Idiopathic retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pericarditis—Retrospective analysis of 11 case histories

Abstract

Retroperitoneal fibrosis, inflammatory aortic aneurysm, and pericardial and mediastinal fibrosis are characterized by infiltration of immuno-inflammatory cells and deposition of thickened fibrous tissues. Several recent studies suggested that an immunoglobulin-G4 (IgG4)-related immunological mechanism may play a role in these diseases. By searching the clinical database of patients admitted to our department between 2000 and 2010, we summarized the clinical data of 11 patients who were diagnosed to have these disorders. The diagnoses were idiopathic retroperitoneal fibrosis (8 cases), mediastinal and/or pericardial fibrosis (4 cases), inflammatory abdominal aneurysm (2 cases), and inflammatory coronary periarteritis (1 case). Hypertension, diabetes, and dyslipidemia were found in 45%, 36%, and 55%, respectively, in these patients, and they were all either current or former smokers. Two patients with pericardial involvement showed a rushed clinical course, resulting in in-hospital death. Serum levels of IgG were elevated in 67%, and soluble interleukin-2 receptor was elevated in 75%, when measured. Immunohistochemical analysis showed marked infiltration of IgG4-positive plasma cells in the pericardium in patients who died of constrictive pericarditis. Our data support the notion that immune-inflammatory mechanism, which might be IgG4-related sometimes, may play a role in idiopathic retroperitoneal fibrosis, inflammatory aortic aneurysm, and mediastinal/pericardial fibrosis, although clinical course may differ substantially.

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).…”

Retroperitoneal Fibrosis in a Patient with Gastric Cancer Manifested by Lower Extremity Edema and Hydrocele

“…On the other hand, however, it has been reported that IgG4-related pathologies may be present in less than 60% in idiopathic retroperitoneal fibrosis (17,18). In addition, IgG4-positivity may not influence the clinical pictures and therapeutic responsiveness in idiopathic retroperitoneal fibrosis (19). Furthermore, a possible relationship between IgG4-related disease and carcinogenesis has been reported (20).…”

IgG4-Related Cardiovascular Disorders

“…24) Mediastinal fibrosis and idiopathic retroperitoneal fibrosis can be associated with patients with IgG4-related pericarditis. 85,86) Idiopathic retroperitoneal fibrosis, the main symptom of which is hydronephrosis by ureteral encasement, is considered to be an IgG4-related disease, whereas secondary retroperitoneal fibrosis is caused by drugs, malignancy, infection, radiotherapy, and surgery. [87][88][89] Retroperitoneal fibrosis sometimes coexists with sclerosing pancreatitis.…”

“…25) Cardiac and pleural effusion sometimes accompanies pericarditis, which could lead to a fatal outcome by causing respiratory insufficiency. 85) Echocardiography can detect it as highly echogenic thickening of the pericardium and cardiac effusion ( Figure 3A). CT imaging is also of help in the diagnosis ( Figure 3B).…”