Evidence map›Paper›PMID 40816861›Full record

ArticleJACC. Case reports2025

Desmoplakin Cardiomyopathy Presenting With Recurrent Myopericarditis Responsive to Interleukin-1 Blockade.

Rocio I Lopez, Michele Marchetta, Michael Ayers, Pamela Mason, Matthew Thomas, Alessio Gasperetti, Nisha A Gilotra, Antonio Abbate

Abstract readCase Reports
In one paragraph

Article in JACC. Case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing 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

3 citing papers in PubMed.

  1. Trial
  2. Review
  3. 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

8 authors.

Rocio I LopezDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA.
Michele MarchettaDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA.
Michael AyersDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA.
Pamela MasonDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA.
Matthew ThomasDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA.
Alessio GasperettiDivision of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
Nisha A GilotraDivision of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
Antonio AbbateDepartment of Medicine, Robert M. Berne Cardiovascular Research Center, University of Virginia, Charlottesville, Virginia, USA. Electronic address: antonio.abbate@virginia.edu.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Acute myopericarditis in patients with pathogenic desmoplakin (DSP) variants are associated with a high risk for heart failure and cardiac arrhythmias. We present the case of a 25-year-old woman who presented with recurrent myopericarditis with chest pain and elevated troponin I levels despite escalating prednisone therapy. Genetic testing revealed a pathogenic variant in DSP along with a pathogenic variant in KCNQ1 associated with long-QT syndrome and a NOD2 variant associated with an increased risk for Crohn disease. A subcutaneous implantable cardioverter-defibrillator was implanted. The patient was started on the interleukin-1 inhibitor rilonacept, resulting in a significant reduction in symptoms and troponin I levels and the ability to wean off prednisone. Nine months after initiating rilonacept, the patient remained asymptomatic, had been off prednisone for 8 months, and demonstrated improved exercise tolerance and normalized troponin I levels. This case highlights the potential benefit of interleukin-1 blockade in managing DSP-related recurrent myopericarditis.

Indexed as

desmoplakininterleukin-1myopericarditis

Identifiers

PMID40816861
PMCPMC12461882

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.