Catastrophic antiphospholipid syndrome presenting with aortic barrage: case report and review of the literature

Author:

Moroni Luca12ORCID,Righini Paolo3,Ramirez Giuseppe A12ORCID,Farina Nicola12,Mancuso Gaia12,Bozzolo Enrica1,Rodríguez-Pintó Ignasi4,Cervera Ricard5ORCID,Nano Giovanni36,Dagna Lorenzo12

Affiliation:

1. Unit of Immunology, Rheumatology, Allergy and Rare Diseases, San Raffaele Hospital, Milano, Italy

2. Faculty of Medicine, Vita-Salute San Raffaele University, Milano, Italy

3. Operative Unit of Vascular Surgery, Cardiovascular Department, IRCCS Policlinico San Donato, Milan, Italy

4. Autoimmune Disease Unit, Internal Medicine Department, Hospital Universitari Mutua de Terrassa, Terrassa, Spain

5. Department of Autoimmune Diseases – Hospital Clínic, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain

6. Department of Biomedical Sciences for Health, University of Milan, Milan, Italy

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition characterized by multiple thromboembolic events occurring in a short period of time, frequently accompanied by significant systemic inflammation. Aortic involvement is rare in antiphospholipid syndrome and it had been never described in the context of its catastrophic variant. Here, we report an unusual case of aortic occlusion as a debut manifestation of CAPS and discuss its clinical features with an up-to-date review of the literature to identify risk factors and clues for clinical practice.

Publisher

SAGE Publications

Subject

Rheumatology

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