TY - JOUR T1 - Large-vessel Vasculitis Affecting the Aorta and its Branches in Relapsing Polychondritis: Case Series and Systematic Review of the Literature JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.190862 SP - jrheum.190862 AU - Alessandro Tomelleri AU - Corrado Campochiaro AU - Silvia Sartorelli AU - Maurizio Papa AU - Giacomo De Luca AU - Giulio Cavalli AU - Elena Baldissera AU - Lorenzo Dagna Y1 - 2019/12/15 UR - http://www.jrheum.org/content/early/2020/05/22/jrheum.190862.abstract N2 - Objective To describe the features of large-vessel vasculitis (LVV) as it affects the aorta and its branches in patients with relapsing polychondritis (RP). Methods Retrospective data and systematic literature review. Results Twenty-one patients were identified. LVV diagnosis was subsequent to RP and associated with extrachondral involvement in the majority of patients. Supraaortic vessels were more frequently involved (82%). Fourteen patients (67%) were treated with a conventional synthetic disease-modifying antirheumatic drug (csDMARD) and 7 (33%) with a biological DMARD (bDMARD). Vascular interventional procedures were performed in 10 patients (48%). Premature death due to cardiovascular complications was reported in 3 cases (14%). Conclusion Extraaortic LVV is a serious and overlooked RP manifestation. All patients with RP should be investigated for LVV. ER -