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Erschienen in: Rheumatology International 4/2021

25.11.2019 | Case Based Review

Aortic involvement in relapsing polychondritis: case-based review

verfasst von: Mustafa Erdogan, Sinem Nihal Esatoglu, Gulen Hatemi, Vedat Hamuryudan

Erschienen in: Rheumatology International | Ausgabe 4/2021

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Abstract

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.
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Metadaten
Titel
Aortic involvement in relapsing polychondritis: case-based review
verfasst von
Mustafa Erdogan
Sinem Nihal Esatoglu
Gulen Hatemi
Vedat Hamuryudan
Publikationsdatum
25.11.2019
Verlag
Springer Berlin Heidelberg
Erschienen in
Rheumatology International / Ausgabe 4/2021
Print ISSN: 0172-8172
Elektronische ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-019-04468-5

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