Evidence map›Paper›PMID 39549055›Full record

ArticleAnnals of hematology2024

Case report: VEXAS syndrome with excellent response to treatment with azacitidine.

Tzvika Porges, Elli Rosenberg, Ofir Wolach, Iftach Sagy, Yehonatan Sherf, Itai Levi

Abstract readCase Reports
PubMed Publisher
In one paragraph

Article in Annals of hematology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing 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

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

  1. Pooled it
  2. Review
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

6 authors.

Tzvika PorgesHematology Department, Soroka University Medical Center, Yitzhack Rager Blvd 151, Beer Sheva, 84101, Israel. tzvipo@clalit.org.il.
Elli RosenbergClinical Immunology Clinic, Soroka University Medical Center, Beer Sheva, Israel.
Ofir WolachHematology Institute, Davidoff Cancer Center, Rabin Medical Center and Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Iftach SagyRheumatology Unit, Soroka University Medical Center, Beer Sheva, Israel.
Yehonatan SherfHematology Department, Soroka University Medical Center, Yitzhack Rager Blvd 151, Beer Sheva, 84101, Israel.
Itai LeviHematology Department, Soroka University Medical Center, Yitzhack Rager Blvd 151, Beer Sheva, 84101, Israel.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Vacuoles, E1 enzyme, X-linked, auto inflammatory, somatic (VEXAS) syndrome is an inflammatory disorder caused by somatic UBA1 variants and is characterized by late-onset systemic autoimmune inflammation and blood abnormalities. Glucocorticoids ameliorate symptoms effectively. However, other treatment options have limited efficacy and a transient effect. Herein, we describe a case of a 69-year-old male patient with VEXAS syndrome with skin, lung and hematologic involvement. He was treated with glucocorticoids and after the failure with anti IL-1 he began treatment with azacitidine with excellent hematological and clinical response. Azacitidine may be a suitable option for treating VEXAS syndrome, especially due to the relationship between inflammatory symptoms and response to azacitidine.

Indexed as

AzacitidineUbiquitin-Activating EnzymesAgedAutoimmune DiseasesGenetic Diseases, X-LinkedGlucocorticoidsHumansMaleTreatment OutcomeAzacitidineGlucocorticoidsUBA1 protein, humanUbiquitin-Activating EnzymesAzacitidineInflammatory syndromeMyelodysplastic syndromeVEXAS syndrome

Identifiers

PMID39549055

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.