Table 1 Characteristics of 9 patients with AAV at the time of PLEX
Variables | Values |
|---|---|
Demographic data at PLEX | |
Age (years) | 71.0 (25.0) |
Male gender (N, (%)) | 5 (55.6) |
AAV variants (N, (%)) | |
MPA | 6 (66.7) |
GPA | 3 (33.3) |
EGPA | 0 (0) |
Gap-time from diagnosis to PLEX (days) | 69.0 (127.0) |
ANCA and anti-GBM within 4 weeks before PLEX (N, (%)) | |
MPO-ANCA | 4 (44.4) |
PR3-ANCA | 2 (22.2) |
ANCA negativity | 3 (33.3) |
Anti-GBM | 0 (0) |
AAV related indices at PLEX | |
BVAS | 20.0 (14.0) |
FFS | 3.0 (1.5) |
Clinical manifestations at PLEX (N, (%)) | |
General | 9 (100) |
Cutaneous | 1 (11.1) |
Mucous membrane/Eyes | 0 (0) |
ENT | 3 (33.3) |
Pulmonary | 7 (77.8) |
Cardiovascular | 3 (33.3) |
Abdominal | 0 (0) |
Renal | 7 (77.8) |
Nervous systemic | 2 (22.2) |
Reason for PLEX (N, (%)) | |
DAH | 6 (66.6) |
RPGN | 2 (22.2) |
Pericarditis with cardiac tamponade | 1 (11.1) |
Methylprednisolone pulse therapy at PLEX (N, (%)) | 9 (100) |
Administered immunosuppressive drugs before PLEX (N, (%)) | |
CYC | 4 (44.4) |
RTX | 3 (33.3) |
AZA | 1 (11.1) |
MMF | 1 (11.1) |
TAC | 1 (11.1) |
None | 4 (44.4) |
Administered immunosuppressant drugs at or after PLEX (N, (%)) | |
CYC | 1 (11.1) |
RTX | 5 (55.6) |
AZA | 1 (11.1) |
MMF | 1 (11.1) |
TAC | 0 (0) |
None | 2 (22.2) |
Follow-up duration (days) | 92.0 (225.5) |
All-cause mortality (N, (%)) | 4 (44.4) |
Cause of death (N, (%)) (N = 4) | |
Sepsis | 3 (75.5) |
No response to PLEX | 1 (25.5) |