AB0540 CLINICAL ANALYSIS OF AORTA INVOLVEMENT IN PATIENTS OF ANCA-ASSOCIATED VASCULITIS
Authors: X. Chen, L. Wang
Journal: Annals of the Rheumatic Diseases (2020)

Background: ANCA-associated vasculitis (AAV) is an autoimmune disease that involves abnormal death of neutrophils and leads to necrotic inflammatory reactions in blood vessels, including microscopic polyangiitis (MPA), Granulomatous polyangiitis (GPA) and Eosinophilic granulomatous polyangiitis (EGPA). AAV is mainly involved in small blood vessels, and intermediate arterial lesions can also occur, but the large arteries and their primary branches are rarely involved. Objectives: To summarize the clinical characteristics of aortic involvement in patients with ANCA-associated vasculitis (AAV). Methods: The clinical manifestations, systemic involvement, laboratory examination, imaging characteristics and treatment of aortic involvement in AAV patients admitted to Peking Union Medical College Hospital from January 2013 to December 2018 were retrospectively analyzed. Results: Nine patients were enrolled in our study. The ratio of male to female was 2:1 and the median age was 47 years old. Of the 9 patients, 4 were GPA (44%), 4 were MPA (44%) and 1 was EGPA (11%). The aorta is involved in an average of 3 locations per case, mainly in 7 locations: 3 ascending aorta and aortic arch, 4 in the head and arm trunk (including carotid and subclavian artery), 2 in the abdominal aorta, and 1 in the abdominal cavity. There were 2 cases of renal artery, 1 case involving brachial radial artery, 2 cases of iliac artery and lower limb artery, and 1 case involving left main coronary artery, anterior descending branch, circumflex branch, and right coronary artery. Aortic lesions: 3 cases had aneurysms, arterial dilatation and / or dissection, 6 cases had arterial stenosis or occlusion and 3 cases had periarteritis.When major arterial involvement was found, the AAV of the patients were mostly active, with an average of 19 points for BVAS vasculitis activity and 1 for FFS score. 6 cases had lung involvement (67%), 6 cases had kidney involvement (67%), 4 cases had ENT involvement (44%), 3 cases had nervous system involvement (33%), and 1 case had gastrointestinal involvement (11%). All patients were treated by steroid and immunosuppressant, while 1 case received the operation of ascending aorta and aortic arch replacement. Conclusion: Mainly involved in small blood vessel inflammation, AAV may also have aorta involvement, which was more common in patients who had active disease and need more positive treatment. The affected aorta areas of these patients were mainly ascending aorta, aortic arch, and head and arm trunk, which can be manifested as aneurysms, dissections, and arterial stenosis Periarteritis, etc. If necessary, surgically treatment of the affected aorta could be considered when the situation of AAV was stable enough. References: [1]Jennette J C, Falk R J, Bacon P A, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides[J]. Arthritis Rheum, 2013,65(1):1-11.DOI:10.1002/art.37715. [2]Eisenberger U, Fakhouri F, Vanhille P, et al. ANCA-negative pauci-immune renal vasculitis: histology and outcome[J]. Nephrol Dial Transplant, 2005,20(7):1392-1399.DOI:10.1093/ndt/gfh830. [3]Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort[J]. Medicine (Baltimore), 2011,90(1):19-27.DOI:10.1097/MD.0b013e318205a4c6. [4]Chirinos J A, Tamariz L J, Lopes G, et al. Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature[J]. Clin Rheumatol, 2004,23(2):152-159.DOI:10.1007/s10067-003-0816-0. [5]Minnee R C, van den Berk G E, Groeneveld J O, et al. Aortic aneurysm and orchitis due to Wegener’s granulomatosis[J]. Ann Vasc Surg, 2009,23(6):715-786.DOI:10.1016/j.avsg.2009.06.011. Disclosure of Interests: None declared