Evidence map›Paper›PMID 41746515›Full record

ArticleJournal of clinical immunology2026

Fatal Systemic Granulomatous Disease Associated with Vaccine-Derived Rubella Virus in AIOLOS-Associated Immunodeficiency.

Linda Zhou, Hye Sun Kuehn, Dayna Gager, Agustin A Gil Silva, Ludmila M Perelygina, LiJuan Hao, Min-Hsin Chen, Julie E Niemela, Jennifer L Stoddard, Matthew Helm and 6 more

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PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of clinical immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

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

16 authors.

Linda ZhouDepartment of Dermatology, University of Pennsylvania, Philadelphia, PA, USA.
Hye Sun KuehnImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Dayna GagerDepartment of Dermatology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.
Agustin A Gil SilvaImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Ludmila M PerelyginaImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
LiJuan HaoImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Min-Hsin ChenImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Julie E NiemelaImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Jennifer L StoddardImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Matthew HelmDepartment of Dermatology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.
Karolyn WanatDepartment of Dermatology, Medical College of Wisconsin, Milwaukee, WI, USA.
Kathleen SullivanDivision of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Galen FoulkeDepartment of Dermatology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.
Thomas H LeungDepartment of Dermatology, University of Pennsylvania, Philadelphia, PA, USA.
Sergio D RosenzweigImmunology Service, Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, USA.
Misha RosenbachDepartment of Dermatology, University of Pennsylvania, Philadelphia, PA, USA. Misha.rosenbach@pennmedicine.upenn.edu.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

We present a case of disseminated granulomatous disease associated with live attenuated vaccine-derived rubella virus (VDRV) in an immunodeficient patient who subsequently developed fatal multi-organ failure, including neurological deficits, with VDRV detected in the cerebrospinal fluid. Posthumous genetic analysis found a heterozygous missense mutation in IKZF3, a gene where haploinsufficiency is linked to immunodeficiency and immune dysregulation. Functional studies demonstrated that this mutation decreases AIOLOS protein stability and half-life, and family members carrying the same mutation exhibited decreased AIOLOS levels. This report underscores the importance of considering persistent rubella virus (RuV) infection in patients with cutaneous granulomatous lesions and highlights the need for comprehensive evaluation to uncover potential underlying immunodeficiencies, which could inform and optimize individualized treatment strategies.

Indexed as

Immunologic Deficiency SyndromesRubellaRubella VaccineRubella virusFatal OutcomeGranulomaHumansVaccines, AttenuatedRubella VaccineVaccines, AttenuatedAIOLOSGranulomaImmunodeficiencyImmunosuppressionRubella

Identifiers

PMID41746515
PMCPMC12975807

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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.