This core set has been approved by the American College of Rheumatology (ACR) Board of Directors asProvisional. This signifies that the core set has been quantitatively validated using patient data, but it has not undergone validation based on an external data set. All ACR-approved core sets are expected to undergo intermittent updates.Objective. To validate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile dermatomyositis (DM). Methods. In 2001, a preliminary consensus-derived core set for evaluating response to therapy in juvenile DM was established. In the present study, the core set was validated through an evidence-based, large-scale data collection that led to the enrollment of 294 patients from 36 countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, concordance in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response. Results. The following clinical measures were found to be feasible, and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) muscle strength, 3) global disease activity measure, 4) parent's global assessment of patient's well-being, 5) functional ability, and 6) health-related quality of life. Conclusion. The members of the Paediatric Rheumatology International Trials Organisation, with the endorsement of the American College of Rheumatology and the European League Against Rheumatism, propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a child with juvenile DM has responded adequately to therapy.

We tested the possible association of the 14-bp polymorphism of the HLA-G gene in the course of two inflammatory diseases, rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA). Patients and controls were genotyped for the 14-bp polymorphism by polymerase chain reaction with specific primers for the exon 8 of the human leukocyte antigen (HLA)-G gene and the amplified fragment was visualized in a 6% polyacrylamide gel. A total of 106 JIA patients, 265 RA patients, 356 healthy adults and 85 healthy children were genotyped for the 14-bp polymorphism. Female JIA patients presented a higher frequency of the -14 bp allele when compared with female healthy children (0.743 and 0.500, corrected P=0.003), which reflected in the JIA group as a whole. This increased frequency of the -14-bp allele was observed in all JIA subtypes. In RA patients, no differences in allelic and genotypic frequencies were observed between patients and controls. No correlations were observed among genotype and disease severity or clinical manifestations. Our data suggest that the HLA-G -14 bp allele is probably a risk factor for JIA, mainly in females. Considering the differences observed in relation to gender, we suggest that hormonal differences can interfere with the development of JIA. Considering the RA patients, our data agree with results from the literature and highlight the differences in the etiology of RA and JIA.

No abstract

Em 1991 avaliou-se a cobertura vacinal em crianças de 12 a 23 meses de idade no território de responsabilidade de um Posto de Atenção Primária à Saúde, na periferia da Zona Norte de Porto Alegre, RS, Brasil, cinco anos após sua implantação, com a finalidade de melhorar a qualidade das ações de saúde desenvolvidas no serviço. Foram investigadas todas as crianças através de um inquérito domiciliar, observando-se a carteira de vacinas e as informações da mãe. Em 1986, um inquérito inicial havia identificado uma cobertura vacinal inferior a 60% para cada uma das vacinas. A atual cobertura vacinal (doses comprovadas) para três doses da vacina DPT (Difteria, Pertussis e Tétano), três doses da Sabin (antipoliomielite), uma dose da anti-sarampo (VAS) e uma dose de BCG são, respectivamente 87, 89, 88 e 79%. Apesar das altas coberturas observadas por tipos de vacinas, quando se verificou para cada criança se o esquema básico do primeiro ano de vida estava completo (3 doses de DPT + 3 doses de Sabin + 1 dose de VAS + 1 dose de BCG), encontrou-se apenas 75% das crianças na citada situação. A cobertura vacinal é heterogênea dentro do território, sendo maior naquelas áreas caracterizadas por piores condições socioeconômicas, onde a equipe de saúde havia intensificado esforços. A comparação com o método administrativo de avaliação de cobertura, realizado mensalmente, mostrou a não-adequação desse, que subestimava a cobertura vacinal. Avaliou-se a situação vacinal das mães, para vacina antitetânica, e apenas 49% das crianças estavam protegidas contra o tétano neonatal. Os dados obtidos subsidiaram a imediata reestruturação das ações do programa, com vistas a atingir uma cobertura vacinal de 100%, e melhorar a qualidade das ações de saúde prestadas pela equipe. Vacinação, estatística, Esquema de imunização. Avaliação de processos e resultados, cuidados de saúde. IntroduçãoNenhuma ação em saúde é tão efetiva e eficiente quanto vacinar crianças contra as principais doenças preveníveis por imunização. Entretanto, ainda existe um hiato entre o potencial dessa práti-ca e suas reais contribuições para a sobrevivência infantil 14,16,21,22 . Anualmente morrem cerca de três milhões de crianças e outras tantas tornam-se inválidas devido a essas doenças 14,22 . A cobertura vacinal -definida como o percentual de uma população-alvo que foi vacinada 19,35 -nos chamados "países em desenvolvimento" ainda está aquém da esperada 21,39 . Para atingir uma cobertura vacinal adequada é preciso fundamentalmente ações institucionais conjugadas e organizadas pelo setor público em diversos níveis 15

This study suggests that in JIA, unlike in RA, CCR5Delta32 does not have a protective effect, but instead it could be a factor associated with more inflammatory forms of the disease. These observations give rise to new questions about the mechanism and the cellular types involved in JIA as well as about the aetiology of JIA.

No abstract

Acrodermatitis Continua of Hallopeau is a rare, chronic, recurrent disorder classified as a form of pustular psoriasis, and most cases affect one or two digits. It tends to be resistant to both topical and systemic treatments for psoriasis. We present an infant with Acrodermatitis Continua of Hallopeau affecting nineteen nails, with an excellent response to the combination of thalidomide and ultraviolet B phototherapy.

Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.