Rare primary vasculitis: update on multiple complex diseases and the new kids on the block.

Systemic vasculitis is a group of rare diseases that share an essential characteristic: inflammation of blood vessel walls. This injury occurs during the disease course, but specific features vary for each entity. In this paper, we will address relevant aspects of the newest monogenic mutation vasculitis, such as deficiency of adenosine deaminase 2 (ADA2) and VEXAS syndrome (UBA1), and other relevant vasculitis, such as Cogan syndrome and Susac syndrome that may share some similarities with them.

Position statement of the Brazilian society of Rheumatology on mesna use as a preventive therapy for bladder disease in patients with systemic autoimmune diseases and systemic vasculitis under cyclophosphamide treatment.

OBJECTIVE: To review current literature to support the use of mesna as a preventive therapy for hemorrhagic cystitis and bladder cancer in patients with systemic autoimmune diseases and systemic vasculitis treated with cyclophosphamide. MATERIALS AND METHODS: The search for articles was conducted systematically through MEDLINE, LILACS, Cochrane Library, and Embase databases. Only articles in English were selected. For available records, titles and abstracts were selected independently by two investigators. RESULTS: Eighteen studies were selected for analysis. The known adverse effects of cyclophosphamide were hematological toxicity, infections, gonadal toxicity, teratogenicity, increased risk for malignancy and hemorrhagic cystitis. Long-term toxicity was highly dependent on cyclophosphamide cumulative dose. The risk of bladder cancer is especially higher in long-term exposure and with cumulative doses above 36 g. The risk remains high for years after drug discontinuation. Hemorrhagic cystitis is highly correlated with cumulative dose and its incidence ranges between 12 and 41%, but it seems to be lower with new regimens with reduced cyclophosphamide dose. No randomized controlled trials were found to analyze the use of mesna in systemic autoimmune rheumatic diseases and systemic vasculitis. Retrospective studies yielded conflicting results. Uncontrolled prospective studies with positive results were considered at high risk of bias. No evidence was found to support the use of mesna during the treatment with cyclophosphamide for autoimmune diseases or systemic vasculitis to prevent hemorrhagic cystitis and bladder cancer. In the scenarios of high cumulative cyclophosphamide dose (i.e., > 30 g), patients with restricted fluid intake, neurogenic bladder, therapy with oral anticoagulants, and chronic kidney disease, mesna could be considered. CONCLUSION: The current evidence was found to be insufficient to support the routine use of mesna for the prophylaxis of hemorrhagic cystitis and bladder cancer in patients being treated for systemic autoimmune diseases and systemic vasculitis with cyclophosphamide. The use may be considered for selected cases.

The favela where I live: the territory from the perspective of young people. / A favela onde moro: o território sob a perspectiva dos jovens.

This article examines a set of photographic productions created by six young people, living in a favela on the outskirts of São Paulo, seeking to reflect on their perception of the territory in which they live. A large part of the population of São Paulo resides in favelas, an urban phenomenon found on the outskirts of large metropolises. Despite the difficulty in accessing basic services, such as health, safety, culture and education, the favela is also a fertile space that nurtures feelings of belonging, solidarity and resistance in its residents. Young people, who also live and circulate in the streets and alleys of the favela, have a specific perspective on their own home territory. This is a qualitative and exploratory study, which uses photography as a research tool. The photographic and cartographic productions of the young people reflected different perspectives of the territory, revealing dimensions of leisure, community articulation, sociability, precariousness of the urban cleaning system, among others. Thus, the undertaking of creating visual narratives made it possible for different aspects of the favela to be exposed, discussed and analyzed.

O presente artigo examina um conjunto de produções fotográficas criadas por seis jovens, moradores de uma favela da periferia de São Paulo, procurando refletir sobre a percepção que eles têm do território em que habitam. Grande parte da população de São Paulo reside em favelas, fenômeno urbano encontrado nas periferias de grandes metrópoles. Apesar da dificuldade do acesso a bens básicos, como saúde, segurança, cultura e educação, a favela também é espaço fértil que nutre sentimentos de pertencimento, solidariedade e resistência em seus moradores. As juventudes, que também vivem e circulam pelas ruas e vielas da favela, detêm um olhar específico sobre seu próprio território de moradia. Esta é uma pesquisa qualitativa exploratória, que utiliza a fotografia como instrumento de pesquisa. As produções fotográficas e cartográficas dos jovens refletiram diferentes perspectivas do território, revelando dimensões de lazer, articulação comunitária, sociabilidade, precariedade do sistema de limpeza urbana, dentre outros. Sendo assim, o empreendimento da criação de narrativas visuais fez com que diferentes aspectos da favela pudessem ser expostos, discutidos e analisados.

A favela onde moro: o território sob a perspectiva dos jovens / The favela where I live: the territory from the perspective of young people

Resumo O presente artigo examina um conjunto de produções fotográficas criadas por seis jovens, moradores de uma favela da periferia de São Paulo, procurando refletir sobre a percepção que eles têm do território em que habitam. Grande parte da população de São Paulo reside em favelas, fenômeno urbano encontrado nas periferias de grandes metrópoles. Apesar da dificuldade do acesso a bens básicos, como saúde, segurança, cultura e educação, a favela também é espaço fértil que nutre sentimentos de pertencimento, solidariedade e resistência em seus moradores. As juventudes, que também vivem e circulam pelas ruas e vielas da favela, detêm um olhar específico sobre seu próprio território de moradia. Esta é uma pesquisa qualitativa exploratória, que utiliza a fotografia como instrumento de pesquisa. As produções fotográficas e cartográficas dos jovens refletiram diferentes perspectivas do território, revelando dimensões de lazer, articulação comunitária, sociabilidade, precariedade do sistema de limpeza urbana, dentre outros. Sendo assim, o empreendimento da criação de narrativas visuais fez com que diferentes aspectos da favela pudessem ser expostos, discutidos e analisados.

Abstract This article examines a set of photographic productions created by six young people, living in a favela on the outskirts of São Paulo, seeking to reflect on their perception of the territory in which they live. A large part of the population of São Paulo resides in favelas, an urban phenomenon found on the outskirts of large metropolises. Despite the difficulty in accessing basic services, such as health, safety, culture and education, the favela is also a fertile space that nurtures feelings of belonging, solidarity and resistance in its residents. Young people, who also live and circulate in the streets and alleys of the favela, have a specific perspective on their own home territory. This is a qualitative and exploratory study, which uses photography as a research tool. The photographic and cartographic productions of the young people reflected different perspectives of the territory, revealing dimensions of leisure, community articulation, sociability, precariousness of the urban cleaning system, among others. Thus, the undertaking of creating visual narratives made it possible for different aspects of the favela to be exposed, discussed and analyzed.

Correlação de indicadores antropométricos de obesidade e prevalência de esteatose hepática não alcoólica diagnosticada por ultrassonografia / Correlation of anthropometric indicators of obesity and prevalence of non-alcoholic hepatic steatosis diagnosed through ultrasonography

OBJETIVO: Correlacionar os índices antropométricos, índice de massa corporal, circunferência da cintura e relação cintura/ quadril com a presença ou não de esteatose hepática diagnosticada pela ultrassonografia. MÉTODOS: Trata-se de estudo descritivo, prospectivo, com abordagem analítica quantitativa, realizado após a aprovação do Comitê de Ética em Pesquisa com Seres Humanos. A amostra contou com 166 pacientes, de ambos os sexos, de 18 a 80 anos de idade. Os pacientes foram diagnosticados como portadores ou não de esteatose hepática na ultrassonografia, e foram analisados os fatores antropométricos citados. Os dados foram analisados por meio do software IBM Stastical Package for Social Science, versão 22.0, para Windows, e o nível de significância utilizado foi p<0,05. A análise das variáveis categóricas foi realizada aplicando-se o teste qui-quadrado de Pearson ou teste exato de Fisher. RESULTADOS: Foram avaliados 166 pacientes pela ultrassonografia e, destes, 39,1% apresentaram esteatose hepática. A presença ou não de esteatose foi correlacionada com os índices antropométricos, e foi obtido resultado estatisticamente significativo para todos estes índices (p<0,0001). A correlação com a gravidade da doença apresentou resultado significativo apenas para a relação cintura/quadril (p=0,0214) e para a circunferência da cintura (p=0,004). CONCLUSÃO: Houve correlação estatística estatisticamente significativa dos índices antropométricos com a presença ou não de esteatose hepática; circunferência da cintura e relação cintura/quadril foram os índices estatisticamente significativos para graus mais avançados, o que mostra a importância da obesidade abdominal na patogênese da doença hepática gordurosa não alcoólica.(AU)

OBJECTIVE: To correlate the anthropometric indexes body mass index (BMI), waist circumference, and waist/hip ratio with the presence or absence of hepatic steatosis diagnosed with ultrasonography. METHODS: This is a descriptive, prospective study with a quantitative analytical approach, carried out after approval by the Human Research Ethics Committee. The sample consisted of 166 patients, of both genders, from 18 to 80 years of age. Patients were diagnosed, through ultrasonography, as having or not having hepatic steatosis and the above-mentioned anthropometric indexes were analyzed. Data was analyzed using the IBM Statistical Package for Social Science® 22.0 software for Windows, and the level of significance used was p<0.05. The analysis of the categorical variables was carried out with the application of either Pearson's chi-squared test or Fisher's exact test. RESULTS: Of the 166 patients evaluated with ultrasonography, 39.1% had hepatic steatosis. The presence or absence of steatosis was correlated with the anthropometric indexes and all of those correlations obtained a statistically significant result (p<0.0001). The correlation with disease severity presented a significant result only for the waist/hip ratio (p=0.0214) and waist circumference (p=0.004). CONCLUSION: There was a statistically significant correlation between the anthropometric indices and the presence or absence of hepatic steatosis, where the waist circumference and waist-to-hip ratio were statistically significant indexes for more advanced degrees of the disease, showing the importance of abdominal obesity in the pathogenesis of non-alcoholic fatty liver disease.(AU)

Lesão renal aguda séptica versus não séptica em pacientes graves: características e desfechos clínicos / Septic versus non-septic acute kidney injury in critically ill patients: characteristics and clinical outcomes

Objetivo: Descrever e comparar as características e os desfechos clínicos de pacientes com lesão renal aguda séptica e não séptica. Métodos: Coorte aberta com 117 pacientes graves com lesão renal aguda consecutivamente admitidos em unidade de terapia intensiva, sendo excluídos aqueles que apresentavam doença renal crônica em estágio avançado, transplante renal, internação ou morte em um período inferior a 24 horas. Presença de sepse e óbito intra-hospitalar representaram, respectivamente, a exposição e o desfecho principal. Análise de confundimento foi realizada com a regressão logística. Resultados: Não houve diferenças na média de idade entre os grupos com lesão renal aguda séptica e não séptica [65,30±(21,27) anos versus 66,35±12,82 anos; p=0,75]. Nos dois grupos, similarmente, observou-se predomínio do sexo feminino (57,4% versus 52,4%; p=0,49) e de afrodescendentes (81,5% versus 76,2%; p=0,49). Os pacientes com sepse apresentaram maiores médias de escore Acute Physiology and Chronic Health Evaluation II [21,73±7,26 versus 15,75± (5,98; p<0,001)] e maiores médias de balanço hídrico (p=0,001). Hipertensão arterial (p=0,01) e insuficiência cardíaca (p<0,001) foram mais frequentes entre os não sépticos. A lesão renal aguda séptica foi associada à maior necessidade de diálise (p=0,001) ...

Objective: This study aimed to describe and compare the characteristics and clinical outcomes of patients with septic and non-septic acute kidney injury. Methods: This study evaluated an open cohort of 117 critically ill patients with acute kidney injury who were consecutively admitted to an intensive care unit, excluding patients with a history of advanced-stage chronic kidney disease, kidney transplantation, hospitalization or death in a period shorter than 24 hours. The presence of sepsis and in-hospital death were the exposure and primary variables in this study, respectively. A confounding analysis was performed using logistic regression. Results: No significant differences were found between the mean ages of the groups with septic and non-septic acute kidney injury [65.30±21.27 years versus 66.35±12.82 years, respectively; p=0.75]. In the septic and non-septic acute kidney injury groups, a predominance of females (57.4% versus 52.4%, respectively; p=0.49) and Afro-descendants (81.5% versus 76.2%, respectively; p=0.49) was observed. Compared with the non-septic patients, the patients with sepsis had a higher mean Acute Physiology and Chronic Health Evaluation II score [21.73±7.26 versus 15.75±5.98; p<0.001)] and a higher mean water balance (p=0.001). Arterial hypertension (p=0.01) and heart failure (p<0.001) were more common in the non-septic patients. Septic acute kidney injury was associated with a greater number of patients who required dialysis (p=0.001) and a ...

Septic versus non-septic acute kidney injury in critically ill patients: characteristics and clinical outcomes.

OBJECTIVE: This study aimed to describe and compare the characteristics and clinical outcomes of patients with septic and non-septic acute kidney injury. METHODS: This study evaluated an open cohort of 117 critically ill patients with acute kidney injury who were consecutively admitted to an intensive care unit, excluding patients with a history of advanced-stage chronic kidney disease, kidney transplantation, hospitalization or death in a period shorter than 24 hours. The presence of sepsis and in-hospital death were the exposure and primary variables in this study, respectively. A confounding analysis was performed using logistic regression. RESULTS: No significant differences were found between the mean ages of the groups with septic and non-septic acute kidney injury [65.30±21.27 years versus 66.35±12.82 years, respectively; p=0.75]. In the septic and non-septic acute kidney injury groups, a predominance of females (57.4% versus 52.4%, respectively; p=0.49) and Afro-descendants (81.5% versus 76.2%, respectively; p=0.49) was observed. Compared with the non-septic patients, the patients with sepsis had a higher mean Acute Physiology and Chronic Health Evaluation II score [21.73±7.26 versus 15.75±5.98; p<0.001)] and a higher mean water balance (p=0.001). Arterial hypertension (p=0.01) and heart failure (p<0.001) were more common in the non-septic patients. Septic acute kidney injury was associated with a greater number of patients who required dialysis (p=0.001) and a greater number of deaths (p<0.001); however, renal function recovery was more common in this group (p=0.01). Sepsis (OR: 3.88; 95%CI: 1.51-10.00) and an Acute Physiology and Chronic Health Evaluation II score >18.5 (OR: 9.77; 95%CI: 3.73-25.58) were associated with death in the multivariate analysis. CONCLUSION: Sepsis was an independent predictor of death. Significant differences were found between the characteristics and clinical outcomes of patients with septic versus non-septic acute kidney injury.

Comparacao dos criterios RIFLE, AKIN e KDIGO quanto a capacidade de predicao de mortalidade em pacientes graves / Comparison of the RIFLE, AKIN and KDIGO criteria to predict mortality in critically ill patients

Objetivo: A lesão renal aguda é uma complicação comum em pacientes gravemente enfermos, sendo os critérios RIFLE, AKIN e KDIGO utilizados para sua classificação. Esse trabalho teve como objetivo a comparação dos critérios citados quanto à capacidade de predição de mortalidade em pacientes gravemente enfermos. Métodos: Estudo de coorte prospectiva, utilizando como fonte de dados prontuários médicos. Foram incluídos todos os pacientes admitidos na unidade de terapia intensiva. Os critérios de exclusão foram tempo de internamento menor que 24 horas e doença renal crônica dialítica. Os pacientes foram acompanhados até a alta ou óbito Para análise dos dados, foram utilizados os testes t de Student, qui-quadrado, regressão logística multivariada e curva ROC. Resultados: A média de idade foi de 64 anos, com mulheres e afrodescendentes representando maioria. Segundo o RIFLE, a taxa de mortalidade foi de 17,74%, 22,58%, 24,19% e 35,48% para pacientes sem lesão renal aguda e em estágios Risk, Injury e Failure, respectivamente. Quanto ao AKIN, a taxa de mortalidade foi de 17,74%, 29,03%, 12,90% e 40,32% para pacientes sem lesão renal aguda, estágio I, estágio II e estágio III, respectivamente. Considerando o KDIGO 2012, a taxa de mortalidade foi de 17,74%, 29,03%, 11,29% e 41,94% para pacientes sem lesão renal aguda, estágio I, estágio II e estágio III, respectivamente. As três classificações apresentaram resultados de curvas ROC para mortalidade semelhantes. Conclusão: Os critérios RIFLE, AKIN e KDIGO apresentaram-se como boas ferramentas para predição de mortalidade em pacientes graves, não havendo diferença relevante entre os mesmos. .

Objective: Acute kidney injury is a common complication in critically ill patients, and the RIFLE, AKIN and KDIGO criteria are used to classify these patients. The present study's aim was to compare these criteria as predictors of mortality in critically ill patients. Methods: Prospective cohort study using medical records as the source of data. All patients admitted to the intensive care unit were included. The exclusion criteria were hospitalization for less than 24 hours and death. Patients were followed until discharge or death. Student's t test, chi-squared analysis, a multivariate logistic regression and ROC curves were used for the data analysis. Results: The mean patient age was 64 years old, and the majority of patients were women of African descent. According to RIFLE, the mortality rates were 17.74%, 22.58%, 24.19% and 35.48% for patients without acute kidney injury (AKI) in stages of Risk, Injury and Failure, respectively. For AKIN, the mortality rates were 17.74%, 29.03%, 12.90% and 40.32% for patients without AKI and at stage I, stage II and stage III, respectively. For KDIGO 2012, the mortality rates were 17.74%, 29.03%, 11.29% and 41.94% for patients without AKI and at stage I, stage II and stage III, respectively. All three classification systems showed similar ROC curves for mortality. Conclusion: The RIFLE, AKIN and KDIGO criteria were good tools for predicting mortality in critically ill patients with no significant difference between them. .

Comparison of the RIFLE, AKIN and KDIGO criteria to predict mortality in critically ill patients.

OBJECTIVE: Acute kidney injury is a common complication in critically ill patients, and the RIFLE, AKIN and KDIGO criteria are used to classify these patients. The present study's aim was to compare these criteria as predictors of mortality in critically ill patients. METHODS: Prospective cohort study using medical records as the source of data. All patients admitted to the intensive care unit were included. The exclusion criteria were hospitalization for less than 24 hours and death. Patients were followed until discharge or death. Student's t test, chi-squared analysis, a multivariate logistic regression and ROC curves were used for the data analysis. RESULTS: The mean patient age was 64 years old, and the majority of patients were women of African descent. According to RIFLE, the mortality rates were 17.74%, 22.58%, 24.19% and 35.48% for patients without acute kidney injury (AKI) in stages of Risk, Injury and Failure, respectively. For AKIN, the mortality rates were 17.74%, 29.03%, 12.90% and 40.32% for patients without AKI and at stage I, stage II and stage III, respectively. For KDIGO 2012, the mortality rates were 17.74%, 29.03%, 11.29% and 41.94% for patients without AKI and at stage I, stage II and stage III, respectively. All three classification systems showed similar ROC curves for mortality. CONCLUSION: The RIFLE, AKIN and KDIGO criteria were good tools for predicting mortality in critically ill patients with no significant difference between them.