Table 1.
Main clinical characteristics and treatments at diagnosis of 727 French Vasculitis Study Group Registry GPA patients according to ANCA specificity
| Characteristic | Overall n=727 | P value (PR3-ANCA+ vs MPO-ANCA+ vs ANCA–) | No ANCA n=62 | P value (ANCA– vs PR3-ANCA+ and MPO-ANCA+) | MPO-ANCA+ n=119 | P value (MPO-ANCA+ vs PR3-ANCA+ and ANCA–) | PR3-ANCA+ n=546 | P value (PR3-ANCA+ vs MPO-ANCA+ and ANCA–) |
| Sex, female | 319 (43.9) | 0.082 | 28 (45.2) | 0.94 | 63 (52.9) | 0.04 | 228 (41.8) | 0.06 |
| Age at diagnosis, years, mean (SD) | 53 (16.3) | <0.001 | 52.5 (16.5) | 0.79 | 59.8 (17.2) | <0.001 | 51.6 (15.7) | <0.001 |
| Limited GPA | 50 (6.9) | 0.002 | 11 (17.7) | 0.001 | 8 (6.7) | 0.55 | 31 (5.7) | 0.04 |
| Severe GPA | 83 (11.4) | 0.08 | 2 (3.2) | 0.09 | 17 (14.3) | 0.71 | 64 (11.7) | 0.75 |
| General | 592 (81.4) | 0.83 | 51 (82.3) | 0.99 | 99 (83.2) | 0.70 | 442 (81) | 0.66 |
| Fever >38.5°C | 304 (41.8) | 0.05 | 23 (37.1) | 0.51 | 39 (32.8) | 0.04 | 242 (44.3) | 0.02 |
| Weight loss >3 kg within 3 months | 329 (45.3) | 0.16 | 21 (33.9) | 0.08 | 53 (44.5) | 0.94 | 255 (46.7) | 0.20 |
| Arthralgias | 376 (51.7) | <0.001 | 27 (43.5) | 0.23 | 41 (34.5) | <0.001 | 308 (56.4) | <0.001 |
| Myalgias | 197 (27.1) | 0.30 | 15 (24.2) | 0.70 | 39 (32.8) | 0.16 | 143 (26.2) | 0.39 |
| Ear, nose and throat | 582 (80.1) | 0.53 | 49 (79.0) | 0.96 | 91 (76.5) | 0.35 | 442 (81) | 0.35 |
| Sinusitis | 297 (40.9) | 0.44 | 28 (45.2) | 0.56 | 43 (36.1) | 0.30 | 226 (41.4) | 0.67 |
| Rhinitis | 390 (53.6) | 0.28 | 29 (46.8) | 0.32 | 59 (49.6) | 0.38 | 302 (55.3) | 0.14 |
| Nasal crusts | 276 (38) | 0.10 | 16 (25.8) | 0.05 | 44 (37) | 0.05 | 216 (39.6) | 0.15 |
| Epistaxis | 138(19) | 0.04 | 7 (11.3) | 0.15 | 16 (13.4) | 0.12 | 115 (21.1) | 0.02 |
| Saddle nose | 9 (1.2) | 0.06 | 0 (0) | 0.75 | 4 (3.4) | 0.07 | 5 (0.9) | 0.33 |
| Nasal obstruction | 183 (25.2) | 0.64 | 18 (29.0) | 0.56 | 27 (22.7) | 0.57 | 138 (25.3) | 0.99 |
| Otitis | 166 (22.8) | 0.32 | 11 (17.7) | 0.40 | 23 (19.3) | 0.38 | 132 (24.2) | 0.16 |
| Lung | 492 (67.7) | 0.35 | 37 (59.7) | 0.21 | 83 (69.7) | 0.67 | 372 (68.1) | 0.72 |
| Alveolar haemorrhage | 130 (17.9) | 0.21 | 7 (11.3) | 0.21 | 26 (21.8) | 0.27 | 97 (17.8) | 0.98 |
| Massive alveolar haemorrhage and/or Hb <90 g/L | 35 (4.8) | 0.16 | 1 (1.6) | 0.36 | 3 (2.5) | 0.30 | 31 (5.7) | 0.09 |
| Lung nodules | 303 (41.7) | 0.61 | 25 (40.3) | 0.93 | 45 (37.8) | 0.41 | 233 (42.7) | 0.39 |
| Lung infiltrate | 134 (18.4) | 0.44 | 6 (9.7) | 0.18 | 18 (15.1) | 0.29 | 62 (11.4) | 0.58 |
| Subglottic stenosis | 11 (1.5) | 0.07 | 3 (4.8) | 0.09 | 2 (1.7) | 1.00 | 6 (1.1) | 0.22 |
| Kidney | 414 (56.9) | <0.001 | 22 (35.5) | 0.001 | 78 (65.5) | <0.05 | 314 (57.5) | 0.66 |
| Serum creatinine median (µmol/L) | 93 (73–177) | <0.001 | 77 (63–100) | <0.001 | 113 (78–199) | <0.001 | 92 (74–174.5) | <0.001 |
| Serum creatinine >150 µmol/L | 162/563 (28.8) | 0.06 | 7/42 (16.7) | 0.10 | 34/94 (36.2) | 0.11 | 121/427 (28.3) | 0.77 |
| Rise in creatinine level >30% | 178 (24.5) | <0.05 | 9 (14.5) | 0.08 | 37 (31.1) | 0.09 | 132 (24.2) | 0.81 |
| Proteinuria | 256 (35.2) | 0.004 | 10 (16.1) | 0.002 | 45 (37.8) | 0.59 | 201 (36.8) | 0.14 |
| Haematuria | 280 (38.5) | <0.001 | 9 (14.5) | 0.001 | 54 (45.4) | 0.11 | 217 (39.7) | 0.27 |
| Need for dialysis | 41 (5.6) | 0.10 | 0 (0) | 0.09 | 9 (7.6) | 0.48 | 32 (5.9) | 0.79 |
| Mucocutaneous | 232 (31.9) | 0.14 | 17 (27.4) | 0.52 | 30 (25.2) | 0.11 | 185 (33.9) | 0.06 |
| Purpura | 114 (15.7) | 0.01 | 6 (9.7) | 0.24 | 10 (8.4) | 0.02 | 98 (17.9) | 0.005 |
| Livedo reticularis | 26 (3.6) | 0.49 | 1 (1.6) | 0.61 | 6 (5.0) | 0.50 | 19 (3.5) | 0.99 |
| Gangrene | 16 (2.2) | 0.33 | 3 (4.8) | 0.30 | 2 (1.7) | 0.94 | 11 (2.0) | 0.76 |
| Eye | 195 (26.8) | 0.07 | 19 (30.6) | 0.58 | 22 (18.5) | 0.03 | 154 (28.2) | 0.17 |
| Exophthalmos | 24 (3.3) | 0.83 | 2 (3.2) | 1.00 | 5 (4.2) | 0.75 | 17 (3.1) | 0.80 |
| Episcleritis | 67 (9.2) | 0.13 | 4 (6.5) | 0.58 | 6 (5.0) | 0.12 | 57 (10.4) | 0.07 |
| Scleritis | 30 (4.1) | 0.16 | 1 (1.6) | 0.48 | 2 (1.7) | 0.22 | 27 (4.9) | 0.09 |
| Cardiovascular | 115 (15.8) | 0.53 | 7 (11.3) | 0.40 | 21 (17.6) | 0.65 | 87 (15.9) | 0.98 |
| Pericarditis | 31 (4.3) | 0.21 | 0 (0) | 0.16 | 5 (4.2) | 1.00 | 26 (4.8) | 0.35 |
| Myocarditis | 9 (1.2) | 0.34 | 1 (1.6) | 1.00 | 3 (2.5) | 0.35 | 5 (0.9) | 0.33 |
| Congestive heart failure | 10 (1.3) | 0.49 | 0 (0) | 0.77 | 1 (0.8) | 0.35 | 9 (1.6) | 0.47 |
| Gastrointestinal | 76 (10.5) | 0.55 | 5 (8.1) | 0.67 | 10 (8.4) | 0.53 | 61 (11.2) | 0.34 |
| Abdominal pain | 41 (5.6) | 0.47 | 2 (3.2) | 0.57 | 9 (7.6) | 0.44 | 30 (5.5) | 0.91 |
| Perforation | 2 (0.3) | 0.042 | 0 (0) | 1.00 | 1 (0.8) | 0.74 | 1 (0.2) | 1.00 |
| Neurological | 215 (29.6) | 0.79 | 16 (25.8) | 0.59 | 35 (29.4) | 1.00 | 164 (30.0) | 0.70 |
| Central nervous system | 21 (2.9) | 0.18 | 4 (6.5) | 0.18 | 2 (1.7) | 0.58 | 15 (2.7) | 0.89 |
| Peripheral neuropathy | 143 (19.7) | 0.06 | 5 (8.1) | 0.025 | 25 (21.0) | 0.78 | 113 (20.7) | 0.27 |
| BVAS at diagnosis, mean (SD) | 17.5 (8.8) | 0.001 | 13.3 (8.1) | <0.001 | 18.2 (8.6) | 0.36 | 17.8 (8.7) | 0.10 |
| Histology supporting GPA diagnosis | 324 (44.6) | 0.935 | 29 (46.8) | – | 53 (44.5) | – | 242 (44.3) | – |
| Intravenous cyclophosphamide | 561 (77.2) | <0.001 | 35 (56.5) | <0.001 | 86 (72.3) | 0.20 | 440 (80.6) | <0.001 |
| Oral cyclophosphamide | 41 (5.6) | 0.001 | 10 (16.1) | 0.001 | 6 (5.0) | 0.93 | 25 (4.6) | <0.05 |
| Rituximab | 50 (6.9) | 0.66 | 3 (4.8) | 0.69 | 10 (8.4) | 0.60 | 37 (6.8) | 0.99 |
| Methotrexate | 33 (4.5) | 0.13 | 6 (9.7) | 0.09 | 5 (4.2) | 1.00 | 22 (4.0) | 0.35 |
| Glucocorticoids | 709 (97.5) | 0.10 | 58 (93.5) | 0.09 | 116 (97.5) | 1.00 | 535 (98) | 0.27 |
| Glucocorticoid, mg/day mean (SD) | 60 (50–70) | 0.006 | 50.2 (29.7) | 0.05 | 52.0 (23.0) | 0.03 | 59.1 (21.4) | 0.002 |
| Methylprednisolone pulse | 208 (28.6) | 0.06 | 10 (16.1) | 0.03 | 39 (32.8) | 0.33 | 159 (29.1) | 0.66 |
| Plasma exchanges | 57 (7.8) | 0.21 | 2 (3.2) | 0.24 | 7 (5.9) | 0.50 | 48 (8.8) | 0.13 |
Values are expressed as number (percentage) unless stated otherwise.
ANCA, antineutrophil cytoplasm antibody; GPA, granulomatosis with polyangiitis; MPO, myeloperoxidase; PR3, proteinase-3.