AbstractBackground 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.