ACR 2020 | Daily Highlights

Isolated aortitis

ISOLATED AORTITIS: SINGLE CENTRE EXPERIENCE OF CLINICAL SPECTRUM AND MANAGEMENT

Abstract: 0404
Authors: Nadia Ahmad et al.

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Key content:
Isolated aortitis was regarded as a rare form of large vessel vasculitis (LVV). It may occur in the context of primary systemic vasculitis, as a part of systemic autoimmune disease or in isolation. This retrospective study aimed to assess: presenting features, time to diagnosis, imaging methods used, treatment and complications.

63 patients were identified over 10 years; the median age at diagnosis was 68 years (range 32 – 88); 36 (62%) were females; the median duration of symptoms was 7 months (range 2-120). The presenting feature was a systemic inflammatory syndrome (SIRS) in 28 patients (49%) followed by back/abdominal pain in 8, chest symptoms in 6 and weight loss in 4. Median CRP was 81mg/l [range16-249]; median ESR 79mm/h [range 11-139]). Dissection/aneurysm was diagnosed in 6 patients who also had histological confirmation of aortitis; 43 patients (74%) were diagnosed on PET-CT, 12 (21%) on CT angiogram aorta (CTA). Prednisolone, methotrexate and other DMARDs were used in the >90% of cases, cyclophosphamide in 11 and tocilizumab in 4 patients. 17 patients developed vascular complications, including aneurysms (8 patients), vascular stenosis/ischaemic complications (9 patients).

Relevance:
The data illustrate the key role of imaging methods, typically PET-CT, to diagnose isolated aortitis. They demonstrate a potentially fatal diagnostic delay, which is due to the lack of pathognomonic signs and/or symptoms of aortal wall inflammation. They show that CRP / ESR are always elevated. Finally, they highlight the need to follow such patients using PET or MR-technology (at least annually), to early detect development of aneurysm or stenosis.

Zusammenfassung und Kommentar von:
Prof. Dr. Peter Villiger
Bern