• Satomi Kasashima, Atsuhiro Kawashima, Fuminori Kasashima, Masamitsu Endo, Yasushi Matsumoto, Kengo Kawakami, Masato Kayahara, Hajime Ohta, Masaaki Yano, and Youhei Marukawa.
• 1 Department of Pathology, National Hospital Organization, Kanazawa Medical Center, Kanazawa, Japan.
• J. Endovasc. Ther. 2014 Aug 1; 21 (4): 589-97.

PurposeTo report a rare and complicated case of immunoglobulin (Ig) G4-related periaortitis involving both the aortic wall and the retroperitoneum without aneurysmal formation.Case ReportA 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.ConclusionsOur 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.

51 keywords...

hide…