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Made available in DSpace on 2022-04-29T08:46:03Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Objective: To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). Methods: This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1). Results: Median disease duration was 2.8 (IQR 1.8–3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773–24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481–16.399, p25 was associated with more overall (SDI ≥ 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). Conclusions: The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo 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 Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Hospital da Criança de Brasília Jose Alencar Post-graduation Program in Medical Science and Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Ribeirao Preto Medical School University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Pedro Ernesto University Hospital 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 Federal University of Pará Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit Hospital Menino Jesus Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Division Sao Paulo State University (UNESP)

Made available in DSpace on 2020-12-10T20:01:32Z (GMT). No. of bitstreams: 0 Previous issue date: 2020-06-05 Objective This study aimed to assess the safety and immunogenicity of the quadrivalent human papillomavirus (qHPV) vaccination in childhood-onset systemic lupus erythematosus (cSLE) patients. Methods Volunteer cSLE patients aged 9-20 years and healthy controls (HC) were enrolled to receive a two- or three-dose qHPV vaccination schedule from March 2014 to March 2016. Study visits were performed before the first dose, one month after the second and third doses and one year after the first dose. In each study visit, disease activity and adverse events following vaccination were analyzed, and a serum sample was collected for testing antibody concentrations. Participant recruitment was conducted in 15 Brazilian paediatric rheumatology units. Of the 256 cSLE patients included, 210 completed the two- or three-dose schedules; 15 had previously received one dose, and 18 had received two doses of the vaccine. The analysis was based on intention-to-treat so that participants who did not complete the entire study protocol were also included. Results No severe adverse events were related to the vaccination. Disease activity was generally low and remained stable or even improved. The HC presented 100% seropositivity to HPV16 and HPV18, whereas the two- and three-dose cSLE groups presented 93% and 83% versus 97% and 91%, respectively. One year after the first dose, seropositivity of the three-dose cSLE group was 91% to HPV16 and 84% to HPV18. Conclusions HPV vaccination in cSLE patients is safe and immunogenic. Since the seropositivity to HPV16 and HPV18 was higher for the three-dose schedule group, this regimen should be recommended for cSLE patients. Univ Med Ctr Utrecht, Wilhelmina Childrens Hosp, Dept Paediat Immunol & Rheumatol, Utrecht, Netherlands Univ Fed Parana, Hosp Clin, Dept Paediat, Curitiba, Parana, Brazil Univ Sao Paulo, Dept Paediat, Fac Med Ribeirao Preto, Sao Paulo, Brazil Univ Estado Rio De Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Sao Paulo, Hosp Clin, Inst Crianca, Dept Paediat Rheumatol,Fac Med, Sao Paulo, Brazil Hosp Infantil Albert Sabin, Dept Paediat Rheumatol, Fortaleza, Ceara, Brazil Univ Florida, Coll Med, Dept Paediat Immunol & Rheumatol, Gainesville, FL USA Univ Estadual Campinas, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Crianca Brasilia Jose Alencar, Dept Paediat Rheumatol, Brasilia, DF, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil Univ Fed Rio de Janeiro, Inst Puericultura & Pediat Martagao Gesteira IPPM, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Fed Rio de Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Hosp Infantil Darcy Vargas, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Estadual Infantil Nossa Senhora da Gloria, Dept Paediat Rheumatol, Vitoria, ES, Brazil Santa Casa Sao Paulo, Dept Paediat Rheumatol, Sao Paulo, Brazil Univ Fed Bahia, Dept Paediat Rheumatol, Fac Med, Salvador, BA, Brazil Univ Fed Sao Paulo, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Pequeno Principe, Dept Paediat Rheumatol, Curitiba, Parana, Brazil Natl Inst Publ Hlth & Environm RIVM, Ctr Infect Dis Control, Bilthoven, Netherlands Fac Med Barretos FACISB, Dept Paediat Rheumatol, Barretos, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil

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: Concerns about the safety and efficacy of vaccinations in patients with pediatric rheumatic diseases, such as juvenile dermatomyositis, have led to contradictions and low vaccination coverage in this group, who are at a higher risk of infections, including by human papillomavirus (HPV). Although HPV vaccine should be recommended for all juvenile dermatomyositis patients, there is a lack of data to support the safety of this vaccine. The aim of this study was to assess the safety and immunogenicity of the quadrivalent HPV vaccination in juvenile dermatomyositis patients.Methods: Juvenile dermatomyositis patients aged from 9-20 years and healthy controls were enrolled to receive a 3-dose schedule of quadrivalent HPV vaccination from March/2014 until March/2016. Study visits were performed before the first dose, one month after the second and third doses and one year after the first dose. Participants completed a diary of possible adverse events following each dose, and disease activity was measured. At each visit, serum samples was collected for testing antibody concentrations. Participants recruitment was conducted in ten Brazilian centers. From 48 eligible patients, 42 completed the 3 doses schedule of the vaccine (5 patients had received doses previously). The McNemar test and the Kappa concordance coefficient were applied to compare the disease activity scores used for juvenile dermatomyositis patients between quadrivalent HPV vaccine doses and before the vaccination. The software used was SAS 9.4.Results: No severe adverse events were related to the vaccination. The disease activity scores were usually low, and remained stable, or even improved during the follow-up. After three vaccine doses the juvenile dermatomyositis group presented seropositivity of 100% for HPV16 and 97% for HPV18, similarly to the control group who presented 100% for both. One year after the first dose the seropositivity for the patients group was 94% for both HPV types.Conclusions: The HPV vaccination in juvenile dermatomyositis patients is safe and immunogenic. Since the seropositivity against HPV16 and HPV18 was very high after the 3-dose schedule, this regimen should be recommended for juvenile dermatomyositis patients.

Introduction Children and adolescents with rheumatic diseases receiving TNF blockers are at risk for the activation of latent Mycobacterium tuberculosis infection (LTBI). Although LTBI treatment is indicated in this group, there are different therapeutic regimens in the literature, without a definite consensus. Objectives To review in the literature therapeutic schemes used and indicated for the treatment of LTBI in these patients. Methods Systematic review of the literature, using health databases, selecting studies that addressed the treatment of LTBI in patients with juvenile rheumatic diseases using TNF blockers, from 1990 to 2015. All study designs were considered. Results A total of 162 studies were identified through the electronic databases and one was found through a manual search by the author, totaling 163 articles. We excluded studies that did not meet the mentioned inclusion criteria, and included a retrospective cohort study and two prospective cohort studies. The three studies addressed treatment with isoniazid (INH) for 9 months and one of them also addressed INH treatment associated with rifampicin for 3 months. Conclusions Only one case of LTBI activation was observed; there was good treatment adherence and absence of complications during follow-up. More studies are necessary to evaluate the response to the other available therapeutic regimens, with better tolerability assessment and a larger sample. However, the results showed that INH therapy for 9 months and INH therapy plus rifampicin for 3 months had a low rate of LTBI activation and complications.

Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents. Resumo As doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta- Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar-se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.

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

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)

ResumoObjetivoDescrever as características clínicas e angiográficas da arterite de Takayasu em crianças e adolescentes brasileiros.MétodosFoi feita coleta retrospectiva de dados de 71 crianças e adolescentes acompanhados em 10 centros brasileiros de referência em reumatologia pediátrica. A avaliação foi feita em três tempos: início dos sintomas até o diagnóstico, do 6° ao 12° mês de diagnóstico e última consulta.ResultadosDos 71 pacientes selecionados, 51 (71,8%) eram meninas. As médias de idade de início dos sintomas e de tempo até diagnóstico foram 9,2 anos (± 4,2) e 1,2 anos (± 1,4), respectivamente. No fim do estudo, 20 pacientes estavam em atividade de doença, 39 em remissão e cinco haviam evoluído a óbito. Os sintomas mais frequentes nas avaliação inicial, segunda avaliação e avaliação final foram, respectivamente, os constitucionais, os musculoesqueléticos e os neurológicos. A redução de pulsos periféricos foi o sinal cardiovascular mais frequente e a elevação da velocidade de hemossedimentação foi o achado laboratorial mais frequente nos três períodos de avaliação. O teste tuberculínico foi reagente em 41%. A estenose foi a lesão angiográfica mais encontrada, a artéria abdominal foi o segmento mais afetado e tipo angiográfico IV o mais frequente. A maioria (90%) fez terapia com glicocorticoides, 85,9% necessitaram de outro imunossupressor e 29,6% foram submetidos à angioplastia.ConclusãoEste é o maior estudo de arterite de Takayasu juvenil e nós observar elevado número de pacientes com idade inferior a 10 anos e a predominância de sintomas constitucionais no início da doença.AbstractObjectiveTo describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents.MethodsA retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit.ResultsOf 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (± 4.2) years and 1.2 (± 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty.ConclusionThis is the largest study on juvenile‐onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

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

Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn's disease, Prader-Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.

Introduction: Children and adolescents with rheumatic diseases receiving TNF blockers are at risk for the activation of latent Mycobacterium tuberculosis infection (LTBI). Although LTBI treatment is indicated in this group, there are different therapeutic regimens in the literature, without a definite consensus. Objectives: To review in the literature therapeutic schemes used and indicated for the treatment of LTBI in these patients. Methods: Systematic review of the literature, using health databases, selecting studies that addressed the treatment of LTBI in patients with juvenile rheumatic diseases using TNF blockers, from 1990 to 2015. All study designs were considered. Results: A total of 162 studies were identified through the electronic databases and one was found through a manual search by the author, totaling 163 articles. We excluded studies that did not meet the mentioned inclusion criteria, and included a retrospective cohort study and two prospective cohort studies. The three studies addressed treatment with isoniazid (INH) for 9 months and one of them also addressed INH treatment associated with rifampicin for 3 months. Conclusions: Only one case of LTBI activation was observed; there was good treatment adherence and absence of complications during follow-up. More studies are necessary to evaluate the response to the other available therapeutic regimens, with better tolerability assessment and a larger sample. However, the results showed that INH therapy for 9 months and INH therapy plus rifampicin for 3 months had a low rate of LTBI activation and complications. RESUMO Introdução: Crianças e adolescentes com doenças reumáticas em terapia anti-TNF-α são grupo de risco para ativação da infecção latente por Mycobacterium tuberculosis (ILTB). Embora o tratamento da ILTB seja indicado nesse grupo, existem diferentes esquemas terapêuticos na literatura, sem um consenso definido. Objetivos: Revisar na literatura esquemas terapêuticos usados e indicados para o tratamento da ILTB nesses pacientes. Métodos: Revisão sistemática da literatura, nas bases de dados em saúde, selecionaram-se estudos que abordaram o tratamento da ILTB em pacientes reumáticos juvenis em uso de anti-TNF-α, de 1990 a 2015. Todos os desenhos de estudo foram considerados. Resultados: Foram identificados através das bases de dados eletrônicas 162 estudos e um foi encontrado por meio de busca manual do autor, total de 163. Foram excluídos os estudos que não atenderam aos critérios de inclusão referidos, incluídos um estudo de coorte retrospectiva e dois de estudos de coorte prospectivas. Os três estudos abordaram o tratamento com isoniazida (INH) por nove meses e um deles abordou também o tratamento com INH associado a rifampicina por três meses. Conclusões: Foi observado apenas um caso de ativação da ILTB; uma boa adesão ao tratamento e ausência de complicações durante o acompanhamento. Mais estudos são necessários para avaliar a resposta aos outros esquemas terapêuticos disponíveis, com melhor avaliação da tolerabilidade e maior amostra. Porém, os resultados mostraram que a terapia com INH por nove meses e a terapia com INH mais rifampicina por três meses têm baixo índice de ativação e complicações.

ObjectivesTo describe the clinical characteristics and the occurrence of atypical arthritis in children diagnosed with rheumatic fever (RF) and followed in tertiary care clinics in Salvador, Bahia, Brazil.MethodologyA descriptive study of a case series, of the initial clinical presentation, and of recurrence in 41 children diagnosed with RF.ResultsOf the patients studied (n=41), 61% were male, mean age of 9.2 years, and mean age at diagnosis between 5 and 16 years. Arthritis was present in 75.6% of patients; carditis in 75.6%; chorea in 31.7%; erythema marginatum in 14.6%; and subcutaneous nodules in 4.9%. An atypical pattern was observed in 22 of 31 cases of arthritis (70.9%): involvement of small joints and/or axial skeleton in 12 cases (38.7%); >3 weeks of duration in 9 (29%); inadequate response to NSAIDs in 2 (6.5%); oligoarthritis (≤4 joints) in 22/31 (71%), with monoarthritis in 6/31 (1 in the foot, 1 in the ankle, and 4 in the knee). Fever was present in 78% of the cases, and 82.9% of patients were regularly on secondary prophylaxis.ConclusionAtypical arthritis was present in most patients presenting with joint involvement, being a confounding factor against a proper diagnosis and of therapeutic delay.

Objective: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. Methods: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6 th to 12th month of diagnosis, and in the last visit. Results: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (± 4.2) years and 1.2 (± 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. Conclusion: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed. RESUMO Objetivo: Descrever as características clínicas e angiográficas da arterite de Takayasu em crianças e adolescentes brasileiros. Métodos: Foi feita coleta retrospectiva de dados de 71 crianças e adolescentes acompanhados em 10 centros brasileiros de referência em reumatologia pediátrica. A avaliação foi feita em três tempos: início dos sintomas até o diagnóstico, do 6º ao 12º mês de diagnóstico e última consulta. Resultados: Dos 71 pacientes selecionados, 51 (71,8%) eram meninas. As médias de idade de início dos sintomas e de tempo até diagnóstico foram 9,2 anos (± 4,2) e 1,2 anos (± 1,4), respectivamente. No fim do estudo, 20 pacientes estavam em atividade de doença, 39 em remissão e cinco haviam evoluído a óbito. Os sintomas mais frequentes nas avaliação inicial, segunda avaliação e avaliação final foram, respectivamente, os constitucionais, os musculoesqueléticos e os neurológicos. A redução de pulsos periféricos foi o sinal cardiovascular mais frequente e a elevação da velocidade de hemossedimentação foi o achado laboratorial mais frequente nos três períodos de avaliação. O teste tuberculínico foi reagente em 41%. A estenose foi a lesão angiográfica mais encontrada, a artéria abdominal foi o segmento mais afetado e tipo angiográfico IV o mais frequente. A maioria (90%) fez terapia com glicocorticoides, 85,9% necessitaram de outro imunossupressor e 29,6% foram submetidos à angioplastia. Conclusão: Este é o maior estudo de arterite de Takayasu juvenil e nós observar elevado número de pacientes com idade inferior a 10 anos e a predominância de sintomas constitucionais no início da doença.

To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers. Pituitary gonadotrophins [follicle-stimulating hormone (FSH) and luteinizing hormone (LH)] and estradiol were evaluated in 32/35 patients, and prolactin and total testosterone in 29/35 patients. Patient's medical records were carefully reviewed according to demographic, clinical and therapeutic findings. The mean duration of amenorrhea was 7.2 +/- A 3.6 months. Low FSH or LH was observed in 7/32 (22%) JSLE patients and normal FSH or LH in 25 (78%). Remarkably, low levels of FSH or LH were associated with higher frequency of current amenorrhea (57% vs. 0%, P = 0.001), higher median disease activity (SLEDAI) and damage (SLICC/ACR-DI) (18 vs. 4, P = 0.011; 2 vs. 0, P = 0.037, respectively) and higher median current dose of prednisone (60 vs. 10 mg/day, P = 0.0001) compared to normal FSH or LH JSLE patients. None of them had decreased ovarian reserve and premature ovarian failure. Six of 29 (21%) patients had high levels of prolactin, and none had current amenorrhea. No correlations were observed between levels of prolactin and SLEDAI, and levels of prolactin and SLICC/ACR-DI scores (Spearman's coefficient). We have identified that amenorrhea in JSLE is associated with high dose of corticosteroids indicated for active disease due to hypothalamic-pituitary-ovary axis suppression.

Submitted by Guilherme Lemeszenski (guilherme@nead.unesp.br) on 2013-08-22T19:02:59Z No. of bitstreams: 1 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) Made available in DSpace on 2013-08-22T19:02:59Z (GMT). No. of bitstreams: 1 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) Previous issue date: 2009-10-01 Made available in DSpace on 2013-09-30T20:04:20Z (GMT). No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Previous issue date: 2009-10-01 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T15:16:32Z No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Made available in DSpace on 2014-05-20T15:16:32Z (GMT). No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Previous issue date: 2009-10-01 Crianças e adolescentes com doenças reumatológicas apresentam maior prevalência de doenças infecciosas quando comparados com a população em geral, em decorrência de atividade da doença, possível deficiência imunológica secundária à própria doença, ou uso de terapia imunossupressora. A vacinação é uma medida eficaz para a redução da morbidade e mortalidade nesses pacientes. O objetivo deste artigo foi realizar um consenso de eficácia e segurança das vacinas em crianças e adolescentes com doenças reumatológicas infantis baseadas em níveis de evidência científica. Imunização passiva para os pacientes e orientações para as pessoas que convivem com doentes imunodeprimidos também foram incluídas. Os 32 pediatras reumatologistas membros do Departamento de Reumatologia da Sociedade de Pediatria de São Paulo (SPSP) e/ou da Comissão de Reumatologia Pediátrica da Sociedade Brasileira de Reumatologia elaboraram o consenso, sendo que alguns desses profissionais estão envolvidos em pesquisas e publicações científicas nesta área. A pesquisa dos termos eficácia e/ou segurança das diferentes vacinas em crianças e adolescentes com doenças reumatológicas foi realizada nas bases de Medline e Scielo, de 1966 até março de 2009, incluindo revisões, estudos controlados e relatos de casos. O grau de recomendação e o nível científico de evidências dos estudos foram classificados em quatro níveis para cada vacina. de um modo geral, as vacinas inativadas e de componentes são seguras nos pacientes com doenças reumatológicas, mesmo em uso de terapias imunossupressoras. Entretanto, vacinas com agentes vivos atenuados são, em geral, contraindicadas para os pacientes imunossuprimidos. Incidence of infectious diseases is higher in children and adolescents with rheumatic diseases than in the general population due to disease activity, possible immune deficiency secondary to the disease itself, or the use of immunosuppressive drugs. Vaccination is effective in reducing morbidity and mortality in those patients. The objective of this study was to establish an evidence-based consensus on the efficacy and safety of vaccination in children and adolescents with rheumatic diseases. Passive immunization of patients and guidelines for people who live with immunosuppressed patients were also included. The 32 pediatric rheumatologists of the Rheumatology Department of the Pediatrics Society of São Paulo, (SPSP, from the Portuguese), São Paulo, SP, Brazil, and/or the Commission on Pediatrics Rheumatology of the Brazilian Society of Rheumatology are responsible for this consensus; some of those professionals are involved on research and scientific publications in this field. The words efficacy and/or safety of different vaccines in children and adolescents with rheumatologic diseases were searched in Medline and Scielo data bases from 1966 to March 2009, including reviews, controlled studies, and case reports. The degree of recommendation and the scientific evidence of the studies were classified in four levels for each vaccine. As a rule, inactive and protein components vaccines are safe for patients with rheumatologic diseases, even in the presence of immunosuppressive therapy. However, live attenuated vaccines are, in general, contraindicated for immunosuppressed patients. Universidade de São Paulo Faculdade de Medicina Instituto da Criança Universidade de São Paulo USP Faculdade de Medicina de Ribeirão Preto FMUSP Universidade Estadual Paulista Faculdade de Medicina de Botucatu Santa Casa de Misericórdia de São Paulo Hospital Municipal Infantil Menino Jesus Universidade Estadual de Campinas Hospital Servidor Público Universidade Federal da Bahia (UFBA) Hospital Materno Infantil de Goiás Universidade Federal do Pará (UFPA) Universidade Federal de Mato Grosso do Sul Universidade Federal do Rio de Janeiro (UFRJ) UFRJ Instituto de Puericultura Martagão Gesteira Universidade Estadual Paulista Faculdade de Medicina de Botucatu

Objetivos: Descrever as características clínicas e a ocorrência de artrite atípica em crianças com diagnóstico de febre reumática (FR) acompanhadas em ambulatórios terciários em Salvador, Bahia. Metodologia: Estudo descritivo, de uma série de casos, do quadro clínico inicial ou recorrência de 41 crianças com diagnóstico de FR. Resultados: Dos pacientes estudados (n=41), 61% eram do sexo masculino; com média de idade de 9,2 anos e idade no momento do diagnóstico entre 5 e 16 anos. Artrite esteve presente em 75,6% dos pacientes; cardite em 75,6%; coreia em 31,7%; eritema marginado em 14,6% e nódulos subcutâneos em 4,9%. Um padrão atípico foi observado em 22 dos 31 casos com artrite (70,9%): envolvimento de pequenas articulações e/ou esqueleto axial em 12 casos (38,7%); duração maior que três semanas em nove (29%); resposta inadequada ao AINH em dois (6,5%); oligoartrite (≤ quatro articulações) em 22/31 (71%), sendo monoartrite em 6/31 (uma em pés, uma em tornozelo e quatro em joelho). A febre esteve presente em 78% dos casos e 82,9% dos pacientes utilizavam a profilaxia secundária de forma regular. Conclusão: Artrite atípica esteve presente na maioria dos pacientes que cursaram com acometimento articular, constituindo um fator de confundimento diagnóstico e atraso terapêutico adequado. Objectives: To describe the clinical characteristics and the occurrence of atypical arthritis in children diagnosed with rheumatic fever (RF) and followed in tertiary care clinics in Salvador, Bahia, Brazil. Methodology: A descriptive study of a case series, of the initial clinical presentation, and of recurrence in 41 children diagnosed with RF. Results: Of the patients studied (n=41), 61% were male, mean age of 9.2 years, and mean age at diagnosis between 5 and 16 years. Arthritis was present in 75.6% of patients; carditis in 75.6%; chorea in 31.7%; erythema marginatum in 14.6%; and subcutaneous nodules in 4.9%. An atypical pattern was observed in 22 of 31 cases of arthritis (70.9%): involvement of small joints and/or axial skeleton in 12 cases (38.7%); >3 weeks of duration in 9 (29%); inadequate response to NSAIDs in 2 (6.5%); oligoarthritis (≤4 joints) in 22/31 (71%), with monoarthritis in 6/31 (1 in the foot, 1 in the ankle, and 4 in the knee). Fever was present in 78% of the cases, and 82.9% of patients were regularly on secondary prophylaxis. Conclusion: Atypical arthritis was present in most patients presenting with joint involvement, being a confounding factor against a proper diagnosis and of therapeutic delay.

This is a case report of juvenile idiopathic arthritis in two pairs of brothers followed in the Department of Pediatric Rheumatology, Universidade Federal da Bahia. Genetic involvement in juvenile idiopathic arthritis pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex polymorphism contribution to juvenile idiopathic arthritis development susceptibility. Despite many advances, the numerous available studies cannot explain several implicit mechanisms in juvenile idiopathic arthritis pathogenesis yet.

Anormalidades na função tireoidiana e presença de autoanticorpos da tireoide têm sido frequentemente descritas em pacientes com doenças reumatológicas autoimunes, como síndrome de Sjögren, artrite reumatoide, lúpus eritematoso sistêmico e esclerodermia. São limitados os dados sobre prevalência e características clínicas de tireoidite autoimune em outras doenças reumatológicas, tais como febre reumática e lúpus eritematoso sistêmico juvenil. Os autores revisaram as associações de doenças autoimunes endócrinas e reumáticas, avaliando as diversas faixas etárias e condições clínicas. O levantamento bibliográfico foi realizado por meio de busca por artigos científicos indexados em bancos de dados de ciências da saúde em geral, como Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS), Medline/PubMed e Scientific Eletronic Library Online (SciELO). Utilizaram-se os seguintes descritores: "rheumatic autoimmune diseases and autoimmune thyroid diseases", "thyroid disorders and rheumatic diseases", "thyroiditis and rheumatic diseases", "autoimmune diseases and thyroid", e "pediatric rheumatic diseases and autoimmune thyroid diseases". Este estudo mostrou que, apesar de resultados contraditórios na literatura, há maior prevalência da associação entre doenças autoimunes da tireoide e doenças reumáticas, destacando-se a possibilidade de mecanismos patogênicos comuns entre as doenças. Thyroid function abnormalities and thyroid autoantibodies have been frequently described in patients with rheumatologic autoimmune diseases, such as Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus and scleroderma. Limited data are available regarding the prevalence and clinical characteristics of autoimmune thyroiditis in other rheumatologic disorders, such as rheumatic fever and juvenile systemic lupus erythematosus. The authors review the association of endocrine autoimmune and rheumatic autoimmune diseases, assessing various age groups and clinical conditions. The bibliographic survey was conducted through the search for scientific articles indexed in the general health sciences databases, such as Latin American and Caribbean Health Sciences Literature (LILACS), Medline/PubMed, and Scientific Electronic Library Online (SciELO). The following descriptors were used: "rheumatic autoimmune diseases and autoimmune thyroid diseases"; "thyroid disorders and rheumatic diseases"; "thyroiditis and rheumatic diseases"; "autoimmune diseases and thyroid"; and "pediatric rheumatic diseases and autoimmune thyroid diseases". This study showed that, despite contradictory results in the literature, there is a greater prevalence of the association between autoimmune thyroid diseases and rheumatic diseases, highlighting the possibility of common pathogenic mechanisms among them.

Thyroid function abnormalities and thyroid autoantibodies have been frequently described in patients with rheumatologic autoimmune diseases, such as Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus and scleroderma. Limited data are available regarding the prevalence and clinical characteristics of autoimmune thyroiditis in other rheumatologic disorders, such as rheumatic fever and juvenile systemic lupus erythematosus. The authors review the association of endocrine autoimmune and rheumatic autoimmune diseases, assessing various age groups and clinical conditions. The bibliographic survey was conducted through the search for scientific articles indexed in the general health sciences databases, such as Latin American and Caribbean Health Sciences Literature (LILACS), Medline/PubMed, and Scientific Electronic Library Online (SciELO). The following descriptors were used: "rheumatic autoimmune diseases and autoimmune thyroid diseases"; "thyroid disorders and rheumatic diseases"; "thyroiditis and rheumatic diseases"; "autoimmune diseases and thyroid"; and "pediatric rheumatic diseases and autoimmune thyroid diseases". This study showed that, despite contradictory results in the literature, there is a greater prevalence of the association between autoimmune thyroid diseases and rheumatic diseases, highlighting the possibility of common pathogenic mechanisms among them.

We report an unusual case of acute suppurative thyroiditis associated with juvenile systemic lupus erythematosus (JSLE) onset. A 10 year-old girl presented with a painful voluminous swelling in the anterior cervical region with fever and dysphagia. She also had dysphonia, myalgia, swelling of the small joints of the hands, weight loss, and erythematous malar rash. Thyroid ultrasonography disclosed an ill-defined solid cystic area containing abundant echoes in suspension suggestive of an abscess in both lobes compatible with suppurative bacterial thyroiditis. Fine-needle aspiration biopsy showed a secretion with positive culture for Staphylococcus aureus. Endoscopic evaluation did not demonstrate pyriform sinus fistula. The child had no previous thyroid disease. Hormonal evaluation showed thyrotrophin (TSH) 13 microIU/ml and free thyroxine (FT4) 0.4 ng/dl. After laboratory confirmation of JSLE, high dose 'pulse' therapy was performed with methylprednisolone (30 mg/kg/day for 3 days), followed by prednisone (2 mg/kg/day). The acute thyroiditis was treated with oxacillin, metronidazole and amikacin which ameliorated the symptoms of infection followed by normalization of thyroid function with levothyroxine. In spite of the known association between autoimmune diseases and thyroid disorders, the coexistence of suppurative thyroiditis and JSLE is rare, generally occurring during the course of lupus and not at diagnosis.

Objective To present an up-to-date and practical review of how to safely withdraw glucocorticosteroid therapy. Sources A review of the published literature identified by searching the MEDLINE and LILACS databases (1997-2007), selecting the most representative articles on the subject. Summary of the findings Three clinical situations may occur during glucocorticoid withdrawal: adrenal insufficiency secondary to negative feedback on the hypothalamic-pituitary adrenal (HPA) axis, steroid withdrawal syndrome and relapse of the disease for which the glucocorticoids were prescribed. Although there is no consensus on how to best discontinue prolonged glucocorticosteroid therapy, there is agreement that this withdrawal should be gradual. This article updates pediatricians on how to recognize these problems and provides recommendations on how to safely suspend glucocorticosteroid therapy. A brief review of the pharmacology of glucocorticoids is also presented. Conclusions There is no good predictive test for predicting the risk of adrenal insufficiency in patients who have been on corticosteroid therapy chronically. There is a need for prospective studies to assess the true incidence of this problem and to propose rational strategies for preventing it. The current recommendation is that patients who have been on chronic and/or high dose glucocorticoids should be administered glucocorticoids during stress situations unless the integrity of the HPA axis has been established by dynamic tests.

OBJETIVO: Apresentar uma revisão atualizada e prática sobre como efetuar de forma segura a retirada da corticoterapia. FONTES DOS DADOS: Revisão da literatura utilizando os bancos de dados MEDLINE e LILACS (1997-2007), selecionando os artigos mais atuais e representativos do tema. SÍNTESE DOS DADOS: Três situações clínicas podem ocorrer durante a retirada da corticoterapia prolongada: insuficiência adrenal secundária à supressão do eixo hipotálamo-hipófise-adrenal, síndrome de retirada ou deprivação dos corticóides e reativação da doença de base. Embora não exista consenso sobre o melhor esquema para descontinuar a terapia prolongada com corticóides, existe concordância quanto ao fato desta retirada ser gradual. Este artigo atualiza o pediatra quanto ao reconhecimento desses problemas e fornece orientações para a suspensão do tratamento prolongado com corticóide. Uma breve revisão da farmacologia dos corticóides também é descrita. CONCLUSÃO: Não existe teste com bom valor preditivo para antecipar o risco de insuficiência adrenal nos pacientes que receberam terapia crônica com corticóide. São necessários estudos prospectivos para avaliar a real incidência desse problema e assim propor estratégias racionais para sua prevenção. No momento, a menos que a integridade do eixo hipotálamo-hipófise-adrenal esteja estabelecida por testes dinâmicos, recomenda-se a administração de corticóide em situações de estresse nos pacientes que fizeram uso de corticoterapia crônica e/ou em doses elevadas. OBJECTIVE: To present an up-to-date and practical review of how to safely withdraw glucocorticosteroid therapy. SOURCES: A review of the published literature identified by searching the MEDLINE and LILACS databases (1997-2007), selecting the most representative articles on the subject. SUMMARY OF THE FINDINGS: Three clinical situations may occur during glucocorticoid withdrawal: adrenal insufficiency secondary to negative feedback on the hypothalamic-pituitary adrenal (HPA) axis, steroid withdrawal syndrome and relapse of the disease for which the glucocorticoids were prescribed. Although there is no consensus on how to best discontinue prolonged glucocorticosteroid therapy, there is agreement that this withdrawal should be gradual. This article updates pediatricians on how to recognize these problems and provides recommendations on how to safely suspend glucocorticosteroid therapy. A brief review of the pharmacology of glucocorticoids is also presented. CONCLUSION: There is no good predictive test for predicting the risk of adrenal insufficiency in patients who have been on corticosteroid therapy chronically. There is a need for prospective studies to assess the true incidence of this problem and to propose rational strategies for preventing it. The current recommendation is that patients who have been on chronic and/or high dose glucocorticoids should be administered glucocorticoids during stress situations unless the integrity of the HPA axis has been established by dynamic tests.

Objective This study was to determine the prevalence and characteristics of the osteoarticular manifestations on initial clinical presentation of acute leukemias (ALs) on childhood in the state of Bahia, Brazil. Materials and methods This retrospective study assessed the medical records of 406 patients with AL from January 1995 to December 2004. Results Acute lymphocytic leukemia (ALL) was diagnosed in 313 (77.1%) patients and acute myeloid leukemia (AML), in 93 (22.9%) patients, including 241 males (59.4%) and 165 females (40.6%). Age ranged from 9 months to 15 years (average: 6.18 y). The most common presenting features were fever (18.5%), musculoskeletal diffuse tenderness (15.0%), pallor (11.4%), and leg tenderness (5.7%). Prior referral to our center, the most frequent initial diagnosis was anemia (15.8%), leukemia (15.0%), amygdalitis (3.7%), and rheumatic fever (2.7%). Osteoarticular manifestations were found on 54.7% of the patients with AL, with a higher frequency among patients between 1 and 9 years of age (58.7%, P=0.0007). The presence of joint tenderness (16.2% in ALLx5.4% in AML), arthritis (26.6% in ALLx9.7 in AML), bone tenderness (26.1% in ALLx16.1% in AML), limb tenderness (49.5% in ALLx25.8% in AML), and antalgic gait (32.8% in ALLx9.7% in AML) had higher prevalence on ALL. The large joints, chiefly the knees (10.6%), ankles (9.4%), elbows (4.4%), and shoulders (3.6%) were more often affected. Conclusions AL should be considered on the differential diagnosis of osteoarticular symptoms of unknown etiology in children.

Background Takayasu’s arteritis (TA) is a chronic granulomatous vasculitis affecting the large and medium arteries and can result in end organ damage. The disease is rare and there are few reports about the clinical features in the pediatric population. Objectives To evaluate and to compare the clinical features of TA in Brazilian children and adolescents. Methods In this Brazilian multicenter retrospective study including 10 pediatric rheumatology centers we identified 71 children and adolescents with TA diagnosed before the age of 18 years according to the validated classification criteria for pediatric age 1 . Patients’ demographic, clinical, laboratory and angiographic data were collected. The angiographic type was classified according to the 1994 International Conference of Takayasu arteritis in Tokyo 2 . Patients were divided in 2 groups: children, younger than 10 years – group 1, and adolescents, older than or equal to 10 years – group 2 and their disease characteristics at initial presentation were compared. Results Thirty-six (50.7%) patients were in group 1 and 35 (49.3%) were in group 2. Twenty-one (58.3%) patients in group 1 and 30 (85.7%) patients in group 2 were girls (p=0.010). The mean onset age was 5.7 and 12.7 in groups 1 and 2 (p Conclusions Children under the age of 10 had more laboratory abnormalities, but similar clinical and angiographic features when compared to adolescents with TA. A delay in diagnosis is more common in younger patients and clinicians must be aware about this disease in this age group. References Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R et al. EULAR/PRINTO/PRES criteria for Henoch-Schoenlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu’s arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69:798-806. Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: New classification. Int J Cardiol.1996;54(suppl):S155-S163. Disclosure of Interest : None Declared

ResumoRelato de casos de ocorrência de Artrite Idiopática Juvenil (AIJ) em dois pares de irmãos acompanhados no serviço de reumatologia pediátrica da Universidade Federal da Bahia. O envolvimento genético na patogênese da AIJ está claro e o risco de recorrência entre irmãos corrobora esta contribuição. Um importante marco dessa descoberta envolve a confirmação da contribuição dos polimorfismos do complexo principal de histocompatibilidade (MHC) na susceptibilidade ao desenvolvimento da AIJ. Apesar de muitos progressos, os inúmeros estudos existentes ainda não são capazes de explicar diversos mecanismos implícitos na patogênese da AIJ.AbstractThis is a case report of juvenile idiopathic arthritis (JIA) in two pairs of brothers followed in the department of pediatric rheumatology, Universidade Federal da Bahia. Genetic involvement in JIA pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex (MHC) polymorphism contribution to JIA development susceptibility. Despite many advances, the numerous available studies cannot explain several implicit mechanisms in JIA pathogenesis yet.

ResumoObjetivoRelatar um caso de coexistência de lúpus eritematoso sistêmico (LES) e doença falciforme (DF) com revisão da literatura sobre o tema.MetodologiaRelato de caso e pesquisa da associação entre LES e DF na literatura, através de artigos científicos nas bases de dados de ciências da saúde, como LILACS, MEDLINE/Pubmed e Scielo, até maio de 2012. Descritores utilizados: 1. anemia falciforme; 2. doença falciforme; 3. lúpus eritematoso sistêmico; 4. hemoglobinopatias.ResultadosOs autores descrevem a associação de LES e hemoglobinopatia SS em paciente do sexo feminino, oito anos, apresentando manifestações articulares, hematológicas e neuropsiquiátricas durante a sua evolução clínica. Na literatura são descritos 45 casos de associação entre LES e DF, sendo a maioria em mulheres (78%) adultas (62,2%), apresentando fenótipo SS em 78% dos casos e com manifestações clínicas variadas. Comparando com a nossa paciente, manifestações articulares, hematológicas e neuropsiquiátricas, estiveram presentes em 76%, 36% e 27% dos casos, respectivamente.ConclusõesLES e DF são doenças crônicas que apresentam diversos achados clínicos e laboratoriais em comum, implicando em dificuldades diagnósticas e na correta condução terapêutica dessas doenças. A associação entre essas enfermidades não é comum, mas está descrita na literatura, por isso é importante que médicos que cuidam dessas enfermidades estejam atentos para tal possibilidade.AbstractObjectiveTo report a case of coexisting systemic lupus erythematosus (SLE) and sickle cell disease (SCD) with a review of the literature on the topic.MethodologyReport of case and research of the association between SLE and SCD in literature through scientific articles in health sciences databases, such as LILACS, MEDLINE/Pubmed and Scielo, until May 2012. Descriptors used: 1. Sickle cell anemia; 2. Sickle cell disease; 3. Systemic lupus erythematosus; 4. Hemoglobinopathies.ResultsThe authors describe an association between SLE and SS hemoglobinopathy in an eight‐year‐old female patient displaying articular, hematologic and neuropsychiatric manifestations during clinical evolution. Forty‐five cases of association between SLE and SCD are described in literature, mostly adult (62.2%), women (78%) and with the SS phenotype in 78% of the cases, and different clinical manifestations. Compared with our patient, articular, hematologic and neuropsychiatric manifestations were present in 76%, 36% and 27% of the cases, respectively.ConclusionSLE and SCD are chronic diseases that have several clinical and laboratory findings in common, meaning difficult diagnosis and difficulty in finding the correct treatment. Although the association between these diseases is not common, it is described in literature, so it is imperative that physicians who treat such diseases be alert to this possibility.

ResumoAs doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta‐ Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar‐se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.AbstractJuvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta‐Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.

We retrospectively evaluated 55 patients with Takayasu’s arteritis from 8 Pediatric Rheumatology centers in Brazil. All patients fulfilled the specific classification criteria for TA that have most recently been developed for the pediatric age group. Clinical, data were collected at two time points (at disease onset and at the last available visit). Results The majority of patients were girls (84%), with mean age at disease onset of 9.3 years, mean age at diagnosis of 10.6 years and a mean follow-up period of 5.6 years. The most common initial clinica lm anifestations included headache (56.3%), malaise (56.3%), weight loss (45.4%), dyspnea (41.8%) and limb pain (41.8%). The most common cardiovascular finding was hypertension (85.4%). The most frequent laboratory abnormality was an increased erythrocyte sedimentation rate (70.9%). The Mantoux test was positive in 38% of patients. The most common alteration was narrowing or stenosis. The most involved vessels were abdominal aorta (71.8%) followed by left (56.2%) and right renal artery (53.1%). At the last visit the most common clinical manifestations were: headache (22%), inferior limb claudication (14%) and arthralgias (12%); Cardiovascular finding included the absence or decrease of the inferior limb pulses (52%). The treatment included imunossupressors other than corticosteroids in 83.6% and anti-TNF antagonists in 7.2% of patients. Seventeen (30.9%) patients underwent surgical intervention and 5 (9%) died of complication from treatment or from disease related complications. Conclusion In this large survey of children and adolescents with Takayasu’s arteritis from Brazil a delay in the diagnosis and a high rate of Mantoux test positivity were observed. A death rate similar to the described in literature was found.