Evidence map›Paper›PMID 38743491›Full record

ArticleJCI insight2024

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

Gabrielle Rabadam, Camilla Wibrand, Emily Flynn, George C Hartoularos, Yang Sun, Chioma Madubata, Gabriela K Fragiadakis, Chun Jimmie Ye, Susan Kim, Zev J Gartner and 2 more

Abstract read
In one paragraph

Article in JCI insight, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.

0numbers the graph read from it
0cells of the map it votes in
12citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

12 citing papers in PubMed.

  1. Review
  2. NMJ-associated transcriptomic remodeling correlates with disease severity in juvenile dermatomyositis.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026
    Article
  3. Article
  4. BATF3 regulates differentiation of CD8Life science alliance · 2026
    Article
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  6. Article
  7. Article
  8. Review
  9. Review
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

12 authors.

Gabrielle RabadamUC Berkeley-UC San Francisco Graduate Program in Bioengineering, and.
Camilla WibrandAarhus University, Aarhus, Denmark.
Emily FlynnCoLabs.
George C HartoularosGraduate Program in Biological and Medical Informatics.
Yang SunDivision of Rheumatology, Department of Medicine.
Chioma MadubataDivision of Pediatric Rheumatology, Department of Pediatrics.
Gabriela K FragiadakisCoLabs.
Chun Jimmie YeDivision of Rheumatology, Department of Medicine.
Susan KimDivision of Pediatric Rheumatology, Department of Pediatrics.
Zev J GartnerDepartment of Pharmaceutical Chemistry, UCSF, San Francisco, California, USA.
Marina SirotaBakar Computational Health Sciences Institute, UCSF, San Francisco, California, USA.
Jessica NeelyDivision of Pediatric Rheumatology, Department of Pediatrics.

Funding

UCSF/UCB Joint Graduate Group in BIoengineeringT32GM008155 · NIGMS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI DESAI, TEJAL A. · 1985 to 2020
$12.2M
RESOURCE-BASED CENTER FOR THE ADVANCEMENT OF PRECISION MEDICINE IN RHEUMATOLOGYP30AR070155 · NIAMS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Mary C Nakamura · 2016 to 2026
$8.2M
The Child Health Research Career Development Program at UCSFK12HD105250 · NICHD · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Raphael Hirsch · 2021 to 2026
$2.5M
MULTIseq: multiplexing massively parallel single cell transcriptional analysis across time, space, and conditionsR01GM135462 · NIGMS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GARTNER, ZEV JORDAN · 2019 to 2022
$1.3M
Universal Sample Multiplexing for Single Cell AnalysisR33CA247744 · NCI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GARTNER, ZEV JORDAN · 2021 to 2023
$1.2M
Harnessing single cell RNA sequencing and integrative bioinformatics to identify precision therapeutics for dermatomyositisK08AR081983 · NIAMS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Jessica Neely · 2023 to 2026
$699k
NCI NIH HHS R33 CA247744NIAMS NIH HHS K08 AR081983NIAMS NIH HHS P30 AR070155NICHD NIH HHS K12 HD105250NIGMS NIH HHS R01 GM135462NIGMS NIH HHS T32 GM008155
6 · The paper itself

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. We applied network analysis to reveal that hyperactivation of the type I IFN response in all immune populations is coordinated with previously masked cell states including dysfunctional protein processing in CD4+ T cells and regulation of cell death programming in NK cells, CD8+ T cells, and γδ T cells. Together, these findings unveil the coordinated immune dysregulation underpinning JDM and provide insight into strategies for restoring balance in immune function.

Indexed as

DermatomyositisSingle-Cell AnalysisAdolescentB-LymphocytesChildChild, PreschoolFemaleGenomicsHumansImmunophenotypingInterferon Type ILeukocytes, MononuclearMaleInterferon Type IAutoimmune diseasesAutoimmunityBioinformaticsRheumatology

Identifiers

PMID38743491
PMCPMC11383589

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.