Demographic data, clinical manifestations, and laboratory data at AAV diagnosis.
| Variables | Total, n = 132 | GPA, n = 51 | MPA, n = 52 | EGPA, n = 29 | p |
|---|---|---|---|---|---|
| Epidemiological data | |||||
| Sex (male/female) | 63/69 | 27/24 | 25/27 | 11/18 | 0.043 |
| Age at diagnosis, yrs, median (IQR) | 57.5 (40.3–68.6) | 38.0 (32.0–56.0) | 67.5 (58.0–75.7) | 55.0 (49.0–67.5) | < 0.001 |
| ANCA-positive | 112 (85) | 42 (82) | 51 (98) | 19 (65) | < 0.001 |
| PR3-ANCA | 39 (30) | 36 (71) | 2 (4) | 1 (3) | < 0.001 |
| MPO-ANCA | 73 (55) | 6 (12) | 49 (94) | 18 (62) | < 0.001 |
| BVAS v.3 | 16 (9–22), median (IQR) | 16.1 ± 1.0, mean ± SEM | 17.1 ± 0.9, mean ± SEM | 15.2 ± 1.1, mean ± SEM | 0.440 |
| FFS 1996, median (IQR) | 0 (0–1) | 0 (0–1) | 1 (0–2) | 0 (0–0) | < 0.05 |
| FFS 2009, median (IQR) | 1 (0–2) | 1 (0–1) | 2 (2–3) | 1 (0–1) | < 0.001 |
| Clinical manifestations | |||||
| Toxic syndrome | 101 (77) | 35 (69) | 44 (85) | 22 (76) | 0.172 |
| Fever | 84 (64) | 28 (55) | 34 (65) | 22 (76) | 0.188 |
| ENT involvement | 62 (47) | 42 (84) | 2 (4) | 18 (62) | < 0.05 |
| Nasal crusting | 28 (21) | 25 (49) | 0 (0) | 3 (10) | < 0.001 |
| Septal perforation | 2 (2) | 2 (4) | 0 (0) | 0 (0) | 0.316 |
| Otitis media | 24 (18) | 19 (37) | 0 (0) | 5 (17) | < 0.05 |
| Paranasal sinus involved | 44 (33) | 27 (53) | 2 (4) | 15 (52) | < 0.001 |
| Subglottic stenosis | 5 (4) | 5 (10) | 0 (0) | 0 (0) | 0.06 |
| Pulmonary infiltrates | 60 (45) | 16 (31) | 24 (46) | 20 (69) | 0.003 |
| Lung nodules | 27 (20) | 23 (45) | 2 (4) | 2 (7) | < 0.001 |
| Alveolar hemorrhage | 24 (18) | 8 (16) | 15 (29) | 1 (3) | 0.013 |
| Acute renal failure | 61 (46) | 20 (39) | 40 (77) | 1 (3) | < 0.001 |
| Pulmonary-renal syndrome | 20 (15) | 7 (14) | 14 (27) | 0 (0) | 0.004 |
| Nephrotic syndrome | 12 (9) | 5 (10) | 7 (14) | 0 (0) | 0.126 |
| Neurologic affection | 50 (38) | 8 (16) | 20 (38) | 22 (76) | < 0.001 |
| Peripheral neuropathy | 11 (8) | 0 (0) | 8 (15) | 3 (10) | 0.026 |
| Mononeuritis multiplex | 39 (30) | 8 (16) | 12 (23) | 19 (66) | < 0.001 |
| Central nervous system | 12 (9) | 9 (18) | 2 (4) | 1 (3) | < 0.05 |
| Stroke | 7 (5) | 5 (10) | 2 (4) | 0 (0) | 0.184 |
| Aseptic meningitis | 5 (4) | 5 (10) | 0 (0) | 0 (0) | 0.026 |
| Optical neuritis | 1 (1) | 0 (0) | 0 (0) | 1 (3) | NS |
| Myocarditis | 3 (2) | 0 (0) | 0 (0) | 3 (10) | NS |
| Pericarditis | 3 (2) | 2 (4) | 1 (2) | 0 (0) | NS |
| Intestinal ischemia | |||||
| Ulcers | 8 (6) | 5 (10) | 2 (4) | 1 (3) | NS |
| Perforation | 2 (2) | 2 (4) | 0 (0) | 0 (0) | NS |
| Laboratory data, mean ± SEM | |||||
| Hemoglobin, g/dl | 10.7 ± 0.2 | 11.2 ± 0.3 | 9.6 ± 0.2 | 12.2 ± 0.3 | < 0.001 |
| Leukocytes, × 109/l | 12.96 ± 5.62 | 11.87 ± 8.59 | 11.81 ± 5.91 | 16.94 ± 1.59 | 0.002 |
| ESR, mmh−1 | 82 ± 3 | 82 ± 5 | 91 ± 3 | 64 ± 5 | 0.016 |
| Creatinine, mg/dl | 2.11 ± 0.22 | 1.62 ± 0.22 | 3.30 ± 0.46 | 0.85 ± 0.03 | < 0.001 |
| Proteinuria, mg/day | 925 ± 131 | 874 ± 218 | 1379 ± 234 | 179 ± 29 | < 0.001 |
Values are n (%) unless otherwise indicated. ANCA: antineutrophil cytoplasmic antibodies; AAV: ANCA-associated vasculitis; BVASv.3: Birmingham Vasculitis Activity Score version 3; ESR: erythrocyte sedimentation rate; FFS: Five Factor Score; GPA: granulomatosis with polyangiitis; EGPA: eosinophilic GPA; MPA: microscopic polyangiitis; MPO: myeloperoxidase; PR3: proteinase 3; IQR: interquartile range; SEM: standard error of the mean.