RT Journal Article
SR Electronic
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
FD The Journal of Rheumatology
SP 1780
OP 1784
DO 10.3899/jrheum.190862
VO 47
IS 12
A1 Tomelleri, Alessandro
A1 Campochiaro, Corrado
A1 Sartorelli, Silvia
A1 Papa, Maurizio
A1 De Luca, Giacomo
A1 Cavalli, Giulio
A1 Baldissera, Elena
A1 Dagna, Lorenzo
YR 2020
UL http://www.jrheum.org/content/47/12/1780.abstract
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.