Summary of the cases of relapsing polychondritis (RP) with extraaortic large-vessel vasculitis in our cohort and reported in the literature.
| Study | Pt. No. | Age at RP Onset, yrs/Sex | Sites of Chondritis | Other Clinical Features | Vasculitis Diagnosis | Temporal Delay, mos | First Vascular Sign/symptom |
|---|---|---|---|---|---|---|---|
| Rabuzzi (1970) | 1 | 25/F | Nose | Arthritis, episcleritis | Concomitant | — | Headache, ischemic stroke |
| Esdaile (1977) | 2 | 46/M | Ear, costosternal | Episcleritis, iritis, orbital pseudotumor | Subsequent | 36 | Angina abdominis |
| Sohi (1981) | 3 | 19/F | Nose, trachea | Keratitis | Subsequent | 252 | Heart failure |
| Giordano (1984) | 4 | 28/F | Nose, ear, trachea | Arthritis, episcleritis | Subsequent | 204 | Vascular bruits |
| Bowness (1991) | 5 | 33/M | Ear, nose, larynx | Arthritis | Subsequent | 5 | Heart failure |
| Yamazaki (2001) | 6 | 59/F | Ear, nose | — | Subsequent | 11 | Headache |
| Barretto (2002) | 7 | 42/M | Ear | Sensorineural deafness | Subsequent | 72 | Lower limb ischemia |
| Sasirekha (2006) | 8 | 24/F | Ear, nose | Arthritis | Subsequent | 12 | Angina pectoris |
| Vaidyanathan (2006) | 9 | 24/F | Ear, nose | — | Subsequent | 24 | Heart failure |
| Butterton (2007) | 10 | 59/M | Ear, nose | Periorbital inflammation | Concomitant | — | Carotidynia |
| Ghosn (2008) | 11 | 7/F | Ear, nose | Pyoderma gangrenosum | Concomitant | — | — |
| Stein (2008) | 12 | 30/M | Ear, nose | Uveitis | Subsequent | 60 | Angina pectoris, heart failure |
| McCarthy (2009) | 13 | 29/M | Ear, nose | Arthritis, sensorineural deafness | Subsequent | 192 | Angina pectoris, heart failure |
| Sugrue (2014) | 14 | 51/M | Ear | Arthritis, oral ulcers | Subsequent | 12 | Heart failure |
| Malik (2015) | 15 | 35/F | Ear, nose | Arthritis, ischemic optic neuropathy | Concomitant | — | Ocular ischemic syndrome |
| Karakaya (2016) | 16 | 26/F | Ear, trachea | — | Concomitant | — | Upper limb numbness |
| Subhadarshani (2017) | 17 | 14/F | Ear, nose | Pyoderma gangrenosum | Subsequent | 96 | Lower limb ischemia |
| Our cohort | 18 | 25/F | Ear, nose | Oral ulcers | Subsequent | 50 | Lower limbs claudication |
| 19 | 37/F | Ear, nose | — | Subsequent | 36 | Ischemic stroke | |
| 20 | 20/F | Ear, nose | Erythema nodosum | Subsequent | 108 | — | |
| 21 | 20/M | Ear, nose | Arthritis, episcleritis, pyoderma gangrenosum | Subsequent | 204 | Arterial hypertension, angina pectoris |