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Cardiac IgG4 Related Disorder Post Mortem Study; A Case Report
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Hamid Reza Eftekhari1    , Mohammad Tofighifard2 , Mehrdad Setareh2 |
1- General Department of Forensic Medicine of East Azerbaijan Province, Tabriz, Iran , dr.hr.eftekhari@gmail.com
2- General Department of Forensic Medicine of East Azerbaijan Province, Tabriz, Iran |
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Abstract: (56 Views) |
Introduction: Various forms of vasculitis can lead to myocardial ischemia by affecting coronary arteries. IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that impacts multiple organs and causes vascular involvement in 15–25% of affected individuals.
Case Report: A 40-year-old man with no known prior medical history was referred to forensic medicine for determination of the cause of death. Autopsy revealed a dense, round lesion measuring 2.5 cm within the heart, with an occlusive thrombus in the right coronary artery. Microscopic examination demonstrated connective tissue proliferation with a storiform pattern and lymphoplasmacytic infiltration, without evidence of obliterative phlebitis. Immunohistochemical analysis (IHC) confirmed strong IgG4 positivity in plasma cells, while ALK staining was negative, ruling out inflammatory myofibroblastic tumor as a differential diagnosis. IgG4-RD can involve various organs, including the heart, and should be considered in the differential diagnosis of tumor-like lesions.
Conclusion: Reviewing patient medical history, assessing serum IgG4 levels, and confirming lesions via IHC can prevent unnecessary surgeries, as these lesions typically show a favorable response to steroid therapy. In forensic medicine, identifying IgG4-RD-related lesions in coronary arteries is crucial. Additionally, raising awareness among cardiologists, especially echocardiography specialists, may play a significant role in preventing sudden cardiac death associated with this disease. |
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| Keywords: Immunoglobulin G4-related disease, Death, Sudden, Cardiac |
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Type of Article: Case Report |
Subject:
Forensic Pathology
Received: 2025/01/11 | Revised: 2025/07/7 | Accepted: 2025/07/1
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