Characteristics/outcome | |
---|---|
Number of patients | 50 |
Gender (F/M), n (%) | 36/14 (72/28) |
Median (range) age at onset (years) | 43 (13–73) |
Median (range) interval from onset to diagnosis (months) | 9 (0–201) |
Median (range) months from onset to diagnosis, diagnosed before 2000 | 12 (0–201) |
Median (range) months from onset to diagnosis, diagnosed after 2000 | 6.7 (0–90) |
Median (range) follow-up (months) | 48 (12–174) |
ANCA status IFT-positive, n (%) | 23 (46) |
C-/PR3-ANCA positive, n (% of ANCA-positive patients) | 19 (82.6) |
P-/MPO-ANCA positive, n (% of ANCA-positive patients) | 3 (13.0) |
P-/PR3-ANCA positive | 1 (4.3) |
Positive ANCA status (IFT+ELISA) | |
At beginning of follow-up, n (% of ANCA-positive patients) | 20 (87) |
At end of follow-up, n (% of ANCA-positive patients) | 20 (87) |
Remission, partial (response), n (%) | 33 (66) |
Remission, complete, n (%) | 17 (34) |
Relapses, n (%) | 24 (48) |
Refractory disease, n (%) | 8 (16) |
Development of systemic disease, n (%) | 5 (10) |
Median (range) time from diagnosis to systemic disease (months) | 72 (48–84) |
Deaths, n (%) | 2 (4) |
ANCA, antineutrophil cytoplasmic autoantibodies.