An 80-year-old woman presented with rare immunoglobulin G4 (IgG4)-related constrictive pericarditis. She underwent pericardiectomy, pericardiotomy, and crosshatching incision of the epicardium using an ultrasonic wave scalpel suitable for the feature of IgG4-related disease. Thereafter, her cardiac function improved remarkably. Additional corticosteroid therapy contributed to maintaining her healthy condition. Definitive diagnosis was based on novel organ-specific criteria. Herein, we highlight that the most important point in the pathological diagnosis of IgG4-related constrictive pericarditis is to determine the presence of IgG4-positive plasma cell infiltration and storiform fibrosis or obstructive phlebitis specific to chronic inflammation.
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http://dx.doi.org/10.1016/j.athoracsur.2021.12.065 | DOI Listing |
Eur Heart J Case Rep
January 2025
Department of Cardiovascular Medicine, Kyoto Chubu Medical Center, 25, Yagi-Ueno, Yagi-cho, Nantan City, Kyoto 629-0197, Japan.
Background: Constrictive pericarditis (CP) can arise from various causes, including post-operative degeneration, tuberculosis, and sequelae of pericarditis. Immunoglobulin (Ig) G4-related disease is a rare but recognized cause of CP. However, the specific mechanisms underlying these aetiologies and pathologies remain unclear.
View Article and Find Full Text PDFMod Rheumatol Case Rep
December 2024
Department of Cardiology, Concord Hospital, The University of Sydney, Concord, NSW, Australia.
IgG-4 related disease (IgG4-RD) is an under recognised multisystem inflammatory disorder that has several typical manifestations. Cardiac manifestations of IgG4-RD are well documented however do not feature in the definition or diagnosis of IgG4-RD according to a recent consensus statement. The most well recognised cardiac manifestation of IgG4-RD, pericardial disease, is outlined in this case report as the initial presenting pathology.
View Article and Find Full Text PDFESC Heart Fail
December 2024
Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Chiba, Japan.
Eur Heart J Case Rep
September 2024
Department of Cardiovascular Surgery, Juntendo University, Hongo 2-1-1, Bunkyo-ku, Tokyo 113-8421, Japan.
Background: Severe liver failure with ascites may be associated with cardiac disease and may be the primary manifestation of constrictive pericarditis or aortic dissection. We report a case of a patient with a chief complaint of ascites for whom close examination revealed that the liver injury was attributed to constrictive pericarditis and chronic aortic dissection, with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) as the primary cause.
Case Summary: A 72-year-old man presented to the emergency department with scrotal oedema and ascites.
J Investig Med High Impact Case Rep
June 2024
Department of Cardiology, St. Joseph's University Medical Center, Paterson, NJ, USA.
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.
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