Unmasking inflammation in juvenile dermatomyositis: myokine profiles of patients and bioengineered human muscle.

ABSTRACT:

Introduction

Juvenile dermatomyositis (JDM), a rare autoimmune disease characterized by a type I interferon (IFN) gene signature and muscle weakness, lacks robust biomarkers and disease models. Myokines-muscle-derived cytokines-are potential biomarkers and therapeutic targets that may clarify muscle's role in JDM. We characterized myokine profiles in treatment-naïve JDM patients and compared them to an IFN-stimulated human tissue-engineered muscle model (myobundles) to identify biomarkers and validate the model.

Methods

Myobundles from four healthy pediatric donors were treated with IFNα, IFNβ, or IFN-stimulant poly(I:C). Sera from treatment-naïve JDM patients (n=21) and controls (n=9) were analyzed. A myokine panel (e.g., IL-6, IL-8, IL-17A, IL-18, CXCL9, CXCL10, TNFα, RANT

SUBMITTER: Covert LT 

PROVIDER: S-EPMC12756377 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature