Pericardial Involvement in IgG4-related Disease

Abstract

We herein report the case of a 65-year-old man with pericardial involvement associated with autoimmune pancreatitis. Chest CT imaging showed pericardial thickening. The patient responded to corticosteroid therapy, and the pericardial thickening resolved. Multiple organs are involved in immunoglobulin G4 (IgG4)-related disease (IgG4-RD); however, only a few cases of IgG4-related chronic constrictive pericarditis have been reported. To our knowledge, this is the first reported case of IgG4-RD with pericardial involvement at an early stage. This case indicates that recognizing pericardial complications in autoimmune pancreatitis is important and that CT imaging may be useful for obtaining the diagnosis and providing follow-up of pericardial lesions in cases of IgG4-RD.

Immunoglobulin G4-related constrictive pericarditis identified by cytological examination of pericardial effusion: a case report

“…IgG4-RD may affect multiple organs in 60 to 90% of patients with IgG4-RD, including cardiovascular organs [3–5]; it responds positively to corticosteroid therapy [1]. Previous studies have suggested that CP could often develop after mediastinal or idiopathic retroperitoneal fibrosis due to IgG4-RD [5] and pleural and/or cardiac effusion was sometimes observed continuously [6].…”

“…Because the findings of the cytological examination did not meet diagnostic criterion (3) properly, we performed an 18 F-FDG-PET scanning to judge the extent of inflammatory foci [7–10]. The 18 F-FDG-PET scanning showed abnormal uptake in atypical organs such as his pericardium, his gastric wall, and in his hilar lymph nodes, but not in his pancreas, his lacrimal and salivary glands, or kidneys that were reported as common sites of IgG4-RD [3]. Finally, we performed a thoracoscopic pericardiectomy because of 18 F-FDG-PET findings.…”

Immunoglobulin G4-Related Cardiovascular Diseases

“…[26][27][28][29][30][31] Matsumiya and colleagues detected IgG4-positive plasma cells in a biopsy specimen from a 50-year-old woman with a thickened pericardium. 26) Clinical fever symptoms and elevated serum IgG4 were promptly resolved by administration of a moderate-dose glucocorticoid.…”

Intrathoracic Manifestations of IgG4-Related Disease

“…17 Several cases of histologically diagnosed (on autopsy or pericardiectomy) IgG4-RD pericardial disease have been reported as well. [18][19][20] IgG4-RD pericardial disease frequently presents as constrictive pericarditis; Pericardial involvement is usually seen as pericardial thickening on CT.…”

“…Pathologically, the pericardial and pleural manifestations of IgG4-RD are characterized by a diffuse fibrinous thickening with a patchy lymphoplasmacytic infiltrate with IgG4 predominance. [18][19][20] Periaortitis and periarteritis are characterized as diffuse lymphoplasmacytic infiltration, numerous IgG4-positive plasma cells, and irregular fibrosis of the adventitia. 21 Pleural effusions have been previously been reported in IgG4-RD and have been described as exudative with a lymphocyte predominance.…”