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The objectives of this study are to evaluate the incidence of aortitis on a surgical population, establish any relationship with systemic diseases, verify early and late surgical results and provide clinical and radiological follow-up to determine factors potentially predicting progression of the disease and influencing late outcome. From 2009 to 2017, 237 patients underwent elective operations on the ascending aorta. Segments of the excised tissues were routinely sent for histologic evaluation, providing adequate data in 178 (75%) for a clinical and pathologic correlation. Patients with aortitis (Group 1) (n = 26) were compared with 152 with atherosclerotic or degenerative disease (Group 2). Incidence of aortitis was 15%, being clinically isolated in 73%. In 24 patients (92%), a giant cell aortitis was found. Actuarial survival at 3 years is 88% in Group 1 and 98% in Group 2 and 74% and 98% at 5 years, respectively (P = 0.016). A control angio-computed tomography revealed an increased descending aorta diameter in 2 out of 14 late survivors. A positron emission tomography showed presence of arteritis in other vascular segments in 3 patients. Clinically isolated aortitis is extremely frequent in patients with inflammatory aortic disease. The diagnosis is often difficult and may be supported by routine pathologic evaluation of surgical explants and by multimodality imaging. The latter should be employed to allow adequate patient follow-up and to disclose potential recurrences in untreated aortic segments. [ABSTRACT FROM AUTHOR]