Background: Immunoglobulin G4 (IgG4)-related disease is increasingly recognized as a systemic autoimmune disorder characterized by increasing level of serum IgG4 and IgG4-positive lymphocyte infiltration. However, there are a few case reports of IgG4 related pericarditis, and clinical course is still unclear. Case Presentation: A 60's-year-old male presented with progressive exertional dyspnea, systemic edema, and pericardial effusion. Cardiac CT showed circumferential pericardial effusion and thickened pericardium. His serum IgG4 levels were mildly increased (174 mg/dl). Cardiac catheterization revealed that both ventricular pressure traces showed an early diastolic dip and plateau. Constrictive pericarditis was diagnosed. A surgical pericardiectomy was performed and the resultant specimen showed significant IgG4-positive plasma cell infiltration and marked fibrous thickening of his pericardium; therefore, a diagnosis of constrictive pericarditis due to IgG4-related disease was made. And also, oral administration of 0.6-mg/kg/day prednisolone resolved his heart failure. Conclusion: Our experience with this case indicates that cardiac CT was useful and supports the diagnosis of IgG4-related pericarditis, which was consistent with the findings of effusive constrictive pericarditis.
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