Search

Abstract YKL-40 increase according to the aging process, and its functions have been associated with tissue remodeling and systemic inflammation. In Rheumatoid Arthritis (RA) it has been proposed as a possible biomarker of activity and severity, however; in the field of idiopathic inflammatory myopathies (IIM) the role of YKL-40 in IIM is not clear. Thus, we aimed to evaluate if there is an association between the serum levels and muscle tissue expression of YKL-40 with age, IIM phenotype, muscle strength and myositis disease activity. The main finding was that age is the most important variable that affects the YKL-40 serum levels. In muscle biopsy, we observed that YKL-40 is mainly expressed in infiltrating lymphoid cells than in muscle tissue. Using ANCOVA according to the b-coefficients, YKL-40 serum levels are predicted by inflammatory state, age, and IIM diagnosis.

Abstract The muscle fiber ultrastructure in Idiopathic Inflammatory Myopathies (IIM) has been scarcely explored, especially in Inclusion Body Myositis. The aim of this study was to implement the Scanning Electron Microscopy (SEM) in a small cohort of IIM patients, together with the characterization of immunological profile for a better understanding of the pathophysiology. For immunological profile characterization, we identified the presence of autoantibodies (Ro-52, OJ, EJ, PL7, PL12, SRP, Jo-1, PMScl75, PMScl100, Ku, SAE1, NXP2, MDA5, TIF1γ, Mi-2α, Mi-2β) and quantified cytokines (IL-1β, IFN-α2, IFN-γ, TNF-α, IL-6, IL-10, IL-12p70, IL-17A, IL-18, IL-23, IL-33) and chemokines (CCL2, CXCL8). The histological analysis was made by hematoxylin–eosin staining while the muscle fiber ultrastructure was characterized by SEM. We observed changes in the morphology and structure of the muscle fiber according to muscle strength and muscle enzymes. We were able to find and describe muscle fiber ultrastructure with marked irregularities, porosities, disruption in the linearity and integrity of the fascicle, more evident in patients with increased serum levels of muscle enzymes and diminished muscle strength. Despite the scarce reports about the use of SEM as a tool in all clinical phenotypes of IIM, our work provides an excellent opportunity to discuss and reframe the clinical usefulness of SEM in the diagnostic approach of IIM.

Abstract Pericardial effusions requiring pericardiocentesis have multiple causes that vary among geographical regions and health contexts. This procedure can be performed for diagnostic or therapeutic indications. The purpose of this study was to identify the principal causes of pericardial effusions and indications for pericardiocentesis, exploring differences among groups. This was a retrospective case series of patients who underwent pericardiocentesis for pericardial effusion in a single center in Latin America. Demographic, clinical, echocardiographic, and procedural variables were recorded and analyzed. The primary outcome was to determine the causes of pericardial effusions in these patients and the indication (diagnostic, therapeutic, or both). The results are presented in two groups (inflammatory and noninflammatory) according to the cause of the pericardial effusion. One hundred sixteen patients with pericardial effusion underwent pericardiocentesis. The median age was 58 years (IQR 46.2–70.7), and 50% were male. In the noninflammatory pericardial effusion group, there were 61 cases (53%), among which neoplastic pericardial effusion was the most frequent cause (n = 25, 40.9%). In the inflammatory group, there were 55 cases (47%), and the main cause was postpericardiectomy syndrome after cardiac surgery (n = 31, 56.4%). In conclusion, the principal indication for pericardiocentesis was therapeutic (n = 66, 56.8%). Large pericardial effusion without hemodynamic effect of cardiac tamponade was significantly more frequent in the inflammatory group (p = 0.03). The principal cause of pericardial effusion in patients who underwent pericardiocentesis was postpericardiectomy syndrome after cardiac surgery, followed by neoplastic pericardial effusion. Pericardiocentesis is mainly a therapeutic procedure.

Abstract Acute lymphocytic leukemia is the most common type of cancer in pediatric individuals. Glucose regulated protein (GRP78) is an endoplasmic reticulum chaperone that facilitates the folding and assembly of proteins and regulates the unfolded protein response pathway. GRP78 has a role in survival of cancer and metastasis and cell-surface associated GRP78 (sGRP78) is expressed on cancer cells but not in normal cells. Here, we explored the presence of sGRP78 in pediatric B-ALL at diagnosis and investigated the correlation with bona fide markers of leukemia. By using a combination of flow cytometry and high multidimensional analysis, we found a distinctive cluster containing high levels of sGRP78, CD10, CD19, and CXCR4 in bone marrow samples obtained from High-risk leukemia patients, which was absent in the compartment of Standard-risk leukemia. We confirmed that sGRP78+CXCR4+ blood-derived cells were more frequent in High-risk leukemia patients. Finally, we analyzed the dissemination capacity of sGRP78 leukemia cells in a model of xenotransplantation. sGRP78+ cells emigrated to the bone marrow and lymph nodes, maintaining the expression of CXCR4. Testing the presence of sGRP78 and CXCR4 together with conventional markers may help to achieve a better categorization of High and Standard-risk pediatric leukemia at diagnosis.