Monophasic (n=26) Remitting (n=13) Persistent (n=21) Recurrent (n=39)
p b
Age, yrs, mean (SD) Female sex, n (%) 59.0 (14.9) 14 (53.8) 57.4 (18.9) 8 (35.9) 50.7 (20.6) 12 (57.1) 57.7 (15.0) 14 (61.5) NS NS
Diagnosis MPA, n (%) GPA, n (%) EGPA, n (%) 17 (65.4) 5 (19.2) 4 (15.4) 10 (76.9) 3 (23.1) 0 9 (42.9) 12 (57.1) 0 19 (48.7) 16 (41.0) 4 (10.3)
0.05
ANCA specificity Anti-MPO, n (%) Anti-PR3, n (%) 23 (88.5) 3 (11.5) 11 (84.6) 2 (15.4) 9 (42.9) 12 (57.1) 24 (61.5) 15 (38.5)
0.004
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 (%) 22 (84.6) 15 (57.7) 22 (84.6) 16 (61.5) 16 (61.5) 7 (26.9) 6 (23.1) 2 (7.7) 5 (19.2) 2 (7.7) 6 (23.1) 2 (7.7) 13 (50.0) 0 9 (69.2) 5 (38.5) 12 (92.3) 11 (84.6) 9 (69.2) 2 (15.4) 0 3 (23.1) 1 (7.7) 0 2 (15.4) 3 (23.1) 3 (23.1) 1 (7.7) 17 (81.0) 11 (52.4) 21 (100) 16 (76.2) 14 (66.7) 6 (28.6) 5 (23.8) 5 (23.8) 4 (19.0) 1 (4.8) 10 (47.6) 5 (23.8) 14 (66.7) 3 (14.3) 24 (61.5) 23 (59.0) 29 (74.4) 25 (64.1) 21 (53.8) 13 (33.3) 11 (28.2) 10 (25.6) 6 (15.4) 2 (5.1) 15 (38.5) 4 (10.3) 19 (48.7) 6 (15.4) NS NS 0.05 NS NS NS NS NS NS NS NS NS NS NS
Treatment Cyclophosphamide, n (%) Azathioprine, n (%) Mycophenolic acid, n (%) Methotrexate, n (%) Plasmapheresis, n (%) Rituximab, n (%) 18 (69.2) 10 (38.5) 3 (11.5) 0 10 (38.5) 10 (38.5) 12 (92.3) 6 (46.2) 3 (23.1) 0 1 (7.7) 2 (15.4) 19 (90.5) 9 (42.9) 7 (33.3) 2 (9.5) 3 (14.3) 6 (28.6) 29 (74.4) 14 (35.9) 7 (17.9) 10 (25.6) 3 (7.7) 14 (35.9) NS NS NS 0.007 0.009 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 (%) 5.7 (3.3) 6 (23.1) 0 7 (26.9) 6 (23.1) 1 (3.8) 6 (23.1) 2 (7.7) 7.1 (7.5) 4 (30.8) 0 1 (7.7) 0 1 (7.7) 4 (30.8) 3 (23.1) 9.6 (7.5) 15 (71.4) 2 (9.5) 3 (14.3) 0 3 (14.3) 5 (23.8) 8 (38.1) 8.6 (8.0) 34 (87.2) 3 (7.7) 8 (20.5) 2 (5.1) 6 (15.4) 14 (35.9) 13 (33.3) 0.07 <0.001 0.06 NS 0.01 NS NS NS