Immunoglobulin G4–related disease is a systemic fibroinflammatory disease; pericardial involvement has occasionally been reported in publications. A 79-year-old man with biopsy-proven immunoglobulin G4–related disease with pleural involvement was admitted in acute heart failure, with imaging and hemodynamic studies consistent with constrictive pericarditis. He was treated with corticosteroids for 2 months with partial response manifest by decreases in pericardial thickening and immunoglobulin G4 levels. However, persistent constriction required pericardiectomy, leading to significant symptomatic improvement. (Level of Difficulty: Intermediate.)
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