Table 1.
Clinical characteristics of patients with VEXAS-RP compared with I-RP (n=95)
| I-RP | VEXAS-RP | P value | |
| N | 40 | 55 | |
| Male gender (%) | 12 (30) | 53 (96) | <0.001 |
| Age at diagnosis of RP (median (IQR)) (years) | 44(38, 52) | 66(61, 72) | <0.001 |
| Fever (%) | 4 (10) | 33 (60) | <0.001 |
| Chondritis (%) | 40 (100) | 52 (98) | 1 |
| Ear chondritis (%) | 36 (90) | 50 (94) | 0.698 |
| Nasal chondritis (%) | 28 (70) | 25 (47) | 0.047 |
| Ocular involvement (%) | 11 (28) | 30 (57) | 0.01 |
| Uveitis (%) | 2 (5) | 9 (17) | 0.148 |
| Scleritis (%) | 4 (10) | 7 (13) | 0.881 |
| Episcleritis (%) | 6 (15) | 15 (28) | 0.205 |
| Retinal vasculitis (%) | 1 (3) | 2 (4) | 1 |
| Peripheral joint involvement (%) | 27 (68) | 36 (67) | 1 |
| Costochondritis (%) | 10 (25) | 3 (12) | 0.339 |
| Skin lesions (%) | 8 (20) | 44 (82) | <0.001 |
| Large airway chondritis (%) | 18 (45) | 13 (25) | 0.064 |
| Pulmonary infiltrates (%) | 0 (0) | 13 (46) | <0.001 |
| Heart involvement (%) | 0 (0) | 6 (11) | 0.0336 |
| Myocarditis (%) | 0 (0) | 3 (6) | 0.349 |
| Pericarditis (%) | 0 (0) | 3 (6) | 0.349 |
| Mitral or aortic valvular disease (%) | 2 (5) | 0 (0) | 0.691 |
| Arterial involvement | |||
| Arterial thrombosis (%) | 0 (0) | 1 (4) | 0.811 |
| Aortitis (%) | 2 (5) | 3 (6) | 1 |
| Venous thrombosis (%) | 8 (20) | 14 (26) | 0.635 |
| MDS (%) | 0 (0) | 41 (75) | <0.001 |
| Renal failure (%) | 0 (0) | 4 (7) | 0.22 |
| Central nervous system involvement (%) | 1 (3) | 3 (6) | 0.835 |
| Vestibular dysfunction (%) | 3 (8) | 2 (8) | 1 |
| Deafness sensorineural (%) | 6 (15) | 4 (16) | 1 |
| Laboratory data | |||
| Haemoglobin (median (IQR)) (g/L) | 137(130, 140) | 103(90, 120) | <0.001 |
| Platelets (median (IQR)) ( ) | 257(209, 303) | 163(115, 236) | <0.001 |
| Neutrophils (median (IQR)) (G/L) | 4(3, 5) | 2.7(2, 4) | 0.018 |
| C-reactive protein (median (IQR)) (mg/L) | 10(2, 23) | 69(30, 107) | <0.001 |
| Treatment data | |||
| Systemic glucocorticoids (%) | 35 (88) | 51 (94) | 0.413 |
| Glucocorticoid-dependency (%) | 24 (62) | 37 (71) | 0.459 |
| Number of immunosuppressive medications (median (IQR)) | 2(1, 3) | 1(1, 3) | 0.85 |
| Remission (%) | 36 (90) | 15 (27) | <0.001 |
| Time to remission (median (IQR)) (months) | 19(11, 49) | 30(12, 69) | 0.387 |
| Duration of remission (median (IQR)) (months) | 24(4, 76) | 7(3, 21) | 0.067 |
| Relapse (%) | 14 (39) | 7 (50) | 0.692 |
| Death (%) | 0 (0) | 6 (11) | 0.083 |
| Duration of follow-up (median (IQR)) (months) | 92(37, 160) | 37(15, 76) | 0.001 |
I-RP, idiopathic RP; MDS, myelodysplastic syndromes; RP, relapsing polychondritis.