From the Research
Cardiac Manifestations of IgG4-Related Disease
The most common cardiac manifestations of Immunoglobulin G4 (IgG4)-related disease include:
- Coronary artery disease, such as coronary artery stenosis and coronary arteritis 1, 2, 3
- Pericardial disease, including pericarditis and pericardial constriction 1, 2, 4, 5
- Cardiac masses and valvular heart disease, although these are less frequently reported 1, 2
Incidence of Cardiac Manifestations
The incidence of coronary periarteritis and pericarditis in IgG4-related disease has been reported as 1.2% and 3.1%, respectively 2. Cardiac involvement in IgG4-related disease is considered rare and may be easily overlooked, as many patients are asymptomatic and diagnosis relies on imaging 2, 4, 5.
Diagnosis and Treatment
Diagnosis of IgG4-related cardiac disease often involves assessment of serum IgG4 levels, organ biopsy, and imaging techniques such as echocardiography, computed tomography (CT), and cardiovascular magnetic resonance (CMR) 4, 5. Treatment typically includes glucocorticoids and immunosuppressants, with patients showing a satisfactory response to treatment 2. Early diagnosis and treatment are crucial to prevent potentially fatal outcomes and irreversible fibrosis 1.