Granulomatosis with poliangiitis (GPA) is a systemic necrotizing vasculitiis of small and medium vessels. Large vessels involvement of GPA is extremely rare - incidence 0.1 per million - but, necrotizing vasculitiis of the aortic wall potentially leads to death in case of rupture. In the literature, two different clinical situation are found: a thickened aortic wall, as aortitis, with or without aneurysm development and the abdominal aorta is the most frequent. Particularly, the ascending tract involvement is almost anecdotic. These patients, are often very fragile and suffering from severe systemic comorbidities. Rupture of ascending aorta is an emergency condition that, in some high risk patients, may require an endovascular or hybrid backup plan, alternatively to ascending aorta replacement, avoiding extracorporeal circulation or cardiac arrest. Some autoimmune systemic disorder might be a severe condition, that results in a contraindication for surgery, due to the frequent concomitant multi-organ impairment.

A 60-years-old male with granulomatosis with polyangiitis (GPA), involving lung and kidney severely, was admitted at our hospital due to chest pain. Five month before, the patient was hospitalized for asthenia and fever and bilateral bleeding lung abscess with hysthological evidence of necrotizing vasculitiis. Furthermore, the computed tomography (CT) scan at that time had shown a suspicion of aortitiis or intramural hematoma (IMH) of ascending aorta, that was treated conservatively, due to the severe lung involvement from the GPA. CT scan, at the time of presentation, showed a severe increase of the aortic wall thickness and a mediastinal hematoma. Therefore the patient was considered a surgical emergency. Due to the several clinical condition, open surgery was considered unaffordable, and, an hybrid solution was planned. Under general anesthesia, the vascular team initially carried out a complete retrograde debranching of supra-aortic vessels by an extra-anatomical bypass from left axillary to right axillary artery with re-implantation of both common carotid arteries; immediately afterwards, a 100 mm-long thoracic aortic stent-graft was deployed in the ascending aorta and right arch with rapid right ventricular pacing (RRVP); at the end, a 14 mm vascular plug was placed in the proximal segment of the innominate artery.

Post-operative imaging proved the efficacy of the hybrid emergency treatment, either surgical and endovascular. Due to a mild-moderate hyposthenia and hypoesthesia of the left arm, a brain CT-scan was done and a right confined ischemic perirolandic area was observed. The patient was dismissed in IX post-operative day. At four months follow-up, the patient was alive, no further complications were observed and he had partially regained motility and sensibility of the left hand.

In patients suffering from a severe and systemic inflammatory disease, as GPA, although the involvement of large vessels is very rare, it is appropriate to consider that chest or abdominal pain may results from aortic involvement. Due to the necrotizing characteristic of GPA vasculitiis, the risk of rupture appears extremely high and may lead to death. Furthermore, In those patients with high surgical risk, unfit for open aortic arch approach, it is mandatory to propose a less invasive alternative plan, as long as it is safe and effective.

1. D. Blockmans, H. Baeyens, R. Van Loon, G. Lauwers & H. Bobbaers. Periaortitis and Aortic Dissection due to Wegener's Granulomatosis. Clinical Rheumatology volume 19, pages161-164 (2000). 2. Shitrit D, Shitrit AB, Starobin D, Izbicki G, Belenky A, Kaufman N, et al. Large vessel aneurysms in Wegener's granulomatosis. J Vasc Surg. 2002;36(4):856-8. 3. Arlet JB MD, Le Thi Huong D MD, PhD, Marinho A MD, Cluzel P MD, Wechsler B MD e Piette JC MD. Arterial Aneurysms in Wegener's Granulomatosis: Case Report and Literature Review. Seminars in Arthritis and Rheumatism, 2008; 37(4):265-268. 4. Durai R, Agrawal R, Piper K, Brohi K. Wegener's granulomatosis presenting as an abdominal aortic aneurysm: a case report. Cases J. 2009;2:9346. 5. Unlu C, Willems M, Ten Berge IJ, Legemate DA. Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis. J Vasc Surg. 2011;54(5):1485-7. 6. Amos LA, Roberts MA, Blair S, McMahon LP. cANCA-associated aortitis. ClinKidney J. 2012;5(1):47-9. 7. Ohta N, Waki T, Fukase S, Suzuki Y, Kurakami K, Aoyagi M, Kakehata S. Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report. J Med Case Rep. 2013;7:202. 8. Roselli EE, Idrees J, Greenberg RK, Johnston DR, Lytle BW. Endovascular stent grafting for ascending aorta repair in high-risk patients. J Thorac Cardiovasc Surg. 2015 Jan;149(1):144-51. 9. Miyawaki M, Oda S, Hirata K, Yuki H, Utsunomiya D, Hayashi H, Sakamoto Y, Okamoto S, Fujii K, Yamashita Y. Granulomatosis with polyangiitis can cause periaortitis and pericarditis. Clin Case Rep. 2017;5(10):1732-3. 10. Niimi N, Miyashita T, Tanji K, Hirai T, Watanabe K, Ikeda K, Morimoto S, Sekigawa I. Aortic aneurysm as a complication of granulomatosis with Polyangiitis successfully treated with prednisolone and cyclophosphamide: a case report and review of the literature. Case Rep Rheumatol. 2018;2018:9682801. 11. Kim WK, Kim JB. Granulomatosis with Polyangiitis involving the ascending aorta. Ann Thorac Surg. 2018;106(1):e11-e3. 12. Lei Pan, Jun-Hong Yan, Fu-Quan Gao, Hong Li, Sha-Sha Han, Guo-Hong Cao, Chang-Jun Lv and Xiao-Zhi Wang. Case report of a 28-year-old man with aortic dissection and pulmonary shadow due to granulomatosis with polyangiitis (BMC Pulmonary Medicine (2019) 19:122).