Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases

• Published in: La Presse médicale (1983) Vol. 49; no. 1; p. 104018
• Main Authors: Marvisi, Chiara, Accorsi Buttini, Eugenia, Vaglio, Augusto
• Format: Journal Article
• Language: English
• Published: Elsevier Masson SAS 01-04-2020
• Subjects: CRP; GCA; ESR; MRI; ECD; RPF; LVV; SLE; TA; CT; 18F-FDG-PET; ANCA; SUV; AAV; IAAA; HLA; IgG4-RD; US
• ISSN: 0755-4982; 2213-0276
• DOI: 10.1016/j.lpm.2020.104018

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.