Abstract 10781: An Unusual Case of Transient Inflammatory Constrictive Pericarditis: IgG4 Disease

Abstract

Case Presentation: A 54-year-old male with a past medical history of hypertension presented with chest tightness, shortness of breath, fatigue, and ankle edema. His vital signs were within normal limits but physical exam was significant for jugular venous distension and bilateral peripheral edema. Laboratory studies were unremarkable except for elevated inflammatory markers (C-reactive protein [23.6 mg/dl] and erythrocyte sedimentation rate [34 mm/hour]). Interferon assay, Influenza A/B antigens and anti-nuclear antibody were negative. The patient was diagnosed with constrictive pericarditis (CP). Although the patient was treated appropriately for pericarditis with a steroid taper, colchicine and ibuprofen, his symptoms recurred. Therefore, he underwent a radical pericardiectomy which resulted in a marked clinical improvement. The final pericardial pathology revealed fibrosis and plasma cell infiltrates with >30% of cells positive for IgG4. An echocardiogram revealed a small pericardial effusion with septal bounce, annulus reversus, and a plethoric inferior vena cava consistent with a diagnosis of CP. Cardiac magnetic resonance imaging showed a small circumferential pericardial effusion and respirophasic septal flattening during inspiration with marked pericardial delayed enhancement (DHE). Discussion: CP is commonly caused by viral or idiopathic etiologies, with infection (tuberculosis) being more prominent in developing countries. IgG4 disease is an uncommon cause of CP with only a few cases of IgG4-related CP have been reported. Pathological findings on histology include IgG4-positive lymphoplasmacytic cells and fibrosis. The disease is characterized by steroid-responsive condition but pericardiectomy is pursued with a suboptimal response. Cardiovascular manifestations of IgG4 disease are under explored and our case illustrates the importance of recognizing and treating the pericardial complications of IgG4 disease.