title: Immunoglobulin G4-related periaortitis complicated by aortic rupture and aortoduodenal fistula after endovascular AAA repair.
abstract: OBJECTIVE
  To report a rare and complicated case of immunoglobulin (Ig) G4-related periaortitis involving both the aortic wall and the retroperitoneum without aneurysmal formation.
  
  
  METHODS
  A 79-year-old man with IgG4-related periaortitis suffered aortic rupture despite a normal caliber aorta after 6 months of steroid therapy (20 mg/d). Endovascular repair with an aortic cuff sealed the rupture. Steroid therapy was halted 2 weeks later due to infection. Four months later, a biopsy during esophagogastroduodenoscopy to investigate gastrointestinal bleeding suggested a relapse of IgG4-RD in the duodenum. Subsequent aortoduodenal fistula formation proved fatal. Generally, IgG4-related periaortitis does not result in such complications due to the absence of aneurysm formation and a thick aortic wall.
  
  
  CONCLUSIONS
  Our report highlights a rare case of IgG4-related periaortitis where complications resulted following steroid therapy and surgical intervention, emphasizing the difficulties in dealing with IgG4-related cardiovascular lesions.
authors.affiliation: 1 Department of Pathology, National Hospital Organization, Kanazawa Medical Center, Kanazawa, Japan.
authors.forename: Satomi
authors.initials: S
authors.lastname: Kasashima
authors.forename: Atsuhiro
authors.initials: A
authors.lastname: Kawashima
authors.forename: Fuminori
authors.initials: F
authors.lastname: Kasashima
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Endo
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Matsumoto
authors.forename: Kengo
authors.initials: K
authors.lastname: Kawakami
authors.forename: Masato
authors.initials: M
authors.lastname: Kayahara
authors.forename: Hajime
authors.initials: H
authors.lastname: Ohta
authors.forename: Masaaki
authors.initials: M
authors.lastname: Yano
authors.forename: Youhei
authors.initials: Y
authors.lastname: Marukawa
mesh_terms: D000368:Aged; D017544:Aortic Aneurysm, Abdominal / Q000175:diagnosis / Q000276:immunology / Q000601:surgery*; D001019:Aortic Rupture / Q000175:diagnosis / Q000276:immunology / Q000601:surgery*; D001025:Aortitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D001027:Aortography / Q000379:methods; D001706:Biopsy; D019917:Blood Vessel Prosthesis Implantation; D004378:Duodenal Diseases / Q000175:diagnosis / Q000276:immunology*; D016099:Endoscopy, Gastrointestinal; D057510:Endovascular Procedures / Q000009:adverse effects*; D004927:Escherichia coli Infections / Q000276:immunology / Q000382:microbiology; D017809:Fatal Outcome; D006471:Gastrointestinal Hemorrhage / Q000276:immunology; D006801:Humans; D016867:Immunocompromised Host; D007074:Immunoglobulin G / Q000032:analysis*; D007412:Intestinal Fistula / Q000175:diagnosis / Q000276:immunology*; D008297:Male; D012307:Risk Factors; D013256:Steroids / Q000009:adverse effects; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016157:Vascular Fistula / Q000175:diagnosis / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G; D013256:Steroids
keywords: IgG4-related disease; IgG4-related periaortitis; aortic rupture; aortoduodenal fistula; corticosteroid; endovascular aortic/aneurysm repair; immunoglobulin G4; inflammatory aortic aneurysm