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Severe Aortic Valvular Incompetence From IgG4-Related Disease: An Unusual Entity.
Bing Wei Thaddeus Soh1, Sophie Catherine Prendergast2, Louise Burke2
1Department of Cardiology, University Hospital Limerick, Limerick, Ireland.
JACC. Case Reports
|October 23, 2023
Summary
IgG4-related disease (IgG4-RD) rarely affects the heart. This case highlights IgG4-RD-associated myocardial mass causing aortic valve issues, successfully treated with surgery and steroids.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition.
- Cardiac involvement in IgG4-RD is exceptionally uncommon.
- Typical IgG4-RD manifestations involve organs like the pancreas, salivary glands, and lymph nodes.
Observation:
- A rare case of IgG4-related disease presenting as a myocardial mass is described.
- The myocardial mass led to severe aortic incompetence.
- This presentation is atypical for IgG4-related disease.
Findings:
- The myocardial mass was confirmed to be associated with IgG4-related disease.
- The patient experienced severe aortic valve dysfunction due to the mass.
- Histopathological examination revealed characteristic IgG4-positive plasma cell infiltration.
Implications:
- This case expands the spectrum of cardiovascular manifestations of IgG4-RD.
- Early diagnosis and intervention are crucial for managing IgG4-RD cardiac complications.
- Combined surgical and corticosteroid therapy can be effective for IgG4-RD-associated myocardial masses.
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