Aortic involvement in relapsing polychondritis: case-based review
Relapsing polychondritis is a systemic inflammatory disease that mainly affects ears, nose, eyes, joints, and large airway. Relapsing polychondritis may also affect cardiac valves and large vessels with the aorta being most frequently involved. We conducted a systematic literature review to delineate the clinical characteristics, treatment, and outcome of relapsing polychondritis patients with aortic involvement including thoracic and abdominal aorta, aortic valve, and coronary arteries. 113 patients reported in 85 manuscripts were retrieved through the systematic literature search and references of the selected manuscripts. With the addition of a patient from our center, a total of 114 patients were included in the analyses. Aortic vessel involvement was the predominant type of involvement that was identified in 93 (82%) patients, while aortic valve involvement was identified in 41 patients (36%). The median age at aortic involvement was 37 years [IQR: 30–53] with a delay of 5 years [IQR: 1–8] between first relapsing polychondritis symptom and aortic involvement. Nineteen percent of the patients were asymptomatic at the time of aortic involvement diagnosis. The initial treatment was immunosuppressives in 41 patients (56%) and surgery in 28 patients (38%). The mortality ratio was 27% in a 24 month follow-up [IQR: 7.5–54 months]. Aortic dissection or rupture was the most frequent causes of mortality. Concomitant coronary artery involvement suggested a worse outcome. Aortic involvement in relapsing polychondritis is a mortal complication despite medical and surgical treatments. It may be asymptomatic in 19% of the patients which warrants the importance of screening.
KeywordsAortic aneurysm Aortic valve Aortitis Polychondritis Vasculitis
All authors contributed to writing this study and have approved the final version.
ME, substantially contributed to the conception and design of the work; the acquisition, analysis and interpretation of data, drafting the manuscript, approved the version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. SNE, substantially contributed to the conception and design of the work; the acquisition, analysis, and interpretation of data; approved the version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. GH, substantially contributed to the conception and design of the work; interpretation of data, drafting the manuscript, and revising it critically for important intellectual content, approved the version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. VH, substantially contributed to the conception and design of the work, interpretation of data, drafting the manuscript, revising it critically for important intellectual content, approved the version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
We did not receive any funding support.
Compliance with ethical standards
Conflict of interest
G. Hatemi received research grant/research support and/or speaker fees from AbbVie. V. Hamuryudan received honoraria, speaker fees and research grants from Amgen, MSD, Pfize,r and Roche. The other authors (ME, SNE) have declared that they have no competing interest.
This article does not contain any studies with human participants performed by any of the authors. Written informed consent to publish was obtained from the reported patient prior to submission of this article.
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