Chronic Periaortitis With Thoracic Aorta Involvement: A New Subtype Of Large Vessel Vasculitis

Alessandra Palmisano¹, Maria Letizia Urban², Domenico Corradi³, Federico Alberici⁴, Carlo Salvarani⁵, Carlo Buzio¹ and Augusto Vaglio⁶, ¹Clinical Medicine, Nephrology and Health Sciences, University of Parma, Parma, Italy, ²Nephrology University of Parma, Parma, Italy, ³Pathology University of Parma, Parma, Italy, ⁴Department of Clinical Medicine, Nephrology and Health Sciences, University of Parma, Parma, Italy, ⁵Rheumatology, Arcispedale S Maria Nuova-IRCCS, Reggio Emilia, Italy, ⁶Unit of Nephrology, University Hospital of Parma, Parma, Italy

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Fibroblasts and vasculitis

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics I

Session Type: Abstract Submissions (ACR)

Background/Purpose: Chronic periaortitis (CP) is characterised by a fibroinflammatory tissue arising from the adventitia of the abdominal aorta and the iliac arteries and spreading into the surrounding retroperitoneum. CP is thought to have an autoimmune origin and to arise as a primary aortitis. Involvement of the thoracic aorta and its main branches has been anecdotally described. We analysed frequency and pattern of involvement of the large thoracic arteries in CP patients.

Methods: We studied 77 consecutive CP patients who had appropriate imaging studies to evaluate thoracic vessel involvement (chest contrast-enhanced CT/MRI, angio-CT/MRI,whole-body CT-PET). All patients underwent routine clinical assessment and laboratory tests.

Results: Twenty-eight patients (36.4%) showed thoracic vessel involvement: 21 had thoracic periaortitis, which surrounded an aneurysmal thoracic aorta in 6 cases and also involved the origin of the epiaortic vessels in 9 cases; 7 patients had thoracic aortic aneurysm without periaortitis. Analysis of demographic and clinical features in the groups with and without thoracic involvement showed, in the former, a higher female prevalence (M/F ratio 14/14 vs 39/10, P=0.010), a more advanced age at diagnosis [median(interquartile range) 64.5 (58.3-69.-5) vs 56.0 (50-59) years, P=0.001], a higher frequency of constitutional symptoms (86% vs. 59%, P=0.021), and a shorter relapse-free survival (log-rank P=0.025).

Conclusion: Involvement of large thoracic arteries occurs in about one-third of CP patients. This subset of patients with systemic large vessel involvement shows distinct clinical features, such as a higher female prevalence, a higher age at diagnosis, and a higher frequency of systemic symptoms; in addition, patients with thoracic involvement tend to have a frequently relapsing course. These findings raise the question as to whether this CP subset represents a distinct form of large-vessel vasculitis.

Disclosure:

A. Palmisano,
None;

M. L. Urban,
None;

D. Corradi,
None;

F. Alberici,
None;

C. Salvarani,
None;

C. Buzio,
None;

A. Vaglio,
None.

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