A Rare Case of Immunoglobulin G4-Related Constrictive Pericarditis Diagnosed Via Multimodality Imaging

• Published in: JIM - high impact case reports Vol. 12; p. 23247096241248969
• Main Authors: Ozgur, Sacide S, Tagliaferri, Ariana, Aiken, Arielle, Desai, Binoy, Abboud, Rachel, Shamoon, Yezin, Akel, Tamer, Singhal, Monisha, Shamoon, Fayez
• Format: Journal Article
• Language: English
• Published: United States SAGE Publications 01-01-2024; SAGE Publishing
• Subjects: Case Report; Diagnosis, Differential; Echocardiography; Humans; Immunoglobulin G - blood; Immunoglobulin G4-Related Disease - complications; Immunoglobulin G4-Related Disease - diagnosis; Immunoglobulin G4-Related Disease - diagnostic imaging; Magnetic Resonance Imaging; Male; Middle Aged; Multimodal Imaging; Pericarditis, Constrictive - diagnosis; Pericarditis, Constrictive - diagnostic imaging; multimodality imaging; polyclonal cells; IgG4-related disease; constrictive pericarditis; gammaglobulinopathy; cardiology
• ISSN: 2324-7096; 2324-7096
• DOI: 10.1177/23247096241248969

Constrictive pericarditis (CP) presents as a pathophysiological state where the pericardium becomes inelastic due to fibrotic changes, most commonly secondary to a protracted inflammatory process. The disease is characterized by compromised diastolic cardiac function due to loss of pericardial compliance. Immunoglobulin G4 (IgG4)-related disease, an entity marked by the insidious proliferation of IgG4-positive plasma cells and subsequent fibrosis within various organs, is an infrequent but recognized cause of CP. A case of a 55-year-old male patient with clinical manifestations of dyspnea and edema in the lower extremities elucidates the diagnostic complexity inherent to CP. Echocardiography revealed a constellation of signs, including annulus reversus, septal bounce, and a congested inferior vena cava; cardiac magnetic resonance imaging (MRI) demonstrated diffuse pericardial thickening with delayed gadolinium enhancement, suggestive of a long-term inflammatory state; and right heart catheterization confirmed the hemodynamic hallmark of CP-equalization of diastolic pressures across the cardiac chambers. The serological analysis elicited elevated serum levels of IgG4 and IgE, pointing to the differential diagnosis of IgG4-related disease. Given the nonspecific clinical presentation of IgG4-related CP, a heightened index of suspicion combined with a systematic approach to imaging and serological evaluation is paramount.