ABSTRACT This report describes a case of a 66-year-old male patient with accidental diagnosis of chronic contained rupture of an aortic aneurysm. Surgery was performed through a median laparotomy. A thick periaortic tissue with fibrosis and lymphnodes covered the AAA. Immunohistochemical examination of the aneurismatic aortic wall revealed intense positivity for inflammatory markers and a large number of immunoglobulin G4 (IgG4) positive cells. The postoperative course was uneventful and patient was discharged in the fifth postoperative day. Patient was then followed periodically at the outpatient rheumatologic clinic. No adverse events occurred during 3 and 6 months follow up. Conclusion: Identification of IgG4-inflammatory aneurysms as an expression of the IgG4-related systemic disease is essential both for clinical follow up and surgical and pharmacological treatment considering the possibility of aneurysm rupture and the involvement of other organs.
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