Table 1.
Variable | Total | ANCA negative | ANCA positive |
---|---|---|---|
Patients, n (%) | 141 | 76 (54) | 65 (46) |
Study visits, n (%) | 892 | 490 (55) | 402 (45) |
Sex, female*, n (%) | 82 (58) | 53 (70) | 29 (45) |
Age at first observation, median (range), years | 55 (21–82) | 51 (21–82) | 57 (23–82) |
Race, Caucasian, n (%) | 131 (93) | 72 (95) | 59 (91) |
Disease duration at first study visit, median (range), years | 1.5 (0–29.8) | 1.6 (0–21.7) | 1.3 (0–29.8) |
Clinical manifestations (prevalence), % | |||
Asthma | 94 | 97 | 91 |
Cardiac | 23 | 27 | 19 |
Dermatological | 58 | 65 | 51 |
ENT | 89 | 90 | 87 |
Gastrointestinal | 18 | 23 | 13 |
Musculoskeletal | 55 | 56 | 54 |
Neurological | 69 | 63 | 76 |
Pulmonary (non-asthma) | 66 | 72 | 60 |
Renal* | 15 | 4 | 27 |
On prednisone* | |||
Study visits, n (%) | 579 (65) | 348 (71) | 231 (57) |
On other immunosuppressants, n (%) | |||
Study visits | 433 (49) | 248 (51) | 185 (46) |
AZA | 205 (23) | 114 (23) | 91 (23) |
CYC | 10 (1) | 3 (1) | 7 (2) |
MTX | 179 (21) | 90 (18) | 89 (22) |
On prednisone or other immunosuppressants, n (%) | 659 (74) | 376 (77) | 283 (70) |
BVAS/WG score, n (%) | 0: 766 (86) | 0: 414 (85) | 0: 352 (88) |
≥1: 99 (11) | ≥1: 56 (11) | ≥1: 43 (11) | |
≥3: 33 (4) | ≥3: 17 (3) | ≥3: 16 (4) | |
Eosinophil count, median (range), 103/mm | 262.0 (0–18 096) | 276.0 (0–7083) | 229.4 (0–18 096) |
IgE, median (range), mg/dl* | 60.0 (0–21 925) | 38.4 (0–21 925) | 85.0 (2–7298) |
ESR, median (range), mm/h | 8.0 (1–94) | 8.0 (1–72) | 8.0 (1–94) |
CRP, median (range), mg/l | 2.2 (0–203) | 2.0 (0–98) | 2.6 (0–203) |
*Indicates statistically significant differences (P < 0.05) between ANCA-positive and ANCA-negative subjects with EGPA. Categorical variables were assessed by Fisher’s exact test and continuous variables were assessed by the Wilcoxon rank sum test. BVAS/WG: BVAS for Wegener’s granulomatosis; EGPA: eosinophilic granulomatosis with polyangiitis.