Total (n=99) Anti-MPO (n=67) Anti-PR3 (n=32)
p b
Age, yrs, mean (SD) Female sex, n (%) 56.5 (16.8) 48 (48.5) 61.3 (14.6) 34 (50.7) 46.4 (17.0) 14 (43.8) <0.001 NS
Diagnosis MPA, n (%) GPA, n (%) EGPA, n (%) 55 (55.6) 36 (36.4) 8 (8.1) 54 (80.6) 5 (7.5) 8 (11.9) 1 (3.1) 31 (96.9) 0 (0) <0.001 <0.001 0.05
Time-course ANCA pattern Monophasic, n (%) Remitting, n (%) Persistent, n (%) Recurrent, n (%) 26 (26,3) 13 (13,1) 21 (21,2) 39 (39,4) 23 (34.3) 11 (16.4) 9 (13.4) 24 (35.8) 3 (9.4) 2 (6.3) 12 (37.5) 15 (46.9) 0.008 NS 0.006 NS
Clinical features Constitutional symptoms, n (%) Fever, n (%) Anemia, n (%) Microhaematuria, n (%) Renal failure, n (%) Alveolar haemorrhage, n (%) Pulmonary infiltrates, n (%) Pulmonary nodules, n (%) Mononeuritis multiplex, n (%) CNS involvement, n (%) ENT involvement, n (%) Purpura, n (%) Arthritis, n (%) Ocular involvement, n (%) 72 (72.7) 54 (54.5) 84 (84.8) 68 (68.7) 60 (60.6) 28 (28.3) 22 (22.2) 20 (20.2) 16 (16.2) 5 (5.1) 33 (33.3) 14 (14.1) 49 (49.5) 10 (10.1) 47 (70.1) 36 (53.7) 56 (83.6) 52 (77.6) 49 (73.1) 19 (28.4) 11 (16.4) 2 (3.0) 11 (16.4) 2 (3.0) 9 (13.4) 6 (9.0) 25 (37.3) 4 (6.0) 25 (78.1) 18 (56.3) 28 (87.5) 16 (50.0) 11 (34.4) 9 (28.1) 11 (34.4) 18 (56.3) 5 (15.6) 3 (9.4) 24 (75.0) 8 (25.0) 24 (75.0) 6 (18.8) NS NS NS 0.006 <0.001 NS 0.04 <0.001 NS NS <0.001 0.03 <0.001 0.05
Treatment Corticosteroids, n (%) Cyclophosphamide, n (%) Azathioprine, n (%) Methotrexate, n (%) Mycophenolic acid, n (%) Plasmapheresis , n (%) Rituximab, n (%) 99 (100) 78 (78.8) 39 (39.4) 12 (12.1) 20 (20.2) 17 (17.2) 32 (32.3) 67 (100) 51 (76.1) 24 (35.8) 2 (3.0) 17 (25.4) 15 (22.4) 19 (28.4) 32 (100) 27 (84.4) 15 (46.9) 10 (31.3) 3 (9.4) 2 (6.3) 13 (40.6) NS NS NS <0.001 NS 0.05 NS
Outcome Follow-up, yrs, median (IQR) Clinical relapsea, n (%) Worsening renal function, n (%) End-stage renal disease, n (%) -ESRD at baseline, n (%) -ESRD during follow-up, n (%) Mortality, n (%) Mortality related to vasculitis, n (%) 9.0 (9.22) 59 (59.6) 26 (26.3) 19 (19.2) 8 (8.1) 11 (11.1) 29 (29.3) 5 (5.1) 7.6 (7.3) 32 (47.8) 21 (31.3) 17 (25.4) 7 (10.4) 10 (14.9) 24 (35.8) 4 (6.0) 12.2 (9.8) 27 (84.4) 5 (15.6) 2 (6.3) 1 (3.1) 1 (3.1) 5 (15.6) 1 (3.1) 0.001 0.001 NS 0.02 NS NS 0.04 NS