Significant Pericardial Involvement of Immunoglobulin G4–Related Disease

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy ¹⁸F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

“…Our literature review found two patients from seven reported cases with IgG4-related pericarditis who did not demonstrate fibrosis in the pericardium (Table) (4,7,9,10,12,13). In addition, four reported patients with IgG4-related pericarditis were not pathologically diagnosed with a pericardial specimen, such as in the present case (Table).…”

“…This indicates that not only the pathological but also the clinical presentation of IgG4-RD may be distinct among the tissues involved. Accordingly, IgG4-related pericardial disease is mostly characterized by fibrosis of the thickened pericardium and clinical presentation of constrictive pericarditis; however, one report has described acute inflammatory pathology in the pericardium (4)(5)(6)(7)(8)(9)(10)(11)(12)(13).…”

Immunoglobulin G4-Related Cardiovascular Diseases

“…28) An open biopsy specimen from the pericardium revealed IgG4-positive plasma cells, and the patient was diagnosed as having an IgG4-related Mikulicz's disease. Seo, Yanagi, and Matsuzaki each presented cases with IgG4-related constrictive pericarditis.…”

“…[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.…”

IgG4-related disease also needs to be ruled out before pericarditis is deemed to be idiopathic

“…Pericardial biopsy showed massive infiltration by lymphocytes and IgG 4 -positive plasma cells. Following treatment with corticosteroids, a significant reduction of pericardial thickness occurred, and there was no recurrence of the effusion 2. IgG 4 -RD also seemed to be implicated in a 64-year-old woman in whom pericardiocentesis yielded 1 L of pericardial fluid which was culture negative and had no malignant cells; IgG 4 -RD was suspected because she also had CT evidence of ureteral wall thickening in the right renal hilum.…”