Table 1.
Summary of the data on the use of rituximab in the treatment of 272 patients with pemphigus vulgaris
| N | Concomitant therapy | Clinical outcome | Mean follow-up (range) | Relapse | SAE | Death | B cell levels | Antibody titers | |
|---|---|---|---|---|---|---|---|---|---|
| Lymphoma protocol—case reports [16–37] | 48 |
10 (20.83%) CS only 36 (75%) CS + ISAs 2 (41.7%) RTX only |
9 (18.75%) CR off 21 (43.75%) CR on 2 (4.17%) CR NM 9 (18.75%) PR 7 (14.58%) NR |
12.91 months (1–36) |
6 patients (12.5%) after 8.73 months 1 patient had 2 relapses Retreated with: 1–RTX infusion 1–ISA 4–2nd RTX cycle |
6 infections (12.5%) 2 LON (4.17%) 1 DVT + PE (2.08%) |
1 (2.08%) from PCP 4 months after RTX 1 (2.08%) from septic shock 16 months after RTX |
Time to depletion in 31 patients (64.58%): 1.76 months (range 0.25–7) Duration of depletion in 18 patients (37.5%): 12.84 months (range 2–23.6) Time to repopulation in 15 patients (31.25%): 12.43 months (range 5.5–23.6) |
IIF: 4 No change, 2 ↑, 19 ↓ Dsg 3: 4 No change, 1 ↑, 25 ↓ Dsg 1: 6 No change, 1 ↑, 17 ↓ |
| Lymphoma protocol—case series [43–49] | 88 |
14 (15.9%) CS only 74 (84.1%) CS + ISAs |
7 (8%) CR off, 34 (38.63%) CR on 15 (17%) CR NM 6 (6.82%) PR 26 (29.55%) clin rem NOS |
21.74 months (10.8–41) |
27 patients (30.7%) after 17.85 months Retreated with: 9–additional RTX cycles 3–2 infusions RTX at 1000mg 3 weeks apart 4–low-dose Prednisone 11–NM |
1 infection (1.14%) | 1 (1.14%) from septicemia 18 months after RTX |
Reported in 35 patients (39.77%) Time to depletion: 0.25–1 month Duration of depletion: 12 months Time to repopulation: 18.93 months (range 12–34) |
Dsg 3: 12 No change, 6 ↑, 66 ↓ Dsg 1: 4 No change, 2 ↑, 20 ↓ |
| Rheumatoid arthritis protocol studies [50–53] | 75 |
42 (56%) CS only 33 (44%) CS + ISAs |
44 (58.67%) CR off 11 (14.67%) CR on 4 (5.23%) CR NM 15 (20%) PR 1 (1.33%) death |
18.66 months (8.35–29) |
28 (37.33%) patients after 18.25 months (range 4–41) 9 patients had 2 relapses 3 patients had 3 relapses Retreated with: 8–2nd RTX cycle 18–500mg RTX infusion/relapse 1–DM + RTX + IA 1–MMF |
14 infection (18.67%) | 1 (1.33%) from sepsis with S. aureus |
Time to depletion in 62 (82.6%) patients: 1–4 weeks Time to repopulation in 42 (56%) patients: 6–14 months |
IIF: 1 ↑, 2 ↓. Dsg 3 + Dsg 1: 66 ↓ |
| Modified protocols—case reports [38–42] | 10 |
2 (20%) CS only 7 (70%) CS + ISAs 1 (10%) NM |
2 (20%) CR off 5 (50%) CR on 1 (10%) CR NM 2 (20%) PR |
14.38 months (6–23) | NM | 1 infection + PE (10%) | NM | NM | NM |
| Modified protocols— case series [54–57] | 51 |
26 (51%) CS + ISAs 25 (49%) NM |
7 (13.73%) CR off 5 (9.8%) CR on 22 (43.14%) CR NM 13 (25.49%) PR 4 (7.84%) NM |
23.8 months (15.72–28.125) |
18 (35.3%) patients after 18.75 months Retreated with: 9–2nd RTX cycle 1–minor therapy NOS 8–NM |
1 LON (1.96%) 1 cardiac (1.96%) |
1 (1.96%) from gastric perforation |
Time to depletion in 18 (35.3%) patients: 1 week Duration of depletion in 7 (13.7%) patients: 23.5 months Time to repopulation in 11 (21.5%) patients: 23.8 months |
Dsg 3 + Dsg 1: 33x ↓ |
clin rem NOS clinical remission not otherwise specified, CR NM complete remission therapy not mentioned, CR off complete remission off systemic therapy, CR on complete remission on systemic therapy, CS corticosteroids, DM dexamethasone, Dsg 1 desmoglein 1, Dsg 3 desmoglein 3, DVT deep vein thrombosis, IA immunoadsorption, IIF indirect immunofluorescence, ISA immunosuppressive therapy, LON late-onset neutropenia, MMF mycophenolate mofetil, N number of patients, NM none mentioned, NR nonresponder, NOS not otherwise specified, PCP Pneumocystis carinii pneumonia, PE pulmonary edema, PR partial remission, RTX rituximab, SAE serious adverse event