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  - 2019 Dec 15
TA  - The Journal of Rheumatology
PG  - jrheum.190862
4099  - http://www.jrheum.org/content/early/2019/12/09/jrheum.190862.short
4100  - http://www.jrheum.org/content/early/2019/12/09/jrheum.190862.full
AB  - Objective To describe the features of large vessel vasculitis (LVV) affecting the aorta and its branches in relapsing polychondritis (RP) patients. Methods Retrospective data and systematic literature review. Results 21 patients were identified. LVV diagnosis was subsequent to RP and associated with extra-chondral involvement in the majority of patients. Supra-aortic vessels were more frequently involved (82%). 14 (67%) patients were treated with a csDMARD and 7 (33%) with a bDMARD. Vascular interventional procedures were performed in 10 (48%) patients. Prematurely death due to cardiovascular complications was reported in 3 (14%) cases. Conclusion Extra-aortic LVV is a serious and overlooked RP manifestation. All RP patients should be investigated for LVV.