Table 1.
AAV patients (n = 11) | Flow cytometry studies (n = 6) | |
---|---|---|
Demographics | ||
Age | 59 (33–77) | 42 (33–77) |
Male | 6 (55%) | 3 (50%) |
Caucasian | 11 (100%) | 6 (100%) |
ANCA associated vasculitis | ||
GPA | 4 (36%) | 2 (33%) |
MPA | 6 (55%) | 3 (50%) |
eGPA | 1 (9%) | 1 (17%) |
Immunology | ||
ANCA immunofluorescence | ||
c-ANCA | 3 (27%) | 1 (17%) |
p-ANCA | 7 (64%) | 4 (67%) |
negative | 1 (9%) | 1 (17%) |
ELISA | ||
PR3 | 4 (36%) | 2 (50%) |
MPO | 6 (55%) | 4 (67%) |
negative | 1 (9%) | 1 (17%) |
Low sensitive flow cytometry CD19+ B-cells (106/L) |
27 (0–311) | 183.5 (1–311) |
Organ involvement | ||
Constitutional symptoms | 6 (55%) | 3 (50%) |
Mucocuteanous | 3 (27%) | 1 (17%) |
Musculoskeletal | 4 (36%) | 2 (33%) |
ENT | 6 (55%) | 2 (33%) |
Renal | 6 (55%) | 3 (50%) |
Respiratory | 7 (64%) | 3 (50%) |
Cardiovascular | 1 (9%) | 1 (17%) |
Central nervous system | 1 (9%) | 0 (0%) |
Peripheral nervous system | 0 (0%) | 0 (0%) |
Ophthalmology | 3 (27%) | 0 (0%) |
Abdominal | 3 (27%) | 2 (33%) |
Disease parameters | ||
BVAS | 6 (0–30) | 11.5 (6–30) |
VDI | 3 (0–12) | 0.5 (0–7) |
Reason for treatment | ||
New diagnosis | 4 (36%) | 4 (67%) |
Relapse | 1 (9%) | 0 (0%) |
Persistent disease | 2 (18%) | 2 (33%) |
Maintenance treatment | 4 (36%) | 0 (0%) |
Treatment | ||
Rituximab | ||
2× 1,000 mg | 6 (55%) | 6 (100%) |
1× 500 mg | 5 (45%) | 0 (0%) |
Methylprednisolone 3× 1,000 mg | 4 (36%) | 4 (67%) |
Plasmapheresis | 1 (9%) | 1 (17%) |
Cyclophosphamide (2× 500 mg) | 1 (9%) | 1 (17%) |
High-dose corticosteroids | 5 (45%) | 5 (83%) |
Previous remission-induction treatment | ||
Rituximab | 5 (45%) | 0 (0%) |
Cyclophosphamide oral | 3 (27%) | 1 (17%) |
Cyclophosphamide IV | 2 (18%) | 0 (0%) |
For numerical values the median with (range) and for categorical values the frequency with percentages is shown.