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Scurfy Mice Develop Features of Connective Tissue Disease Overlap Syndrome and Mixed Connective Tissue Disease in the Absence of Regulatory T Cells
Discussion Treg represent a lineage of T cells which play a fundamental role in maintaining humoral tolerance in the periphery. This subset of “suppressor T cells” is identified as FoxP3-expressing CD4+ T cells (16, 17). The unrestrained expression of FoxP3 is essential for the development and function of Treg (4). Accordingly, a disruption of the Foxp3 gene in scurfy mice results in an autoimmune lymphoproliferative disorder with fatal multi-organ inflammation (18). Since the causative mutation occurs in orthologous genes, the scurfy phenotype is indicated as the murine equivalent of the human IPEX sy...
Source: Frontiers in Immunology - April 23, 2019 Category: Allergy & Immunology Source Type: research

Anti-NT5c1A Autoantibodies as Biomarkers in Inclusion Body Myositis
Conclusions: Anti-NT5c1A has moderate sensitivity and high specificity for sIBM using ALBIA. The presence of anti-NT5c1A antibodies may be associated with muscle weakness. Anti-NT5c1A antibodies were not associated with a specific IIF staining pattern, hence screening using HEp-2 substrate is unlikely to be a useful predictor for presence of these autoantibodies. Introduction Sporadic Inclusion Body Myositis (sIBM) is one subset of idiopathic inflammatory myopathies (IIM) that is characterized by a clinical presentation of asymmetrical muscle involvement, predominantly affecting the long finger flexors, quadriceps mu...
Source: Frontiers in Immunology - April 8, 2019 Category: Allergy & Immunology Source Type: research

18F-FDG PET/CT versus conventional investigations for cancer screening in autoimmune inflammatory myopathy in the era of novel myopathy classifications
Conclusion 18F-FDG PET/CT does not appear to be useful in cancer screening for AIM patients compared with conventional screening and carries potential harms associated with follow-up investigations. The risk of cancer in AIM differs by myositis-specific antibodies-defined subsets and cancer screening is likely to be indicated only in high-risk patients, particularly DM. These results, replicated in larger, multicentered studies, may carry significant consequences for optimal management of AIM and health resource utilization.
Source: Nuclear Medicine Communications - March 16, 2019 Category: Nuclear Medicine Tags: ORIGINAL ARTICLES Source Type: research

Extensive interstitial lung disease in inflammatory myopathy is a strong predictor of mortality
The objective of this study was to identify clinical predictors of mortality in a prospective cohort of subjects with IIM.Subjects were from the Canadian Inflammatory Myopathy Study group, a multicenter, prospective cohort of incident IIM. Clinical data, lung function were obtained per study protocol. Computed tomography scans (CT) of the chest done for clinical indications were reviewed by a respirologist for presence of ILD. Total lung involvement was estimated to the nearest 5%. Kaplan-Meier was used to identify survival differences between groups with and without ILD. Cox proportional hazard model identified predictors...
Source: European Respiratory Journal - December 6, 2017 Category: Respiratory Medicine Authors: Assayag, D., Hirsch, A., Baron, M., Vinet, E., Albert, A., Fortin, P., Hudson, M. Tags: Diffuse Parenchymal Lung Disease Source Type: research