Patient characteristics of the included studies
| Study | Patients (n) | GPA n (%) | MPA n (%) | EGPA n (%) | PR3+ n (%) | MPO+ n (%) | Age (years*) | Sex (male/female) (n) |
| Watanabe et al11 | 271 | 34 (12.5) | 183 (67.5) | 15 (5.5) | 0 | 271 (100) | 73 (65–78) | 106/165 |
| Bunch et al12 | 54 | 18 (33.3)† | 18 (33.3)† | 0 | 23 (42.6) | 20 (37.0) | Active: 58 (48–68) Remission: 58 (38–66) | 30/24 |
| Matsumoto et al13 | 29 | 13 (44.8) | 16 (55.2) | 0 | 7 (24.1) | 21 (72.4) | 70 (57–79) | 13/16 |
| Chen et al14 | 82 | 14 (17.1) | 68 (82.9) | 0 | 0 | 82 (100) | 60±13 | 41/41 |
| Gou et al15 | 100‡ | NR | NR | NR | 0 | 100 (100) | Active: 55±36 Remission: 59±15 | Active: 29/37 Remission: 21/33 |
| Grayson et al16 | 112 | 87 (77.7) | 25 (22.3) | 0 | 78 (69.6) | 34 (30.4) | Non-responder: 51±15 Responder: 52±18 | 53/59 |
| Yanaoka et al17 | 26 | 23 (88.5) | 3 (11.5) | 0 | 8 (30.8) | 18 (69.2) | 68 (58–75) | 7/19 |
| Monach et al18 | 186 | 139 (74.7) | 46 (24.7) | 0 | 124 (66.7) | 62 (33.3) | 52 (44–66) | 91/95 |
| Monach et al19 | 74 | 57 (77.0) | 16 (21.6) | 0 | 54 (73.0) | 20 (27.0) | 51 (40–63) | 33/41 |
| Ahn et al20 | 60 | 17 (28.3) | 32 (53.3) | 11 (18.3) | 7 (11.7)§ | 37 (61.7)§ | 59±14 | 20/40 |
| Yoon et al21 | 59 | 18 (30.5) | 30 (50.8) | 11 (18.6) | 7 (11.9) | 35 (59.3) | 60±14 | 21/38 |
| Sun et al22 | 32 | NR | NR | NR | 2 (6.3) | 30 (93.8) | 59±12 | 13/19 |
| Kambas et al23 | 17 | 11 (64.7) | 6 (35.3) | 0 | 9 (52.9) | 8 (47.1) | 58±12 | 5/12 |
| Aendekerk et al24 | 95 | 55 (57.9) | 37 (38.9) | 3 (3.2) | 45 (47.4)§ | 47 (49.5)§ | NR | 62/33 |
| O’Reilly et al, inception25 | 177¶ | 101 (57.1) | 54 (30.5) | 12 (6.8) | NR | NR | 64 (range 21–90) | 87/90 |
| O’Reilly et al, validation 125 | 155¶ | 94 (60.6) | 49 (31.6) | 6 (3.9) | NR | NR | 60 (range 17–95) | 97/58 |
| O’Reilly et al, validation 225 | 133** | 57 (42.9) | 59 (44.4) | 8 (6.0) | NR | NR | 60 (range 18–91) | 80/53 |
| O’Reilly et al, Ext. validation25 | 39 | 28 (71.8) | 11 (28.2) | 0 | NR | NR | 59 (range 31–82) | 16/23 |
| Dekkema et al26 | 154 | NR | NR | NR | 93 (60.4) | 61 (39.6) | Inception: 58 (47–67) Validation: 61 (48–74) | 87/67 |
| Wu et al27 | 256†† | |||||||
| Active | 173 | NR | NR | NR | 11 (6.4) | 162 (93.6) | 59±14 | 66/107 |
| Remission | 143 | NR | NR | NR | 4 (2.8) | 53 (37.1) | 60±12 | 61/82 |
| Gou et al28 | 29 | NR | NR | NR | 0 | 29 (100) | 58±15 | 12/17 |
| Sonnemann et al29 | 87 | 53 (60.9) | 34 (39.1) | 0 | 48 (55.2) | 39 (44.8) | 57 (48–64)‡‡ 44 (38–51)§§ 63 (55–71)¶¶ 67 (56–73)*** 50 (44–58)††† 64 (52–68)‡‡‡ | 54/33 |
| Sparding et al30 | 47 | NR | NR | NR | 21 (44.7) | 23 (48.9) | 62 (55–69) | 28/19 |
| Matsuda et al31 | 49 | 0 | 49 (100) | 0 | 3 (6.1)§§§ | 48 (98.0)§§§ | ILD: 71 (68–77) No ILD: 76 (71–82) | 25/24 |
| Wu et al32 | 39 | NR | NR | NR | 0 | 39 (100) | 58±100¶¶¶ 63±14**** 57±14†††† | 22/17 |
*Median (IQR) or mean±SD.
†Seven renal limited patients.
‡Twenty patients had both active and remission samples.
§One patient MPO-ANCA and PR3-ANCA positive.
¶Three anti-GBM disease patients were included.
**Five anti-GBM disease patients were included.
††Sixty patients had samples at both active disease stage and remission.
‡‡Active renal disease, inception cohort.
§§Active non-renal disease, inception cohort.
¶¶Remission, inception cohort.
***Active renal disease, validation cohort.
†††Active non-renal disease, validation cohort.
‡‡‡Remission, validation cohort.
§§§Two patients MPO-ANCA and PR3-ANCA positive.
¶¶¶Group of patients without kidney dysfunction .
****Group of patients who did not need dialysis.
††††Group of patients requiring dialysis.
ANCA, anti-neutrophil cytoplasmic antibody ; EGPA, eosinophilic granulomatosis with polyangiitis; Ext., external; GPA, granulomatosis with polyangiitis; ILD, interstitial lung disease; MPA, microscopic polyangiitis; MPO, myeloperoxidase; n, number; NR, not reported; PR3, roteinase 3.