Journal of Clinical Immunology

, Volume 31, Issue 2, pp 155–166

CD8+ T Cells in Facioscapulohumeral Muscular Dystrophy Patients with Inflammatory Features at Muscle MRI

Authors

  • Giovanni Frisullo
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Roberto Frusciante
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Viviana Nociti
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
  • Giorgio Tasca
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Rosaria Renna
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Raffaele Iorio
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Agata Katia Patanella
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
  • Elisabetta Iannaccone
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Alessandro Marti
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Monica Rossi
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
  • Assunta Bianco
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Mauro Monforte
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Pietro Attilio Tonali
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
  • Massimiliano Mirabella
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
  • Enzo Ricci
    • Institute of Neurology, Department of NeurosciencesCatholic University of Rome
    • Fondazione Don Carlo Gnocchi
Article

DOI: 10.1007/s10875-010-9474-6

Cite this article as:
Frisullo, G., Frusciante, R., Nociti, V. et al. J Clin Immunol (2011) 31: 155. doi:10.1007/s10875-010-9474-6

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 CD8+pSTAT1+, CD8+T-bet+ T cells and CD14+pSTAT1+, CD14+T-bet+ cells percentages and IL12p40, IFNγ and TNFα levels than patients without muscles displaying hyperintense features and controls. Moreover, the percentages of CD8+pSTAT1+, CD8+T-bet+ and CD14+pSTAT1+ 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.

Keywords

T-betpSTAT1cytokinesFSHDmuscle MRICD8 T cellsinflammation

Copyright information

© Springer Science+Business Media, LLC 2010