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OBJECTIVES: To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients. METHODS: We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots. RESULTS: A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively. CONCLUSIONS: This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.

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BACKGROUND: Inflammatory bowel diseases (IBD), comprising Crohn's disease and ulcerative colitis, are chronic inflammatory diseases of the gastrointestinal tract that often have their onset among adolescents and young adults (AYA). IBD are characterized by episodes of active disease interspersed with periods of remission, and its activity is inversely correlated with health-related quality of life (HRQL). OBJECTIVE: This study aimed to determine whether AYA in remission or with low IBD activity would exhibit HRQL similar to that of age-matched healthy individuals, and whether demographic and disease factors could affect HRQL using a 'patient-reported outcome' instrument. METHODS: This study enrolled only AYA with IBD, with low activity. This research included five multidisciplinary clinics of two academic hospitals: Paediatric Gastroenterology, Gastroenterology, Coloproctology, Paediatric Rheumatology and Adolescent divisions, São Paulo, Brazil. A total of 59 AYA with IBD (age, 13-25 years) and 60 healthy AYA (age, 13-25 years) completed the Pediatric Quality of Life Inventory 4.0 and 36-Item Short-Form Health Survey questionnaires and the visual analogue scale (VAS) for pain. Demographic data, extra-intestinal manifestations, treatment, and outcomes regarding CD and UC were evaluated. RESULTS: AYA with IBD and healthy controls were similar with respect to median ages (18.63 [13.14-25.80] years vs 20.5 [13.68-25.84] years, P=0.598), proportion of female sex (42% vs 38%, P=0.654), and percentage of upper middle/middle Brazilian socioeconomic classes (94% vs 97%, P=0.596). The school/work score was significantly lower in AYA with IBD than in healthy controls (70 [10-100] vs 75 [5-100], P=0.037). The 'general health-perception' score was significantly lower in AYA with IBD than in healthy controls (50 [10-80] vs 0 [25-90], P=0.0002). The median VAS, FACES pain rating scale, and total VAS scores were similar between the two groups (2 [0-10] vs 3 [0-9], P=0.214). No association between HRQL and clinical and demographic parameters was identified among IBD patients. CONCLUSION: AYA with low IBD activity reported poor HRQL in school/work and general health perception domains, which highlights a disability criterion in this vulnerable population.

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ABSTRACT BACKGROUND: Inflammatory bowel diseases (IBD), comprising Crohn's disease and ulcerative colitis, are chronic inflammatory diseases of the gastrointestinal tract that often have their onset among adolescents and young adults (AYA). IBD are characterized by episodes of active disease interspersed with periods of remission, and its activity is inversely correlated with health-related quality of life (HRQL). OBJECTIVE: This study aimed to determine whether AYA in remission or with low IBD activity would exhibit HRQL similar to that of age-matched healthy individuals, and whether demographic and disease factors could affect HRQL using a 'patient-reported outcome' instrument. METHODS: This study enrolled only AYA with IBD, with low activity. This research included five multidisciplinary clinics of two academic hospitals: Paediatric Gastroenterology, Gastroenterology, Coloproctology, Paediatric Rheumatology and Adolescent divisions, São Paulo, Brazil. A total of 59 AYA with IBD (age, 13-25 years) and 60 healthy AYA (age, 13-25 years) completed the Pediatric Quality of Life Inventory 4.0 and 36-Item Short-Form Health Survey questionnaires and the visual analogue scale (VAS) for pain. Demographic data, extra-intestinal manifestations, treatment, and outcomes regarding CD and UC were evaluated. RESULTS: AYA with IBD and healthy controls were similar with respect to median ages (18.63 [13.14-25.80] years vs 20.5 [13.68-25.84] years, P=0.598), proportion of female sex (42% vs 38%, P=0.654), and percentage of upper middle/middle Brazilian socioeconomic classes (94% vs 97%, P=0.596). The school/work score was significantly lower in AYA with IBD than in healthy controls (70 [10-100] vs 75 [5-100], P=0.037). The 'general health-perception' score was significantly lower in AYA with IBD than in healthy controls (50 [10-80] vs 0 [25-90], P=0.0002). The median VAS, FACES pain rating scale, and total VAS scores were similar between the two groups (2 [0-10] vs 3 [0-9], P=0.214). No association between HRQL and clinical and demographic parameters was identified among IBD patients. CONCLUSION: AYA with low IBD activity reported poor HRQL in school/work and general health perception domains, which highlights a disability criterion in this vulnerable population.

RESUMO CONTEXTO: As doenças inflamatórias intestinais (DII), que englobam a doença de Crohn e a colite ulcerativa, são doenças inflamatórias crônicas do trato gastrointestinal que frequentemente se manifestam em adolescentes e adultos jovens (AAJ). As DII são caracterizadas por episódios de doença ativa intercalados com períodos de remissão, e sua atividade se correlaciona inversamente com a qualidade de vida relacionada à saúde (QVRS). OBJETIVO: Este estudo teve como objetivo determinar se AAJ em remissão ou com baixa atividade de DII exibiria QVRS semelhante à de indivíduos saudáveis pareados por idade, e se fatores demográficos da doença poderiam afetar a QVRS usando um instrumento de medidas de desfecho relatadas pelo paciente. MÉTODOS: Este estudo envolveu apenas AAJ com DII, com baixa atividade. Esta pesquisa incluiu cinco clínicas multidisciplinares de dois Hospitais Universitários: Divisões de Gastroenterologia Pediátrica, Gastroenterologia, Coloproctologia, Reumatologia Pediátrica e Adolescentes, São Paulo, Brasil. Um total de 59 AAJ com DII (13-25 anos de idade) e 60 AAJ controle saudáveis (13-25 anos de idade) responderam os questionários Pediatric Quality of Life Inventory 4.0 e 36-Item Short-Form Health Survey e as escalas visuais de dor. Dados demográficos, manifestações extra intestinais, tratamentos e desfechos da doença de Crohn e a colite ulcerativa foram avaliados. RESULTADOS: AAJ com DII e os controles saudáveis foram grupos semelhantes com relação à média de idade (18,63 [13,14-25,80] vs 20,5 [13,68-25,84] anos, P=0,598), quanto à proporção de pacientes do sexo feminino (42% vs 38%, P=0,654), e quanto à porcentagem da classe socioeconômica brasileira média elevada/ média (94% vs 97%, P=0,596). Os escores escola/trabalho foram significativamente mais baixos nos AAJ com DII do que nos controles saudáveis (70 [10-100] vs 75 [5-100], P=0,037). O escore 'percepção geral de saúde' foi significativamente mais baixo nos AAJ com DII do que no agrupamento controle saudável (50 [10-80] vs 0 [25-90], P=0,0002). As escalas de avaliação visual de dor foram semelhantes entre os dois grupos (2 [0-10] vs 3 [0-9], P=0,214). Nenhuma associação entre QVRS e parâmetros clínicos e demográficos foi identificada entre os pacientes com DII. CONCLUSÃO: AAJ com baixa atividade das DII relataram baixa QVRS nos domínios da escola/trabalho e percepção geral da saúde, o que destaca um critério de incapacidade nesta vulnerável população.

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Abstract Objective: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. Methods: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. Results: A total of 150 patients with a mean age of 17.0 years (SD = 2.2 years, range 14-21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension "Talking with providers" consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. Conclusions: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non-specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases.

Resumo Objetivo: Traduzir para o português brasileiro e validar o Questionário de Avaliação do Preparo para a Transição em uma população de adolescentes e adultos jovens com doenças reumáticas crônicas. Este questionário avalia o preparo do paciente para realizar a transição do serviço de saúde pediátrico para a assistência ao adulto. Métodos: Seguimos a metodologia de quatro etapas para a tradução e validação de questionários genéricos que inclui tradução, retrotradução, teste piloto e validação clínica do instrumento final. Utilizamos Análise Fatorial Confirmatória e Coeficiente Alfa de Cronbach para testar a validade do instrumento e sua consistência interna. Resultados: Responderam ao questionário traduzido e adaptado 150 pacientes. A média de idade foi de 17,0 anos (DP = 2,2 anos, variação 14-21 anos). Tinham o diagnóstico de lúpus eritematoso sistêmico juvenil 71 pacientes (47,3%), 64 (42,7%) artrite idiopática juvenil e 15 (10%) dermatomiosite juvenil. Durante a análise fatorial confirmatória, a dimensão "Falando com a Equipe Médica" contendo duas questões teve que ser removida devido à presença de expressivo efeito teto. Todas as outras questões restantes contribuíram favoravelmente para aumentar a consistência interna do questionário, obteve-se um Coeficiente Alfa de Cronbach de 0,776. Conclusões: O Questionário de Avaliação do Preparo para a Transição na sua versão em português brasileiro pode ser validado em uma população de pacientes com doenças reumáticas crônicas em transição, com a exclusão de uma dimensão do questionário original. Por ser um questionário não específico para doenças reumáticas, poderá ser utilizado para avaliar o preparo para a transição de outros pacientes brasileiros com doenças crônicas.

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INTRODUCTION: Musculoskeletal pain is a constant complaint in pediatric practice. The pain may be related to a number of organic diseases and / or be part of the amplified musculoskeletal pain syndromes. Idiopathic musculoskeletal pain (IMSP) is defined as the presence of intermittent pain in three or more body regions for at least three months, excluding organic diseases that could explain the symptoms. OBJECTIVE: To study the gait of children and adolescents with IMSP by dynamic baropodometry. METHODOLOGY: Thirty-two patients with IMSP and 32 healthy controls, matched by age, sex, social class, and body mass index (BMI) were enrolled. All were evaluated for pain intensity through the visual analogue scale (VAS) and gait evaluation using dynamic baropodometry. RESULTS: The mean age of the IMSP group was 13.6 years (SD = 2.1, range 9.8-16.9) and of the control group was 13.5 years (SD = 2.0, range 9.6-16.5). The mean pain scale was 5.4 cm in the IMSP group and 0 cm in the control group (p < 0.001). In gait, the mean right foot velocity of the IMSP group was significantly lower (p = 0.034), the time of the step of the IMSP group was significantly higher (p = 0.003) and the pace of the IMSP group was significantly lower (p = 0.001). CONCLUSION: In our study we observed differences between the gait of children with IMSP and healthy controls according to the dynamic baropodometry. This finding indicates the need for individualized attention to the gait of children with musculoskeletal pain.

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OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. RESULTS: A total of 150 patients with a mean age of 17.0 years (SD=2.2 years, range 14-21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension "Talking with providers" consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. CONCLUSIONS: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non-specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases.

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OBJECTIVE: To report cases of children and adolescents diagnosed with pseudotumor cerebri associated or not with rheumatic disease. METHODS: This was a retrospective study based on medical reports of 29 patients, up to 18 years of age and diagnosed with pseudotumor cerebri, followed up in the Pediatric Rheumatology and Neurology outpatient clinics of a tertiary hospital, until December 2016. RESULTS: Among the 29 patients diagnosed with pseudotumor cerebri, 51.7% were girls and the mean age at the disease onset was 12.3 years. In 18 patients (62%) where an etiology was found, four were associated with a rheumatic disease. The most common symptom was headache (69%) and acetazolamide was the most used medication (69%). Two patients developed blindness and 10 are still being followed up. CONCLUSION: Although rare, pseudotumor cerebri should be considered in children with headaches, especially in patients with rheumatic disease.

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ABSTRACT Objective: To report cases of children and adolescents diagnosed with pseudotumor cerebri associated or not with rheumatic disease. Methods: This was a retrospective study based on medical reports of 29 patients, up to 18 years of age and diagnosed with pseudotumor cerebri, followed up in the Pediatric Rheumatology and Neurology outpatient clinics of a tertiary hospital, until December 2016. Results: Among the 29 patients diagnosed with pseudotumor cerebri, 51.7% were girls and the mean age at the disease onset was 12.3 years. In 18 patients (62%) where an etiology was found, four were associated with a rheumatic disease. The most common symptom was headache (69%) and acetazolamide was the most used medication (69%). Two patients developed blindness and 10 are still being followed up. Conclusion: Although rare, pseudotumor cerebri should be considered in children with headaches, especially in patients with rheumatic disease.

RESUMO Relatar os casos de crianças e adolescentes com diagnóstico de pseudotumor cerebral com ou sem doença reumática. Métodos: Estudo retrospectivo através de revisão de prontuários, 29 pacientes com idade até 18 anos e diagnóstico de pseudotumor, atendidos nos ambulatórios de Reumatologia Pediátrica e Neurologia de um hospital terciário, registrados até dezembro de 2016. Resultados: Dentre os 29 pacientes com diagnóstico de pseudotumor cerebral, 51,7% eram meninas. A média de idade de aparecimento dos sintomas foi de 12,3 anos. Em relação à etiologia do pseudotumor cerebral, em 18 pacientes (62%) foi possível identificar uma causa, sendo o diagnóstico de doença reumática associada em quatro desses casos. Cefaléia foi o sintoma mais frequente (69%), e a medicação mais utilizada foi a acetazolamida (69%). Dois pacientes evoluíram para cegueira e 10 ainda se encontram em seguimento ambulatorial. Conclusão: Concluímos que, apesar de raro, o diagnóstico de pseudotumor cerebral deve ser considerado em crianças com cefaleia, principalmente nos pacientes com doença reumática.

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BACKGROUND: Studies on functional magnetic resonance imaging (fMRI) have shown that adults with musculoskeletal pain syndromes tolerate smaller amount of pressure (pain) as well as differences in brain activation patterns in areas related to pain.The objective of this study was to evaluate, through fMRI, the brain activation in adolescents with idiopathic musculoskeletal pain (IMP) while performing an experimental paradigm of pain. METHODS: The study included 10 consecutive adolescents with idiopathic musculoskeletal pain (average age 16.3±1.0) and 10 healthy adolescents age-matched. fMRI exams were performed in a 3 T scanner (Magnetom Trio, Siemens) using an event-related design paradigm. Pressure stimuli were performed in the nondominant hand thumb, divided into two stages, fixed pain and variable pain. The two local Research Ethics Committees (Ethics Committee from Universidade Federal de São Paulo- Brazil, process number 0688/11, on July 1st, 2011 and Ethics Committee from Hospital Israelita Albert Einsten - Brazil, process number 1673, on October 19th, 2011) approved the study. RESULTS: The idiopathic musculoskeletal pain (IMP) group showed a reduced threshold for pain (3.7 kg/cm2 versus 4.45 kg/cm2, p = 0.005). Control group presented increased bain activation when compared to IMP group in the following areas: thalamus (p = 0.00001), precentral gyrus (p = 0.0004) and middle frontal gyrus (p = 0.03). In intragroup analysis, IMP group showed greater brain activation during the unpredictable stimuli of the variable pain stage, especially in the lingual gyrus (p = 0.0001), frontal lobe (p = 0.0001), temporal gyrus (p = 0.0001) and precentral gyrus (p = 0.03), when compared to predictable stimulus of fixed pain. The same intragroup analysis with the control group showed greater activation during the unpredictable stimuli in regions of the precentral gyrus (p = 0.0001), subcallosal area (p = 0.0001), right and left occipital fusiform gyrus (p = 0.0001; (p = 0.0007), middle gyrus (p = 0.01) and precuneus p = (0.02). CONCLUSION: Adolescents with idiopathic musculoskeletal pain (IMP) tend to request higher brain function in cognitive-emotional areas when interpreting unpredictable sensory-perceptual situations. Therefore, it is assumed that this difference in pain processing in adolescents with IMP make the subjective experience of pain something more intense and unpleasant.

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INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients. METHOD: 22 patients younger than 21 years old with MS or NMO diagnosed before the age of 18 years were evaluated regarding epidemiological data, clinical presentation, association with autoimmune diseases, family history of autoimmune diseases, laboratory findings, imaging studies and presence of auto-antibodies. RESULTS: Among the patients studied, there was a prevalence of females (68.1%). The mean age of symptoms onset was 8 years and 9 months and the mean current age was 16 years and 4 months. Two patients (9%) had a history of associated autoimmune rheumatic disease: one case of juvenile dermatomyositis in a patient with NMO and another of systemic lupus erythematosus in a patient with MS. Three patients (13%) had a family history of autoimmunity in first-degree relatives. Antinuclear antibody was found positive in 80% of patients with NMO and 52% of patients with MS. About 15% of antinuclear antibody-positive patients were diagnosed with rheumatologic autoimmune diseases. CONCLUSION: Among patients with demyelinating diseases diagnosed in childhood included in this study there was a high frequency of antinuclear antibody positivity but a lower association with rheumatologic autoimmune diseases than that observed in studies conducted in adults.

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