Baseline phenotypic characteristics and follow-up data of 673 patients with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), within subgroups with GPA and MPA and within each cluster generated by cluster model 2 including nine clinical variables plus PR3-ANCA and MPO-ANCA positivity as input variables
| Variable | All | By clinical diagnosis | Clusters | |||||
|---|---|---|---|---|---|---|---|---|
| GPA | MPA | Non-renal AAV | Renal AAV with PR3-ANCA | Renal AAV without PR3-ANCA | CV AAV | GI AAV | ||
| No | 673 (100) | 396 (58.8) | 277 (41.2) | 84 (12.5) | 270 (40.1) | 212 (31.5) | 58 (8.6) | 49 (7.3) |
| Diagnosis | ||||||||
| GPA | 396 (58.8) | 396 (100) | 0 | 80 (95.2) | 217 (80.4) | 38 (17.9) | 37 (63.8) | 24 (49.0) |
| MPA | 277 (41.2) | 0 | 277 (100) | 4 (4.8) | 53 (19.6) | 174 (82.1) | 21 (36.2) | 25 (51.0) |
| Age at diagnosis, mean (SD) (year) | 57.9 (14.1) | 55.2 (14.3) | 61.8 (12.9) | 52.3 (14.1) | 55.3 (14.7) | 61.7 (12.4) | 60.1 (14.3) | 62.1 (12.1) |
| Male sex* | 358 (53.2) | 215 (54.3) | 143 (51.6) | 42 (50.0) | 155 (57.4) | 101 (47.6) | 33 (56.9) | 27 (55.1) |
| Symptoms | ||||||||
| Kidney* | 572 (85.0) | 303 (76.5) | 269 (97.1) | 0 | 270 (100) | 210 (99.1) | 48 (82.8) | 44 (89.8) |
| Lung* | 394 (58.5) | 264 (66.7) | 130 (46.9) | 50 (59.5) | 164 (60.7) | 99 (46.7) | 51 (87.9) | 30 (61.2) |
| ENT* | 393 (58.4) | 334 (84.3) | 59 (21.3) | 74 (88.1) | 194 (71.9) | 56 (26.4) | 41 (70.7) | 28 (57.1) |
| Eye* | 194 (28.8) | 156 (39.4) | 38 (13.7) | 30 (35.7) | 112 (41.6) | 27 (12.7) | 16 (27.6) | 9 (18.4) |
| Skin* | 173 (25.7) | 119 (30.1) | 54 (19.5) | 16 (19.1) | 84 (31.1) | 33 (15.6) | 22 (37.9) | 18 (36.7) |
| Neuropathy* | 164 (24.4) | 112 (28.3) | 52 (18.8) | 26 (31.0) | 63 (23.3) | 37 (17.5) | 22 (37.9) | 16 (32.7) |
| CV* | 64 (9.5) | 41 (10.4) | 23 (8.3) | 0 | 0 | 0 | 58 (100) | 6 (12.2) |
| GI* | 49 (7.3) | 24 (6.1) | 25 (9.0) | 0 | 0 | 0 | 0 | 49 (100) |
| ANCA positivity | ||||||||
| PR3-ANCA* | 376 (55.9) | 311 (78.5) | 65 (23.5) | 68 (81.0) | 255 (94.4) | 5 (2.4) | 34 (58.6) | 33 (67.3) |
| MPO-ANCA* | 212 (31.5) | 42 (10.6) | 170 (61.4) | 7 (8.3) | 0 | 176 (83.0) | 16 (27.6) | 13 (26.5) |
| PR3-ANCA and MPO-ANCA | 19 (2.8) | 14 (3.5) | 5 (1.8) | N/A* | N/A* | N/A* | N/A* | N/A* |
| Neither | 66 (9.8) | 29 (7.3) | 37 (13.4) | 9 (10.7) | 15 (5.6) | 31 (14.6) | 8 (13.8) | 3 (6.1) |
| BVAS, mean (SD) | 19.5 (8.5) | 20.6 (8.6) | 17.9 (8.2) | 12.1 (6.0) | 20.7 (8.2) | 17.6 (7.1) | 26.2 (8.2) | 25.1 (8.5) |
| Creatinine, mean (SD) (µl/litre) | 295.0 (294.1) | 214.2 (234.2) | 408.1 (330.4) | 88.4 (56.0) | 271.4 (269.6) | 392.8 (335.6) | 298.0 (276.2) | 330.3 (293.5) |
| Trial | ||||||||
| Early systemic AAV | 92 (13.7) | 86 (21.7) | 6 (2.2) | 50 (59.5) | 30 (11.1) | 5 (2.4) | 3 (5.2) | 4 (8.2) |
| Generalised AAV | 148 (22.0) | 91 (23.0) | 57 (20.6) | 5 (6.0) | 81 (30.0) | 49 (23.1) | 7 (12.1) | 6 (12.2) |
| Generalised renal AAV | 147 (21.8) | 56 (14.1) | 91 (32.9) | 4 (4.8) | 58 (21.5) | 71 (33.5) | 6 (10.3) | 8 (16.3) |
| Systemic AAV | 172 (25.6) | 127 (32.1) | 45 (16.3) | 25 (29.8) | 57 (21.1) | 33 (15.6) | 31 (53.5) | 26 (53.1) |
| Severe renal AAV | 114 (16.9) | 36 (9.1) | 78 (28.2) | 0 | 44 (16.3) | 54 (25.5) | 11 (19.0) | 5 (10.2) |
| Events | ||||||||
| Death | 137 (20.4) | 55 (13.9) | 82 (29.6) | 5 (6.0) | 36 (13.3) | 66 (31.1) | 15 (25.9) | 15 (30.6) |
| Relapse | 238 (35.4) | 184 (46.5) | 54 (19.5) | 46 (54.8) | 116 (43.0) | 46 (21.7) | 22 (37.9) | 8 (16.3) |
| Follow-up, mean (SD) (year) | 4.45 (3.03) | 4.75 (2.98) | 4.02 (3.05) | 4.68 (2.65) | 5.21 (3.06) | 4.09 (3.09) | 2.82 (2.41) | 3.35 (2.64) |
Variables marked with an asterisk were used as input variables. Data are number (%) unless indicated.
*For the groups generated by cluster analysis, patients with double PR3-ANCA and MPO-ANCA positivity were assigned to the PR3-ANCA group (see text in the corresponding Results section for more detailed explanation).
BVAS, Birmingham Vasculitis Activity Score; CV, cardiovascular; ENT, ear, nose, throat; GI, gastrointestinal; GPA, granulomatosis with polyangiitis (Wegener's); MPA, microscopic polyangiitis, MPO, myeloperoxidase; N/A, not applicable; PR3, proteinase 3.