Background and aim: Isolated thoracic aortitis (ITA) is a newly-defined pathological entity with a still-unknown etiopathology and with some potential relationship to IgG4-related systemic disease. We investigated patients on whom the ascending aorta was operated to identify the clinical and histopathological features of ITA and its relationship to IgG4-related systemic disease.
Methods: Two hundred fifty-one patients underwent replacement of the ascending aorta. Retrospective review of all histological reports was done to identify noninfectious aortitis. Immunohistochemical analysis of resected specimens was performed in all cases.
Results: We found 11 (4.4%) patients with noninfectious aortitis aged 52 to 79 years; nine of the patients were female. All patients underwent ascending aorta replacement. The 30-day mortality was 0. During the follow-up period (median 12 months) four patients died (two of them because of progression of aortic disease). None of the seven living patients developed any IgG4-related diseases, and all had normal serum levels of IgG and IgG4.
Conclusions: Surgical treatment of ITA has acceptable short- and mid-term results. Because follow-up serum levels of IgG and IgG4 were normal in survivors, postoperative corticosteroid therapy may not be indicated in patients in the absence of active vasculitis.
© 2012 Wiley Periodicals, Inc.