Unveiling the Mystery of IgG4-Related Disease: Aortic Regurgitation and Ventricular Tachycardia
A rare case of IgG4-related disease (IgG4-RD) presenting as aortic regurgitation and ventricular tachycardia is reported, highlighting the importance of considering IgG4-RD in patients with cardiac manifestations.
Introduction
IgG4-RD is a complex immune disorder causing inflammation and fibrosis in various organs. While it typically affects the meninges, orbits, and glands, its impact on the cardiovascular system is less common. This case study focuses on a 68-year-old woman who developed aortic regurgitation and ventricular tachycardia due to IgG4-RD, a rare occurrence.
Case Presentation
The patient sought medical attention for chronic chest discomfort and was diagnosed with severe aortic regurgitation. Despite normal laboratory findings, echocardiography revealed a decreased left ventricular ejection fraction and an abnormal aortic valve. Interestingly, she had previously suffered from severe headaches and was treated for suspected autoimmune encephalitis, with elevated IgG and IgG4 levels in the cerebrospinal fluid.
Surgical Intervention and Diagnosis
The patient underwent aortic valve replacement, and histopathology confirmed IgG4-RD with fibromyxoid degeneration and IgG4-positive plasma cells. This diagnosis was further supported by the patient’s history of IgG4-related encephalitis and elevated IgG levels in the cerebrospinal fluid.
Postoperative Course and Management
After surgery, the patient experienced ventricular tachycardia, requiring an implantable cardioverter defibrillator and radiofrequency catheter ablation. Additional immunosuppressive therapy was initiated, and the patient’s condition stabilized. The case emphasizes the need to consider IgG4-RD in patients with valvular disease or arrhythmia, especially with a history of IgG4-RD.
Discussion
IgG4-RD can lead to valvulopathy, as demonstrated by Yamauchi et al. in 2013. However, the diagnosis requires specific histopathological features, including dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. In this case, the patient’s valvular tissue characteristics may have masked these features, making diagnosis challenging.
Implications and Future Considerations
Uncontrolled IgG4-related valvulitis can cause prosthetic valve failure. Regular echocardiographic monitoring and inflammation control are essential to prevent this complication. Additionally, the patient’s ventricular tachycardia suggests myocardial involvement, which can lead to cardiomyopathy. This case underscores the importance of thorough cardiac evaluation and systemic inflammation management in IgG4-RD patients with cardiac manifestations.
Conclusion
This report presents the first case of concurrent valvulopathy and ventricular arrhythmia associated with IgG4-RD. As IgG4-RD can affect multiple organs, it should be considered in patients with cardiac symptoms, especially those with a history of IgG4-RD. The case highlights the need for comprehensive cardiac evaluation and careful perioperative management of systemic inflammation.
But here’s where it gets controversial…
Could IgG4-RD be an underrecognized cause of cardiac issues? How should clinicians approach the diagnosis and management of IgG4-RD-related cardiac complications? Share your thoughts in the comments!