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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.

To determine the influence of breastfeeding duration in the clinical activity of low-income juvenile idiopathic arthritis (JIA). Ninety-one JIA patients followed in Fortaleza-CE, Brazil, were cross-sectionally evaluated from May 2015 to April 2016. Breastfeeding duration was obtained by interviewing mothers. Mean age was 14.6 ± 5.2 years with 10.31 ± 3.7 years of disease duration. Polyarticular category predominated, with 39 (42.8%) patients, followed by 23 (25.3%) oligoarticular and 17 (18.7%) enthesitis-related. Forty-seven (61.8%) were receiving methotrexate isolated or combined to leflunomide, which was used by 12 (15.4%); 30 (32.9%) were on biologic DMARD with 16 (53.3%) etanercept, 8 (26.7%) adalimumab, 3 (10%) tocilizumab, and 1 (3.3%) each on infliximab, abatacept, and canakinumab. Mean(SD) CHAQ and JADAS27 were 0.37 ± 0.36 and 5.03 ± 6.1, respectively and 22 (24%) had permanent joint deformities. No family declared monthly income over US$900.00 and 32 (37.2%) earned less than US$300.00. Eighty-three (91%) were ever breastfed; over two-thirds were breastfed for more than 3 months. Those breastfed for more than 6 months had less joint deformities and a tendency to lower JADAS27 and CHAQ scores using minimally adjusted general linear or logistic models, as appropriate. Parental smoking or literacy and family income did not differ regarding breastfeeding time. This is a low-income JIA cohort with the highest breastfeeding prevalence ever reported. Breastfeeding over 6 months was associated with less disease activity.Key Point• Long-term breastfeeding benefits juvenile idiopathic arthritis.

To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population.A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients.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.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.

Background To our knowledge the influence of ethnic background in childhood-onset SLE (cSLE) presentation was not evaluated in a large population of Latin American country. Objectives To assess demographic data, clinical manifestations, laboratory abnormalities and disease activity score in cSLE patients according to ethnic groups at diagnosis Methods This multicenter study included cSLE patients(ACR 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 Results According to ethnic groups, 1,537 cSLE patients were classified in: Caucasian (n=786), African-Latin American(n=526), Asian(n=8) and others/unknown(n=217). Comparisons between 1,312 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 Conclusion Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable to 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. Disclosure of Interests None declared

ResumoA contracepção é uma questão importante e deve ser um motivo de preocupação em toda consulta médica de pacientes adolescentes e jovens com doenças reumáticas crônicas. Esta revisão narrativa discute métodos contraceptivos em adolescentes com lúpus eritematoso sistêmico (LES), síndrome antifosfolipídica (SAF), artrite idiopática juvenil (AIJ) e dermatomiosite juvenil (DMJ). Os métodos de barreira são seguros e todos os adolescentes com doenças reumáticas crônicas devem ser incentivados a usá‐los. Os contraceptivos orais combinados (COC) são estritamente proibidos para pacientes com LESJ e SAF com anticorpos antifosfolípides positivos. A contracepção reversível de ação prolongada pode ser incentivada e oferecida rotineiramente a paciente adolescente com LES e outras doenças reumáticas. As pílulas que contêm somente progestina são seguras na maior parte das doenças reumáticas, embora a principal preocupação relacionada com seu uso por adolescentes seja a baixa adesão em decorrência da irregularidade menstrual. As injeções de acetato de medroxiprogesterona de depósito a cada três meses são uma estratégia altamente eficaz de contracepção, embora o seu uso em longo prazo esteja associado à diminuição na densidade mineral óssea. Contraceptivos orais combinados ou outros contraceptivos hormonais combinados podem ser opções para pacientes com AIJ e DMJ. O levonorgestrel oral deve ser considerado como um método de contracepção de emergência para todas as adolescentes com doenças reumáticas crônicas, incluindo pacientes com contraindicação para COC.AbstractContraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies. Reversible long‐acting contraception can be encouraged and offered routinely to the JSLE adolescent patient and other rheumatic diseases. Progestin‐only pills are safe in the majority of rheumatic diseases, although the main concern related to its use by adolescents is poor adherence due to menstrual irregularity. Depot medroxyprogesterone acetate injections every three months is a highly effective contraception strategy, although its long‐term use is associated with decreased bone mineral density. COC or other combined hormonal contraceptive may be options for JIA and JDM patients. Oral levonorgestrel should be considered as an emergency contraception method for all adolescents with chronic rheumatic diseases, including patients with contraindication to COC.

ResumoAngiíte primária do sistema nervoso central juvenil (APSNCJ) é uma doença inflamatória cerebral rara e de etiologia desconhecida. Hemorragia cerebral tem sido raramente reportada em pacientes com APSNCJ, geralmente associada com atraso diagnóstico, ou com um diagnóstico somente por necrópsia. Relata‐se um caso de um paciente do gênero masculino com APSNCJ e que previamente sofreu um acidente vascular cerebral isquêmico. Aos 7 anos e 10 meses de idade, o menino apresentou subitamente cefaleia intensa, vômitos e redução do nível de consciência (escala de coma de Glasgow 7), requerendo imediata intubação traqueal. Uma tomografia computadorizada cerebral demonstrou hematoma intraparenquimatoso no lobo parieto‐occipital direito e um pequeno foco de sangramento no lobo frontal direito, edema vasogênico, herniação do úncus e um desvio de 10mm da linha média para a esquerda. A proteína C‐reativa (9.2mg/dL) e o fator antígeno de von Willebrand (202%) estavam elevados. Foi realizada uma craniotomia descompressiva, seguida pela administração de metilprednisolona e ciclofosfamida. Transcorrida uma semana, o paciente apresentava hemiparesia esquerda, sem outras sequelas. É digno de nota que o déficit motor tem melhorado progressivamente. Nosso caso reforça a inclusão dessa vasculite como diagnóstico diferencial em crianças e adolescentes com hemorragia do sistema nervoso central.AbstractChildhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto‐occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C‐reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

Made available in DSpace on 2019-10-06T16:49:50Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-01-01 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. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César Pediatric Rheumatology Unit Hospital Jose Alencar Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Ribeirao Preto Medical School – University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Pontifícia Catholic University of Sorocaba Pediatric Rheumatology Division Sao Paulo State University (UNESP)

Made available in DSpace on 2018-12-11T17:24:18Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-09-01 Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Objective: The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods: A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions: Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition. Pediatric Rheumatology Unit Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Hospital Jose Alencar Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Hospital Municipal Piedade Pediatric Rheumatology Division Sao Paulo State University (UNESP) FAPESP: 03756-4

Objective To assess the quality of medical care in childhood-onset systemic lupus erythematosus (SLE) at tertiary pediatric rheumatology centers as measured by observance of SLE quality indicators (SLE-QIs). Methods International consensus has been achieved for childhood-onset SLE-QIs capturing medical care provision in 9 domains: diagnostic testing, education of cardiovascular (CV) risk and lifestyles, lupus nephritis (LN), medication management, bone health, ophthalmologic surveillance, transition, pregnancy, and vaccination. Using medical record information, the level of performance of these childhood-onset SLE-QIs was assessed in childhood-onset SLE populations treated at 4 tertiary pediatric rheumatology centers in the US, 2 in Brazil, and 1 center in India. Results A total of 483 childhood-onset SLE patients were assessed. Care for the 310 US patients differed markedly for childhood-onset SLE-QIs addressing LN, bone health, vaccinations, education on CV risk, and transition planning. Performance of safety blood testing for medications was high at all centers. Despite often similar performance on the childhood-onset SLE-QI, access to kidney biopsies was lower in Brazil than in the US. Irrespective of the country of practice, larger centers tended to meet the childhood-onset SLE-QIs more often than smaller centers. Conclusion The childhood-onset SLE-QIs, evidence-based minimum standards of medical care, are not consistently met in the US or some other countries outside the US. This has the potential to contribute to suboptimal childhood-onset SLE outcomes.

Objective.To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE).Methods.A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group Consensus Group criteria (proven, probable, and possible). Also evaluated were demographic, clinical, and laboratory data, and disease activity [SLE Disease Activity Index 2000 (SLEDAI-2K)], cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), treatment, and outcomes.Results.IFI were observed in 33/852 patients (3.9%) with cSLE. Proven IFI was diagnosed in 22 patients with cSLE, probable IFI in 5, and possible IFI in 6. Types of IFI were candidiasis (20), aspergillosis (9), cryptococcosis (2), and 1 each disseminated histoplasmosis and paracoccidioidomycosis. The median of disease duration was lower (1.0 vs 4.7 yrs, p < 0.0001) with a higher current SLEDAI-2K [19.5 (0–44) vs 2 (0–45), p < 0.0001] and current prednisone (PRED) dose [50 (10–60) vs 10 (2–90) mg/day, p < 0.0001] in patients with IFI compared with those without IFI. The frequency of death was higher in the former group (51% vs 6%, p < 0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR 1.108, 95% CI 1.057–1.163, p < 0.0001), current PRED dose (OR 1.046, 95% CI 1.021–1.071, p < 0.0001), and disease duration (OR 0.984, 95% CI 0.969–0.998, p = 0.030) were independent risk factors for IFI (R2 Nagelkerke 0.425).Conclusion.To our knowledge, this is the first study to characterize IFI in patients with cSLE. We identified that disease activity and current glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course, with a high rate of fatal outcome.

RESUMO Objetivo Avaliar as dimensões do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico (LESp) e controles saudáveis. Métodos Foram submetidos a uma ultrassonografia do abdome 30 pacientes com LESp e 30 voluntários saudáveis controle. Foram feitas duas medições do fígado no lobo hepático esquerdo (craniocaudal e anteroposterior) e três no lobo hepático direito (LHD) (craniocaudal posterior [CCP-LHD], craniocaudal anterior e anteroposterior). Foram também avaliadas três medidas das dimensões do baço: longitudinal, transversal e anteroposterior. Foram avaliados dados demográficos, clínicos e laboratoriais, SLEDAI-2K, ECLAM, SLAM e tratamento. Resultados A idade média foi semelhante nos pacientes com LESp e controles (170,31 ± 27,81 vs. 164,15 ± 39,25 meses; p = 0,486). A média da dimensão CCP-LHD foi significativamente maior no grupo LESp em comparação com os controles (13,30 ± 1,85 vs. 12,52 ± 0,93, p = 0,044). Não houve diferenças nos outros parâmetros biométricos do fígado e do baço (p > 0,05). Uma análise especifica realizada apenas nos pacientes com LESp de acordo com a dimensão CCP-LHD ≥ 13,3 cm versus < 13,3 cm mostrou que a mediana do SLEDAI-2K [8 (0-18) vs. 2 (0-8), p = 0,004], ECLAM [4 (0-9) vs. 2 (0-5), p = 0,019] e SLAM [5 (1-13) vs. 2 (0-14), p = 0,016] era significativamente maior em pacientes com maior dimensão CCP-LHD, do mesmo modo que a frequência de nefrite (77% vs. 29%, p = 0,010). As enzimas hepáticas foram semelhantes nos dois grupos (p > 0,05). Foi observada uma correlação positiva entre o SLEDAI-2K e a dimensão CCP-LHD (p = 0,001, r = +0,595). Evidenciou-se uma correlação negativa entre a duração da doença e a dimensão longitudinal do baço (p = 0,031, r = −0,394). Conclusão Os dados levantam a possibilidade de que a atividade da doença pode levar a uma hepatomegalia subclínica e localizada durante o curso da doença. A duração da doença resultou em atrofia do baço em pacientes com LESp. ABSTRACT Objective To evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls. Methods 30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed. Results Mean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3 cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p = 0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = −0.394). Conclusion Our data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.

Made available in DSpace on 2018-12-11T17:16:07Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-11-01 Objectives To assess clinical digital vasculitis (DV) as an initial manifestation of childhood‐onset systemic lupus erythematosus (cSLE) within a large population. Methods Multicenter cross‐sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi‐organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI‐2 K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10(0‐28) vs. 14(0‐58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients. Universidade Federal de São Paulo (Unifesp) Unidade de Reumatologia Pediátrica Universidade de São Paulo (USP) Faculdade de Medicina Unidade de Reumatologia Pediátrica Universidade de São Paulo (USP) Faculdade de Medicina Divisão de Reumatologia Universidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de Botucatu Universidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de Botucatu

Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Among them, invasive aspergillosis (IA), which is usually related to immunosuppressed patients, has been rarely reported in JSLE. From 1983 to 2011, 5604 patients were followed at our institution and 283 (5%) met the American College of Rheumatology (ACR) classification criteria for SLE. Six (2.1%) of our JSLE patients had IA. One of them was previously reported and five will be described herein. Four of them were female. The median age at JSLE diagnosis was 12 years (8–16) and the median interval between diagnosis of JSLE and IA was 6 months (1–38). All had pulmonary involvement and three of them had systemic involvement. The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was 19 (7–22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and/or immunosuppressive drugs at IA diagnosis (azathioprine and/or intravenous cyclophosphamide). They all required treatment in the pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B, itraconazole and/or voriconazole); nonetheless, none of them survived. In conclusion, this was the first report that evaluated the prevalence of IA in a large population of JSLE patients from a tertiary pediatric hospital, and clearly showed the severity of the outcome, especially in patients with active disease and treated with immunosuppressive agents. This study reinforces the importance of early diagnosis and treatment with certain antifungals, especially in critically ill patients.

To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (6 years); group B, school age (≥6 to12 years); and group C, adolescent (≥12 to18 years).This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients.Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages2 years, 4 (10%) were ≥2 to3 years, and 32 (82%) were ≥3 and6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score ≥8, autoantibody profile, elevated acute phase proteins, and low complement levels (P 0.05). However, the frequency of fever (78% versus 61% versus 47%; P 0.0001), hepatomegaly (42% versus 29% versus 14%; P 0.0001), splenomegaly (28% versus 12% versus 4%; P 0.0001), and discoid lupus (13% versus 4% versus 4%; P = 0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss2 kg (19% versus 28% versus 36%; P = 0.017), photosensitivity (34% versus 41% versus 51%; P = 0.006), leukopenia4,000/mmOur large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.

Made available in DSpace on 2018-12-11T17:22:56Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-01-01 Background: Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment. Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment. Results: CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU. Conclusions: To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement. Unit of Pediatric Rheumatology, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar Unit of Pediatric Allergy and Immunology, Faculdade de Medicina da Universidade de São Paulo (FMUSP) Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo (FMUSP) Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP) Faculdade de Medicina de Botucatu, São Paulo State University (UNESP) Irmandade da Santa Casa de Misericórdia de São Paulo State University of Campinas Ribeirão Preto Medical School, University of São Paulo Hospital Infantil Darcy Vargas Hospital Municipal Infantil Menino Jesus Faculdade de Medicina de Botucatu, São Paulo State University (UNESP)

Involvement of the reticuloendothelial system occurs in 20-50% of patients with childhood-onset systemic lupus erythematosus (C-SLE) at disease onset. However, a systematic evaluation of liver and spleen sizes has never been performed in a pediatric population with lupus.

Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient’s symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.

Background The majority of infections in childhood-onset systemic lupus erythematosus (cSLE) patients are caused by virus and bacteria, and less frequently by opportunistic agents, such as fungi. 1 However, studies evaluating solely invasive fungal infections (IFI) in cSLE patients are restricted to case reports or case series 1 without any systematic evaluation of the possible associated risk factors and outcomes in pediatric lupus population. Objectives To study the prevalence, risk factors and mortality of IFI in cSLE patients. Methods A retrospective multicenter cohort study was performed in 852 cSLE patients from 10 Pediatric Rheumatology services of Sao Paulo state, Brazil. An investigator meeting was held and all participants received database training. IFI were diagnosed according to EORTC/MSG Consensus Group (proven, probable and possible). 2 Demographic data, clinical, laboratorial, SLEDAI-2K, SLICC/ACR-DI, treatment and outcome were also evaluated. Results IFI were recorded in 33/852 (3.9%) cSLE patients. Proven IFI was evidenced in 22 cSLE patients, probable IFI in 5 and possible IFI in 6. The most frequent types of IFI were candidiasis (n=20) and aspergillosis (n=9). The median of disease duration was lower (1.0 vs. 4.7 years, p 2 Nagelkerke 0.425). Conclusions This was the first study that characterized IFI in a large cSLE population. We identified that disease activity and glucocorticoid use were the main risk factors for these life-threatening infections, mainly in early disease course and with a high rate of fatal outcome. References Silva MF, Ribeiro AS, Fiorot FJ, et al. Invasive aspergillosis: a severe infection in juvenile systemic lupus erythematosus patients. Lupus 2012;21:1011-6. De Pauw B, Walsh TJ, Donnelly JP, et al. Revised definitions of invasive fungal disease from the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group. Clin Infect Dis 2008;46:1813-21. Disclosure of Interest None declared

Background/Purpose: Recently, 26 quality indicators (QI) categorized into 9 domains for childhood-onset systemic lupus erythematous (cSLE) have been developed based on international consensus and scientific evidence. QI are defined as minimum standards of medical care in support of optimal disease outcomes. The current level at which these QI are followed has not been well documented. Hence, the objective of this study is to assess the current quality of medical care received by patients with cSLE at tertiary pediatric rheumatology centers. Methods: Cross-sectional data pertaining to the QI were acquired via chart review and analyzed collectively in 290 cSLE patients followed at four tertiary pediatric rheumatology centers—two in the United States and two in Brazil. Results: Adherence to the QI differed by QI domain, ranging from 41 to 100%. The QI domain with the highest adherence 100% was Laboratory Testing at the time of diagnosis, and the lowest domain 41% was Medication Management. The recommended kidney biopsies for newly diagnosed lupus nephritis were done in only 67% of the patients and 85% of the patients received at least one of the recommended vaccinations (Table 1). Cardiovascular risks factors were discussed in 86% of cases when patient was 13 years or older. Adherence to the QI differences between countries and insurance status will be presented, supporting that the set of current QI are suitable for international use. Table 1. Adherence to QI by Domain Quality Indicators by Domain Results Lab testing at diagnosis & screening Obtained diagnostic/confirmatory labs within first two visits 100% Obtained lab surveillance of CBC, renal, LFT every 12 months 85% General prevention Prescribed influenza and/or encapsulated organisms vaccination, unless contraindicated 85% Discussed and documented education on sun avoidance at least once in the medical record (e.g., wearing protective clothing, applying sunscreens whenever outdoors, and avoiding sunbathing) 89% Discussed transition plan to appropriate adult healthcare providers with patient age ≥14 years. 78% Lupus nephritis (LN) and hypertension management Kidney biopsy discussed/ordered/performed if developed proteinuria >500 mg/day, or worsening GFR, or urinary sediment 60% Evaluated by a nephrologist in the last year of care for lupus nephritis (LN) and of hypertension 65% Evaluated by rheumatologist every 3 months in last year if a patient has known LN regardless of disease activity. 96% Received kidney biopsy when diagnosed with LN 67% Class III/IV, treated with immunosuppressive + glucocorticoids within 1 month 92% Angiotensin receptor blocker (ARB) or, Angiotensin–converting enzyme (ACE) inhibitor were prescribed if ongoing proteinuria >500 mg/day or worsening GFR in last year of care. 96% Check assessments (Kidney function, urine sediment, and proteinuria) every three months. 99% Medication Management If started new meds, discussed risk vs. benefit of therapy 97% Currently prescribed any antimalarial therapy 92% Attempted to taper a dose of steroids not acceptable for chronic use. 89% Attempted to taper and unable to decrease steroid; added/increased steroid sparing agent. 41% Surveillance for medication safety done at regular intervals. 99% Bone Health Received at least one bone mineral density testing DEXA scan 72% Repeat bone mineral density testing if baseline testing outside normal limits (Z score ≤2) 62% Prescribed calcium/vitamin D if a patient is on any steroid therapy. 78% Ophthalmological surveillance Receives eye exams annually while on anti-malarial therapy 82% Receives eye exams annually while on glucocorticoids 85% Education on cardiovascular risk factors Education on cardiovascular risk factors (smoking, hypertension, high BMI) every 1 year with patient and parent if patient is 13 years or older. 86% Discussed lifestyle modifications (smoking cessation, weight control, exercise) every 2 years with parent and patient 13 years or older. 87% Pregnancy Anti-SSA, anti-SB and anti-phospholipid antibodies have been assessed during pregnancy 100% CNS health Prescribed Immunosuppressive therapy if patient has major neuropsychiatric manifestations in last year of care (Optic neuritis, coma, psychosis … etc.) 94% Conclusion: Based on this initial benchmarking effort, the medical care of patients with cSLE at tertiary pediatric rheumatology centers is very good. Systematic planning and documentation of patient education on lifestyle modifications seems warranted and is expected to improve the self-management skills of cSLE patients. Furthermore, increased focus on bone health, eye exams annually, and adequate vaccination of cSLE patients are areas to continue to monitor.

Objective To evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls. Methods 30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed. Results Mean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25 months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters ( p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥13.3 cm versus p = 0.004], ECLAM [4 (0–9) vs. 2 (0–5), p = 0.019] and SLAM [5 (1–13) vs. 2 (0–14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups ( p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL ( p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen ( p = 0.031, r = −0.394). Conclusion Our data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.

Made available in DSpace on 2018-11-26T17:42:33Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-11-01 Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) Federico Foundation Nucleo de Apoio a Pesquisa Saude da Crianca e do Adolescente of USP (NAP-CriAd) Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in Sao Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients. (C) 2017 Published by Elsevier Editora Ltda. Univ Fed Sao Paulo UNIFESP, Unidade Reumatol Pediat, Sao Paulo, SP, Brazil Univ Sao Paulo, Fac Med, Unidade Reumatol Pediat, Sao Paulo, SP, Brazil Univ Sao Paulo, Fac Med, Div Reumatol, Sao Paulo, SP, Brazil Univ Estadual Paulista, UNESP, Fac Med Botucatu, Hosp Clin Botucatu, Botucatu, SP, Brazil Univ Estadual Paulista, UNESP, Fac Med Botucatu, Hosp Clin Botucatu, Botucatu, SP, Brazil CNPq: CNPq 303422/2015-7 CNPq: 301805/2013-0 CNPq: 305068/2014-8 CNPq: 301479/2015 CNPq: 303752/2015-7

Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10 mm deviation to the left from the midline. C-reactive protein (9.2 mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.