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

JACC Case Rep. 2020 Aug 19;2(10):1558-1563. doi: 10.1016/j.jaccas.2020.06.038. eCollection 2020 Aug.

Abstract

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.).

Keywords: CP, constrictive pericarditis; IgG4 related disease; IgG4-RD, immunoglobulin G4–related disease; TTE, transthoracic echocardiogram; constrictive pericarditis.

Publication types

  • Case Reports