RESUMO
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, p<0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114-5.3381, p = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2-51) vs 14 (0-51), p<0.001). 2019-EULAR/ACR score >25 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.
Assuntos
Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Reumatologia , DNA , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
OBJECTIVE: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. METHODS: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. RESULTS: cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),pâ¯<â¯0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,pâ¯<â¯0.0001), polyneuropathy (9% vs. 1%,pâ¯<â¯0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, pâ¯<â¯0.0001) and intravenous cyclophosphamide use (59% vs. 37%, pâ¯<â¯0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.
Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Complicações na Gravidez , Adulto , Idade de Início , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/epidemiologia , Brasil/epidemiologia , Criança , Estudos de Coortes , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Morbidade , Gravidez , Estudos RetrospectivosRESUMO
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.
Assuntos
Humanos , Criança , Adolescente , Artrite Juvenil , Consenso , Imunização Passiva , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Vacinação , VacinasRESUMO
A ocorrência de manifestações clínicas e laboratoriais semelhantes às encontradas na doença de Lyme e da coinfecção com a babesiose já foi demonstrada em trabalhos anteriores em adultos, porém não existem estudos desta natureza em crianças. OBJETIVO: Caracterizar o perfil clínico-epidemiológico da síndrome de Lyme-Símile em crianças do Estado de Mato Grosso do Sul e avaliar a prevalência dos anticorpos anti-Borrelia burgdorferi e anti-Babesia bovis no soro de pacientes que preencheram os critérios estabelecidos. MÉTODOS: 100 pacientes entre 9 meses e 16 anos de idade foram submetidos à pesquisa dos anticorpos pela técnica de ELISA e a soroprevalência foi comparada com um grupo-controle. RESULTADOS: Positividade para anticorpos anti-B. burgdorferi ocorreu em 27 por cento dos pacientes com suspeita clínica, sendo 17 por cento IgM e 12 por cento IgG. As manifestações articulares ocorreram em 21 pacientes, as manifestações cutâneas em três pacientes e as manifestações neurológicas em três pacientes. A prevalência de anticorpos contra B. burgdorferi nos pacientes do grupo-controle foi de 15 por cento (p < 0,05). Os anticorpos anti-B. bovis estiveram presentes em 24 por cento dos pacientes suspeitos e em 3 por cento do grupo-controle (p < 0,05). Concomitância dos anticorpos ocorreu em 10 pacientes com suspeita clínica e em nenhum controle. CONCLUSÃO: Os resultados deste estudo evidenciam a etiologia de caráter infeccioso e reacional das manifestações clínicas encontradas nos pacientes, podendo caracterizar uma síndrome, que possivelmente ocorra na presença de múltiplos microrganismos e está relacionada com carrapatos. Foi encontrada freqüência superior de anticorpos contra Borrelia e Babesia nos pacientes em relação aos controles. Embora pequena, essa diferença foi estatisticamente significante. Este achado não atesta que o quadro clínico dos pacientes seja causado por espécies de Borrelia ou Babesia, mas levantam a possibilidade de participação...
The occurence of clinical and laboratory manifestations similar to the Lyme disease and the coinfection with babesiosis was already demonstraded in previous researches with adult patients. However, there are no studies in children. OBJECTIVE: To identify the clinical-epidemiological profile of the Lyme Simile Syndrome in children of the State of Mato Grosso do Sul and to evaluate the prevalence of antibodies against Borrelia burgdorferi and Babesia bovis in the serum of patients that had fulfilled the established criteria. METHODS: One hundred patients (age range: 9 months and 16 years-old) were screened for the presence of those antibodies, using ELISA, comparing to a control group. RESULTS: Antibodies against B. burgdoferi were found in 27 percent of the patients with a clinical picture. The articular manifestations occurred in 21 patients, cutaneous in 3 and neurological in 3. The prevalency in the control group was 15 percent (p < 0.05). The antibodies for Babesia bovis were present in 24 percent of suspect patients and in 3 percent of the control group (p < 0.05). Concomitance of the two antibodies occurred in 10 patients with clinical suspect and no one of control group. CONCLUSION: The results found in this study suggest the infective and reactive character of the clinical manifestations of the patients, which can characterize a Syndrome, which possibly occurs in the presence of multiple microorganisms that can be associated with tick-borne. It was found a higher frequency of antibodies against borrelia and babesia in patients in relation to the controls. Although small, this difference was statistically significant. This finding does not certify that the clinical features of patients is caused by species of borrelia or babesia, but raised the possibility of involvement of some infectious agent that could be related to these micro-organisms. The studies should continue in order to improve the delineation of this syndrome and to...
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Babesiose , Borrelia , Borrelia burgdorferi , Grupo Borrelia Burgdorferi , Infecções por Borrelia , Doença de Lyme , Doenças Transmitidas por CarrapatosRESUMO
A síndrome CINCA (crônico-infantil-neurológica-cutâneaarticular) é uma enfermidade inflamatória multissistêmica rara, de início no período neonatal e caracterizada por febre, exantema cutâneo, envolvimento articular e do sistema nervoso central. É também conhecida pela literatura médica norte-americana como NOMID (doença multissistêmica inflamatória de início neonatal). Relatamos o caso de uma criança de 3 anos de idade admitida em nosso serviço com história de febre e exantema cutâneo desde o período neonatal. Apresentou crises convulsivas no sexto mês de vida e artrite simétrica de joelhos desde o nono mês. Na admissão, mostrava-se toxemiada, pálida, com um exantema maculopapular generalizado e artrite de joelhos e tornozelos. Apresentava ainda retardo de crescimento e desenvolvimento. Achados laboratoriais incluíram anemia, leucocitose, trombocitose, níveis elevados de proteína C reativa e meningite asséptica no exame do liquor. Os outros exames foram negativos. Os achados radiográficos dos joelhos, quadris e tornozelos foram anormais. A criança recebeu tratamento com antiinflamatório não hormonal, corticosteróide e metotrexato, com melhora apenas da dor e da febre. A etiologia da síndrome CINCA permanece desconhecida e nenhum tratamento tem se mostrado eficaz. Essa doença deve ser distinguida da forma sistêmica da artrite idiopática juvenil (AIJ), o principal diagnóstico diferencial.
CINCA syndrome (chronic-infantile-neurological-cutaneousarticular) is a rare multisystemic inflammatory disease with neonatal onset characterized by fever, skin rash, articular, and central nervous system involvement. This syndrome is known in the North American medical literature as infantile onset multisystem inflammatory disease (NOMID). We describe the case of a 3-yearold child admitted in our service with fever and skin rash since the neonatal period. She presented seizures at 6 months-old and bilateral arthritis of the knees since her 9 months. On admission she looked sick, pale and presented generalized erythematosus maculopapular rash, arthritis of the knees and ankles. She presented growth and mental retardation too. Laboratory Endings included anemia, leukocytosis, thrombocytosis, elevated C-reactive protein levels and the examination Endings from the cerebrospinal fluid showed an aseptic meningitis. Other test results were negative. Radiographs of the knees, hips and ankles revealed abonrmalities. The child received treatment with nonsteroidal anti-inflammatory drugs, corticosteroids and methotrexate, with partial response to treatment, leading to improvement in pain and fever defervescence. The etiology of CINCA syndrome remains unknown and no single treatment has been found to be effective. This disease should be distinguished from systemic onset juvenile idiophatic arthritis (JIA), the main differential diagnosis.
Assuntos
Humanos , Feminino , Pré-Escolar , Artrite , Artrite Juvenil , Artrite Juvenil/diagnóstico , Diagnóstico Diferencial , ExantemaRESUMO
PURPOSE: To determine the consequences of the chronic use of systemic corticosteroids in children with juvenile rheumatoid arthritis by means of evaluating osteochondral effects depicted by magnetic resonance imaging. PATIENTS AND METHODS: We reviewed clinical and magnetic resonance imaging findings in 69 children (72 knees) with juvenile rheumatoid arthritis. Two groups were studied. Group I: 34 (49.3 percent) children had previous or current use of systemic corticotherapy (22 girls; 12 boys; mean age: 11.3 years; mean disease duration: 5.9 years; mean corticotherapy duration: 2.9 years; mean cumulative dose of previous corticosteroids: 5000 mg); Group II: 35 (50.7 percent) children had no previous use of corticosteroids (27 girls; 8 boys; mean age: 11.7 years; mean disease duration: 5.3 years). The groups were compared statistically. RESULTS: In the group that had received corticotherapy (Group I), osteochondral abnormalities were significantly correlated to long-standing disease (>3.5 years; p<0.001). This correlation was not found in the group that had no previous history of corticotherapy (Group II). No correlations were established between median dose of corticosteroids and magnetic resonance imaging findings. CONCLUSION: It is important to further investigate the long-term intra-articular effects of systemic corticotherapy to ensure that the side effects of the aggressive therapy will not be more harmful for the joints than the symptoms suffered over the natural course of the disease
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adulto , Adolescente , Corticosteroides , Artrite Juvenil , Doenças Ósseas/diagnóstico , Joelho , Corticosteroides , Progressão da Doença , Doenças Ósseas/induzido quimicamente , Imageamento por Ressonância MagnéticaRESUMO
Os autores pretendem, neste estudo transversal ("cross-sectional") de pacientes co artrite reumatóide juvenil, comparar a análise bidimensional de espessura sinovial máxima obtida de cortes de sequencia axial sinovial (tridimensional) obtida de cortes da sequencia axial T1 SPIR ("spectral presaturation with inversion recovery") SE pós-gadolino. Estas duas formas de avaliaçäo imagenológica säo associadas a parâmetros clínicos, laboratoriais e á presença de realce sinovial á ressonância magnética, com objetivo de avaliaçäo de seu custo-benefício.