RT Journal Article
SR Electronic
T1 Architectural B-cell organization in skeletal muscle identifies subtypes of dermatomyositis
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e451
DO 10.1212/NXI.0000000000000451
VO 5
IS 3
A1 Josefine Radke
A1 Randi Koll
A1 Corinna Preuße
A1 Debora Pehl
A1 Kremena Todorova
A1 Constanze Schönemann
A1 Yves Allenbach
A1 Eleonora Aronica
A1 Marianne de Visser
A1 Frank L. Heppner
A1 Joachim Weis
A1 Soroush Doostkam
A1 Thierry Maisonobe
A1 Olivier Benveniste
A1 Hans-Hilmar Goebel
A1 Werner Stenzel
YR 2018
UL http://nn.neurology.org/content/5/3/e451.abstract
AB Objective To study the B-cell content, organization, and existence of distinct B-cell subpopulations in relation to the expression of type 1 interferon signature related genes in dermatomyositis (DM).Methods Evaluation of skeletal muscle biopsies from patients with adult DM (aDM) and juvenile DM (jDM) by histology, immunohistochemistry, electron microscopy, and quantitative reverse-transcription PCR.Results We defined 3 aDM subgroups—classic (containing occasional B cells without clusters), B-cell–rich, and follicle-like aDM—further elucidating IM B-lymphocyte maturation and immunity. The quantity of B cells and formation of ectopic lymphoid structures in a subset of patients with aDM were associated with a specific profile of cytokines and chemokines involved in lymphoid neogenesis. Levels of type 1 interferon signature related gene expression paralleled B-cell content and architectural organization and link B-cell immunity to the interferon type I signature.Conclusion These data corroborate the important role of B cells in DM, highlighting the direct link between humoral mechanisms as key players in B-cell immunity and the role of type I interferon–related immunity.aDM=adult dermatomyositis; CK=creatine kinase; DM=dermatomyositis; ELS=ectopic lymphoid structure; EM=electron microscopy; ENMC=European Neuromuscular Centre; HEV=high endothelial venule; IFN=interferon; jDM=juvenile dermatomyositis; MHC=major histocompatibility complex; mRNA=messenger RNA; MxA=myxovirus resistance gene A; qPCR=quantitative PCR; RIN=RNA integrity number; TRI=tubuloreticular inclusion; VAS=visual analog scale