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Abstract
Background: Arterial aneurysms are one of the unique features of Behçet’s disease (BD). Although arterial aneurysms are relatively rare, they carry risk of rupture and have poor prognosis.
Objectives: To evaluate the medical and interventional therapies and long-term outcomes of BD patients with extracranial arterial aneurysms.
Methods: We retrospectively reviewed the medical records of 441 BD patients according to ISG criteria between 2013 and 2018 at the Hacettepe University Vasculitis Center (HUVAC). We determined 45 BD patients with an arterial aneurysm. Six patients with isolated carotid and/or cranial arterial aneurysms who were followed by other clinics excluded from the analysis. Overall, 39 BD patients with an extracranial and extra carotidal aneurysm included in the study. Data regarding demographic characteristics, clinical, laboratory and vascular imaging findings, history of medical treatments and outcomes were collected. Vascular intervention of patients were grouped as surgery or endovascular intervention. Radiological response after vascular intervention divided into 3 groups as regression, stable and progression according to radiological evaluation. Regression was defined as a decrease or disappearance of the aneurysm. Stable was defined as no change in the aneurysm diameter. Progression was defined as an increase in diameter of a previous aneurysm or occurrence of a new vascular event. We colligated the stable and regression groups and compared with the progression group. Relapse was defined the progression of the aneurysm which underwent an intervention.
Results: A total of 39 BD patients (Male, 80%) with an arterial aneurysm were analyzed in this study. Mean age and mean age of diagnosis patients were 40.9±11.0 and 29.7±7.7 years, respectively. The clinical features of BD was as follows; oral ulcer 100%, genital ulcer 80.6%, acneiform lesion 47.2%, erythema nodosum 33.3%, pathergy positivity 34.5%, arthritis 27.8%, fever 24.3%, uveitis 34.5%, neurological involvement 30.6% and, gastrointestinal involvement 7.7%. Distribution of arterial aneurysms were 5.1% subclavian, 5.1% coronary artery & cardiac, 38.8% pulmonary, 10.3% thoracic aorta, 33.3% abdominal aorta, 5.1% renal, 25.6% iliac and 12.8% femoral. Induction and maintenance treatments are shown in figure 1. In our cohort, 25 (64%) patients needed a vascular intervention, including 9 (23%) open operations and 16 (41%) endovascular interventions. Of 7 (18%) patients had to perform an urgent open operation or endovascular intervention but we didn’t find any relation between the progression of an aneurysm and urgent intervention. First induction treatment options and prognosis of arterial aneurysms shown in figure 2. At median 71.8 months follow up, 7 (18%) patient died in the BD aneurysm group. The rate of progression of the aneurysm was 20% in BD patients with pulmonary artery involvement and 64% in BD patients with aortic and its branch involvement (p=0.038). In the absence of medical induction treatment, 5 patients had progression, 1 had regression and 1 had no follow-up.
Conclusion: Arterial aneurysms in BD have poor prognosis and high mortality. Glucocorticoids, cyclophosphamide and interferon-α are the most preferred treatments for induction and maintenance therapy for aneurysm of the BD. Approximately 2/3 of the patients required a vascular intervention. Clinical and radiological progression was more prominent in the aorta and its branches. As expected, there is more progression in interventional procedures without medical treatment.
Disclosure of Interests: Berkan-Armagan: None declared, Ertuğrul Çağrı Bölek: None declared, Alper Sarı: None declared, Gözde Kübra Yardımcı: None declared, Bayram Farisoğulları: None declared, Emre Bilgin: None declared, Fatma Gonca Eldem: None declared, Levent Kılıç: None declared, Omer Karadag: None declared, Bora Peynircioğlu: None declared, Barbaros Erhan Çil: None declared, Metin Demircin: None declared, Ali Akdoğan: None declared, Şule Apraş Bilgen: None declared, Ali İhsan Ertenli: None declared, Sedat Kiraz: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim