Evidence map›Paper›PMID 39119941›Full record

ArticleClinical and experimental immunology2024

Unexplained fever with consumptive syndrome in the elderly: two cases of VEXAS syndrome with inflammasome dysregulation.

Leonardo Oliveira Mendonça, Vinicius N C Leal, Mariela E G V Roa, Samar Freschi Barros, Jorge Kalil, Alessandra Pontillo

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Clinical and experimental immunology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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.

Leonardo Oliveira MendonçaDivisão de Imunologia Clínica e Alergia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, Brazil.
Vinicius N C LealLaboratório de Imunogenética, Departamento de Imunologia, Instituto de Ciências Biomédicas (ICB), Universidade de São Paulo (USP), São Paulo, Brazil.
Mariela E G V RoaLaboratório de Imunogenética, Departamento de Imunologia, Instituto de Ciências Biomédicas (ICB), Universidade de São Paulo (USP), São Paulo, Brazil.
Samar Freschi BarrosLaboratório de Investigação Médica (LIM)-19, Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, Brazil.
Jorge KalilDivisão de Imunologia Clínica e Alergia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, Brazil.
Alessandra PontilloLaboratório de Imunogenética, Departamento de Imunologia, Instituto de Ciências Biomédicas (ICB), Universidade de São Paulo (USP), São Paulo, Brazil.ORCID 0000-0002-7883-0640

Funding

São Paulo Research Foundation 2021/05420-4
6 · The paper itself

Abstract

The aim of this study is to investigate the inflammasome dysregulation in peripheral blood leukocytes of VEXAS patients. The constitutive and in vitro triggered activation of inflammasome in PBMC and neutrophils was analyzed in two Brazilian patients with typical UBA1 mutations, and compared with healthy donors. Our findings highlight the constitutive activation of caspase-1 in VEXAS leukocytes, accompanied by increased plasma levels of IL-18. Furthermore, upon stimulation of isolated peripheral blood mononuclear cells (PBMC) and neutrophils, we observed not only the exhaustion of NLRP3 and NLRP1/CARD8 pathways in VEXAS PBMC but also a significant increase in NLRP3-mediated NETs release in VEXAS neutrophils. These findings support previous studies on the contribution of the inflammasome to VEXAS pathogenesis, identifying at least two profoundly affected pathways (NLRP3 and NLRP1/CARD8) in VEXAS peripheral blood.

Indexed as

CARD Signaling Adaptor ProteinsInflammasomesInterleukin-18Leukocytes, MononuclearNeutrophilsNLR Family, Pyrin Domain-Containing 3 ProteinNLR ProteinsAdaptor Proteins, Signal TransducingAgedApoptosis Regulatory ProteinsBrazilCaspase 1FemaleFeverHumansMaleAdaptor Proteins, Signal TransducingApoptosis Regulatory ProteinsCARD8 protein, humanCARD Signaling Adaptor ProteinsCaspase 1IL18 protein, humanInflammasomesInterleukin-18Neoplasm ProteinsNLR Family, Pyrin Domain-Containing 3 ProteinNLRP1 protein, humanNLRP3 protein, humanNLR ProteinsUBA1 protein, humanUbiquitin-Activating EnzymesCARD8inflammasomeNETsNLRP1NLRP3UBA1VEXAS

Identifiers

PMID39119941
PMCPMC11482498

What OpenQuestion holds

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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.