Additional details about cases of relapsing polychondritis with extraaortic large-vessel vasculitis in our cohort and reported in the literature.
| Study | Pt. No. | Imaging for Vasculitis Diagnosis | Aortitis | Coronary Involvement | Large Arteries Vasculitis | Vascular Intervention | csDMARD | bDMARD |
|---|---|---|---|---|---|---|---|---|
| Rabuzzi (1970) | 1 | Angiography | — | — | Right CCA | — | — | — |
| Esdaile (1977) | 2 | Angiography | Ascending, abdominal | — | Bilateral RA and IA; IMA | — | AZA, CYC | — |
| Sohi (1981) | 3 | Angiography | Ascending, abdominal | — | Bilateral IA | Aortic aneurysm repair | — | — |
| Giordano (1984) | 4 | Angiography | Arch | — | BCT; left CCA; left SA | — | — | — |
| Bowness (1991) | 5 | Postmortem examination | — | Right and left CA | — | — | CYC | — |
| Yamazaki (2001) | 6 | MRA | — | — | Bilateral ICA | — | AZA | — |
| Barretto (2002) | 7 | CTA, angiography | Entire aorta | Left and circumflex CA | Bilateral IA, FA, and SA; left RA | Aortofemoral bypass | CSA, CYC, chlorambucil | — |
| Sasirekha (2006) | 8 | Angiography | Ascending | Right and left CA | — | CABG | * | — |
| Vaidyanathan (2006) | 9 | Angiography | Ascending | Right and left CA | — | — | — | — |
| Butterton (2007) | 10 | CTA | — | — | Left ICA and VA | — | MMF | — |
| Ghosn (2008) | 11 | CTA, angiography | Ascending | — | Right CCA and AA; bilateral SA | Aortic aneurysm repair | — | IFX |
| Stein (2008) | 12 | CTA, angiography | Ascending | Right and left CA | — | CABG | MTX, CYC | IFX |
| McCarthy (2009) | 13 | Angiography | — | Right and left CA | — | CABG | MTX, AZA, CSA | IFX, RTX |
| Sugrue (2014) | 14 | CTA, PET, angiography | Ascending | Left CA | — | PTCA | MTX | IFX |
| Malik (2015) | 15 | Doppler US | — | — | Bilateral CCA, VA, SA | — | — | — |
| Karakaya (2016) | 16 | MRA | — | — | Bilateral ICA; left VA; right SA | — | — | — |
| Subhadarshani (2017) | 17 | CTA | Arch | — | Bilateral SA; left CCA; BCT | — | CYC, AZA | — |
| Our cohort | 18 | MRA, PET | Abdominal | — | SMA; CT; bilateral IA | Aneurysm repair + aortofemoral bypass | AZA, MTX | GOL, TCZ |
| 19 | MRA | Ascending, arch | — | Left CCA and SA; BCT; CT | — | AZA | — | |
| 20 | Angiography, MRA | Ascending, abdominal | Left CA | Bilateral CCA; left IA | Aortic aneurysm repair; coronary PTA | CSA, MTX, CYC | ANK, TCZ | |
| 21 | Angiography, US | — | Right and left CA | Bilateral RA; CT; SMA | CABG, Renal PTA | AZA, MMF, MTX | ETN, IFX, ADA, TCZ, ANK |
↵* Diaminodiphenylsulfone. csDMARD: conventional synthetic disease-modifying antirheumatic drug; bDMARD: biological DMARD; AA: axillary artery; BCT: brachiocephalic trunk; CA: coronary artery; CCA: common carotid artery; MRA: magnetic resonance angiography; CT: celiac trunk; FA: femoral artery; IA: iliac artery; ICA: internal carotid artery; IMA: inferior mesenteric artery; RA: renal artery; AZA: azathioprine; CYC: cyclophosphamide; SA: sub clavian artery; VA: vertebral artery; CTA: computed tomography; CSA: cyclosporine; CABG: coronary artery bypass grafting; MMF: mycophenolate mofetil; IFX: infliximab; MTX: methotrexate; RTX: rituximab; PET: positron emission tomography; PTCA: percutaneous transluminal coronary angioplasty; US: ultra-sound; SMA: superior mesenteric artery; GOL: golimumab; TCZ: tocilizumab; PTA: percutaneous transluminal angioplasty; ANK: anakinra; ETN: etanercept; ADA: adalimumab.