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-This study was based on a prospective and a retrospective analysis of 35 patients who met Bohan and Peter criteria for juvenile dermatomyositis diagnosis.The mean follow-up time was three years ten months. Calcinosis was present in five (14.28 %) patients, cutaneous ulcers in four (11.42%), and systemic involvement in nine (27.71%) patients. All patients presented alterations in the serum levels of muscle enzymes, and all of them were submitted to muscle biopsy as a diagnostic procedure. Nine (25.71%) patients received corticotherapy prior to and 26 (74.28%) after the muscle biopsy. Chloroquine, methotrexate, cyclosporine, cyclophosphamide and intravenous immunoglobulin were used in patients with poor response to corticotherapy. Continuation of cutaneous manifestations was observed in 4 (11.43%) patients, laboratorial activity in 1 (2.85%), cutaneous and laboratorial activities in 3 (8.57%). Ten (28.57%) patients were out of activity, and 17 (48.57%) in remission at study end-point, on March 2002. Two (5.71%) patients died.KEY WORDS: juvenile dermatomyositis, clinical findings, muscle biopsy, treatment, evolution, outcome. Dermatomiosite juvenil: parâmetros clínicos, laboratoriais, histológicos, terapêuticos e evolutivos de 35 pacientesRESUMO -Este estudo foi baseado na análise prospectiva e retrospectiva de 35 pacientes que preencheram os critérios diagnósticos de Bohan e Peter de dermatomiosite juvenil. O tempo médio de seguimento foi de 3 anos e 10 meses. Foi observado calcinose em 5 (14,28%) pacientes, úlcera cutânea em 4 (11,42%) e envolvimento sistêmico em 9 (22,71%). Todos os pacientes apresentavam alterações nos níveis séricos das enzimas musculares e todos foram submetidos a biópsia muscular como procedimento diagnóstico. Nove (25,71%) pacientes receberam corticoterapia antes e 26 (74,28%) depois da realização da biópsia muscular. Foram utilizados cloroquina, metotrexato, ciclosporina, ciclofosfamida e imunoglobulina endovenosa em todos os pacientes que não apresentaram boa resposta ao corticóide. Houve manutenção das manifestações cutâneas em 4 (11,43%) pacientes, atividade laboratorial em 1 (2,85%), atividades cutânea e laboratorial em 3 (8,57%). Dez (28,57%) pacientes estavam fora de atividade e 17 (48,57%) em remissão por ocasião do término do estudo em março de 2002. Dois (5,71%) pacientes faleceram.

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Objective. To investigate the effects of a supervised exercise training program on health parameters, physical capacity, and health-related quality of life in patients with mild and chronic juvenile dermatomyositis (DM). Methods. This was a prospective longitudinal study following 10 children with mild and chronic juvenile DM (disease duration >1 year). The exercise program consisted of twice-a-week aerobic and resistance training. At baseline and after the 12-week intervention, we assessed muscle strength and function, aerobic conditioning, body composition, juvenile DM scores, and health-related quality of life. Results. Child self-report and parent proxy-report Pediatric Quality of Life Inventory scores were improved after the intervention (؊40.3%; P ‫؍‬ 0.001 and ؊48.2%; P ‫؍‬ 0.049, respectively). Importantly, after exercise, the Disease Activity Score was reduced (؊26.9%; P ‫؍‬ 0.026) and the Childhood Muscle Assessment Scale was improved (؉2.5%; P ‫؍‬ 0.009), whereas the Manual Muscle Test presented a trend toward statistical significance (؉2.2%; P ‫؍‬ 0.081). The peak oxygen consumption and time-to-exhaustion were increased by 13.3% (P ‫؍‬ 0.001) and 18.2% (P ‫؍‬ 0.003), respectively, whereas resting heart rate was decreased by 14.7% (P ‫؍‬ 0.006), indicating important cardiovascular adaptations to the exercise program. Upper and lower extremity muscle strength and muscle function were also significantly improved after the exercise training (P < 0.05). Both the whole-body and the lumbar spine bone mineral apparent density were significantly increased after training (1.44%; P ‫؍‬ 0.044 and 2.85%; P ‫؍‬ 0.008, respectively). Conclusion. We showed for the first time that a 12-week supervised exercise program is safe and can improve muscle strength and function, aerobic conditioning, bone mass, disease activity, and health-related quality of life in patients with active and nonactive mild and chronic juvenile DM with near normal physical function and quality of life.

This study has identified low lean mass and GC pulse therapy use as the major factors for low hip BMAD in JDM patients.

Objective. To investigate the association between exposure to air pollutants in the Sao Paulo metropolitan area and disease activity in juvenile-onset systemic lupus erythematosus (SLE) patients. Methods. A longitudinal panel study based on 409 consecutive visits of juvenile-onset SLE patients living in the Sao Paulo metropolitan area was carried out. Disease activity was evaluated in accordance with the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), and the patients were divided into 2 groups: those with SLEDAI scores £8 and those with SLEDAI scores >8. Daily concentrations of inhaled particulate matter (PM 10 ), sulfur dioxide, nitrogen dioxide (NO 2 ), ozone, and carbon monoxide (CO) were evaluated on the 21 days preceding the medical visits. A generalized estimation equation model was used to assess the impact of these measurements on SLEDAI-2K scores, considering the fixed effects for repetitive measurements. The models were adjusted for erythrocyte sedimentation rate, corticosteroid use (daily and cumulative doses), antimalarial use, the use of immunosuppressive agents, the presence of infection 20 days preceding the medical appointment, and the minimum temperature and relative humidity outdoors. Results. PM 10 , NO 2 , and CO were risk factors for juvenile-onset SLE disease activity (SLEDAI-2K score >8) approximately 2 weeks after exposure. A 13.4 mg/m 3 increase in the PM 10 moving average (from lag 12 to lag 15) was associated with a 34% increase (95% confidence interval 7.0-68.0) in the risk of a SLEDAI-2K score >8. Conclusion. This is the first study to show that exposure to inhaled pollutants may increase the risk of disease activity in children with juvenile-onset SLE in a large urban center.