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- Title
CD8 T Cells in Facioscapulohumeral Muscular Dystrophy Patients with Inflammatory Features at Muscle MRI.
- Authors
Frisullo, Giovanni; Frusciante, Roberto; Nociti, Viviana; Tasca, Giorgio; Renna, Rosaria; Iorio, Raffaele; Patanella, Agata; Iannaccone, Elisabetta; Marti, Alessandro; Rossi, Monica; Bianco, Assunta; Monforte, Mauro; Tonali, Pietro; Mirabella, Massimiliano; Batocchi, Anna; Ricci, Enzo
- Abstract
Facioscapulohumeral muscular dystrophy (FSHD) is an inherited disease, and although strongly suggested, a contribution of inflammation to its pathogenesis has never been demonstrated. In FSHD patients, we found by immunohistochemistry inflammatory infiltrates mainly composed by CD8 T cells in muscles showing hyperintensity features on T2-weighted short tau inversion recovery magnetic resonance imaging (T2-STIR-MRI) sequences. Therefore, we evaluated the presence of circulating activated immune cells and the production of cytokines in patients with or without muscles showing hyperintensity features on T2-STIR-MRI sequences and from controls. FSHD patients displaying hyperintensity features in one or more muscles showed higher CD8pSTAT1, CD8T-bet T cells and CD14pSTAT1, CD14T-bet cells percentages and IL12p40, IFNγ and TNFα levels than patients without muscles displaying hyperintense features and controls. Moreover, the percentages of CD8pSTAT1, CD8T-bet and CD14pSTAT1 cells correlated with the proportion of muscles displaying hyperintensity features at T2-STIR sequences. These data indicate that circulating activated immune cells, mainly CD8 T cells, may favour FSHD progression by promoting active phases of muscle inflammation.
- Subjects
FACIOSCAPULOHUMERAL muscular dystrophy; MUSCLES; T cells; GLYCOPROTEINS; INFLAMMATION; CYTOKINES; GENETIC disorders; IMMUNOHISTOCHEMISTRY; MAGNETIC resonance imaging
- Publication
Journal of Clinical Immunology, 2011, Vol 31, Issue 2, p155
- ISSN
0271-9142
- Publication type
Article
- DOI
10.1007/s10875-010-9474-6