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Aortic aneurysm and dissection in systemic lupus erythematosus

Aortenaneurysma und -dissektion bei systemischem Lupus erythematosus

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Abstract

Aortic aneurysm and dissection are rare complications of systemic lupus erythematosus (SLE). The incidence, etiology, risk factors, and outcomes of this entity were largely unknown.

The study materials were based on the publications of aortic aneurysm or dissection due to SLE published between 2000 and 2017.

A total of 36 articles reporting a single case or case series involving 40 patients were collected. The patients showed an absolute female dominance at a mean aneurysm age of 44.6 years. Steroid use was 13.3 ± 9.4 years prior to admission for management of aortic aneurysm or dissection. Aortic aneurysm occurred more commonly in abdominal than other segments of the aorta, whereas aortic dissection did not show any location predilection. Patients with open aortic operations showed a higher mortality rate than other groups; however, no statistical significance was reached. Interventional therapy was minimally invasive, but postinterventional endoleaks were a concerning problem.

SLE patients had significant risks for developing aortic aneurysm and dissection. Hypertension, long-term steroid use, and aortic pathological changes related to SLE seemed to be predominant risk factors for the occurrence of aortic aneurysm and dissection. Upon diagnosis, a surgical, interventional, or hybrid treatment should be performed to prevent severe sequelae and sudden deaths.

Zusammenfassung

Das Aortenaneurysma und die Aortendissektion sind seltene Komplikationen des systemischen Lupus erythematosus (SLE). Inzidenz, Ätiologie, Risikofaktoren und Ergebnisse von dieser Entität waren bisher weitgehend unbekannt.

Diese Studie basiert auf Publikationen zu Aortenaneurysma und -dissektion in Verbindung mit SLE aus den Jahren 2000–2017.

Insgesamt wurden 36 Artikel mit 40 Patienten als Beschreibung eines Einzelfalls oder einer Fallserie in die Auswertung einbezogen. Bei den Patienten herrschte das weibliche Geschlecht vor, das Durchschnittsalter bei Auftreten des Aneurysmas lag bei 44,6 Jahren. Die Dauer einer Steroidbehandlung betrug 13,3 ± 9,4 Jahre bis zur stationären Aufnahme wegen eines Aortenaneurysmas oder einer Aortendissektion. Ein Aortenaneurysma trat häufiger im abdominalen als in anderen Segmenten der Aorta auf, während für eine Aortendissektion keine Prädilektionsstellen festgestellt wurden. Patienten mit offener Aortenoperation wiesen eine höhere Mortalitätsrate auf als andere Gruppen; es wurde jedoch keine statistische Signifikanz erreicht. Interventionell therapiert wurde minimalinvasiv, ein Problem stellten allerdings postinterventionelle Endoleckagen dar.

Bei SLE-Patienten bestanden signifikante Risiken für die Entwicklung eines Aortenaneurysmas und einer Aortendissektion. Hypertonie, Langzeitsteroidtherapie und pathologische Veränderungen der Aorta durch den SLE schienen vorherrschende Risikofaktoren für das Auftreten von Aortenaneurysma und -dissektion zu sein. Bei Diagnosestellung sollte eine chirurgische, interventionelle oder Hybridtherapie erfolgen, um schwere Folgen und plötzlichen Tod zu verhindern.

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Correspondence to Shi-Min Yuan MD, PhD.

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S.-M. Yuan declares that he has no competing interests.

This article does not contain any studies with human participants or animals performed by any of the authors.

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U. Müller-Ladner, Bad Nauheim

U. Lange, Bad Nauheim

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Yuan, SM. Aortic aneurysm and dissection in systemic lupus erythematosus. Z Rheumatol 78, 287–294 (2019). https://doi.org/10.1007/s00393-018-0555-x

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