PT  - JOURNAL ARTICLE
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
TI  - Large-vessel Vasculitis Affecting the Aorta and its Branches in Relapsing Polychondritis: Case Series and Systematic Review of the Literature
AID  - 10.3899/jrheum.190862
DP  - 2020 Dec 01
TA  - The Journal of Rheumatology
PG  - 1780--1784
VI  - 47
IP  - 12
4099  - http://www.jrheum.org/content/47/12/1780.short
4100  - http://www.jrheum.org/content/47/12/1780.full
SO  - J Rheumatol2020 Dec 01; 47
AB  - 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.