Table 1

Main clinical characteristics and treatments at diagnosis of 727 French Vasculitis Study Group Registry GPA patients according to ANCA specificity

CharacteristicOverall n=727P value (PR3-ANCA+ vs MPO-ANCA+ vs ANCA)No ANCA n=62P value (ANCA vs PR3-ANCA+ and MPO-ANCA+)MPO-ANCA+ n=119P value (MPO-ANCA+ vs PR3-ANCA+ and ANCA)PR3-ANCA+ n=546P value (PR3-ANCA+ vs MPO-ANCA+ and ANCA)
Sex, female319 (43.9)0.08228 (45.2)0.9463 (52.9)0.04228 (41.8)0.06
Age at diagnosis, years, mean (SD)53 (16.3)<0.00152.5 (16.5)0.7959.8 (17.2)<0.00151.6 (15.7)<0.001
Limited GPA50 (6.9)0.00211 (17.7)0.0018 (6.7)0.5531 (5.7)0.04
Severe GPA83 (11.4)0.082 (3.2)0.0917 (14.3)0.7164 (11.7)0.75
General592 (81.4)0.8351 (82.3)0.9999 (83.2)0.70442 (81)0.66
 Fever >38.5°C304 (41.8)0.0523 (37.1)0.5139 (32.8)0.04242 (44.3)0.02
 Weight loss >3 kg within 3 months329 (45.3)0.1621 (33.9)0.0853 (44.5)0.94255 (46.7)0.20
 Arthralgias376 (51.7)<0.00127 (43.5)0.2341 (34.5)<0.001308 (56.4)<0.001
 Myalgias197 (27.1)0.3015 (24.2)0.7039 (32.8)0.16143 (26.2)0.39
Ear, nose and throat582 (80.1)0.5349 (79.0)0.9691 (76.5)0.35442 (81)0.35
 Sinusitis297 (40.9)0.4428 (45.2)0.5643 (36.1)0.30226 (41.4)0.67
 Rhinitis390 (53.6)0.2829 (46.8)0.3259 (49.6)0.38302 (55.3)0.14
 Nasal crusts276 (38)0.1016 (25.8)0.0544 (37)0.05216 (39.6)0.15
 Epistaxis138(19)0.047 (11.3)0.1516 (13.4)0.12115 (21.1)0.02
 Saddle nose9 (1.2)0.060 (0)0.754 (3.4)0.075 (0.9)0.33
 Nasal obstruction183 (25.2)0.6418 (29.0)0.5627 (22.7)0.57138 (25.3)0.99
 Otitis166 (22.8)0.3211 (17.7)0.4023 (19.3)0.38132 (24.2)0.16
Lung492 (67.7)0.3537 (59.7)0.2183 (69.7)0.67372 (68.1)0.72
 Alveolar haemorrhage130 (17.9)0.217 (11.3)0.2126 (21.8)0.2797 (17.8)0.98
 Massive alveolar haemorrhage and/or Hb <90 g/L35 (4.8)0.161 (1.6)0.363 (2.5)0.3031 (5.7)0.09
 Lung nodules303 (41.7)0.6125 (40.3)0.9345 (37.8)0.41233 (42.7)0.39
 Lung infiltrate134 (18.4)0.446 (9.7)0.1818 (15.1)0.2962 (11.4)0.58
 Subglottic stenosis11 (1.5)0.073 (4.8)0.092 (1.7)1.006 (1.1)0.22
Kidney414 (56.9)<0.00122 (35.5)0.00178 (65.5)<0.05314 (57.5)0.66
 Serum creatinine median (µmol/L)93 (73–177)<0.00177 (63–100)<0.001113 (78–199)<0.00192 (74–174.5)<0.001
 Serum creatinine >150 µmol/L162/563 (28.8)0.067/42 (16.7)0.1034/94 (36.2)0.11121/427 (28.3)0.77
 Rise in creatinine level >30%178 (24.5)<0.059 (14.5)0.0837 (31.1)0.09132 (24.2)0.81
 Proteinuria256 (35.2)0.00410 (16.1)0.00245 (37.8)0.59201 (36.8)0.14
 Haematuria280 (38.5)<0.0019 (14.5)0.00154 (45.4)0.11217 (39.7)0.27
 Need for dialysis41 (5.6)0.100 (0)0.099 (7.6)0.4832 (5.9)0.79
Mucocutaneous232 (31.9)0.1417 (27.4)0.5230 (25.2)0.11185 (33.9)0.06
 Purpura114 (15.7)0.016 (9.7)0.2410 (8.4)0.0298 (17.9)0.005
 Livedo reticularis26 (3.6)0.491 (1.6)0.616 (5.0)0.5019 (3.5)0.99
 Gangrene16 (2.2)0.333 (4.8)0.302 (1.7)0.9411 (2.0)0.76
Eye195 (26.8)0.0719 (30.6)0.5822 (18.5)0.03154 (28.2)0.17
 Exophthalmos24 (3.3)0.832 (3.2)1.005 (4.2)0.7517 (3.1)0.80
 Episcleritis67 (9.2)0.134 (6.5)0.586 (5.0)0.1257 (10.4)0.07
 Scleritis30 (4.1)0.161 (1.6)0.482 (1.7)0.2227 (4.9)0.09
Cardiovascular115 (15.8)0.537 (11.3)0.4021 (17.6)0.6587 (15.9)0.98
 Pericarditis31 (4.3)0.210 (0)0.165 (4.2)1.0026 (4.8)0.35
 Myocarditis9 (1.2)0.341 (1.6)1.003 (2.5)0.355 (0.9)0.33
 Congestive heart failure10 (1.3)0.490 (0)0.771 (0.8)0.359 (1.6)0.47
Gastrointestinal76 (10.5)0.555 (8.1)0.6710 (8.4)0.5361 (11.2)0.34
 Abdominal pain41 (5.6)0.472 (3.2)0.579 (7.6)0.4430 (5.5)0.91
 Perforation2 (0.3)0.0420 (0)1.001 (0.8)0.741 (0.2)1.00
Neurological215 (29.6)0.7916 (25.8)0.5935 (29.4)1.00164 (30.0)0.70
 Central nervous system21 (2.9)0.184 (6.5)0.182 (1.7)0.5815 (2.7)0.89
 Peripheral neuropathy143 (19.7)0.065 (8.1)0.02525 (21.0)0.78113 (20.7)0.27
BVAS at diagnosis, mean (SD)17.5 (8.8)0.00113.3 (8.1)<0.00118.2 (8.6)0.3617.8 (8.7)0.10
Histology supporting GPA diagnosis324 (44.6)0.93529 (46.8)53 (44.5)242 (44.3)
 Intravenous cyclophosphamide561 (77.2)<0.00135 (56.5)<0.00186 (72.3)0.20440 (80.6)<0.001
 Oral cyclophosphamide41 (5.6)0.00110 (16.1)0.0016 (5.0)0.9325 (4.6)<0.05
 Rituximab50 (6.9)0.663 (4.8)0.6910 (8.4)0.6037 (6.8)0.99
 Methotrexate33 (4.5)0.136 (9.7)0.095 (4.2)1.0022 (4.0)0.35
 Glucocorticoids709 (97.5)0.1058 (93.5)0.09116 (97.5)1.00535 (98)0.27
 Glucocorticoid, mg/day mean (SD)60 (50–70)0.00650.2 (29.7)0.0552.0 (23.0)0.0359.1 (21.4)0.002
 Methylprednisolone pulse208 (28.6)0.0610 (16.1)0.0339 (32.8)0.33159 (29.1)0.66
 Plasma exchanges57 (7.8)0.212 (3.2)0.247 (5.9)0.5048 (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.