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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: Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients. METHODS: We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients. Data were collected at cSLE diagnosis, during follow-up, and at last visit or death, between September 2016 and May 2019. RESULTS: Of 1077 patients with LN, 59 (5.4%) presented with CKD, 36/59 (61%) needed dialysis, and 7/59 (11.8%) were submitted for kidney transplantation. After Bonferroni's correction for multiple comparisons (p < 0.0013), determinants associated with CKD were higher age at last visit, urinary biomarker abnormalities, neuropsychiatric involvement, higher scores of disease activity at last visit and damage index, and more frequent use of methylprednisolone, cyclosporine, cyclophosphamide, and rituximab. In the regression model analysis, arterial hypertension (HR = 15.42, 95% CI = 6.12-38.83, p ≤ 0.001) and biopsy-proven proliferative nephritis (HR = 2.83, 95%CI = 1.70-4.72, p ≤ 0.001) increased the risk of CKD, while children using antimalarials had 71.0% lower CKD risk ((1.00-0.29) × 100%) than children not using them. The Kaplan-Meier comparison showed lower survival in cSLE patients with biopsy-proven proliferative nephritis (p = 0.02) and CKD (p ≤ 0.001). CONCLUSIONS: A small number of patients manifested CKD; however, frequencies of dialysis and kidney transplantation were relevant. This study reveals that patients with cSLE with hypertension, proliferative nephritis, and absence of use of antimalarials exhibited higher hazard rates of progression to CKD. A higher resolution version of the Graphical abstract is available as Supplementary information.

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ABSTRACT BACKGROUND AND OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease which may persist into adulthood. Pain and joint deformities affect quality of life (QoL). The objective was to study the influence of JIA in QoL of children and adults, comparing the two groups. METHODS: Cross-sectional study of 47 JIA patients (20 children and 27 adults) using the following questionnaires: Juvenile Arthritis Multidimensional Assessment Report (JAMAR), Health Assessment Questionnaire (HAQ) in adults and Childhood Health Assessment Questionnaire CHAQ) in children. Epidemiological, clinical and treatment data was collected. RESULTS: No differences were found in gender, arthritis subset, presence of uveitis and fever, visual analog scale for pain and duration of morning stiffness. Adult patients required more biologics in the treatment (p=0.02) and had higher age at disease onset than children (p=0.001). About 45% of children and 51.8% of adults complained having pain and 40% of children and 48% of adults were not satisfied with the current status of their disease. CONCLUSION: Children and adult patients with JIA had similar degree of pain, morning stiffness and functional impairment. Functional impairment was considered mild but the level of pain found was higher than in other studies. Almost half of patients were not satisfied with the treatment.

RESUMO JUSTIFICATIVA E OBJETIVOS: A artrite idiopática juvenil (AIJ) é uma doença reumática crônica que pode persistir na idade adulta. Dor e deformidades articulares afetam a qualidade de vida (QV). O objetivo foi estudar a influência da AIJ na QV de crianças e adultos, comparando os dois grupos. MÉTODOS: Estudo transversal de 47 pacientes com AIJ (20 crianças e 27 adultos) utilizando os questionários: Juvenile Arthritis Multidimensional Assessment Report (JAMAR), Health Assessment Questionnaire (HAG) em adultos e Childhood Health Assessment Questionnaire (CHAQ) em crianças. Dados epidemiológicos, clínicos e de tratamento foram coletados. RESULTADOS: Não foram encontradas diferenças em sexo, subconjunto de artrite, presença de uveíte e febre, escala analógica visual para dor e duração da rigidez matinal. Pacientes adultos necessitaram mais fármacos biológicos no tratamento (p=0,02) e tinham maior idade de início da doença do que crianças (p=0,001). Cerca de 45% das crianças e 51,8% dos adultos queixaram-se de dor e 40% das crianças e 48% dos adultos não estavam satisfeitos com o estado atual de sua doença. CONCLUSÃO: Pacientes crianças e adultos com AIJ apresentaram graus semelhantes de dor, rigidez matinal e comprometimento funcional. O comprometimento funcional foi considerado leve, mas o nível de dor encontrado foi maior do que em outros estudos. Quase metade dos pacientes não ficou satisfeita com o tratamento.

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OBJECTIVE: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. METHODS: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. RESULTS: cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),p < 0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p < 0.0001), polyneuropathy (9% vs. 1%,p < 0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, p < 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p < 0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.

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OBJECTIVE: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents' and all four grandparents' self-reported ethnicity. The statistical analysis was performed using the Bonferroni's correction (p < 0.0027). RESULTS: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6-18) vs. 12.1(0.3-18) years, p = 0.234], time interval to diagnosis [0.25(0-12) vs. 0.3(0-10) years, p = 0.034], and SLEDAI-2K score [14(0-55) vs. 14(0-63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. CONCLUSIONS: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Key Points • Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. • Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients. • African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia. • The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.

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É relatado um caso de escolar com abscesso de espaço perigoso de pescoço, provavelmente oriundo de uma sinusite inicial, com complicação posterior à mediastinite. Através de drenagem cirúrgica e antibioticoterapia de amplo espectro instituídas precocemente, evitou-se um desfecho fatal, apesar do índice de mortalidade nesses casos chegar até 50% dos casos em alguns serviços.

It is reported a case with school abscess neck dangerous space, probably originating from an initial sinusitis with subsequent complication to mediastinitis. By surgical drainage and antibiotics broad spectrum started early, avoided a fatal outcome, although the mortality rate in these cases reach 50% of cases in some services.

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É apresentado relato de caso de uma pré-escolar grande queimada encaminhada a um serviço hospitalar de referência, a qual evoluiu com óbito devido à sepse subsequente à infecção de cateter, a despeito da utilização de terapia antimicrobiana guiada por antibiograma. O objetivo desse estudo é alertar a equipe pediátrica quanto às particularidades da criança grande queimada, principalmente quando em vigência de infecção, e levantar discussões quanto ao uso precoce, mas não profilático, de antibióticos.

It is presented a case report of a major burn in a preschool referred to a hospital service reference, which evolved to death due to subsequent sepsis infection catheter, despite the use of antimicrobial therapy guided by antibiogram. The aim of this study is to alert the pediatric team and the particularities of major burned kid, especially when in presence of infection, and raise discussions on the early use, but not prophylactic, antibiotics.

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OBJETIVO: Avaliar o estresse psicológico do cuidador do paciente pediátrico com artrite idiopática juvenil (AIJ). MÉTODOS: Estudo observacional analítico transversal não controlado de 40 cuidadores de pacientes com AIJ, que foram avaliados pelo questionário Caregiver Burden Scale. Esta escala analisa cinco domínios do estresse: tensão geral, isolamento, decepção, envolvimento emocional e estresse causado por barreiras impostas pelo meio ambiente, graduando-os de 1 a 4. Os dados obtidos foram submetidos a análise estatística. RESULTADOS: Os cuidadores de pacientes com AIJ são principalmente do sexo feminino (87,5 por cento), casados (92,1 por cento) e com parentesco direto com o paciente (90 por cento). O grau de estresse é maior nos cuidadores de pacientes com AIJ forma poliarticular (p = 0,006), nos solteiros (p = 0,019) e naqueles do sexo feminino (p = 0,017). A dimensão analisada na qual se observou maior nível de estresse foi a de dificuldades relacionadas com o meio ambiente. CONCLUSÃO: Mulheres casadas e com parentesco direto com o paciente são os maiores cuidadores do paciente de AIJ. Cuidado com pacientes de forma poliarticular causa mais estresse do que o de pacientes de forma oligoarticular. Barreiras impostas pelo meio ambiente são responsáveis pelo maior índice de estresse nestes cuidadores.

OBJECTIVE: To assess psychological stress in primary caregivers of juvenile idiopathic arthritis (JIA) pediatric patients. METHODS: Uncontrolled cross-sectional analytical study of 40 caregivers of JIA patients. Caregivers were evaluated using the Caregiver Burden Scale, which analyzes five domains of stress on a scale of 1 to 4: general strain, isolation, disappointment, emotional involvement and strain caused by environmental barriers. The data were subjected to statistical analysis. RESULTS: Caregivers of JIA patients were mainly female (87.5 percent), married (92.1 percent) and close relatives (90 percent). Stress levels were higher in caregivers of polyarticular JIA patients (p = 0.006), single caregivers (p = 0.019) and female caregivers (p = 0.017). Environment-related difficulties were reported as the most stressful category by caregivers. CONCLUSION: Caregivers of JIA patients are usually married female relatives. Caring for polyarticular JIA patients is more stressful than caring for oligoarticular JIA patients. Strain caused by environmental barriers accounts for the highest levels of stress among the caregivers included in this study.

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OBJECTIVE: To assess psychological stress in primary caregivers of juvenile idiopathic arthritis (JIA) pediatric patients. METHODS: Uncontrolled cross-sectional analytical study of 40 caregivers of JIA patients. Caregivers were evaluated using the Caregiver Burden Scale, which analyzes five domains of stress on a scale of 1 to 4: general strain, isolation, disappointment, emotional involvement and strain caused by environmental barriers. The data were subjected to statistical analysis. RESULTS: Caregivers of JIA patients were mainly female (87.5%), married (92.1%) and close relatives (90%). Stress levels were higher in caregivers of polyarticular JIA patients (p = 0.006), single caregivers (p = 0.019) and female caregivers (p = 0.017). Environment-related difficulties were reported as the most stressful category by caregivers. CONCLUSION: Caregivers of JIA patients are usually married female relatives. Caring for polyarticular JIA patients is more stressful than caring for oligoarticular JIA patients. Strain caused by environmental barriers accounts for the highest levels of stress among the caregivers included in this study.

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OBJETIVO: Determinar os valores séricos do ligante do receptor do ativador do fator nuclear Kappa B (RANKL) e da osteoprotegerina (OPG) em pacientes com Artrite Idiopática Juvenil de início poliarticular (AIJp) em atividade e avaliar a possível correlação destes valores com a presença radiológica de erosões ósseas. MÉTODOS: Trinta pacientes do sexo feminino com diagnóstico de AIJ de início poliarticular segundo os critérios da ILAR (International League of Associations for Rheumatology) em atividade e trinta crianças saudáveis (controles) pareadas por idade e sexo foram selecionadas consecutivamente para este estudo. Todas as articulações comprometidas foram radiografadas e avaliadas, com especial interesse, para a presença de erosões ósseas. Concentrações séricas do RANKL e OPG foram medidas por enzima-imunoensaio (Biomedica, Vienna, Austria). RESULTADOS: Pacientes com AIJp em atividade apresentaram altos valores séricos de RANKL e menores taxas de OPG/RANKL comparadas com controles [2,90 (0,1-37,4) vs. 0,25 (0,1-5,7) pg/ml, p=0,007 e 21,25 (1,8- 897,6) vs. 347,5 (9-947,8)] pg/ml, p=0,005). Diferentemente, não houve diferença em relação à concentração sérica de OPG quando se comparou os pacientes e controles [55,24 (28,34-89,76) vs. 64,42 (30,68-111,28) pg/ml, p=0,256]. Maiores concentrações de RANKL e menores taxas de OPG/RANKL também foram observadas em pacientes com AIJp em atividade com erosões ósseas comparadas com controles [3,49 (0,1-37,4) vs. 0,25 (0,1-5,7) pg/ml, p=0,0115 e 14,3 (1,8-897.6) vs. 347,5 (9-947,8) p=0,016]. Em contraste, valores séricos de RANKL e a taxa de OPG/RANKL foram semelhantes em pacientes com AIJp sem erosões ósseas comparadas com controles...

OBJECTIVE: To determine the serum levels of receptor activator of nuclear factor kB-ligand (RANKL) and osteoprotegerin (OPG) in active polyarticularonset Juvenile Idiopathic Arthritis patients (pJIA) and evaluate its possible correlation with bone erosions on the X-ray. METHODS: Thirty female girls with active pJIA diagnosis according ILAR criteria (International League of Associations for Rheumatology) and 30 healthy children gender and agematched controls were consecutively selected for this study. All involved articulations were evaluated by X-ray and analyzed for the presence of bone erosions. The serum levels of RANKL and OPG were measured using an enzyme-linked immunosorbent (Biomedica, Vienna, Austria). RESULTS: Results: Patients with active pJIA had higher levels of serum RANKL and lower OPG/RANKL ratio compared to controls [2.90 (0.1-37.4) vs. 0.25 (0.1- 5.7) pg/ml, p=0.007] and 21.25 (1.8-897.6) vs. 347.5 (9-947.8) pg/ml, p=0.005]. However, levels of OPG were comparable in both groups [55.24 (28.34-89.76) vs. 64.42 (30.68-111.28) pg/ml, p=0.256]. Higher levels of serum RANKL and lower OPG/RANKL ratio was also observed in active pJIA patients with bone erosions compared to controls [3.49 (0.1-37.4) vs. 0.25 (0.1-5.7) pg/ml, p=0.0115 and 14.3 (1.8-897.6) vs. 347.5 (9-947.8), p=0.016]. In contrast, RANKL levels and OPG/RANKL ratio were alike in pJIA patients without bone erosion and controls [1.75 (0.1-10.9) vs. 0.25 (0.1- 5.7) pg/ml, p=0.055 and 29.2 (3.3-756.8) vs. 347.5 (9-947.8), p=0.281]. CONCLUSION: These data suggest that active pJIA with bone erosion is associated with high serum levels of RANKL and low OPG/RANKL ratio indicating that these alterations may reflect bone damage in this disease.

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Objetivo: avaliar o etanercepte no tratamento da artrite idiopática juvenil (AIJ) não-responsiva aos imunossupressores. Casuística e métodos: foram selecionados os casos de AIJ não-responsivos ao...

Objective: to evaluate the enercept therapeutic effect on pattients with juvenile idiopathic arthritis (JIA) non-responsive to immunossupressors. Casuistic and methods: patients with JIA non-responsive to...

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A febre reumática (FR) permanece um problema de Saúde Pública nos países subdesenvolvidos e mesmo nos desenvolvidos ela está ressurgindo. Portanto, a proposta deste estudo é delinear a epidemiologia dessa patologia em nossa cidade para que se possa desenvolver futuramente estratégias de prevençäo adequadas. Procedeu-se a análise retrospectiva de uma amostra de 267 crianças abaixo de 15 anos atendidas no Serviço de Reumatologia Pediátrica do Do Hospital Universitário Evangélico de Curitiba, no período de abril de 1986 a abril de 1997, dos quais 81 (30,3 por cento) apresentaram diagnóstico de FR e tiveram seus prontuários analisados detalhadamente, quanto aos aspectos clínicos, diagnósticos e terapêuticos. Na análise dos pacientes com FR, observou-se uma incidência significativamente maior nas crianças com idade entre 9 e 11 anos (53,1 por cento) (p<0,001), bem como a raça branca foi a prevalente (95,0 por cento) (p<0,001). Os meses de junho, julho e agosto constituem o período em que mais se giagnosticou a FR, sendo o mês de junho o de maior frequência (18,5 por cento) (p<0,005). Houve um pico de casos nos anos de 1988 e 1990 (13,6 por cento) em cada ano (p<0,005). A manifestaçäo clínica mais incidente foi a cardite com 54 casos (66,7 por cento) do total e associada a poliartrite também apresentou-se como a mais frequente com 49,4 por cento (p<0,001). Oexame laboratorial de VHS encontrou-se aumentado em um número significativo de pacientes, 71,8 por cento (28 de 39) (p<0,01). Atravé deste estudo vemos que a prevençäo pode ser feita com maior eficácia concentrando esforeços principalmente à prevençäo primária, alertando tanto profissionais da saúde, como as crianças de todas as idades durante o ano todo

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