title: 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.
authors.affiliation: Department of Cardiovascular Medicine, University of Tokyo Graduate School of Medicine, Tokyo, Japan.
authors.forename: Aiko
authors.initials: A
authors.lastname: Sakamoto
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
authors.forename: Kan
authors.initials: K
authors.lastname: Saito
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Imai
authors.forename: Masao
authors.initials: M
authors.lastname: Takahashi
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Hosoya
authors.forename: Norifumi
authors.initials: N
authors.lastname: Takeda
authors.forename: Kenji
authors.initials: K
authors.lastname: Hirano
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Koike
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Enomoto
authors.forename: Haruki
authors.initials: H
authors.lastname: Kume
authors.forename: Yukio
authors.initials: Y
authors.lastname: Homma
authors.forename: Daichi
authors.initials: D
authors.lastname: Maeda
authors.forename: Hideomi
authors.initials: H
authors.lastname: Yamada
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Hirata
authors.forename: Nobukazu
authors.initials: N
authors.lastname: Ishizaka
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001014:Aortic Aneurysm / Q000276:immunology / Q000473:pathology*; D017544:Aortic Aneurysm, Abdominal / Q000000981:diagnostic imaging / Q000473:pathology; D003327:Coronary Disease / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007150:Immunohistochemistry; D007249:Inflammation; D007376:Interleukin-2 / Q000097:blood; D008297:Male; D008480:Mediastinitis / Q000473:pathology; D008875:Middle Aged; D010493:Pericarditis / Q000276:immunology / Q000473:pathology*; D010496:Pericardium; D049268:Positron-Emission Tomography; D012185:Retroperitoneal Fibrosis / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D012189:Retrospective Studies; D012598:Sclerosis / Q000473:pathology; D012907:Smoking / Q000009:adverse effects; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G; D007376:Interleukin-2