diferenciados da Tuberculose. Foram criados critérios diagnósticos clínicos e laboratoriais que serão discutidos detalhadamente. O tratamento depende da condição clínica do doente e da micobactéria isolada, sendo importante ressaltar que muitas vezes acontece apenas a contaminação do paciente, não havendo infecção (AU)
Asunto(s)
Humanos , Infecciones por Mycobacterium no Tuberculosas , Micobacterias no Tuberculosas , Infecciones por Mycobacterium no Tuberculosas/cirugíaRESUMEN
A micobacteriosa atípica é uma doença causada por micobactérias não tuberculosas. Os microorganismos são encontrados na natureza e podem causar doença tanto em imunocomprometidos quanto em imunocompetentes. Alguns pacientes são mais susceptíveis, como os pneumopatas. O acometimento é principalmente pulmonar, mas também pode haver infecção do tecido linfático, pele, subcutâneo e ossos. Os sintomas são inespecíficos e difíceis de serem diferenciados da Tuberculose. Foram criados critérios diagnósticos clínicos e laboratoriais que serão discutidos detalhadamente. O tratamento depende da condição clínica do doente e da micobactéria isolada, sendo importante ressaltar que muitas vezes acontece apenas a contaminação do paciente, não havendo infecção