Table 1.
Demographics and baseline clinical characteristics of patients at the time of diagnosis or relapse
| AAV-specific variables | Number of subjects | Entire cohort |
United States |
United Kingdom |
Europe |
P value |
|---|---|---|---|---|---|---|
| n = 191 | n = 44 | n = 83 | n = 64 | |||
| Age at diagnosis | Mean years (SD) | 65 (14) | 68 (12) | 65 (15) | 61 (13) | 0.038 |
| Sex, n (%) | Female | 99 (52) | 22 (50) | 40 (48) | 27 (42) | 0.493 |
| Race, n (%) | White | 170 (89) | 38 (86) | 68 (82) | 64 (100) | <0.001 |
| Black | 5 (3) | 4 (9) | 1 (1) | 0 | ||
| Asian | 16 (8) | 2 (5) | 14 (17) | 0 | ||
| Diagnosis, n (%) | GPA | 97 (51) | 25 (57) | 43 (52) | 29 (45) | 0.09 |
| MPA | 84 (44) | 19 (43) | 32 (39) | 33 (52) | ||
| EGPA | 10 (5) | 0 | 8 (10) | 2 (3) | ||
| ANCA type, n (%) | PR3 | 90 (47) | 17 (40) | 42 (51) | 30 (47) | 0.366 |
| MPO | 94 (49) | 25 (58) | 36 (43) | 33 (52) | ||
| Negative | 7 (4) | 1 (2) | 5 (6) | 1 (2) | ||
| Organs involved during study period, n (%) | Kidney | 155 (81) | 41 (93) | 61 (73) | 53 (83) | 0.024 |
| Lung | 96 (50) | 19 (43) | 44 (53) | 33 (52) | 0.555 | |
| Sinus | 44 (23) | 7 (16) | 26 (31) | 11 (17) | 0.058 | |
| Eyes | 13 (7) | 2 (5) | 9 (11) | 2 (3) | 0.146 | |
| Ears | 12 (6) | 3 (7) | 7 (8) | 2 (3) | 0.415 | |
| Peripheral nerve | 20 (10) | 6 (14) | 8 (10) | 6 (9) | 0.736 | |
| Joints | 31 (16) | 3 (7) | 9 (11) | 19 (30) | 0.001 | |
| Skin | 22 (12) | 4 (9) | 10 (12) | 8 (13) | 0.845 | |
| Cardiac | 8 (4) | 1 (2) | 7 (8) | 0 | 0.031 | |
| Gastrointestinal | 3 (2) | 0 | 0 | 3 (5) | 0.049 | |
| Underwent kidney biopsy, n (%) | n = 155 patients with kidney disease on entry | 111 (72) | 35 (85); n = 41 | 44 (73); n = 61 | 32 (60); n = 53 | 0.027 |
| Proportion of patients presenting in relapse, n (%) | 59 (31) | 6 (14) | 31 (37) | 22 (34) | 0.017 | |
| Comorbidities, n (%) | Diabetes | 27 (14) | 5 (11) | 13 (16) | 9 (14) | 0.803 |
| Hypertension | 111 (58) | 30 (68) | 42 (51) | 39 (61) | 0.139 | |
| Heart disease | 35 (18) | 13 (30) | 9 (11) | 13 (20) | 0.031 | |
| Lung disease | 46 (24) | 11 (25) | 20 (24) | 15 (23) | 0.983 | |
| Cerebrovascular disease | 9 (5) | 4 (9) | 2 (2) | 3 (5) | 0.239 | |
| Chronic kidney disease | 52 (27) | 16 (36) | 19 (23) | 17 (27) | 0.265 | |
| Cancer | 18 (9) | 8 (18) | 4 (5) | 6 (9) | 0.049 | |
| No comorbidities | 52 (27) | 8 (18) | 24 (29) | 20 (31) | 0.292 | |
| Alveolar hemorrhage, n (%) | 28 (15) | 8 (18) | 10 (12) | 10 (16) | 0.626 | |
| Dialysis on admission, n (%) | n = 155 patients with kidney disease on entry | 33 (21) | 12 (29); n = 41 | 11 (18); n = 61 | 10 (19); n = 53 | 0.345 |
| GFR (ml/min per 1.73 m2) at baseline | Mean (SD) | 34 (31); n = 189 | 21 (28); n = 44 | 41 (32); n = 83 | 35 (31); n = 62 | 0.0027 |
| Creatinine (mg/dl) | Mean (SD) | 3.46 (2.97); n = 189 | 4.81 (3.27); n = 44 | 2.91 (2.7); n = 83 | 3.2 (2.8); n = 62 | 0.0019 |
AAV, antibody-associated vasculitis; ANCA, anti-neutrophil cytoplasmic autoantibody; EGPA, eosinophilic granulomatosis with polyangiitis; GFR, glomerular filtration rate; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; MPO, myeloperoxidase; PR3, proteinase 3.