Abstract

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by proximal muscle weakness and cutaneous manifestations, typically heliotrope rash and Gottron's papules. Previous studies have identified an increase in circulating B cells in JDM patients, but their provenance and functional characteristics have not been examined. In this study we investigated whether a recently identified immature B cell subset (CD24hiCD38hi) with known regulatory function (Breg) was enriched in JDM and correlated with disease outcome measures.

Highlights

  • Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by proximal muscle weakness and cutaneous manifestations, typically heliotrope rash and Gottron’s papules

  • Ex-vivo analysis of pre-treatment JDM peripheral blood mononuclear cells (PBMC) displayed a significant increase in CD19+ B cells when compared to post-Rx JDM samples

  • The immature B cell compartment was expanded in pre-Rx JDM patients

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Summary

Introduction

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by proximal muscle weakness and cutaneous manifestations, typically heliotrope rash and Gottron’s papules. Previous studies have identified an increase in circulating B cells in JDM patients, but their provenance and functional characteristics have not been examined. In this study we investigated whether a recently identified immature B cell subset (CD24hiCD38hi) with known regulatory function (Breg) was enriched in JDM and correlated with disease outcome measures

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