RESUMO
We describe a 9-year-old girl who initially presented at age 4 with evidence of arthritis in her hands, feet, and large joints. Although she had a partial response to anti-inflammatory medications and had some laboratory results consistent with inflammatory disease, radiographs showed carpal and tarsal osteolysis associated with interphalangeal joint erosions. There was also widening of the shafts of the metacarpals and metatarsals with thinning of the cortices. Based on both the clinical progression of her illness and the radiologic characteristics, this child most likely has the Torg syndrome.
Assuntos
Osteólise/congênito , Anticorpos Antinucleares/imunologia , Artrite Juvenil/diagnóstico por imagem , Artrite Juvenil/imunologia , Artrite Reumatoide , Criança , Feminino , Deformidades Congênitas do Pé/diagnóstico por imagem , Deformidades Congênitas da Mão/diagnóstico por imagem , Humanos , Interleucina-1/sangue , Interleucina-6/sangue , Osteólise/diagnóstico por imagem , Radiografia , SíndromeRESUMO
OBJECTIVE: To investigate whether changes in peripheral blood lymphocytes correlate with changes in disease activity in juvenile dermatomyositis (JDM). METHODS: Clinical changes in disease expression measured by a disease activity score were correlated with changes in percentages of peripheral blood lymphocyte subsets. RESULTS: Changes in the percentage of peripheral blood CD19 positive lymphocytes (B cells) correlated with changes in disease activity (Spearman rank coefficients = 0.47, p = 0.02). There were no significant correlations in disease activity with changes of T cell subsets or the T cell activation markers CD25 or DR. CONCLUSION: Change in the percentage of peripheral blood B cells correlates with change in disease activity in patients with JDM. This variable may be of use as an indicator of immunologic activity and response to therapy.