Constrictive Pericarditis Caused by IgG4-Related Disease Requiring Pericardiectomy After Partial Response to Corticosteroids

• Main Authors: Eugene Yuriditsky, MD, Aeshita Dwivedi, MD, Navneet Narula, MD, Leon Axel, MD, James M. Horowitz, MD, Mikhail Vaynblat, MD
• Format: Article
• Language: English
• Published: Elsevier 2020-08-01
• Series: JACC: Case Reports
• Subjects: constrictive pericarditis; IgG4 related disease
• Online Access: http://www.sciencedirect.com/science/article/pii/S2666084920308639
• ISSN: 2666-0849

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.)