Original contributionAortitis and ascending aortic aneurysm: description of 52 cases and proposal of a histologic classification
Introduction
Noninfectious aortitis is a heterogeneous group of diseases. There is a predilection for the proximal aorta and formation of aneurysms. Noninfectious aortitis is typically classified as Takayasu disease or giant cell aortitis, largely dependent on patient age and other nonspecific clinical criteria [1]. It has been stated, based on relatively few cases, that histologic features of Takayasu disease and giant cell aortitis are sufficiently similar that histologic criteria cannot separate the two. Because many patients with noninfectious aortitis are young and have no extra-aortic manifestations of Takayasu disease, a variety of synonymous overlapping terms have been used, including nonspecific aorto-arteritis and granulomatous aortitis [2], [3], [4]. The most recent series proposes a classification of aortitis that relies entirely on clinical features [5], [6].
The purpose of the current study is to retrospectively analyze a series of aortitis, with an attempt at histologic classification that will enable the surgical pathologist to provide a descriptive diagnosis that will aid in the eventual clinical subclassification of the patient's rheumatologic disease. The use of a 50-year cutoff point was analyzed by histogram, and the presence of medial zonal necrosis is a starting point for histologic subclassification, adding other histologic and immunohistochemical findings.
Section snippets
Case selection
A retrospective search for aortitis was performed at 3 institutions, and cases were retrieved from 1989 to 2005. Fifty-seven cases were initially retrieved. Inclusion criteria were diagnoses with the term “aortitis,” with histopathologic verification of the sections demonstrating primary noninfectious aortitis and excluding mycotic aneurysm, syphilitic aortitis, and inflammation secondary to atherosclerosis or organizing dissection. Five aortitis with predominant adventitial fibrosis and
Results
Based on light microscopic features, cases of aortitis were separated into NA and NNA. There were 43 patients with NA; these patients were separated into 2 groups: those older than 65 years and those younger than 65 years. There were 24 patients younger than 65 years and 18 patients older than 65 years (elderly NA). There was no sex predilection for adult NA; however, women predominated in the group older than 65 years (P = .003 versus adult NA) (Table 1). There was a higher proportion of
Discussion
The current study shows a spectrum of pathologic stages in aortitis that include necrosis, inflammation, regenerative changes, and fibrosis. However, 2 basic types emerge: NNA, which does not result in medial laminar necrosis or elastic loss, and NA. NNA has several features that immediately separate it from NA: exclusive occurrence in the elderly, higher association with giant cell arteritis, a higher rate of dissection, and several histologic features. Significant intimal and adventitial
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