Coordinated immune dysregulation in juvenile dermatomyositis revealed by single-cell genomics.

ABSTRACT: Juvenile dermatomyositis (JDM) is one of several childhood-onset autoimmune disorders characterized by a type I IFN response and autoantibodies. Treatment options are limited due to an incomplete understanding of how the disease emerges from dysregulated cell states across the immune system. We therefore investigated the blood of patients with JDM at different stages of disease activity using single-cell transcriptomics paired with surface protein expression. By immunophenotyping peripheral blood mononuclear cells, we observed skewing of the B cell compartment toward an immature naive state as a hallmark of JDM at diagnosis. Furthermore, we find that these changes in B cells are paralleled by T cell signatures suggestive of Th2-mediated inflammation that persist despite disease quiescence.

SUBMITTER: Rabadam G 

PROVIDER: S-EPMC11383589 | biostudies-literature | 2024 May

REPOSITORIES: biostudies-literature