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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)

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.

Made available in DSpace on 2019-10-06T15:31:10Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-11-23. Added 1 bitstream(s) on 2021-07-15T15:23:32Z : No. of bitstreams: 1 S2523-31062018000100228.pdf: 598953 bytes, checksum: c510a60909892e9f6d7f26d4469f267b (MD5) Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) OBJECTIVE: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. METHODS: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p

Background The influence of psychosocial aspects on Juvenile Systemic Lupus Erythematosus (JSLE) is known both in the triggering of the disease and in reactivation. Since it is a chronic disease in pediatric patients, its diagnosis has an important impact on both the patient and the family. The understanding of the diagnosis, evolution and treatment of the disease leads to a better adherence to the treatment, consequently better evolution.Objective: To evaluate the impact of the diagnosis of JSLE in the life of patients and parents and the degree of understanding about the disease and treatment. Methods Pilot study with application of a questionnaire containing epidemiological data, questions about the understanding of the disease, psychological impact on diagnosis and currently and association with a stressor event. This questionnaire was applied to the jSLE patients and their parents accompanied at pediatric rheumatology department of Santa Casa de Sao Paulo. Qualitative data were submitted to exploratory descriptive analysis. Results 24 patients and 21 parents answered the questionnaire. 84% of the patients were female and 95% of the relatives were mothers. The age ranged from 9 to 17 and from 28 to 46 years for the patients and the parents, respectively. The follow-up time was 33.3±5.7 months. Only 21% of the patients and 38% of the parents were able to define SLE as an autoimmune disease. Regarding the cause of SLE, 29% of parents and 13% of children do not know but many parents associated with the emotional issue, while patients related to altered immune system. Both parents and patients associated a stressor event with the onset of jSLE. Regarding treatment both children and parents demonstrated an awareness of the need for appropriate medication and follow-up, but also described the importance of sun protection. The main feeling reported at the time of diagnosis was sadness for both parents and patients. Already today, 50% of children and parents express a feeling of conformity and tranquility, knowing how to deal better with the disease, but still have some degree of concern. Conclusions jSLE is a disease that brings a sense of sadness and there is an association of a stressor event for both patients and parents. The health team must be able to clarify the lupus disease, considering limitations of understanding of the patients and parents, which allows a better control of the disease, bringing tranquility. Funding Source(s): None

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 2015-12-07T15:38:44Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-10-01. Added 1 bitstream(s) on 2019-10-09T18:24:49Z : No. of bitstreams: 1 S0482-50042016000100052.pdf: 646225 bytes, checksum: 1638a2e27ec46d822bf61de374bb4355 (MD5) Objetivo Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development; 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes; 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients; 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA; 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Electronic address: teterreri@terra.com.br. Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil. Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil. Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil. Universidade Federal do Pará (UFPA), Belém, PA, Brasil. Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (Uerj), Rio de Janeiro, RJ, Brasil. Serviço de Reumatologia Pediátrica, Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil. Hospital Pequeno Príncipe, Curitiba, PR, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil.

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. Recomendações: 1. O diagnóstico da FFM é baseado nas manifestações clínicas, caracterizadas por episódios febris recorrentes associados a dor abdominal, torácica ou artrite de grandes articulações; 2. A FFM é uma doença genética que apresenta traço autossômico recessivo ocasionada por mutação no gene MEFV; 3. Exames laboratoriais são inespecíficos e demonstram níveis séricos elevados de proteínas inflamatórias na fase aguda da doença, mas também, com frequência, níveis elevados mesmo entre os ataques. Níveis séricos de SAA podem ser especialmente úteis no monitoramento da eficácia do tratamento; 4. A colchicina é a terapia de escolha e demonstrou eficácia na prevenção dos episódios inflamatórios agudos e progressão para amiloidose em adultos; 5. Com base na informação disponível, o uso de medicamentos biológicos parece ser opção para pacientes com FFM que não respondem ou que são intolerantes à terapia com colchicina. Abstract Objective: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints; 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene; 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment; 4. The therapy of choice is colchicine; this drug has proven effectiveness in preventing acute inflammatory episodes and progression towards amyloidosis in adults; 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine.

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.

Objective To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus(cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. Methods A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p Conclusions Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.

ResumoA arterite de Takayasu (AT) e a febre reumática (FR) são doenças que podem ter início com manifestação cardíaca, o que dificulta o diagnóstico. Há relatos de associação de FR com AT que se inicia com comprometimento cardíaco na faixa etária pediátrica. O objetivo deste estudo é relatar a possibilidade da associação de FR e AT em paciente com alteração cardíaca. Descrevemos o caso de uma adolescente diagnosticada inicialmente como FR que apresentou na evolução alterações que permitiram o diagnóstico de AT. A AT e a FR são duas causas importantes de envolvimento valvular que podem apresentar manifestações sistêmicas.AbstractTakayasu's arteritis (TA) and rheumatic fever are diseases that can start with cardiac features, making the diagnosis difficult. There are reports of association of RF with Takayasu's arteritis beginning with cardiac involvement in pediatric patients. The aim of this study is to report the possible association of RF and TA in patients with cardiac abnormalities. We describe the case of an adolescent initially diagnosed with RF who progressed with changes that allowed making the diagnosis of TA. TA and RF are two important causes of valve involvement that may have systemic manifestations.

Background Data of diffuse alveolar hemorrhage (DAH) in childhood-onset systemic lupus erythematosus (cSLE) patients are limited due to the small representation of this complication in previous case series or the focus on the comparison to adult SLE, precluding an accurate analysis of associated factors and outcomes in patients with and without this severe complication. Objectives To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 cSLE patients with and without diffuse DAH, as well as concomitant parameters of severity. Methods DAH was defined as the presence of at least three respiratory symptoms or signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels with no other source of bleeding. Holm-Bonferroni correction for multiple comparisons was performed adjusting the significance level (p Results DAH was evidenced in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1–151) vs. 4 (1–151) months, p=0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p Conclusions This is the largest study to evaluate DAH. This complication, although not a disease activity score descriptor, occurs in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition is associated with serious disease flare complicated by sepsis and with high mortality rate. Acknowledgements This study was supported by research grants from Conselho Nacional de Desenvolvimento Cientifico e Tecnolόgico (CNPq 301805/2013-0 to RMRP, 303752/2015-7 to MTT, 301479/2015-1 to CSM, 305068/2014-8 to EB and 303422/2015-7 to CAS), Federico Foundation (to EB, RMRP and CAS) and by Nucleo de Apoio a Pesquisa “Saude da Crianca e do Adolescente” da USP (NAP-CriAd) to CAS. Disclosure of Interest : None declared

This study investigated some immunological features by experimental periodontitis (EP) and rheumatoid arthritis (RA) disease interact in destructive processes in arthritic rats. Rats were assigned to the following groups: EP +RA; RA; EP; and Negative Control. RA was induced by immunizations with type-II collagen and a local immunization with Complete Freund's adjuvant in the paw. Periodontitis was induced by ligating the right first molars. The serum level of rheumatoid factor (RF) and anti-citrullinated protein antibody (ACCPA) were measured before the induction of EP (T1) and at 28 days after (T2) by ELISA assay. ACCPA levels were also measured in the gingival tissue at T2. The specimens were processed for morphometric analysis of bone loss, and the gingival tissue surrounding the first molar was collected for the quantification of interleukin IL-1β, IL-4, IL-6, IL-17 and TNF-α using a Luminex/MAGpix assay. Paw edema was analyzed using a plethysmometer. Periodontitis increased the RF and ACCPA levels in the serum and in the gingival tissue, respectively. Besides, the level of paw swelling was increased by EP and remained in progress until the end of the experiment, when EP was associated with RA. Greater values of IL-17 were observed only when RA was present, in spite of PE. It can be concluded that periodontitis increases rheumatic factor serum levels and citrullinated proteins level in gingival tissues and alter cytokine balance in arthritic rats; at the same time, arthritis increases periodontal destruction, confirming the bidirectional interaction between diseases.

Made available in DSpace on 2018-12-11T17:08:54Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-03-01 Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) The aim of this study is to assess uveitis prevalence in a large cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. A retrospective multicenter cohort study including 852 cSLE patients was performed in ten pediatric rheumatology centers (Brazilian cSLE group). An investigator meeting was held and all participants received database training. Uveitis was diagnosed through clinical assessment by the uveitis expert ophthalmologist of each center. Patients with and without uveitis were assessed for lupus clinical/laboratory features and treatments. Uveitis was observed in 7/852 cSLE patients (0.8%). Two of them had ocular complications: cataract and irreversible blindness in one patient and retinal ischemia with subsequent neovascularization and unilateral blindness in another. Uveitis was identified within the first 6 months of cSLE diagnosis in 6/7 patients (86%). Comparison of a subgroup of cSLE patients with (n = 7) and without uveitis (n = 73) and similar length of disease duration showed that patients with uveitis had increased SLEDAI-2K score (19 vs. 6; p

INTRODUÇÃO: A reumatologia pediátrica (RP) é uma especialidade emergente, com número restrito de especialistas, e ainda não conta com uma casuística brasileira sobre o perfil dos pacientes atendidos e as informações sobre a formação de profissionais capacitados. OBJETIVO: Estudar o perfil dos especialistas e dos serviços em RP e as características dos pacientes com doenças reumáticas nessa faixa etária a fim de estimar a situação atual no estado de São Paulo (ESP). PACIENTES E MÉTODOS: No ano de 2010 o departamento científico de RP da Sociedade de Pediatria de São Paulo encaminhou um questionário respondido por 24/31 especialistas com título de especialização em RP que atuam no ESP e por 8/12 instituições com atendimento nesta especialidade. RESULTADOS: A maioria (91%) dos profissionais exerce suas atividades em instituições públicas. Clínicas privadas (28,6%) e instituições (37,5%) relataram não ter acesso ao exame de capilaroscopia e 50% das clínicas privadas não tem acesso à acupuntura. A média de tempo de prática profissional na especialidade foi de 9,4 anos, sendo 67% deles pós-graduados. Sete (87,5%) instituições públicas atuam na área de ensino, formando novos reumatologistas pediátricos. Cinco (62,5%) delas têm pós-graduação. Dois terços dos especialistas utilizam imunossupressores e agentes biológicos de uso restrito pela Secretaria da Saúde. A doença mais atendida foi artrite idiopática juvenil (29,1%-34,5%), seguida de lúpus eritematoso sistêmico juvenil (LESJ) (11,6%-12,3%) e febre reumática (9,1%-15,9%). Vasculites (púrpura de Henoch Schönlein, Wegener, Takayasu) e síndromes autoinflamatórias foram mais incidentes nas instituições públicas (P = 0,03; P = 0,04; P = 0,002 e P < 0,0001, respectivamente). O LESJ foi a doença com maior mortalidade (68% dos óbitos), principalmente por infecção. CONCLUSÃO: A RP no ESP conta com um número expressivo de especialistas pós-graduados, que atuam especialmente em instituições de ensino, com infraestrutura adequada ao atendimento de pacientes de alta complexidade. INTRODUCTION: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. OBJECTIVE: To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. PATIENTS AND METHODS: In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. RESULTS: Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as restricted use by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. CONCLUSION: The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients. Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Setor de Reumatologia Pediátrica Universidade de São Paulo Faculdade de Medicina Hospital das Clínicas Santa Casa de Misericórdia de São Paulo Departamento de Pediatria Setor de Reumatologia Universidade Estadual de Campinas Unidade de Reumatologia Pediátrica Universidade de São Paulo Faculdade de Medicina de Ribeirão Preto Departamento de Puericultura e Pediatria Santa Casa de Misericórdia de Santos Setor de Reumatologia Pediátrica Faculdade de Medicina de Botucatu Hospital Estadual de Bauru Serviço de Reumatologia Pediátrica Faculdade de Medicina de Botucatu UNIFESP, Depto. de Pediatria Setor de Reumatologia Pediátrica SciELO

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.

Made available in DSpace on 2015-12-07T15:39:10Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-10-09. Added 1 bitstream(s) on 2019-10-09T18:24:50Z : No. of bitstreams: 1 S0482-50042016000100037.pdf: 781208 bytes, checksum: b70672ad129c7cebf8eba7da1b7f87b9 (MD5) Objetivo Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints; 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene; 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment; 4. The therapy of choice is colchicine; this drug has proven effectiveness in preventing acute inflammatory episodes and progression towards amyloidosis in adults; 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Electronic address: teterreri@terra.com.br. Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil. Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil. Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil. Universidade Federal do Pará (UFPA), Belém, PA, Brasil. Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (Uerj), Rio de Janeiro, RJ, Brasil. Instituto de Puericultura e Pediatria Martagão Gesteira, Serviço de Reumatologia Pediátrica, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil. Hospital Pequeno Príncipe, Curitiba, PR, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil.

Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions. Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das Sín dromes periódicas associadas à criopirina (Criopirinopatias – CAPS). Descric¸ão do método de coleta de evidência: A Diretriz foi elaborada a partir de 4 questões clínicas que foram estruturadas por meio do P.I.C.O. (Paciente, Intervenc¸ão ou Indicador, Comparac¸ão e Outcome), com busca nas principais bases primárias de informac¸ão científica. Após definir os estudos potenciais para sustento das recomendac¸ões, estes foram graduados pela forc¸a da evidência e grau de recomendac¸ão. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1215 artigos, tendo sido selecionados 42 trabalhos, para sustentar as recomendac¸ões. Recomendac¸ões: 1. O diagnóstico de CAPS é baseado na anamnese e manifestac¸ões clíni cas, sendo posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e CINCA (forma grave). Avaliac¸ões neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinc¸ão entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de CAPS, isto é, indivíduos que apresentam, antes dos 20 anos de idade, episódios recorrentes de inflamac¸ão expressa por urticária e febre moderada; 3. As alterac¸ões laboratoriais incluem leucocitose e elevac¸ão nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitac¸ões importantes em relac¸ão à seguranc¸a em longo prazo. Nenhuma das três medicac¸ões anti-IL1 evita progressão das lesões ósseas

INTRODUÇÃO/OBJETIVOS: Avaliar a prática clínica com relação à verificação do cartão vacinal e à indicação de vacinas específicas em pacientes com doenças reumáticas pediátricas em uso de diferentes drogas, e evidenciar a possível associação entre frequência de vacinação e tempo de prática clínica dos reumatologistas pediátricos do estado de São Paulo. MATERIAL E MÉTODOS: Um questionário foi enviado para os reumatologistas pediátricos do Departamento de Reumatologia da Sociedade de Pediatra de São Paulo. Esse instrumento incluiu questões sobre tempo de prática em Reumatologia Pediátrica, vacinação de pacientes com Lúpus Eritematoso Sistêmico Juvenil (LESJ), artrite idiopática juvenil (AIJ), dermatomiosite juvenil (DMJ) e imunização de acordo com os tratamentos utilizados. RESULTADOS: Cartão de vacinação foi visto por 100% dos profissionais na primeira consulta e por 36% anualmente. Vacinas de agentes vivos não foram recomendadas para pacientes com LESJ, AIJ e DMJ em 44%, 64% e 48%, respectivamente. Os profissionais foram divididos em dois grupos: A (< 15 anos de prática, n = 12) e B (> 16 anos, n = 13). Nenhuma diferença estatística foi observada no uso de vacinas de agentes vivos e vacinas de agentes inativos ou componentes proteicos em relação ao tratamento nos dois grupos (P > 0,05). Além disso, os grupos foram similares em relação à opinião sobre a gravidade de imunossupressão em pacientes com LESJ, AIJ e DMJ com ou sem atividade e a terapêutica utilizada (P > 0,05). CONCLUSÕES: A frequência de vacinação por reumatologistas pediátricos de São Paulo é baixa, especialmente após a primeira consulta, e não é influenciada pelo tempo de prática profissional.INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL AND METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (< 15 years of practice, n = 12) and B (> 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.

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

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.

We evaluated the prevalence and clinical associations of amenorrhea in 298 female juvenile systemic lupus erythematosus (JSLE) patients (ACR criteria) followed in 12 Brazilian Paediatric Rheumatology centres. Amenorrhea was observed in 35 patients (11.7%) with a mean duration of 7.2 ± 3.6 months. The hormones were performed in 32/35 patients and none of them had FSH and LH levels above and estradiol below the normal range according to pubertal changes. JSLE patients with amenorrhea were younger (15.04 ± 2.5 versus 17.8 ± 3.1 years; P = 0.001), and had a shorter period of time between menarche and current age (3.4 ± 2.9 versus 6.7 ± 5.4 years; P = 0.001). Interestingly, the frequency, cumulative dose, number of pulses and duration of intravenous cyclophosphamide treatment were alike in patients with and without amenorrhea ( P > 0.05). In contrast, patients with amenorrhea had significantly higher SLEDAI ( P = 0.01) and SLICC/ACR-DI ( P = 0.024) scores compared to those without this condition. Independent risk factors identified by multivariate analysis were higher SLEDAI (OR = 1.059; CI = 1.004—1.116; P = 0.034) and SLICC/ACR-DI (OR = 2.125; IC = 1.373—3.291; P = 0.001) scores. Our data suggest that in spite of immunosuppressive therapy, JSLE patients have an adequate ovarian follicular reserve and amenorrhea is particularly associated with disease activity and damage. Lupus (2007) 16, 531—536.

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.

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos. Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.

To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes.The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation.1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations.1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions.

Made available in DSpace on 2015-12-07T15:31:47Z (GMT). No. of bitstreams: 0 Previous issue date: 2015. Added 1 bitstream(s) on 2019-10-09T18:24:47Z : No. of bitstreams: 1 S0482-50042016000100044.pdf: 771872 bytes, checksum: 98287e6415e7d85100770f0f1070148f (MD5) Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das síndromes periódicas associadas à criopirina (criopirinopatias – Caps). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de quatro questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1.215 artigos e selecionados 42 trabalhos para sustentar as recomendações. Recomendações: 1. O diagnóstico de Caps é baseado na anamnese e nas manifestações clínicas e posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e Cinca (forma grave). Avaliações neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinção entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de Caps, isto é, indivíduos que apresentam, antes dos 20 anos, episódios recorrentes de inflamação expressa por urticária e febre moderada; 3. As alterações laboratoriais incluem leucocitose e elevação nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitações importantes em relação à segurança em longo prazo. Nenhuma das três medicações anti-IL1 evita progressão das lesões ósseas. To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. The Guideline was prepared from 4 clinical questions that were structured through Pico (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. 1. The diagnosis of Caps is based on clinical history and clinical manifestations, and later confirmed by genetic study. Caps may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and Cinca (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of Caps, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brasil Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brasil Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto (FMRP), Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil Universidade Federal do Pará (UFPA), Belém, PA, Brasil Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brasil Instituto de Puericultura e Pediatria Martagão Gesteira, Serviço de Reumatologia Pediátrica, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil Hospital Pequeno Príncipe, Curitiba, PR, Brasil Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu (FMB), Universidade Estadual Paulista (UNESP), Botucatu, SP, Brasil Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu (FMB), Universidade Estadual Paulista (UNESP), Botucatu, SP, Brasil

INTRODUÇÃO/OBJETIVO: Avaliar a frequência do comprometimento da habilidade verbal e possíveis fatores associados em pacientes portadores de Lúpus Eritematoso Sistêmico Juvenil (LESJ). PACIENTES E MÉTODOS: Estudo transversal de 36 crianças e adolescentes com LESJ, de um grupo de 57 pacientes, do Ambulatório de Reumatologia do Departamento de Pediatria e Clínica Médica da Santa Casa de Misericórdia de São Paulo. Por ocasião do diagnóstico e do estudo, foram analisados aspectos epidemiológicos, clínicos, socioeconômicos e de escolaridade. Os pacientes foram submetidos a testes cognitivos e exames laboratoriais, e foram avaliadas medidas de atividade da doença (SLEDAI) e do dano cumulativo (SLICC-DI) e o tratamento com corticoesteroide. Os pacientes foram submetidos a testes cognitivos (escalas Weschler de inteligência: WISC III e WAISS III), e os resultados foram avaliados de acordo com os aspectos epidemiológicos, clínicos, laboratoriais e terapêuticos. RESULTADOS: A média de idade ao diagnóstico foi de 11,2 ± 2 anos, a idade na época do estudo de 15,4 ± 4,7 anos, com 89% do sexo feminino. Houve predomínio de pacientes da classe socioeconômica C (61,1%). O comprometimento cognitivo detectado nesses pacientes foi frequente (58,3%), sendo o comprometimento da habilidade verbal um dos domínios cognitivos mais constantes. Encontrou-se associação do comprometimento da habilidade verbal com baixa condição socioeconômica e dano cumulativo (P < 0,05), mas não com atividade da doença, presença de autoanticorpos e dose de corticoesteroide (P > 0,05). CONCLUSÕES: Alteração da habilidade verbal é frequente no LESJ e está associada à condição socioeconômica e ao dano cumulativo, devendo ser suspeitada e investigada, principalmente por se tratar de pacientes pediátricos, para que não haja comprometimento da qualidade de vida na fase adulta. Como não está relacionado à atividade da doença ou à presença de autoanticorpos, deve ser sempre avaliado na presença ou não desses fatores. Da mesma forma, deve-se avaliar independentemente das doses de corticoesteroide por não haver associação. INTRODUCTION/OBJECTIVE: Evaluate the frequency of verbal ability impairment and associated factors in patients with juvenile systemic lupus erythematosus (JSLE). PATIENTS AND METHODS: Cross sectional study of 36 children and adolescents with JSLE of a group of 57 patients at the Clinic of Rheumatology, Department of Pediatrics and Medical Clinic of Santa Casa de Misericórdia de São Paulo. At the time of diagnosis and study, we analyzed the following epidemiological features: clinical, socioeconomic, and educational level. Patients underwent cognitive and laboratory tests and we assessed disease activity (SLEDAI), cumulative damage (SLICC-DI), and treatment with corticosteroids. The patients underwent cognitive tests (Wechsler Intelligence Scales: WISC III and Waiss III), and the results were evaluated according to epidemiological, clinical, laboratory and treatment features. RESULTS: The mean age at diagnosis was 11.2 ± 2 years and at the time of the study the mean age was 15.4 ± 4.7 years. There was predominance of women (89%) and of socioeconomic class C patients (61.1%). The cognitive impairment found in these patients was frequent (58.3%), affecting more often the verbal ability. We found association of verbal ability impairment with low socioeconomic status and cumulative damage (P < 0.05), but not with disease activity, presence of autoantibodies, and dose of corticosteroids (P > 0.05). CONCLUSIONS: Change in verbal ability is frequent in JSLE and is associated with socioeconomic status and cumulative damage, and should be suspected and investigated, particularly in pediatric patients to avoid quality of life impairment in adulthood. As it is not related with disease activity or presence of autoantibodies, it should always be assessed in the presence or absence of these factors. Likewise, the doses of corticosteroids should be independently evaluated.

Submitted by Guilherme Lemeszenski (guilherme@nead.unesp.br) on 2013-08-22T19:03:00Z No. of bitstreams: 1 S0482-50042010000400002.pdf: 313332 bytes, checksum: 96870657aea3327450d91534cda09689 (MD5) Made available in DSpace on 2013-08-22T19:03:00Z (GMT). No. of bitstreams: 1 S0482-50042010000400002.pdf: 313332 bytes, checksum: 96870657aea3327450d91534cda09689 (MD5) Previous issue date: 2010-08-01 Made available in DSpace on 2013-09-30T20:04:20Z (GMT). No. of bitstreams: 2 S0482-50042010000400002.pdf: 313332 bytes, checksum: 96870657aea3327450d91534cda09689 (MD5) S0482-50042010000400002.pdf.txt: 28357 bytes, checksum: ca76c2ae8ebb973bad986ae5922eefcf (MD5) Previous issue date: 2010-08-01 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T15:16:32Z No. of bitstreams: 2 S0482-50042010000400002.pdf: 313332 bytes, checksum: 96870657aea3327450d91534cda09689 (MD5) S0482-50042010000400002.pdf.txt: 28357 bytes, checksum: ca76c2ae8ebb973bad986ae5922eefcf (MD5) Made available in DSpace on 2014-05-20T15:16:32Z (GMT). No. of bitstreams: 2 S0482-50042010000400002.pdf: 313332 bytes, checksum: 96870657aea3327450d91534cda09689 (MD5) S0482-50042010000400002.pdf.txt: 28357 bytes, checksum: ca76c2ae8ebb973bad986ae5922eefcf (MD5) Previous issue date: 2010-08-01 INTRODUÇÃO/OBJETIVOS: Avaliar a prática clínica com relação à verificação do cartão vacinal e à indicação de vacinas específicas em pacientes com doenças reumáticas pediátricas em uso de diferentes drogas, e evidenciar a possível associação entre frequência de vacinação e tempo de prática clínica dos reumatologistas pediátricos do estado de São Paulo. MATERIAL E MÉTODOS: Um questionário foi enviado para os reumatologistas pediátricos do Departamento de Reumatologia da Sociedade de Pediatra de São Paulo. Esse instrumento incluiu questões sobre tempo de prática em Reumatologia Pediátrica, vacinação de pacientes com Lúpus Eritematoso Sistêmico Juvenil (LESJ), artrite idiopática juvenil (AIJ), dermatomiosite juvenil (DMJ) e imunização de acordo com os tratamentos utilizados. RESULTADOS: Cartão de vacinação foi visto por 100% dos profissionais na primeira consulta e por 36% anualmente. Vacinas de agentes vivos não foram recomendadas para pacientes com LESJ, AIJ e DMJ em 44%, 64% e 48%, respectivamente. Os profissionais foram divididos em dois grupos: A (< 15 anos de prática, n = 12) e B (> 16 anos, n = 13). Nenhuma diferença estatística foi observada no uso de vacinas de agentes vivos e vacinas de agentes inativos ou componentes proteicos em relação ao tratamento nos dois grupos (P > 0,05). Além disso, os grupos foram similares em relação à opinião sobre a gravidade de imunossupressão em pacientes com LESJ, AIJ e DMJ com ou sem atividade e a terapêutica utilizada (P > 0,05). CONCLUSÕES: A frequência de vacinação por reumatologistas pediátricos de São Paulo é baixa, especialmente após a primeira consulta, e não é influenciada pelo tempo de prática profissional. INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL and METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (< 15 years of practice, n = 12) and B (> 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice. Universidade de São Paulo Faculdade de Medicina Instituto da Criança Universidade Federal de São Paulo (UNIFESP) USP FM USP Faculdade de Medicina de Ribeirão Preto Universidade Estadual Paulista Faculdade de Medicina de Botucatu Santa Casa de Misericórdia de São Paulo Hospital Municipal Infantil Menino Jesus Universidade Estadual de Campinas Faculdade de Ciências Médicas Universidade Estadual Paulista Faculdade de Medicina de Botucatu

Evaluate the frequency of verbal ability impairment and associated factors in patients with juvenile systemic lupus erythematosus (JSLE).Cross sectional study of 36 children and adolescents with JSLE of a group of 57 patients at the Clinic of Rheumatology, Department of Pediatrics and Medical Clinic of Santa Casa de Misericórdia de São Paulo. At the time of diagnosis and study, we analyzed the following epidemiological features: clinical, socioeconomic, and educational level. Patients underwent cognitive and laboratory tests and we assessed disease activity (SLEDAI), cumulative damage (SLICC-DI), and treatment with corticosteroids. The patients underwent cognitive tests (Wechsler Intelligence Scales: WISC III and Waiss III), and the results were evaluated according to epidemiological, clinical, laboratory and treatment features.The mean age at diagnosis was 11.2 ± 2 years and at the time of the study the mean age was 15.4 ± 4.7 years. There was predominance of women (89%) and of socioeconomic class C patients (61.1%). The cognitive impairment found in these patients was frequent (58.3%), affecting more often the verbal ability. We found association of verbal ability impairment with low socioeconomic status and cumulative damage (P0.05), but not with disease activity, presence of autoantibodies, and dose of corticosteroids (P0.05).Change in verbal ability is frequent in JSLE and is associated with socioeconomic status and cumulative damage, and should be suspected and investigated, particularly in pediatric patients to avoid quality of life impairment in adulthood. As it is not related with disease activity or presence of autoantibodies, it should always be assessed in the presence or absence of these factors. Likewise, the doses of corticosteroids should be independently evaluated.

OBJETIVO: Avaliar a freqüência e a gravidade dos efeitos adversos durante a infusão de infliximabe. MATERIAL E MÉTODOS: Avaliação retrospectiva de prontuários de 58 pacientes acompanhados em cinco serviços de reumatologia pediátrica. Todos apresentavam doença refratária ou eram intolerantes ao uso de uma ou mais drogas modificadoras de doença e receberam uma ou mais infusões de infliximabe. A análise estatística foi descritiva, levando-se em conta a freqüência e o tipo dos efeitos adversos, assim como sua relação com o número de infusões, a dose de infliximabe e a indicação. RESULTADOS: A média da idade na época da introdução do infliximabe foi de 12 anos e 9 meses e a média do tempo de evolução da doença foi de 7 anos e 7 meses. A principal indicação da medicação foi artrite idiopática juvenil (43). Dos 58 pacientes avaliados, 25 (43,1%) apresentaram efeitos adversos durante a infusão e em 17 (29,3%), a medicação teve de ser suspensa. As reações que ocorreram foram: dispnéia (dez), náuseas e vômitos (oito), rash cutâneo (sete), choque anafilático (seis), rubor facial (cinco), angioedema (quatro), dor torácica (quatro), urticária e hipertensão, entre outras. A reação anafilática foi mais freqüente entre a quarta e a sexta infusão. CONCLUSÃO: A freqüência e a gravidade dos efeitos adversos ao infliximabe devem ser levadas em conta, não apenas por ocasião da sua indicação, mas principalmente por ocasião da escolha do local para a sua aplicação, que deve contar com estrutura para atendimento de urgência. OBJECTIVE: To evaluate the frequency and the severity of the adverse reactions during the infusion of infliximab. METHODS: We performed a retrospective chart review of 58 patients, followed up at 5 paediatric rheumatology centers. All patients presented refractory disease or were intolerant to one or more disease modifying drugs and received one or more infliximab infusions. The data analysis was descriptive, considering the frequency and the kind of adverse reactions as well as the number of the infusions, the infliximab dose and the indication. RESULTS: The mean age at the onset of infliximab was 12 years and 9 months and the mean of the disease duration was 7 years and 7 months. The main indication of the drug was juvenile idiopathic arthritis (43). Twenty five out of 58 patients (43.1%) presented adverse reactions during the infusion and 17 (29,3%) had to withdraw the treatment. The reactions were: dyspnea (ten), nausea and vomiting (eight), rash (seven), anaphylaxis (six), flush (five), angioedema (four), chest pain (four) besides urticaria, hypertension, among others. The anaphylaxis was more frequent between the fourth and the sixth infusion. CONCLUSION: The frequency and the severity of adverse reactions to infliximab must be considered not only in relation to its indication, but mainly for the choice of the center to the drug infusion where emergency treatment may be performed. Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Setor de Reumatologia Pediátrica Universidade Federal do Rio de Janeiro Faculdade de Medicina UFRJ Instituto de Puericultura e Pediatria Martagão Gesteira Serviço de Reumatologia Pediátrica UFRJ Faculdade de Medicina setor de Reumatologia do Adolescente Irmandade da Santa Casa de Misericórdia de São Paulo Departamento de Pediatria Serviço de Reumatologia Pediátrica PMSP SMS Hospital Infantil Menino Jesus UNIFESP, Depto. de Pediatria Setor de Reumatologia Pediátrica SciELO

Takayasu's arteritis (TA) and rheumatic fever are diseases that can start with cardiac features, making the diagnosis difficult. There are reports of association of RF with Takayasu's arteritis beginning with cardiac involvement in pediatric patients. The aim of this study is to report the possible association of RF and TA in patients with cardiac abnormalities. We describe the case of an adolescent initially diagnosed with RF who progressed with changes that allowed making the diagnosis of TA. TA and RF are two important causes of valve involvement that may have systemic manifestations.

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.