Increased Soluble Cytoplasmic Bcl-2 Protein Serum Levels and Expression and Decreased Fas Expression in Lymphocytes and Monocytes in Juvenile Dermatomyositis.

Objective: To evaluate soluble Fas antigen (sFas), sFas ligand (sFasL), soluble tumor necrosis factor-related apoptosis-inducing ligand, and soluble cytoplasmic Bcl-2 protein (sBcl-2) serum levels, Fas and Bcl-2 expressions in T and B lymphocytes and monocytes and relations with erythrocyte sedimentation rate, C-reactive protein (CRP), Childhood Myositis Assessment Scale, and manual muscle testing in juvenile dermatomyositis (JDM).

Methods: Serum levels were determined by ELISA and peripheral cell expressions by flow cytometry for patients with JDM or juvenile idiopathic arthritis (JIA), and healthy controls.

Results: Patients with JDM had increased sBcl-2, which correlated with CRP.

Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus.

The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA.

Autoinflammatory diseases: mimics of autoimmunity or part of its spectrum? Case presentation.

Introduction: Autoinflammatory diseases are very rare diseases presenting within a wide clinical spectrum. Recognition of the main clinical features are challenging due to overlapping or mimicking with autoimmune diseases.

Discussion: A case series is reviewed to illustrate typical and atypical features and the difficulties of these diagnoses in the low prevalence areas--a typical unrecognized case of familial Mediterranean fever (FMF) in a youngster, an atypical adult case with overlapping of FMF with Behçet disease, and an early presentation of FMF in infant presenting with inflammatory colitis, as well as the overlapping features within the cryopirin diseases spectrum in an 8-year-old boy who presented with systemic onset arthritis.

[Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis].

Objective: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis.

Description: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.

Nitric oxide-derived species in synovial fluid from patients with juvenile idiopathic arthritis.

Objective: To evaluate superoxide anion (O2-), nitrite/nitrate (NO2-/NO3-), and nitrotyrosine (NT) production and the contribution of myeloperoxidase (MPO) to the production of NT-containing proteins in the synovial fluid (SF) of patients with juvenile idiopathic arthritis (JIA). The affected tissues in inflammatory arthritis produce large amounts of nitric oxide (NO) or peroxynitrite (ONOO-) but there are no reports of NO or ONOO- participation in JIA. We also attempted to correlate our findings with variables of disease activity and articular damage.

Inter- and intrareader variability in the interpretation of two radiographic classification systems for juvenile rheumatoid arthritis.

Objective: To evaluate the inter- and intrareader variability for interpretation of a modified Larsen's radiographic classification system for juvenile rheumatoid arthritis (JRA) focused on osteochondral lesions and a conventional Larsen's classification system, compared to a reference MR scoring system of corresponding images.

Materials And Methods: Seventy-five radiographs of 60 children with JRA, performed within a short interval of time from the MR examinations, were independently evaluated by three experienced radiologists, three diagnostic imaging residents and three rheumatologists, in two separate sessions, according to the two different classification methods, blinded to the corresponding MR images.

Results: The inter- and intrareader concordance rates between the two radiographic classification systems and the MR-related radiographs were respectively poor and poor/moderate.

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus.

Objective: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus.

Methods: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.

Analysis of human leukocyte antigens class II-DR in Brazilian children and adolescents with systemic lupus erythematosus.

Objective: To analyze the frequency of human leukocyte antigens class II-DR in children and adolescents with systemic lupus erythematosus.

Patients And Methods: Fifty-fiveBrazilian systemic lupus erythematosus children and adolescents and 308 healthy individuals were studied. Gender, race, and age of onset of systemic lupus erythematosus were recorded.

Immunohistological analysis of CD59 and membrane attack complex of complement in muscle in juvenile dermatomyositis.

Objective: To assess the presence of CD59 and the deposition of membrane attack complex (MAC) of complement system in skeletal muscle from patients with juvenile dermatomyositis (JDM), in comparison to patients with muscular dystrophies (MD) and children with normal muscle biopsies.

Methods: Muscle specimens obtained for diagnostic purposes from 10 patients with JDM, 6 with MD, and 7 children whose biopsies showed normal histology were analyzed. Immunohistological staining was performed using Mab against CD59 (YTH 53.