Evidence map›Paper›PMID 40581580›Full record

ArticleAnnals of the rheumatic diseases2026

Disease Trajectories and Glucocorticoid Exposure in VEXAS Syndrome Treated with Cytokine-Directed Therapies.

Benjamin A Turturice, Alice Fike, Bhavisha A Patel, Emma M Groarke, Fernanda Gutierrez-Rodriguez, Karyssa Stonick, Hannah Berrett, Shanni Liu, Ivana Darden, Kaitlin A Quinn and 2 more

Abstract read
In one paragraph

Article in Annals of the rheumatic diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
8citing papers in PubMed, 1 pooled it
–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

8 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Myelodysplasia in VEXAS syndrome.Blood advances · 2026
    Review
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors.

Benjamin A TurturiceNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Alice FikeNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Bhavisha A PatelNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Emma M GroarkeNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Fernanda Gutierrez-RodriguezNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Karyssa StonickNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Hannah BerrettNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Shanni LiuNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Ivana DardenNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Kaitlin A QuinnNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Neal S YoungNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Peter C GraysonNational Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. Electronic address: peter.grayson@nih.gov.

Funding

Vasculitis and Translational MedicineZIAAR041204 · NIAMS · NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES · PI GRAYSON, PETER · 2015 to 2025
$15.4M
Intramural NIH HHS Z99 AR999999Intramural NIH HHS ZIA AR041204
6 · The paper itself

Abstract

objectivesTo establish the long-term impact of cytokine-directed therapies on glucocorticoid use and clinical outcomes in Vacuoles, E1-enzyme, X-linked, Autoinflammatory, Somatic (VEXAS).

methodsPatients with VEXAS were prospectively followed for events of transfusion dependence, haematopoietic stem cell transplantation or death. Laboratory results, glucocorticoid exposure and clinical measures were retrospectively assessed in relationship to treatment initiation with interleukin-6-directed therapies (anti-IL6R) or Janus kinase inhibitors (JAKi). Patients were stratified by UBA1 variants and presence of typical clonal haematopoiesis with variant allele fraction ≥ 10% (CH

resultsIn 71 VEXAS patients (81.7% with anti-IL6R or JAKi exposure), event-free survival differed by genotype and presence of concomitant CH

conclusionsCytokine-directed therapies alone do not alter the risk of haematologic disease progression or significantly reduce glucocorticoid exposure in VEXAS. These data provide benchmarks for future interventional studies.

Indexed as

Genetic Diseases, X-LinkedGlucocorticoidsHereditary Autoinflammatory DiseasesJanus Kinase InhibitorsAdolescentAdultFemaleHumansInterleukin-6MaleMiddle AgedMyelodysplastic SyndromesProspective StudiesReceptors, Interleukin-6Retrospective StudiesSkin Diseases, GeneticGlucocorticoidsInterleukin-6Janus Kinase InhibitorsReceptors, Interleukin-6

Identifiers

PMID40581580
PMCPMC12276827

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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.