Case report
Multiple aortic aneurysms in relapsing polychondritis

https://doi.org/10.1016/0002-9149(76)90432-XGet rights and content

Abstract

A patient with relapsing polychondritis and thoracic and abdominal aortic aneurysms is described. The aortic changes were due to aortitis, which primarily involved the media, with increased vascularization, perivascular infiltration of mononuclear cells, increased amounts of collagen and decreased amounts of elastic tissue and sulfated acid mucopolysaccharides. Aortic aneurysms frequently occur in relapsing polychondritis; they are usually in the ascending aorta but may be multiple and involve the abdominal aorta; involvement of the ascending aorta results in aortic regurgitation and left ventricular failure, and involvement of the abdominal aorta may be clinically silent and result in fatal rupture.

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Cited by (44)

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    Case reports have described patients with RP-related aortic aneurysm/dissection who underwent surgical procedures. Indeed, among 40 RP patients with aortic involvement, who underwent surgical aortic procedures, 3 patients (7.5%) died peri-operatively and 10 patients (25%) exhibited relapses of aortic localizations within a median time of 7 months [range: 2–24 months] after surgical procedures [2–8,10,11,14,15,17,18,20,22,23,27,29–33,35–37,39]. In the present study, we have interestingly found that vascular complications were less frequent in RP patients without biological inflammatory syndrome at time of aortic surgery; thus, we suggest that aortic surgical procedures should be performed when RP is inactive.

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    However, cusp rupture with normal aortic root has also been reported [52]. Aortic aneurysms occur frequently in RP, may be multiple and involve all parts of the aorta, even resulting in fatal rupture in asymptomatic patients [53]. Furthermore, several cases of atrioventricular block, comprising complete heart block, mitral regurgitation, obstructive lesions, acute pericarditis, myocarditis, and silent myocardial infarction have been reported [5,26,50,54,55].

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    2002, Mayo Clinic Proceedings
    Citation Excerpt :

    Multiple aneurysms along the entire length of the aorta can also occur, as seen in our patient. Involvement of the ascending aorta can produce dilatation and aortic regurgitation, and aneurysm rupture or dissection may lead to sudden death.8,10 An overlap with other vasculitides, including Behçet syndrome, giant cell arteritis, and Takayasu arteritis, has been reported.1

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This study was partially supported by Grant 1 TO 1 HL05966-02 CAR from the National Institutes of Health, Bethesda, Md.

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