Architectural B-cell organization in skeletal muscle identifies subtypes of dermatomyositis.

Abstract

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.

• Authors: Josefine Radke, Randi Koll, Corinna Preuße, Debora Pehl, Kremena Todorova, Constanze Schönemann, Yves Allenbach, Eleonora Aronica, Marianne de Visser, Frank L Heppner, Joachim Weis, Soroush Doostkam, Thierry Maisonobe, Olivier Benveniste, Hans-Hilmar Goebel, Werner Stenzel
• Journal: Neurology(R) neuroimmunology & neuroinflammation (Neurol Neuroimmunol Neuroinflamm) Vol. 5 Issue 3 Pg. e451 (May 2018) ISSN: 2332-7812 [Print] United States
• PMID: 29520367 (Publication Type: Journal Article)