Severe Cardiac Involvement in a Young Woman with Mixed Connective Tissue Disease Complicated by Macrophage Activation Syndrome

Clicks: 4
ID: 320082
2026
Free PDF
Article Quality & Performance Metrics
Overall Quality
Not rated
Combines reader engagement with the AI quality analysis. This article has not been analysed, so there is no overall score — reader engagement is measured and shown alongside.
AI Quality Assessment
Not analyzed
Readership in this journal

Ranked #2 of 44 articles by views in Modern Rheumatology Case Reports

Most read Least read

Bar heights use a square-root scale.

Mint this article as an NFT
Not yet minted

Create a permanent, verifiable on-chain record of this article on the Scimatic Network. The NFT is held in your Journament account, and you can withdraw it to your own wallet at any time.

5 SUSD one-off · no wallet required
Sign in to mint
Abstract
Reports of severe mixed connective tissue disease (MCTD) or macrophage activation syndrome (MAS)-associated cardiac involvement are limited. A 37-year-old woman with MCTD was transferred to a local hospital with fever and headache. She was diagnosed with meningitis and treated with glucocorticoid (GC) pulse therapy (methylprednisolone 1,000 mg/day for 3 days), followed by GC tapering. Despite a normal left ventricular ejection fraction on admission, her left ventricular ejection fraction abruptly declined to 7%, and she subsequently developed cardiogenic shock. She then experienced sudden cardiac arrest, which required initiation of veno-arterial extracorporeal membrane oxygenation and insertion of an Impella CP. Intravenous cyclophosphamide (1,000 mg) was administered for suspected myocarditis, although endomyocardial biopsy showed no specific positive staining. Cardiac function did not improve after immunosuppressive therapy. Therefore, plasma exchange was performed five times considering for MAS, which resulted in recovery of the left ventricular ejection fraction to 71%. Veno-arterial extracorporeal membrane oxygenation was successfully ceased, followed by veno-venous extracorporeal membrane oxygenation and mechanical ventilation to facilitate respiratory recovery for 39 days. The etiology of the myocardial dysfunction remains unclear; however, both cytokine-mediated myocardial dysfunction associated with MAS and immune-mediated microvascular injury could not be excluded. We successfully managed with combined immunosuppressive therapy, including GC, intravenous cyclophosphamide, and plasma exchange, under mechanical circulatory support.
Reference Key
openalex_W7167735774 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Kosuke Muto, Michiru Nomoto, Yasuto Araki, Yu Miyama, T Arai, Shintaro Nakano
Journal Modern Rheumatology Case Reports
Year 2026
DOI
10.1093/mrcr/rxag061
URL
https://doi.org/10.1093/mrcr/rxag061
Keywords Keywords not found

Citations

No citations found. To add a citation, contact the admin at info@scimatic.org

Comments

No comments yet. Be the first to comment on this article.