Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients

Abstract

-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.

Fatores de risco associados à calcinose na dermatomiosite juvenil

“…8,11 However, in this sample, evidence of gastrointestinal involvement was observed in 40% of the population in contrast with the 17.1 to 26% described in the literature. 8,11 Calcinosis affects between 10 and 70% of patients with JDM.…”

“…8,11 However, in this sample, evidence of gastrointestinal involvement was observed in 40% of the population in contrast with the 17.1 to 26% described in the literature. 8,11 Calcinosis affects between 10 and 70% of patients with JDM. 8,11,12 Our study identified this disorder in 43% of the patients and it was associated with severe cases of the disease, where there was cardiac involvement and immunosuppressors had been required.…”

“…8,11 Calcinosis affects between 10 and 70% of patients with JDM. 8,11,12 Our study identified this disorder in 43% of the patients and it was associated with severe cases of the disease, where there was cardiac involvement and immunosuppressors had been required.…”

“…10 Calcinosis are more common in the pediatric age group, affecting from 10 to 70% of children and adolescents with JDM, compared with 30% of adults with dermatomyositis (DM). 8,11,12 The etiopathogenesis of calcinosis is unknown.…”

Tacrolimus tópico nas lesões cutâneas refratárias da dermatomiosite juvenil

“…Carlos Nobre Rabelo Júnior (1) , Nádia Emi Aikawa (1) , Kátia Tomie Kozu (1) , Adriana Maluf Elias Sallum (2) , Lúcia Maria Arruda Campos (2) , Clovis Artur Almeida da Silva (3) RESUMO A dermatomiosite juvenil (DMJ) é uma doença inflamatória crônica idiopática que afeta principalmente músculos e pele. As lesões cutâneas podem persistir apesar do controle bem-sucedido da miosite.…”