Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report

Abstract

Background Constrictive pericarditis remains a problematic diagnosis and a thorough investigation is critical. Among possible aetiologies, immunoglobulin-G4 (IgG4)-related pericardial disease is an unusual cause of pericardial constriction. We report a challenging diagnostic case of pericardial constriction due to IgG4-related disease. Case presentation A 68-year old male with a history of inferior myocardial infarction with right ventricle (RV) involvement was thrice-hospitalized due to marked ascites and peripheral oedema. Systemic congestion was initially attributed to RV dysfunction due to previous infarction. Yet, at the final admission, a re-assessment echocardiogram followed by cardiac computed tomography, magnetic resonance and right heart catheterization raised a possible diagnosis of constrictive pericarditis with a finding of abnormal pulmonary venous return. Patient therefore underwent pericardiectomy and surgical correction of pulmonary venous return. Pericardium histology revealed an IgG4-related pericardial constriction. Patient was later discharged on corticosteroids with marked symptomatic improvement. Conclusion IgG4-related disease remains a rare cause of pericardium constriction while also presenting a challenging diagnosis in everyday clinical practice. This case exemplifies the difficulties faced by clinicians when reviewing a possible case of constrictive pericarditis, while highlighting the importance of a multimodality assessment.

Semiquantitative 18F-FDG PET/CT in monitoring glucocorticoid response of immunoglobulin G4-related effusive constrictive pericarditis: a case report

“…IgG4-RD is a fibroinflammatory condition with multiorgan involvement. ECP, although rare, is a potentially life-threatening condition requiring clinical acumen for diagnosis [6]. Definite diagnosis of IgG4-RD can be made according to the 2020 Revised Comprehensive Diagnostic criteria incorporating clinical, serological, and pathological indicators [7].…”

Rituximab and pericardiectomy with waffle procedure in constrictive pericarditis due to IgG4‐related disease: A case report

“… 2 Reports on CP caused by IGRD are limited. 3 This study reports a case of CP caused by IGRD and describes the successful treatment with pericardiectomy via the waffle procedure and rituximab administration to avoid long‐term steroid use.…”

“…IgG4‐related disease (IGRD) is a multiorgan fibroinflammatory condition that mimics many malignant, infectious, and inflammatory disorders 2 . Reports on CP caused by IGRD are limited 3 . This study reports a case of CP caused by IGRD and describes the successful treatment with pericardiectomy via the waffle procedure and rituximab administration to avoid long‐term steroid use.…”